RADIATION EXPOSURE GUIDELINES
DESCRIPTION The National Council on Radiation Protection and Measurements has recommended maximum permissible dose limits for occupational exposure to members of the public, which apply to the sum of the effective doses from external radiation and the committed effective doses from internal exposures.
Occupational exposure
• The individual worker’s lifetime effective dose should not exceed age in years times 10 mSv
• An annual effective dose limit of 50 mSv
• An annual dose limit of 150 mSv for the lens of the eye
• An annual dose limit of 500 mSv for localized areas of the skin and the hands and feet
• A monthly dose limit of 0.5 mSv to the fetus once a pregnancy is declared
• No occupational exposure should be permitted until age 18 yr
Public exposure
• An annual effective dose limit of 1 mSv for continuous exposure and 5 mSv for infrequent exposure
• An annual dose limit of 50 mSv for the hands and feet and localized areas of the skin and 15 mSv for the lens of the eye
• For educational and training purposes involving people aged <18 yr, an annual effective dose limit of 1 mSv
REFERENCE
Cuaron JJ, Hirsch AE, Medich DC, et al. Introduction to Radiation Safety and Monitoring. J Am Coll Radiol. 2011;8:259.
RADIATION, PELVIC, UROLOGIC CONSIDERATIONS
DESCRIPTION Pelvic radiation is commonly used as both a primary treatment for localized prostate cancer and also as a treatment for local recurrence after surgery or nodal disease. Muscle-invasive bladder cancer is also treated with radiation. Urologists often encounter patients who have received previous pelvic radiation for gynecologic cancers such as cervical, ovarian, or rectal cancer. Radiation can cause several urologic complications, including cystitis, ureteral stricture, secondary bladder malignancy, fistula, and retroperitoneal fibrosis. Previous pelvic radiation is a contraindication for orthotopic neobladder reconstruction, and urinary diversion should be performed using bowel and with placement of the stoma outside the radiation field.
REFERENCE
Duggan B, Nambirajan T, Johnston SR. Radiation-induced haemorrhagic cystitis. Eur Urol. 2003;43:111–123.
RADIATION PROCTITIS, UROLOGIC CONSIDERATIONS
DESCRIPTION One potentially serious complication of radiation therapy for prostate and other pelvic malignancies is radiation proctitis. Radiation proctitis refers to radiation-induced injury to the rectal mucosa beginning 3 mo after treatment has ended. The incidence varies from 5–20%. Predisposing factors include prior lower abdominal surgery, diabetes, hypertension and possibly chemotherapy. Symptoms of radiation proctitis include tenesmus, bleeding, low-volume diarrhea, rectal pain, and less commonly, low-grade obstruction or fistula. Diagnosis is made with sigmoidoscopy.
TREATMENT
• Corticosteroids
• Sucralfate enemas
• Argon laser
• Bipolar electrocoagulation
• Formalin-soaked gauze
• Hyperbaric oxygen
• Surgery
REFERENCE
Babb R: Radiation proctitis: A review. Am J Gastroenterol. 1996;91:1309.
RADIATION, RENAL AND RETROPERITONEAL, UROLOGIC CONSIDERATIONS
DESCRIPTION Radiation therapy (RT) for RCC as primary therapy is ineffective, but it is useful for palliation of bone metastases. It can be utilized for renal sarcomas or lymphoma. Retroperitoneal radiation is used for seminoma but not for nonseminomatous testis tumors. Nonseminomatous germ cell tumors (NSGCT) are generally less radiosensitive, and RT is typically not used. Side effects of renal and retroperitoneal RT include retroperitoneal fibrosis, ureteral stricture or obstruction, hematuria, enteritis, cardiovascular complications, and secondary malignancy.
REFERENCE
Garcia-Serra AM, Zlotecki RA, Morris CG, et al. Long-term results of radiotherapy for early-stage testicular seminoma. Am J Clin Oncol. 2005;28:119–124.
RADIOPHARMACEUTICALS, UROLOGIC CONSIDERATIONS (STRONTIUM89, SAMARIUM153, RADIUM223)
DESCRIPTION Strontium89 and Samarium153 ethylene diamine tetramethylene phosphonate (EDTMP) are FDA-approved radioisotopes approved for the treatment of bony metastatic pain. Phosphorus32 has been used for bony metastases but is infrequently employed today. All of these agents are β-emitters that can cause significant bone marrow suppression, although myelosuppression has limited its application. These radioisotopes are infused intravenously and taken up in bony areas of high metabolic activity. The radioactive decay has a toxic effect on tumor cells, and relief of symptoms generally begins 1–4 wk after initial infusion.
Radium 223 is a newly approved α-emitting radioisotope for metastatic castrate resistant prostate cancer without visceral disease. Can palliate boney disease and also extend survival. Less likely to impact bone marrow due to larger particle size and limited field effect. (See also Section VI: “Urologic Drug Reference.”)
• α-emitters: Consists of helium nuclei; high linear energy transfer, will not penetrate a sheet of paper; number of DNA hits to kill a cell:1–10; induces double strand breaks that are more lethal and difficult to repair
• β-emitters: Consists of electrons; relatively low linear energy transfer; may be halted by an aluminum plate; 100–1,000 hits to kill cells; single strand breaks that are more easily repaired
REFERENCES
Finlay IG, Mason MD, Shelley M. Radioisotopes for the palliation of metastatic bone cancer: A systematic review. Lancet Oncol. 2005;6:392–400.
Parker C, Nilsson S, Heinrich D, et al. Alpha emitter radium-223 and survival in metastatic prostate cancer. N Engl J Med. 2013;369(3):213–223.
Perez, et al. Principles and Practice of Radiation Oncology. 5th ed. Lippincott Williams & Wilkins; 2007.
RAPID PLASMA REAGIN (RPR) BLOOD TEST
DESCRIPTION The RPR test is a screening test for syphilis (T. pallidum infection). RPR detects serum antibodies to substances released by cells damaged by T. pallidum. It is 78%, 100%, and 95% sensitive in screening for primary, secondary, and tertiary syphilis, respectively. If a patient tests positive, a confirmatory treponemal particle agglutination or fluorescent treponemal antibody test should be ordered. False positives can be seen in some viral infections, and HIV can cause a false-negative reaction.
REFERENCE
Frenkl TL, Potts J. Sexually transmitted infections. Urol Clin North Am. 2008;35:233–246.
RAZ BLADDER NECK SUSPENSION (URETHROPEXY)
DESCRIPTION This is 1 of many surgical bladder neck suspension techniques aiming to fix the vesicourethral junction in a physiologic position to correct genuine stress incontinence in females. It is a modification of the Pereyra needle suspension. Through an inverted U-shaped incision in the anterior vaginal wall, the operator performs (1) retropubic urethrolysis, (2) fingertip guidance of a double-pronged suture carrier placed through a suprapubic opening, and (3) placement of helical nonabsorbable sutures through the urethropelvic ligament, otherwise known as the endopelvic fascia. Cystoscopy is performed after the sutures are placed. Best suited for patients with urethral and bladder neck hypermobility and no cystocele.
REFERENCE
Stothers L, Chopra A, Raz S. Surgery for female stress urinary incontinence. Can J Urol. 1995;2(Supp1):33–37.
RAZ VAGINAL WALL SLING
DESCRIPTION Technique to treat urinary incontinence due to intrinsic sphincter dysfunction or anatomic incontinence, this is a modification of the original Raz bladder neck suspension. This technique provides support for both the bladder neck and mid-urethra. In addition to the principal maneuvers described in the Raz urethropexy, the author incorporates a patch of anterior vaginal wall with the suspension sutures at the level of the bladder neck, which, in effect, creates a hammock that serves as a backboard to the bladder neck and mid-urethra.
REFERENCE
Raz S, Stothers L, Young GP, et al. Vaginal wall sling for anatomical incontinence and intrinsic sphincter dysfunction: Efficacy and outcome analysis. J Urol. 1996;156(1):166–170.
REACTIVE ARTHRITIS/REACTIVE ARTHRITIS TRIAD (FORMERLY REITER SYNDROME)
DESCRIPTION The preferred term today is reactive arthritis, a classic triad of polyarthritis, conjunctivitis, and nongonococcal urethritis; in women, cervicitis. Anterior uveitis and skin or genital rash may be seen. Thought to be a systematic inflammatory response triggered by microbial infection in the GU or GI tracts, the condition is a member of the spondyloarthritis family of disorders. The arthritis is usually asymmetric, with predominately lower extremity involvement. Joint aspiration is typically sterile. Association with HLA-B27 is noted, and may confer susceptibility. 2 forms exist: Sexually transmitted, in which symptoms emerge 10–14 days after exposure. Causes include C. trachomatis, Salmonella sp., Shigella sp., Yersinia sp., and Ureaplasma urealyticum. The overall prognosis is good, with spontaneous remission or remission following NSAID therapy within 6 mo of onset. A small proportion have chronic persistent arthritis; a few will develop ankylosing spondylitis more frequent (if HLA-B27 positive). Usual treatment includes supportive care NSAIDs, intra-articular or systemic steroids for polyarthritis. Antibiotic treatment is initiated for identified organisms, if possible, such as C. trachomatis doxycycline 100 mg PO BID for 7–14 days. (Note: The name change from Reiter syndrome to reactive arthritis is based in part on allegations that Dr. Reiter was an un-convicted war criminal.)
REFERENCE
Hannu T, Inman R, Granfors K, et al. Reactive arthritis or postinfectious arthritis? Best Pract Res Clin Rheumatol. 2006;20(3):419–433.
RECTOCELE, UROLOGIC CONSIDERATIONS
DESCRIPTION Rectocele generally presents as a posterior vaginal wall defect with varying degrees of prolapse. It can present with urologic symptoms including sexual dysfunction and voiding symptoms, as well as constipation. If a patient presents with voiding dysfunction, a urodynamic study should be performed with the rectocele reduced to unmask the patient’s underlying urodynamic parameters. Prior to repair, it is important to determine if there is evidence of enterocele or cystocele, to determine the appropriate reconstructive procedure. Treatment can be conservative using a vaginal pessary or surgical using 1 of several techniques, including open or laparoscopic, transvaginal, or endorectal. (See also Section I: “Pelvic Organ Prolapse [Cystocele and Enterocele].”)
REFERENCE
Hall GM, Shanmugan S, Nobel T, et al. Symptomatic rectocele: What are the indications for repair? Am J Surg. 2014;207(3):375–379.
RED SCROTUM SYNDROME
DESCRIPTION The external genitalia can be affected by many inflammatory processes such as atopic and irritant dermatitis, psoriasis, or ichthyosis. A rare scrotal disease Red Scrotum Syndrome (RSS) affects males in their 2nd half of life and typically runs a chronic course. It may represent a localized phenotypical expression of erythromelalgia. RSS is characterized by persistent redness of the anterior 1/2 of the scrotum and may also involve the base of the penis. Symptoms include itching, burning, and pain sensations. It can develop after prolonged use of topical corticosteroids. It is often mistaken for eczema but itching is not common in RSS. Burning and hyperalgesia are typical in RSS and are not usual with eczema. The pain is aggravated by warmth, relieved by cold. Differential diagnosis includes atopic dermatitis, contact dermatitis, psoriasis, ichthyosis, mycosis such as tinea cruris, secondary syphilis and Langerhans cell histiocytosis among others. Oral doxycycline is used initially with gabapentin a 2nd-line therapy. (See also Section II: “Scrotal Skin Lesion.”)
REFERENCE
Wollina U. Red scrotum syndrome. J Dermatol Case Rep. 2011;5(3): 38–41.
REED SYNDROME
DESCRIPTION Multiple cutaneous and uterine leiomyomatosis, also known as Reed syndrome, is an autosomal dominant condition. Individuals have an increased predisposition to develop benign smooth muscle tumors (leiomyomas) in the skin and uterus (fibroids). Subsets of these patients are at risk for renal cell cancer and have been determined to have mutations in the fumarate hydratase gene. The term hereditary leiomyomatosis and renal cell cancer refers to families with an increased prevalence of smooth muscle tumors and renal cell cancer as a result of the fumarate hydratase genetic defect. (See also Section II: “Leiomyomatosis, Hereditary” and “Renal Cell Carcinoma, Familial.”)
REFERENCE
Emer JJ, Solomon S, Mercer SE. Reed’s Syndrome A Case of multiple cutaneous and uterine leiomyomas. J Clin Aesthet Dermatol. 2011;4(12):37–42.
REFLUX NEPHROPATHY
DESCRIPTION Renal scarring secondary to reflux of sterile or infected urine from the bladder to the kidney. Girls are at increased risk of developing reflux nephropathy because of the increased incidence of UTI’s. Most cases are associated with vesicoureteral reflux, and children are usually asymptomatic or may present with infection, hypertension, or renal failure in cases of severe scarring. Usually a radiographic diagnosis; US and voiding cystourethrography identify the reflux, and renal scarring is detected radiographically by a cortical imaging agent such as DMSA technetium99m (dimercaptosuccinic acid). Treatment is directed at the cause (such as vesicoureteral reflux antibiotic suppression or surgical correction). (See also Section I: “Vesicoureteral Reflux.” and (Image 
))
REFERENCE
Polito C, La Manna A, Rambaldi PF, et al. Long-term evolution of renal damage associated with unilateral vesicoureteral reflux. J Urol. 2007;178(3):1043–1047.
REIFENSTEIN SYNDROME
DESCRIPTION A form of incomplete male pseudohermaphroditism, usually presenting with perineoscrotal hypospadias and frequently cryptorchidism at birth, azoospermia and incomplete virilization at puberty, and infertility and gynecomastia at or after puberty. Caused by mutations in the DNA-binding domain of androgen receptor, with varying degrees of androgen receptor dysfunction. Patients are usually assigned to male sex at birth, and they exhibit elevated levels of testosterone and luteinizing hormone. Surgical repair of hypospadias and cryptorchidism as the treatment and supplemental testosterone is not beneficial. (See also Section II: “Androgen Insensitivity Syndrome [AIS or Androgen Resistance Syndrome], Complete and Partial.”)
SYNONYMS
• Lubor Gilbert–Dreyfus Syndrome
• Type 1 incomplete male pseudohermaphroditism
REFERENCE
Androgen Insensitivity, Partial; PAIS: in Online Mendelian Inheritance in Man http://www.omim.org/entry/312300, Accessed March 6, 2014.
REINKE CRYSTALS
DESCRIPTION Cytoplasmic crystalloid inclusions found in human Leydig cells. The crystals are large, distinctive, and easily visible under light microscopy. It has been noted that their numbers increase with age; their function or significance is unknown.
REFERENCE
Kerr JB. Ultrastructure of the seminiferous epithelium and intertubular tissue of the human testis. J Electron Microsc Technique. 1991;19(2):215–240.
RENAL ADENOMA (PAPILLARY ADENOMA)
DESCRIPTION The most common renal epithelial neoplasm and found in 4–37% of autopsy specimens. Controversial if these are small adenocarcinomas. Strict diagnostic criterion include papillary, tubular, or tubulopapillary architecture, <5 mm and no resemblance to any renal malignancy.
REFERENCE
Grignon DJ, Eble JN. Papillary and metanephric adenomas of the kidney. Semin Diagn Pathol. 1998;15(1):41–53.
RENAL AGENESIS (BILATERAL AND UNILATERAL)
DESCRIPTION This condition is defined by the congenital absence of 1 or both kidneys:
• Bilateral renal agenesis: Incompatible with life as kidney function in utero is necessary in development of the lungs. Infants born with bilateral agenesis have hypoplastic lungs, oligohydramnios, anuria, and renal failure, as well as a well-described group of physical findings such as a flattened nose, low-set ears, bowed limbs, and a small chest collectively referred to as Potter syndrome. Bilateral agenesis is reported in 1 in 3,000 births but the actual incidence is unknown since many fetuses are believed to spontaneously abort without a diagnosis.
• Unilateral renal agenesis: In contrast, unilateral agenesis is usually asymptomatic and is often undetected throughout life. It occurs in 1 in 1,100 births, and is more common in males (1.8:1); the left kidney is more commonly missing. Commonly associated with müllerian duct, wolffian duct, and ureteric bud abnormalities and thus may involve the vas deferens, ureter, trigone, vagina, and seminal vesicles. 65% of patients have another urologic anomaly. Associated anomalies include reflux; contralateral UPJ obstruction including single umbilical artery; absence of ipsilateral ureter and hemi-trigone; vaginal atresia/agenesis (Mayer–Rokitansky syndrome); unilateral vas deferens agenesis/atresia; and seminal vesicle cysts. Unilateral renal agenesis is also associated with anomalies of other systems (such as cardiovascular in 30% valvular or septal cardiac anomalies); GI in 25%; imperforate anus or atresia of anus or esophagus; and vertebral or pharyngeal anomalies. If diagnosed, some clinicians propose yearly screening of BP and urinary protein levels because of the risk of hypertension, renal insufficiency, and proteinuria found in some adult studies. (See also Section II: “Potter Syndrome/Potter Facies.” and (Image ))
REFERENCE
Uetani N, Bouchard M. Plumbing in the embryo: Developmental defects of the urinary tracts. Clin Genet. 2009;75(4):307–317.
RENAL ANATOMY, NORMAL RADIOGRAPHIC FINDINGS (SIZES, CALYCES)
DESCRIPTION The kidney can be imaged by plain film, US, CT, MRI, radionuclide scanning, or pyelography, either IV excretory, antegrade, or retrograde. A normal adult kidney should measure 10–13 cm vertically, 5–7 cm transversely, and 3 cm anteroposteriorly. The right kidney tends to be shorter and wider than the left due to hepatic compression. The renal pedicle usually consists of a single renal artery and vein, although many normal anatomic variants exist. Typically each kidney has 2–3 major calyces and 8–14 minor calyces. Many normal variants of calyceal anatomy also exist, but pathologic findings include debris and filling defects; calyceal diverticulum, dilation of calyces or a single calyx suggests ureteral and infundibular obstruction, respectively (Image ).
REFERENCE
Singer A, Simmons MZ, Maldjian PD. Spectrum of congenital renal anomalies presenting in adulthood. Clin Imaging. 2008;32:183–191.
RENAL ARTERY ANEURYSM
DESCRIPTION A renal artery aneurysm is defined as a dilated segment of renal artery that exceeds twice the diameter of a normal renal artery. Although rare, the diagnosis and incidence of this entity have been steadily increasing due to the routine use of cross-sectional imaging. Incidence ranges from 0.3–1.0% on radiographic studies, accounting for 1% of all arterial aneurysms and 10% of visceral aneurysms. They are commonly bilateral or multiple 20% and 30%, respectively, and occur typically in the 5th–6th decades of life, slightly more frequently on the right. They are associated with hypertension (HTN), but a causal effect has not been shown. Spontaneous rupture is rare, but risk is increased during pregnancy. Presentation is usually secondary to HTN, flank pain, hematuria, or an incidental finding on radiographic study. Causes include atherosclerosis, congenital fibromuscular dysplasia, trauma, infectious disease (syphilis or TB), intrarenal aneurysms, collagen vascular diseases (PAN, Wegener granulomatosis).
TREATMENT
• Most studies recommend repair of renal artery aneurysms (RAAs) >2 cm, spontaneous rupture, or asymptomatic lesions in high-risk patients women of childbearing age or patients with a functional or anatomic solitary kidney. Recent data suggests that many of these patients with RAA >2 cm can be observed if symptomatic.
• Repair includes primary repair with excision of aneurysmal segment, or aortorenal bypass with vein. Extra-anatomic bypass (hepatorenal, gastroduodenal-renal, or splenorenal) is useful for a severely calcified aorta or when aortic cross-clamping is undesirable.
• Autotransplantation is useful for complex repairs with long ischemic time.
• Percutaneous treatment with embolization or occlusion of aneurysmal segments is reserved for high-risk surgical candidates.
• Intraluminal vascular stent: Investigational
REFERENCES
González J, Esteban M, Andrés G, et al. Renal artery aneurysms. Curr Urol Rep. 2014;15(1):376. doi: 10.1007/s11934-013-0376-z.
Klausner JQ, Harlander-Locke MP, Plotnik AN, et al. Current treatment of renal artery aneurysms may be too aggressive. J Vasc Surg. 2014 Jan 22. pii: S0741–5214(13)02157–5. doi: 10.1016/j.jvs.2013.11.062. [Epub ahead of print]
RENAL ARTERY FIBROMUSCULAR DYSPLASIA
DESCRIPTION Fibromuscular diseases of the renal artery account for 1/3 of cases of renovascular hypertension. Treatment consists of antiplatelet therapy for asymptomatic individuals and percutaneous balloon angioplasty for patients with indications for intervention. Patients with macroaneurysms should be treated with either a covered stent or surgery. BP control with ACE inhibitor or angiotensin II receptor blocker. 4 pathologic entities have been described.
• Intimal fibroplasia affects mainly children and young male adults. Angiographically, a smooth focal stenosis is typically seen at the mid renal artery or its branches. Prompt repair is advised because of the progressive nature of the disease.
• Fibromuscular hyperplasia of smooth muscle and fibrous tissue is rare, progressive, and affects mainly children and young adults.
• Medial fibroplasia is the most common (80%), affecting women in their 30s. On angiogram, it has the appearance of a string of beads. This lesion does not dissect, and complete occlusion has not been reported. Angioplasty is the treatment of choice.
• Perimedial fibroplasia is a tightly stenotic, progressive lesion, affecting women 15–30 yr of age. On angiography, extensive collateral vessels are commonly identified. Follow potassium and creatinine after initiation of therapy. Revascularization using percutaneous angioplasty is the definitive treatment of choice.
REFERENCE
Olin JW, Pierce M. Contemporary management of fibromuscular dysplasia. Curr Opin Cardiol. 2008;23(6):527.
RENAL CARCINOID TUMOR
DESCRIPTION Rare tumor derived from enterochromaffin or amine precursor uptake and decarboxylation (APUD) cells, occurring most commonly in the GI tract and lung, but also in ovaries, testes, thymus, pancreas, and hepatobiliary system. Primary renal lesions are extremely rare, with only 32 cases reported. The lesions are thought to originate in renal collecting cells undergoing intestinal metaplasia or from teratomatous epithelial cells within the kidney. Horseshoe kidneys are shown to have a markedly elevated risk of carcinoid tumor, although still very rare, and may have a more benign course.
REFERENCE
Krishnan B, Truong LD, Saleh G, et al. Horseshoe kidney is associated with an increased relative risk of primary renal carcinoid tumor. J Urol. 1997;(1576):2059–2066.
RENAL CELL CARCINOMA, CHROMOPHOBE
DESCRIPTION Chromophobe RCC is a subtype of RCC derived from the distal renal tubules, and it comprises about 3–5% of all RCCs. Tumor cells display a transparent cytoplasm with a plant cell appearance and a characteristic perinuclear halo. On electron microscopy, microvesicles are seen that stain positive for Hale’s colloidal iron. There are 2 types. Type 1, or classic, is defined by loss of chromosomes 1, 2, 6, 10, 13, 17, or 21 and has a better prognosis than type 2, or eosinophilic variant. The eosinophilic variant is part of Birt–Hogg–Dubé syndrome. In general, chromophobe RCC has a better prognosis than other RCC histologies. Chromophobe RCC is difficult to distinguish from oncocytoma on biopsy, making definitive diagnosis difficult without a complete pathologic exam. CD82 and epithelial-related antigen (MOC31) may be helpful in the distinction between chromophobe RCC and renal oncocytoma. (See also Section I: “Renal Cell Carcinoma, General” and (Image ).)
REFERENCE
Klatte T, Han KR, Said JW, et al. Pathobiology and prognosis of chromophobe renal cell carcinoma. Urol Oncol. 2008;266:604–609.
RENAL CELL CARCINOMA, CLEAR CELL
DESCRIPTION This represents up to 85% of renal tumors. It is thought to arise from the proximal renal tubule and is characterized by a 3p deletion in over 90%. The Von Hippel–Lindau (VHL) gene is found on chromosome 3 (3p25–26) and is involved in the development of clear cell RCC in patients with VHL disease. In addition, VHL gene alterations are involved in the pathogenesis of sporadic RCC. The diffuse positivity for carbonic anhydrase 9 (CA9) is diagnostic for clear cell RCC. A worse prognosis is associated with higher nuclear grade or the presence of a sarcomatoid pattern. Tumors with predominant eosinophilic cells were classified as “granular cell” carcinoma but the 2004 WHO classification of renal tumors based on the presence of vasculature and genetic alterations included these under clear cell RCC. Treatment and staging are discussed elsewhere. (See also Section I: “Renal cell carcinoma, General” and “Von Hippel–Lindau Disease/Syndrome” and Section II: “Renal Cell Carcinoma, Familial.” and (Image ))
SYNONYMS
• Hypernephroma (once thought to be adrenal origin)
• Grawitz tumor
REFERENCE
Delahunt B, Eble Jn. History of the development of the classification of renal cell neoplasia. Clin Lab Med. 2005;25(2):231–246.
RENAL CELL CARCINOMA, FAMILIAL
DESCRIPTION Approximately 2% of renal cell carcinomas (RCC’s) can be inherited. Familial renal cancers are characterized by an early onset compared with sporadic cases and frequently comprise bilateral and multicentric tumors. Moreover, extrarenal features suggestive of a described familial renal cancer syndrome might be present. See table for common renal tumor familial syndromes. (See also Section I: “Renal Cell Carcinoma, General.”)
REFERENCES
Lopez-Beltran A, Scarpelli M, Montironi R, et al. 2004 WHO classification of the renal tumors of the adults. Eur Urol. 2006;49(5):798–805.
Refae MA, Wong N, Patenaude F, et al. Hereditary leiomyomatosis and renal cell cancer: An unusual and aggressive form of hereditary renal carcinoma. Nat Clin Pract Oncol. 2007;4:256–261.
RENAL CELL CARCINOMA, PAPILLARY TYPES 1 AND 2
DESCRIPTION An uncommon variant of RCC, representing ∼10–15% of cases. The lesions exhibit a tubulo-papillary growth pattern. A hereditary pattern is demonstrated in a small number of families, tending to be multifocal, bilateral, and associated with a loss of short arm of chromosome 3. Subtypes are cytologically classified as type 1 with small single-layer cells, and type 2 with large pseudostratified cells. Type 1 papillary RCC typically exhibits genetic alterations including gains of chromosome 7 and 17 and loss of Y, which has a better prognosis; type 2 has a more heterogeneous genetic alterations and a poorer prognosis. CK7 and AMACR are excellent markers for papillary RCC. (See also Section I: “Renal Cell Carcinoma, General” and (Image ).)
REFERENCE
Pignot G, Elie C, Conquy S, et al. Survival analysis of 130 patients with papillary renal cell carcinoma: Prognostic utility of type 1 and type 2 subclassification. Urology. 2007;69(2):230–235.
RENAL CELL CARCINOMA, SARCOMATOID
DESCRIPTION An uncommon histologic variant of RCC, with an approximate incidence of 5–10% of all cases. Histologically, the lesion is composed of clear cells and pleomorphic spindle cells resembling sarcoma. It tends to have a more malignant behavior and worse prognosis, with higher local recurrence, more frequent metastasis, and shorter survival. Sunitinib shows efficacy in advanced renal tumors with sarcomatoid differentiation particularly in patients with good performance status. (See also Section I: “Renal Cell Carcinoma, General” and “Renal Sarcoma, Adult and Pediatric.” and (Image ))
REFERENCES
Kunene V, Miscoria M2, Pirrie S3, et al. Sarcomatoid renal cell carcinoma: Clinical outcome and survival after treatment with sunitinib. Clin Genitourin Cancer. 2013;pii: S1558–7673(13)00312–1. doi: 10.1016/j.clgc.2013.12.001. [Epub ahead of print]
Oda H, Machinami R. Sarcomatoid renal cell carcinoma. A study of its proliferative activity. Cancer. 1993;71(7):2292–2298.
RENAL CELL CARCINOMA, TUBULOCYSTIC
DESCRIPTION Tubulocystic carcinoma of the kidney (TCCK), a newly described entity, is well-demarcated multicystic lesions giving a “wrapped bubble” or “spongy” appearance. Microscopically, the tumors were composed of tubules and cysts lined by a single layer of eosinophilic, columnar, cuboidal, flat, or hobnail cells with large nuclei and prominent nucleoli separated by a thin fibrotic stroma. In all TCCKs, the majority of neoplastic cells showed immunohi-stochemical (CD10(+), RCC(+), vimentin(+), and AMACR(+)) and ultrastructural (abundant long brush border microvilli) characteristics of proximal renal tubules, but the cell of origin is unclear. The major differential diagnostic considerations are oncocytoma, multilocular cystic RCC, and cystic nephroma/mixed epithelial and stromal tumor of the kidney. It generally pursues an indolent clinical course. Nephrectomy/ partial nephrectomy of involved renal unit is usually necessary (Image ).
REFERENCE
Alexiev BA, Drachenberg CB. Tubulocystic carcinoma of the kidney: a histologic, immunohistochemical, and ultrastructural study. Virchows Arch. 2013;462(5):575–581.
RENAL CELL CARCINOMA, UNCLASSIFIED
DESCRIPTION A diagnostic category to which renal carcinomas should be assigned when they do not fit into 1 of the other categories, even after genetic analysis. In some series, this group has amounted to 3–5% of cases. Features which might prompt the assignment of a carcinoma to this category include apparent composites of recognized types, sarcomatoid morphology without recognizable epithelial elements, mucin production, mixtures of epithelial and stromal elements, and unrecognizable cell types. Unclassified RCC is associated with distinct and highly aggressive biologic behavior with poor clinical outcomes. Whenever feasible, immunotherapy with nephrectomy is warranted.
REFERENCES
Kovacs G, Akhtar M, Beckwith BJ, et al. The Heidelberg classification of renal cell tumours. J Pathol. 1999;183:131–133.
Zisman A, Chao DH, Pantuck AJ, et al. Unclassified renal cell carcinoma: Clinical features and prognostic impact of a new histological subtype. J Urol. 2002;168:950.
RENAL CELL CARCINOMA, XP11.2;TFE3 TRANSLOCATIONS
DESCRIPTION A number of sporadic cases of RCC have chromosomal translocations involving the TFE3 gene at chromosome Xp11.2. Children and young adults are affected without predilection for gender however there appears to be a female predilection in the adult population. The histologic features of such cases have been variably described as papillary RCC, clear cell RCC, or a unique type of pathology. Papillary RCC is the most reported subtype associated with the translocation. The TFE3 gene encodes a transcription factor related to the proto-oncogene product c-myc. RCC is likely due to TFE3 overexpression. TFE3, TFEB, and ALK protein expression are crucial in establishing the diagnosis. This is an aggressive disease and targeted therapy (sorafenib, sunitinib, temsirolimus) and VEGF-targeted therapy demonstrate some activity in advanced disease (Image ).
REFERENCE
Malouf GG, Camparo P, Oudard S, et al. Targeted agents in metastatic Xp11 translocation/TFE3 gene fusion renal cell carcinoma (RCC): A report from the Juvenile RCC Network. Ann Oncol. 2010;21(9):1834–1838.
RENAL CHOLESTEROL EMBOLISM SYNDROME
DESCRIPTION Cholesterol microembolism (also called cholesterol emboli and cholesterol crystal emboli) of the kidney is an uncommon cause of hypertensive emergencies, affecting mainly elderly men with atherosclerotic vascular disease. It is increasingly associated with thrombolytic therapy. Clinical findings include severe hypertension, digital gangrene, livedo reticularis, cerebrovascular accidents, GI hemorrhage or infarction, bowel perforation, retinal emboli, and eosinophilia. Dialysis for renal insufficiency might be necessary. Diagnosis is made from clinical history, physical exam, lab findings, and selective renal angiogram, and is confirmed by renal biopsy. In the kidneys, intralobular and arcuate arteries are most frequently affected. Treatment is supportive and preventative with management of hypertension and renal insufficiency through control of the underlying pathology.
CAUSES
• Angiographic manipulation
• Anticoagulant medications
• Cardiovascular surgery
• Iatrogenic
• Spontaneous
REFERENCE
Hitti WA, Anderson J. Cholesterol emboli-induced renal failure and gastric ulcer after thrombolytic therapy. South Med J. 2005;98(2):235–237.
RENAL CORTICAL ADENOMA
DESCRIPTION Also called papillary adenoma of the kidney this is a type of benign renal neoplasm. It falls under the general category of renal adenomas and is considered one of the most common of renal epithelial neoplasms. These small papillary tumors are characterized by scant cytoplasma (chromophilic cells), occasionally somewhat eosinophilic, with tubular-papillary patterns, limited but not encapsulated. Renal papillary adenoma defines papillary or tubular architecture of low nuclear grade with a maximum diameter of 5 mm. Based on autopsy series prevalence rate is 40% in patients older than 70. It has been postulated that papillary adenoma may progress to papillary RCC because of high coexistence, histopathologic similarity, and similar genetic alterations between papillary adenoma and papillary RCC. Cytogenetic changes of papillary adenomas include loss of the Y chromosome and combined trisomy of chromosomes 7 and 17. Histologic and genetic abnormalities of small renal adenomas are indistinguishable from larger papillary RCCs. Based on these findings it is now considered that these small benign lesions can increase in size and transform into papillary carcinomas. For this reason, the WHO 2004 renal cell tumors classification considered tumors with a maximum diameter of 5 mm as papillary adenomas. Renal papillary adenoma frequently develops in patients with acquired renal cystic disease and long-term hemodialysis and may have a different pathogenesis (Image ).
REFERENCES
Algaba F. Renal adenomas: pathological differential diagnosis with malignant tumors. Adv Urol. 2008:974848. doi: 10.1155/2008/974848. Epub 2008 Oct 8.
Wang KL, Weinrach DM, Luan C, et al. Renal papillary adenoma–a putative precursor of papillary renal cell carcinoma. Hum Pathol. 2007;38(2):239–246.
RENAL HEMANGIOMA
DESCRIPTION These benign vascular neoplasms are usually diagnosed in the 3rd–5th decades, with no sex predilection. The most common presenting symptom is intermittent hematuria. Angiographic appearance varies markedly, with hypervascular, hypovascular, and normal lesions being reported. In the past, a clinical finding of unilateral hematuria and a suggestive angiographic pattern were the basis of preoperative diagnosis. Currently, hemangioma can be identified ureteroscopically, without the need for a biopsy, where they may appear as small, red or bluish spots on the tip or base of a papilla, or they may be large, bulbous, erythematous lesions on the papillary tips. Pathologically, hemangiomas have the gross appearance of a well-demarcated lesion that shows a cluster of blood-filled vascular channels. Microscopically, the majority of cases conform to the typical features of cavernous hemangioma, with variable, large, blood-filled vascular tributaries in a disorganized tangle. Variation in vascular wall thickness and structure indicates arterial and venous components. The benign cytologic feature of flat lining endothelial cells allows for differentiation of this lesion from angiosarcoma.
TREATMENT
• Ureteroscopic electrocauterization or laser ablation using holmium, ND:YAG, or a combination of both
• Surgical resection
REFERENCE
Waller JI, Throckmorton MA, Barbosa E. Renal hemangioma. J Urol. 1995;74:186.
RENAL HEMANGIOPERICYTOMA
DESCRIPTION A rare primary sarcoma of the kidney, accounting for 1–3% of renal malignancies. Solid hypervascular mass with calcifications, originating from pericytes, located external to endothelial cells of capillaries, and enveloped by basement membrane. It can be metabolically active, secreting renin. Common presenting signs include flank pain, flank mass, hypertension, hypoglycemia, and hematuria. It can be treated by radical nephrectomy, with reports of nephron-sparing surgery.
REFERENCE
Brescia A, Pinto F, Gardi M, et al. Renal hemangiopericytoma: Case report and review of the literature. Urology. 2008;71(4):755.e9–e12.
RENAL LEIOMYOMA
DESCRIPTION A rare benign renal tumor, with an incidence of 5% on autopsy series, it originates from smooth muscle, usually from renal capsule or vessels, and less commonly from the renal pelvis. Radiographically, it can appear as a solid or cystic mass, difficult to differentiate from RCC, thus usually prompting radical nephrectomy. Imaging findings that can help to suggest the diagnosis of renal leiomyomas are tumors that are hyperdense before contrast, with density similar to that of muscles, and with lower enhancement than the adjacent renal parenchyma. Most are peripheral, without involvement of the renal cortex and with well-defined margins. (See also Section I: “Renal Mass.”)
REFERENCE
Derchi LE, Grenier N, Heinz-Peer G, et al. Imaging of renal leiomyomas. Acta Radiol. 2008;49(7):833–838.
RENAL LEIOMYOSARCOMA
See Section I: “Renal Sarcomas, Adult and Pediatric.”
RENAL LYMPHANGIECTASIA
DESCRIPTION A very uncommon benign disorder of renal lymphatics with unknown pathophysiology, the condition can present with perinephric collections, ascites, abdominal pain, and reversible hypertension. Imaging demonstrates enlarged kidneys with fluid collections seen to be abutting the surrounding structures. Perinephric fluid analysis usually demonstrates elevated protein levels with leucocytes (mostly lymphocytes). Management alternatives range from percutaneous drainage in symptomatic cases to medical therapy (antihypertensives and diuretics).
REFERENCE
Ashraf K, Raza SS, Ashraf O, et al. Renal lymphangiectasia. Br J Radiol. 2007;8095(4):e117–118.
RENAL MALROTATION
DESCRIPTION The abnormal orientation of the kidney, such that the position of the renal pelvis is not antero-medial, and the calyces are not pointing laterally. This condition may occur in cases of ectopia, fusion, and incomplete renal ascent; it has an incidence of 1 in 390. Three types of renal malrotation have been described
• Nonrotation: The renal pelvis is anterior.
• Reverse rotation: The renal pelvis is lateral.
• Hyper-rotation: The renal pelvis is posterior.
Symptoms are usually absent; occasionally, vague abdominal pain and/or vomiting due to renal obstruction may occur. Patients may develop ureteral obstruction, infections, or calculi. Diagnosis is made typically on excretory urography with altered orientation of the calyces and pelvices. Retrograde pyelography is often useful in defining the anatomy. No treatment is necessary unless symptoms, stones, or obstruction become problematic. Follow-up US to evaluate for stones or hydronephrosis is recommended. (See also Section I: “Renal Ectopia.”)
REFERENCE
Kelalis P, et al., eds. Clinical Pediatric Urology. 3rd ed. Vol. 1. Informa Healthcare; 1992:505–507.
RENAL MASS, INDETERMINATE
DESCRIPTION An indeterminate renal mass is one that cannot be diagnosed confidently as benign or malignant at the time it is discovered. The American College of Radiology has provided appropriateness criterion for the radiographic evaluation of the indeterminate renal mass. The utility of radiologic procedures in the evaluation of the indeterminate renal mass are summarized in the table on the next page.
REFERENCE
Israel GM, et al. ACR Appropriateness Criteria® indeterminate renal masses. Reston (VA): American College of Radiology (ACR); 2010. Online Publication Available at AHRQ (Agency for Healthcare Research and Quality) http://www.guideline.gov/content.aspx?id=32641&search=renal+masses (Accessed August 30, 2014)
RENAL MEDULLARY CARCINOMA
DESCRIPTION This is an aggressive renal malignancy limited almost exclusively to young black patients with sickle cell trait and, less commonly, sickle cell disease. Typical age of presentation is 21–24 yr of age with a male predominance. Over 75% occur in the right kidney. Genetic testing has suggested a potential association with various chromosomal anomalies. It is considered a variant of collecting duct carcinoma. Patients usually present with gross hematuria, UTI, flank pain, an abdominal mass, and/or weight loss. Metastasis in the cervical nodes or brain are often the initial finding. Hematuria in the target population should prompt evaluation for renal medullary carcinoma. Imaging typically demonstrates a centrally located infiltrative lesion invading the renal sinus with peripheral caliectasis. Size ranges from 4–12 cm (averaging 7 cm) and usually associated with hemorrhage and necrosis. Metastatic disease is commonly present at diagnosis. Surgical resection is not usually curative. There is limited experience in the treatment of metastatic disease. Survival after diagnosis is usually <6–12 mo (Image ).
REFERENCE
Dimashkieh H, Choe J, Mutema G, et al. Renal medullary carcinoma a report of 2 cases and review of the literature. Arch Pathol Lab Med. 2003;127:E135–E138.
RENAL OSTEODYSTROPHY
DESCRIPTION Renal osteodystrophy is defined by the National Kidney Foundation as bone morphology alterations observed in chronic kidney disease. Several changes can occur, including osteitis fibrosa cystica due to secondary hyperparathyroidism, osteomalacia, or low bone turnover. The most common presenting symptoms are bone fracture and pain, and usually occurring once a patient is on dialysis. Treatment may include treating hyperphosphatemia, Calcitrol, vitamin D analogs, calcimimetics, or parathyroidectomy.
REFERENCE
Moe S, Drüeke T, Cunningham J, et al. Definition, evaluation, and classification of renal osteodystrophy: A position statement from Kidney Disease: Improving Global Outcomes (KDIGO). Kidney Int.2006;69:1945–1953.
RENAL PSEUDOTUMOR
DESCRIPTION A benign condition of the kidney mimicking a renal neoplasm on radiographic studies. Most commonly is a hypertrophied column of Bertin (sometimes referred to as an anomalous calyx), a prominent medullary column usually located between the upper and middle pole calyceal infundibula that can appear as a renal mass but is homogeneous with surrounding renal parenchyma, with normal-appearing calyces. Other conditions giving appearance of renal tumor include persistent fet al lobulation, dromedary or splenic humps, arteriovenous malformation (AVM), extramedullary hematopoiesis, splenorenal fusion (extremely rare), lobar nephronia (focal glomerulonephritis), renal abscess, scarred kidney, regenerating nodule after reflux. xanthogranulomatous pyelonephritis (XGP), granulomatous diseases (sarcoidosis and malakoplakia) subepithelial renal pelvic hematoma (due to anticoagulant or vasculitis) and renal sinus lipomatosis. Contrast-enhanced ultrasound, CT, or MRI can all assist in characterization, but final diagnosis may require percutaneous biopsy or surgery. (See also Section I: “Renal Mass” and Section II: “Renal Mass Indeterminate.”)
REFERENCES
Bhatt S, MacLennan G, Dogra V. Renal Pseudotumors. Am J Roentgenol. 2007;188:1380–1387.
Mazziotti S, Zimbaro F, Pandolfo A, et al. Usefulness of contrast-enhanced ultrasonography in the diagnosis of renal pseudotumors. Abdom Imaging. 2010;35(2):241–245.
RENAL SINUS ABNORMALITIES
DESCRIPTION The renal sinus is a central spacious cavity in which major branches of the renal artery and vein, along with the major and minor calyces of the collecting system, are located. It is usually filled with adipose tissue, lymphatic channels, nerve fibers, and fibrous tissue. Lesions that may develop in the sinus could be benign, including parapelvic simple cysts, lipomatosis, cysts, urinomas, and vascular lesions such as renal artery aneurysm or AV fistula. Malignant tumors originating in renal pelvis or parenchyma, such as TCC and RCC, may also protrude or even invade and obliterate renal sinus fat. Primary tumors, such as hemangioma, fibroma, leiomyoma, and MFH are rare but may develop in the space.
REFERENCE
Rha SE, Byun JY, Jung SE, et al. The renal sinus: Pathologic spectrum and multimodality imaging approach. Radiographics. 2004;24(Suppl 1):S117–S131.
RENAL TRANSPLANT TYPES (STANDARD/EXTENDED/DONOR AFTER DEATH)
DESCRIPTION Renal transplant donors can be by living donors or deceased donors. Deceased donors are individuals who meet the criteria for brain death, but whose organs are being perfused by life-support methods, allowing adequate time for procurement. Donor after cardiac death is an expanded criteria donor. Nonheart beating donor (NHBD) death is characterized by irreversible absence of circulation. NHBDs are less than ideal because organs suffer ischemia during prolonged periods of circulatory dysfunction. Nearly /2 of all renal transplants are from deceased donors. Living donors can be either related or unrelated. On preoperative evaluation, normal renal function after donation is the goal. In line with this, the better functioning kidney is left with the donor. HLA-identical siblings have the highest graft survival rates followed by 1-haplotype siblings. In paired donor exchange, also known as kidney swap, 2 kidney recipients exchange willing donors whom they are incompatible with. This allows the donors to provide 2 recipients with grafts where previously no transplant would have been possible. Overall, living donor grafts have higher survival rates, with 80% surviving at the 5-yr mark compared to 67% of deceased donor grafts (Image ).
REFERENCE
Dunn D. Kidney Transplantation. In: Schwartz SI, et al. Schwartz’s Principles of Surgery. 9th ed. New York, NY: McGraw-Hill; 2010.
RENAL TRANSPLANTATION AND NEOPLASIA
DESCRIPTION Transplant recipients have an elevated risk of cancer. There is a very small risk of primary malignancy harbored in the graft being transferred to the recipient. The most common malignancies that form after transplantation are de novo malignancies. They are thought to occur secondary to chronic use of immunosuppressive drugs. After 10 yr of immunosuppression, kidney transplant recipients have a cumulative incidence of cancer as high as 20%. The most common are Kaposi’s sarcoma and non-Hodgkin lymphoma. Compared to age and sex matched general population, kidney cancer recipients are 3–5-fold increased risk of developing skin cancers and urologic malignancies. Recipients who have been on hemodialysis are also at a higher risk of acquired cystic disease and subsequent primary renal malignancy.
REFERENCE
Piselli P, Serraino D, Segoloni GP, et al. Risk of de novo cancers after transplantation: Results from a cohort of 7217 kidney transplant recipients, Italy 1997-2009. Eur J Cancer. 2013;49(2):336–344.
RENAL TUMORS, WHO 2004 CLASSIFICATION
DESCRIPTION The 2004 World Health Organization (WHO) classification of the adult renal epithelial neoplasms replaced the previous 1998 WHO classification. It incorporates other classifications (Mainz, Heidelberg) and describes entities based on both pathologic and genetic analyses. (See table)
REFERENCE
Eble JN, et al. Pathology and Genetics. Tumors of the urinary system and male genital organs. Lyon: IARC Press; 2004.
RENAL VEIN, LEIOMYOSARCOMA
DESCRIPTION A rare tumor arising from the smooth muscle element of the renal vein, occurring most commonly on the left side. The highest incidence is seen in 60–69 year olds and is twice as common in women. Only 32 cases of renal vein leiomyosarcoma have been reported in the literature. The renal vein is the most common site of venous leiomyosarcoma outside of the vena cava. Presenting symptoms include flank or abdominal pain, weight loss, and a palpable abdominal mass. 3-dimensional abdominal imaging imaging reveals a homogeneous, well-circumscribed mass at or near the renal hilum, commonly encasing the renal vein. Mean survival is 28 mo, with an aggressive malignant pattern to distant sites, including lung, liver, bone, skin and soft tissue, and brain.
TREATMENT
• Aggressive surgical resection with nephrectomy and en-bloc resection
• Nonsurgical treatment with XRT and chemotherapy can be instituted but the efficacy is limited.
REFERENCE
Gage MJ, Patel AV, Koenig KL, et al. Non-vena cava venous leiomyosarcomas: a review of the literature. Ann Surg Oncol. 2012;19(11):3368–3374.
RENAL–RETINAL SYNDROME
DESCRIPTION Also called juvenile nephronophthisis with retinal disease or Senior-Loken syndrome (SLS) it is a subtype of juvenile nephronophthisis. (See Section II: “Juvenile Nephronophthisis.”) It is an autosomal recessive disease characterized by development of retinitis pigmentosa- or Leber congenital amaurosis-like retinal dystrophy and a medullary cystic kidney disease. These patients have concomitant retinitis pigmentosa, which is slowly progressive and bilateral, with retinal degeneration. Rods are affected, leading to defective night vision that becomes symptomatic in early childhood. The disc may look yellow and waxy. Treatment is supportive, with renal replacement therapy as needed.
SYNONYMS
• Juvenile nephronophthisis with retinal disease
• Senior–Loken syndrome
REFERENCE
Ronguillo CC, Bernstein PS, Baehr W. Senior-Loken syndrome: A syndromic form of retinal dystrophy associated with nephronophthisis. Vision Res. 2012;75:88–97.
RENIN, PLASMA AND RENAL VEIN
DESCRIPTION In suspected renovascular hypertension, an elevated renin level of >50% in the renal vein compared to the renal artery plasma level (estimated from the IVC renin level) of the affected kidney is diagnostic. Individual renal veins can be sampled to isolate ischemic individual renal segments using this same technique. Normal, morning plasma renin activity for seated subjects ranges from about 1– 4 ng/mL/h (0.8–3.0 nmol/L/h). Corresponding active renin concentrations are 8–35 mU/L. Renin is increased with diuretics (including spironolactone), dihydropyridine calcium channel blockers, angiotensin converting enzyme inhibitors and angiotensin receptor antagonists. Levels are decreased by β-blockers, clonidine, or α-methyldopa (all of which reduce β-sympathetic stimulation of renin release), or nonsteroidal anti-inflammatory agents (which promote salt retention and also inhibit renal prostaglandin production).
• Normal renin: Secondary adrenal insufficiency (ie, hypopituitarism or isolated ACTH deficiency); Cushing syndrome, but they can be low when there is a marked degree of hypercortisolism.
• Low renin: Primary aldosteronism; with 11β-hydroxylase or 17α-hydroxylase deficiency (due to mutations in CYP11B1 and CYP17, respectively), which are hypertensive forms of CAH; primary glucocorticoid resistance; DOC-producing adrenal tumors; ectopic ACTH syndrome.
REFERENCES
Rossi GP, Cesari M, Chiesura-Corona M, et al. Renal vein renin measurements accurately identify renovascular hypertension caused by total occlusion of the renal artery. J Hypertens. 2002;2:975–984.
Stowasser, M. Assays of the renin-angiotensin-aldosterone system in adrenal disease. In UpToDate.com, Accessed March 7, 2014.
RENINOMA (RENIN-SECRETING JUXTAGLOMERULAR CELL TUMOR)
DESCRIPTION A rare renin-secreting tumor of the juxtaglomerular apparatus. Its usual presentation is in a young female with severe, refractory, frequently paroxysmal hypertension with hypokalemia, hyperaldosteronism, and elevated plasma renin levels. Selective renal vein sampling for renin may help localize the lesion. It is generally a benign tumor, although untreated hypertension can be fatal. Surgical intervention (open or laparoscopic) may be curative and in high-risk patients, radiofrequency ablation may be a less invasive alternative to radical nephrectomy (Image ).
REFERENCE
Chao CT, Chang FC, Wu VC, et al. Reninoma. Kidney Int. 2011;79(2);260.
RENO-ALIMENTARY FISTULA
DESCRIPTION A broad group of nonanatomic communications between the upper urinary collecting system and the alimentary canal, with nephrocolic and right renal pelvis and duodenal fistulas being the most common presentations. Symptoms vary from GI symptoms, such as nausea, vomiting, and diarrhea, to recurrent UTIs with flank pain and fever. Retrograde ureterography is generally needed to visualize the fistula. (See also Section II: “Fistula, Enterovesical.”)
CAUSES
• Renal inflammatory disease acute/chronic)
• Malignancy of either intestinal or renal origin
• Iatrogenic (eg, percutaneous surgery)
• Trauma
• Ulcer disease
TREATMENT
• Conservative: Stenting or nephrostomy tube
• Nephrectomy with removal of fistula tract and bowel resection
REFERENCE
Bissada NK, Cole AT, Fried FA. Reno-alimentary fistula: An unusual urological problem. J Urol. 1973;110:273–276.
RENO-BRONCHIAL FISTULA
DESCRIPTION Fistulous communication between pleural cavity and kidney, associated with pyelonephritis and perinephric abscess. Usually presents with flank or abdominal pain with an ipsilateral pneumonia. Patients commonly have a history of pyelonephritis or abdominal abscess. Fistulas involving the kidney are most commonly caused by iatrogenic trauma. These may include percutaneous nephrostomy or nephrolithotomy tract placement. Reno-bronchial fistulas usually develop from an upper pole approach to the kidney, where the pleural cavity may be traversed. It can involve pleural space alone or erode into lung parenchyma and bronchial tree. Diagnosis is made with CT.
SYNONYMS
• Nephrobronchial fistula
• Renal, bronchopleural fistula
CAUSES
• Pyelonephritis with perinephric abscess
• Xanthogranulomatous pyelonephritis
• Most common pathogens: E. coli and Proteus sp account for 1/3 of cases
• Tubercular infections also reported
• Iatrogenic trauma: Percutaneous nephrostomy, nephrolithotomy tract placement
TREATMENT
• Percutaneous or open drainage and antibiotic therapy acutely
• Nephrectomy may ultimately be required
REFERENCE
Qazi H, Manikandan R, Holmes ME, et al. Nephrobronchial fistula- A case report. Int Urol Nephrol. 2007;39(1):31–32.
RENOMEDULLARY INTERSTITIAL CELL TUMOR (MEDULLARY FIBROMA, RENAL HAMARTOMA)
DESCRIPTION Also referred to as medullary fibromas and renal hamartomas, this is a benign renal mesenchymal neoplasm. Rarely they are large and symptomatic. It is a common incidental finding in as many as 50% of adults at autopsy. Renomedullary interstitial cell tumor arises from interstitial cells of the medulla. They do not appear to have any effect on blood pressure. At gross exam, medullary fibromas are white or gray nodules within the renal pyramid. At histologic exam, this tumor is characterized by stellate spindle cells in the background of a basophilic stroma. It can be seen on CT as a small nonenhancing noncalcified hypoattenuating solid mass within the renal medulla. Although it is a benign tumor, it is difficult to differentiate this lesion from other malignancies of the kidney on radiologic basis and hence many patients undergo radical nephrectomy (Image ).
REFERENCES
Katabathina V, Vikram R, Nagar AM, et al. Mesenchymal neoplasms of the kidney in Adults: Imaging spectrum with radiologic-pathologic correlation. Radiographics. 2010;30(6):1525–1540.
Kumar S, Choudhary GR, Nanjappa B, et al. Benign medullary fibroma of the kidney: A rare diagnostic dilemma. J Clin Imaging Sci. 2013;3:43.
REPERFUSION INJURY, RENAL (RENAL ISCHEMIA AND REPERFUSION INJURY)
DESCRIPTION Ischemic acute kidney injury (AKI) is a syndrome that develops following a transient drop in total or regional blood flow to the kidney. Although reperfusion is essential for the survival of ischemic tissue, there is evidence that reperfusion itself causes additional cellular injury after a period of ischemia. This can occur during renal transplantation and with vascular clamping during partial nephrectomy. Studies comparing partial nephrectomy with and without clamping show that ischemia is associated with an increased risk of ARF and advanced chronic kidney disease. Oncologic outcomes and complications in partial nephrectomy without clamping are similar to those with clamping. The clinical sequelae of warm ischemia reperfusion renal injury of approximately 30 min in one study of laparoscopic partial nephrectomy are minimal, however advanced age and pre-existing azotemia increase the risk of renal dysfunction after partial nephrectomy, especially when the warm ischemia time exceeds 30 min. No renoprotective drug therapy has proven clinical utility and renal cooling has not been proven uniformly beneficial. Attempting partial nephrectomy without vascular clamping is optimal. If renal vascular clamping is necessary, it seems that this should be for <30 min to limit renal reperfusion injury.
REFERENCES
Desai MM, Gill IS, Ramani AP, et al. The impact of warm ischaemia on renal function after laparoscopic partial nephrectomy. BJU Int. 2005;95(3):377–383.
Wszolek MF, Kenney PA, Libertino JA. Nonclamping partial nephrectomy: towards improved nephron sparing. Nat Rev Urol. 2011;8(9):523–527.
RESIDUAL URINE (POSTVOID RESIDUAL [PVR])
DESCRIPTION PVR is the amount of urine remaining immediately after a void. It can be measured by US (bladder scan) or directly by catheterization. Significant variability occurs between PVR measurements in any 1 individual, and several separate measurements should be performed. The absolute value of PVR has little significance but can indicate voiding dysfunction in symptomatic individuals. Therefore, PVR should be part of any assessment of patients with incontinence, LUTS, recurrent UTI, suspected bladder outlet obstruction, or neurogenic bladder.
REFERENCE
Kaplan SA, Wein AJ, Staskin DR, et al. Urinary retention and postvoid residual urine in men: Separating truth from tradition. J Urol. 2008;180:47–54.
RESISTIVE INDICES (RI)
DESCRIPTION The RI is a measurement of renal blood flow using Doppler ultrasound (US). It is defined as the PSV minus the end-diastolic velocity divided by PSV. It used to evaluate upper tract urinary obstruction as well as graft function in transplanted kidneys. Although there are no accepted parameters of normal RI, values >0.7 suggest obstruction or graft hypoperfusion.
REFERENCE
Nezami N, Tarzamni MK, Argani H, et al. Doppler ultrasonographic indices after renal transplantation as renal function predictors. Transplant Proc. 2008;40:94–99.
RETE TESTIS, ADENOCARCINOMA
DESCRIPTION A rare, highly malignant tumor arising from rete testis that usually occurs in older men, but has been reported in men as young as 17. It commonly presents with a painless scrotal mass or symptoms related to metastasis. Pathology reveals papillary adenocarcinoma in the rete testis, commonly with local invasion. May be associated with maldescended testis or adenomatous hyperplasia of the rete testis. Prognosis is poor, with <50% survival at 1 yr. Metastatic sites include retroperitoneal lymph nodes, lungs, bone, and liver. The diagnostic criteria include:
• Tumor in mediastinum separate from the body of testis
• Transition in rete testis from normal epithelium to neoplastic cells
• No evidence of teratoma
• No primary tumor elsewhere
• Intact pariet al tunica
TREATMENT
• Radical orchiectomy is the mainstay of treatment.
• XRT and chemotherapy have limited efficacy for metastatic disease.
• Retroperitoneal lymph node dissection may have a role in the absence of metastasis.
REFERENCE
Sogni F. Primary adenocarcinoma of the rete testis: Diagnostic problems and therapeutic dilemmas. Scand J Urol Nephrol. 2008;(421):83–85.
RETE TESTIS, TUBULAR ECTASIA AND CYSTIC DYSPLASIA
DESCRIPTION Tubular ectasia of the rete testis is a benign condition (sometime called cystic ectasia of the rete testes) seen mostly in older men, which involves cystic dilatation of the rete testis often detected on ultrasound. Ovoid cluster of anechoic cystic spaces located peripherally in the mediastinum testis (without any solid component) and no flow within the lesion on Doppler imaging and normal adjacent testicular parenchyma are pathognomic for tubular ectasia of the rete testis. It is believed that this is secondary to obstruction of the epididymis as 85% of cases have coexisting epididymal abnormalities like epididymal cysts. Vasectomy, spermatoceles or epididymitis, may also be associated with dilatation of the rete testis. It must be differentiated from testicular neoplasm and MRI is potentially helpful. It is unclear whether this lesion is a cause of infertility.
Cystic dysplasia of the rete testis is similar sonographically and histologically, but it is a congenital lesion that occurs in children. It is associated with ipsilateral renal or urogenital excretory duct malformations.
Both of these lesions (tubular ectasia and cystic dysplasia) must be differentiated from benign intratesticular varicocele and tumors such as adenocarcinoma of the rete testis.
REFERENCES
Gadodia A, Goyal A, Thulkar S. Ectasia of the rete testis: Beware of this masquerader. Indian J Urol. 2010;26(4):593–594.
Jequier AM, Phillips N. Cystic dilatation of the rete testis: a hidden diagnosis among infertile men. Reprod Biomed Online. 2009;18(2):190–194.
RETROCAVAL/CIRCUMCAVAL URETER
DESCRIPTION A rare congenital anomaly in which the infrarenal vena cava (IVC) is derived from the right subcardinal or postcardinal vein, anterior to the ureter. The termcircumcaval ureter refers to the ureter emerging medial to IVC after running behind it, whereas the term retrocaval applies to those ureters that only knuckle behind the IVC but re-emerge laterally. Males are affected 3 times more often than are females. Not all retrocaval/circumcaval ureters are obstructed, but if obstruction exists, surgical repair is typically warranted. CT is the best imaging modality for identification. Despite its congenital origin, symptoms are usually absent in childhood and present later in life. Less commonly, patients present with hematuria or UTI.
REFERENCE
Qian ZY, Yang MF, Zuo KQ, et al. Computed tomography manifestations of common inferior vena cava dysplasia and its clinical significance. Exp Ther Med. 2013;5(2):631–635.
RETROGRADE URETHROGRAM (RUG), TECHNIQUE
DESCRIPTION RUG is used to radiographically evaluate the urethra. It is most commonly used to evaluate urethral stricture disease or trauma. It is commonly performed by inserting a Foley catheter or Brodney clamp into the Fossa Navicularis. The balloon is inflated with a few milliliters of water to create a seal. Then 50 cc of contrast solution is injected into the urethra under low pressures while obtaining a series of x-rays. An oblique view allows best visualization of the entire urethra (Image ).
REFERENCE
Berná JD, Berná JD Jr, Aparicio Meson M. Urethrography in the male: The clamp method. Acta Radiol. 2009;50(2):233–237.
RETROPERITONEAL HEMATOMA
DESCRIPTION A retroperitoneal hematoma is hemorrhage contained within the retroperitoneum. Etiologies include disruption of the kidney or renal pedicle from trauma, postoperative hemorrhage, spontaneous hemorrhage of a renal mass typically angiomyolipoma (or RCC), or abdominal vessel hemorrhage. Ecchymosis may be observed around the umbilicus (Cullen sign) or flank (Grey–Turner sign). Management is primarily conservative, including frequent hemoglobin levels, resuscitation, and transfusion, as necessary. However, if the patient is hemodynamically unstable and the bleeding is from a renal source, and they have an expanding pulsatile hematoma, or renal hemorrhage cannot be stopped with selective embolization, then surgical exploration is indicated. Further evaluation of the underlying pathology and follow-up imaging for resolution is warranted. (See also Section I: “Renal Angiomyolipoma”; “Retroperitoneal Abscess”; “Retroperitoneal Masses and Cysts” and (Image ).)
REFERENCE
Heyns CF. Renal trauma: Indications for imaging and surgical exploration. BJU Int. 2004;93:1165–1170.
RETROPERITONEAL LIPOSARCOMA
DESCRIPTION Retroperitoneal liposarcoma is the most common retroperitoneal sarcoma arising from adipose tissue. However retroperitoneal sarcomas are rare, with just 2.7 new cases per 1 million persons reported annually. They are usually identified incidentally or at a locally advanced stage when they cause symptoms from adjacent tissue invasion or compression. Compression of ureters can cause obstruction, but other symptoms can include early satiety, obstruction, or retroperitoneal bleeding. Contrast-enhanced CT or MRI should be used for diagnosis, staging, and surgical planning. On CT it appears as a large encapsulated mass containing variable amount of fatty (<–20 Hounsfield units) and soft tissue components. A germ cell tumor (GCT) must be ruled out by tumor markers, and a biopsy is necessary if there is diagnostic uncertainty. Complete resection is the only curative treatment. Radiotherapy can be used preoperatively for local control. (See also Section I: “Retroperitoneal Masses and Cysts.” and (Image ))
REFERENCE
Chang IY, Herts BR. Retroperitoneal liposarcoma. J Urol. 2013;189(3):1093–1094.
RETROPERITONEAL LYMPHOMA
DESCRIPTION Lymphoma involving retroperitoneal lymph nodes; it can be the primary site of involvement or a site of metastasis. The lesion can cause extrinsic compression of ureters with obstructive uropathy. Positive diagnosis is made when a mass is visualized on CT or a ureteral obstruction is visualized with US or IVP. Differential diagnosis may include retroperitoneal fibrosis, retroperitoneal fat necrosis, lymphangiomas, ganglioneuroma, sarcomas, metastasis form other tumors such as prostate, or bladder or germ cell tumor metastasis. (See also Section I: “Retroperitoneal Masses and Cysts” and (Image ).)
TREATMENT
• CHOP chemotherapy (cyclophosphamide, adriamycin, vincristine, prednisolone) and radiation therapy
• Obstructive uropathy may require ureteral stenting or percutaneous decompression prior to chemotherapy.
REFERENCE
Terao T, Fujii Y, Ikeda I, et al. Retroperitoneal malignant lymphoma showing follicular type: Report of a case. Hinyokika Kiyo. 1992;38(10):1151–1155.
RETROPERITONEAL RHEUMATOID NODULES
DESCRIPTION Rheumatoid nodules (necrobiotic granulomas) are a common extra-articular manifestation of rheumatoid arthritis, usually found in subcutaneous tissue. They have been reported in numerous other locations, including blood vessels, larynx, pharynx, sclera, and extradural space. GU involvement is rare and includes renal cortex and bladder. Retroperitoneal occurrence has been reported and can cause ureteral compression and obstruction requiring ureterolysis and repair. (See also Section I: “Retroperitoneal Masses and Cysts.”)
REFERENCE
Adelson GL, Saypol DC, Walker AN. Ureteral stenosis secondary to retroperitoneal rheumatoid nodules. J Urol. 1982;127(1):124–125.
RETROPERITONEAL SARCOMA
DESCRIPTION Retroperitoneal sarcomas are mesenchymal neoplasms. Approximately ½ of retroperitoneal sarcomas are high-grade tumors, with the most common type being liposarcoma, followed by leiomyosarcoma. Median age at presentation is, although they can occur at any age. At the time of presentation, more than 50% are >20 cm in size. They are typically incidentally diagnosed but when patients do present with symptoms they are abdominal or back pain and increased abdominal girth. Differential diagnosis of a retroperitoneal mass includes neoplasm from a retroperitoneal visceral structure, lymphoma, or a metastatic lesion. Retroperitoneal sarcomas carry a worse prognosis than extremity sarcomas due to the difficulty of complete resection, involvement of critical structures, and delay of diagnosis. Contrast-enchanced CT is the imaging modality of choice. In patients whom systemic therapy or radiation is deemed to be potentially beneficial, a biopsy is mandatory. Complete surgical resection is considered the only curative modality. Neoadjuvant chemotherapy or radiation should be based on optimizing the patient for surgical resection. (See also Section I: “Retroperitoneal Masses and Cysts.”)
REFERENCE
Mullinax JE, Zager JS, Gonzalez RJ. Current diagnosis and management of retroperitoneal sarcoma. Cancer Control. 2011;18(3):177–187.
RETROPERITONEUM, FAT NECROSIS
DESCRIPTION Retroperitoneal fat necrosis is a pathologic syndrome characterized by the histologic hallmarks of coalescence of fat cells into fat cysts bordered by foreign-body giant cell granulomas. Local injury to fat cells from trauma appears to be the initiating event of fat necrosis in the retroperitoneum. In addition, an inflammatory trigger such as acute pancreatitis may also be an initiating event. Its presentation is similar to that of retroperitoneal fibrosis with insidious onset of abdominal or flank pain with the possibility of extrinsic ureteral obstruction.
REFERENCES
Ross JS, Prout GR Jr. Retroperitoneal fat necrosis producing ureteral obstruction. J Urol. 1976;115(5):524–529.
Howard JM. Studies of acute pancreatitis with retroperitoneal necrosis: “The suet syndrome”. Improvements in patient survival Journal of Hepato-Biliary-Pancreatic Surgery 1996, Volume 3, Issue 3, pp. 195–202.
RHABDOID TUMOR, MALIGNANT
DESCRIPTION The most lethal renal neoplasm, formerly believed to be a form of Wilms tumor. It comprises 2% of all renal tumors and primarily affects children and 85% are diagnosed before the age of 5. The incidence has increased from <0.1 per million in 1986 to 0.6 per million in 2005. It has a tendency to early metastatic spread. The most common presentation is an abdominal mass detected in these young patients. Extrarenal sites include central nervous system (35%), liver, and gastrointestinal tract. Presence of mutations in the hSNF5/INI1 gene on chromosome 22 is the hallmark. The lack of staining of the INI1 gene product is diagnostic. Younger patients have a worse prognosis compared with older patients. Radiotherapy is an essential part of multimodality therapy.
REFERENCE
Zhuge Y, Cheung MC, Yang R, et al. Pediatric non-Wilms renal tumors: subtypes, survival, and prognostic indicators. J Surg Res. 2010;163(2):257–263.
RIEGER SYNDROME
DESCRIPTION Also known as the Axenfeld–Rieger Syndrome, this is an autosomal dominant syndrome affecting multiple organ systems. It is manifested by ocular anomalies such as glaucoma; cardiovascular outflow tract malformations, craniofacial abnormalities and pituitary abnormalities which can result in severe endocrinologic sequelae. Genitourinary anomalies occur in the form of hypospadias. 2 major genes have been identified, PITX2 and FOXC1 on chromosome 25.
REFERENCE
Chang TC, Summers CG, Schimmenti LA, et al. Axenfeld-Rieger syndrome: New perspectives. Br J Ophthalmol. 2012;96(3):318–322.
RIFLE CRITERION FOR ACUTE RENAL INJURY
DESCRIPTION Also referred to as the RIFLE Classification System for AKI, it assesses levels of renal injury (Risk, Injury, and Failure) based on the degree of elevation in serum creatinine or urine output, and 2 outcome measures (Loss and End-stage renal disease):
• Risk: 1.5× increase in creatinine or 25% GFR decrease by 25% or urine output <0.5 mL/kg/h for 6 hr
• Injury: 2× increase in creatinine or 50% GFR decrease or urine output <0.5 mL/kg/h for 12 hr
• Failure: 3× increase in creatinine or 75% GFR decrease or urine output of <0.5 mL/kg/h for 24 hr, or anuria for 12 hr
• Loss: Complete loss of kidney function (eg, renal replacement therapy necessary) for >4 wk
• End stage renal disease (ESRD): Complete loss of kidney function (eg, renal replacement therapy necessary) for >3 mo
The RIFLE criteria correlates with prognosis, with a stepwise increase in the risk of death in patients who met the RIFLE criteria for various stages of AKI. Compared to patients without AKI, patients in the RIFLE stages of risk, injury, and failure had increased mortality risks of 2.4, 4.15, and 6.37, respectively. (See also Section I: “Acute Kidney Injury, Adult [Renal Failure, Acute].”)
REFERENCE
Bellomo R, Ronco C, Kellum JA, et al. Acute renal failure–definition, outcome measures, animal models, fluid therapy and information technology needs: The Second International Consensus Conference of the Acute Dialysis Quality Initiative (ADQI) Group. Crit Care. 2004;8(4):R204.
RIM SIGN (RIM NEPHROGRAM)
DESCRIPTION A radiographic appearance in which only a thin peripheral rind of renal tissue is visible. This condition occurs as a result of marked thinning of the parenchyma due to end-stage obstructive atrophy, and it usually denotes irretrievable renal function.
REFERENCE
Bedon WE, Levitt SB, Baker DH, et al. Hydronephrosis in infants and children: Value of high dosage excretory urography in predicting renal salvageability. AJR Am J Roentgenol Radium Ther Nucl Med. 1970;109:380–389.
ROBINOW SYNDROME
DESCRIPTION A skelet al dysplasia with both autosomal dominant and recessive inheritance pattern. It is characterized by short stature, limb shortening, genital hypoplasia (micropenis), and craniofacial abnormalities. The more phenotypically severe autosomal recessive form has been associated with mutations in the ROR2 gene on the long arm of chromosome 9.
REFERENCE
Person AD, Beiraghi S, Sieben CM, et al. WNT5A mutations in patients with autosomal dominant Robinow syndrome. Dev Dyn. 2010;239(1):327–337.
ROBSON STAGING SYSTEM
DESCRIPTION Robson’s modification of Flocks and Kadesky’s staging system for renal cell carcinoma (RCC) was the most commonly used in the United States. The fact that long-term evaluation of patients with stage IIIA lesions, without disease extension into perinephric fat and lymph nodes, has shown survivals comparable to those of stages I and II, has currently led many investigators to prefer the TNM system prosed by IUAC. (See Section I: “Renal Cell Carcinoma, General”; Section VII: “TNM Kidney.”)
• Stage I: Tumor is confined within the kidney parenchyma (no involvement of perinephric fat, renal vein, or regional nodes).
• Stage II: Tumor involves the perinephric fat but is confined within Gerota fascia (including adrenal).
• Stage IIIA: Tumor involves the renal vein or inferior vena cava.
• Stage IIIB: Tumor involves regional lymph nodes.
• Stage IIIC: Tumor involves both local vessels and regional lymph nodes.
• Stage IVA: Tumor involves adjacent organs other than the adrenals (colon, pancreas, etc.).
• Stage IVB: Distant metastases.
REFERENCE
Robson CJ, Churchill BM, Anderson W. The results of radical nephrectomy for renal cell carcinoma. Trans Am Assoc Genitourin Surg. 1968;60:122–129.
ROKITANSKY–KUSTER–HAUSER SYNDROME
DESCRIPTION Pathologic condition characterized by primary amenorrhea and infertility and by congenital aplasia of the uterus and upper 2/3 of the vagina. Also referred to in the literature as Mayer–Rokitansky–Kuster–Hauser Syndrome. The condition is usually discovered during evaluation of a normal-appearing girl who presents with failure of menstruation at the time of expected puberty. They have a normal female karyotype 46, XX and do develop normal secondary sexual characteristics. The syndrome may be caused by the lack of development of the müllerian ducts between the 5th and 6th wk of gestation. In some patients, cyclic abdominal pain suggestive of some functional endometrium is noted. Vaginal reconstruction with bowel or skin grafting is performed. It can also be associated with renal and skelet al abnormalities.
REFERENCE
Pizzo A, Laganà AS, Sturlese E, et al. Mayer-Rokitansky-Kuster-Hauser Syndrome: Embryology, genetics and clinical and surgical treatment. ISRN Obstet Gynecol. 2013;628717.
ROSEWATER SYNDROME
DESCRIPTION Rosewater syndrome is an infertility disorder associated with germ cell aplasia. On testicular biopsy, the seminiferous tubules contain only Sertoli cells. This is considered irreversible and precludes germ cell restoration. Not infrequently, tubular and peritubular fibrosis is associated with germ cell aplasia.
REFERENCE
Craig JM. The pathology of infertility. Pathol Ann. 1975;10:299.
ROVSING POLYCYSTIC KIDNEY OPERATION
DESCRIPTION A historically important surgical procedure that entails unroofing multiple renal cysts. This procedure does not prevent deterioration of renal function but may improve flank pain if the cysts cause obstruction of the collecting system.
REFERENCE
Yates-Bell JG. Rovsing’s Operation For Polycystic Kidney The Lancet , Volume 269, Issue 6960, pp. 126–128; 1957.
ROVSING SYNDROME
DESCRIPTION This is a symptom complex associated with horseshoe kidney, in which the patient experiences pain (often periumbilical), nausea, and vomiting on hyperextension of the spine. It is due to compression of the isthmus of the fused kidney on the vena cava and aorta, accentuated by hypertension and accompanied by a sensation of fullness.
REFERENCE
Glenn JF. Analysis of 51 patients with horseshoe kidney. N Engl J Med. 1959;261:684.