TABES DORSALIS
DESCRIPTION Tertiary syphilis involving the dorsal spinal roots and posterior spinal column. This condition can present with voiding dysfunction, presumably due to loss of bladder sensation, with high residual volumes and urinary retention. Urodynamic evaluation reveals detrusor atony and detrusor areflexia. (See Section I: “Syphilis.”)
SYNONYMS
• Neurosyphilis
• Tabetic bladder
• Tertiary syphilis
TREATMENT
• Penicillin for syphilis
• Clean intermittent catheterization (CIC) for bladder atony
REFERENCE
Erturk E, Sheinfeld J, Davis RS. Voiding dysfunction in tertiary syphilis. Urology. 1987;30(3):284–286.
TAGHAANDAN
DESCRIPTION The practice of forcibly snapping or cracking an erect penis to achieve rapid detumescence. This is a common cause of penile fracture in Middle Eastern countries. In Iran, 69% of penile fractures are due to this mechanism and were encountered at an average of one per wk. Treated as described in Section I: “Penis, Trauma.”
REFERENCE
Zargooshi J. Penile fracture in Kermanshah, Iran: Report of 172 cases. J Urol. 2000;164(2):364–366.
TAKAYASU ARTERITIS, UROLOGIC CONSIDERATIONS
DESCRIPTION A vasculitis of unknown origin involving mainly major vessels, which results in stenosis and aneurysmal dilatation. Involvement of renal arteries might lead to renovascular hypertension. The disease is progressive and difficult to manage and is often treated and diagnosed with angiography and surgery.
REFERENCES
Di Luccio GM, Lopes de Souza SA, Lopes FP, et al. Self-kidney transplantation in Takayasu arteritis. Transplantation. 2012;94(7):e47–e49.
Kumar S, Mandalam KR, Rao VR, et al. Percutaneous transluminal angioplasty in non-specific aortoarteritis (Takayasu’s disease): Experience in 16 cases. Cardiovasc Intervent Radiol. 1990;12:321.
TANNER STAGES/CLASSIFICATION OF SEXUAL MATURITY
DESCRIPTION The Tanner scale defines physical measurements of the onset and development of pubertal changes based on external primary and secondary sex characteristics, such as breast and genitalia development and the growth of pubic hair. It is useful in the evaluation of delayed or precocious puberty.
• Pubic hair (boys and girls)
– Stage 1: Prepubertal (none or vellus hair similar to abdominal wall)
– Stage 2: Sparse growth of long, slightly pigmented hair, straight or curled, at base of penis or along labia
– Stage 3: Darker, coarser, and more curled hair, spreading sparsely over junction of pubes
– Stage 4: Hair is adult in type, but covering smaller area than in adult; no spread to medial surface of thighs
– Stage 5: Adult in type and quantity, with horizontal distribution (feminine type)
• External genitalia (boys)
– Stage 1: Prepubertal
– Stage 2: Enlargement of scrotum and testes; scrotum skin reddens and changes in texture
– Stage 3: Enlargement of penis (length at 1st); further growth of testes
– Stage 4: Increased size of penis, with growth in breadth and development of glans; testes and scrotum larger, scrotum skin darker
– Stage 5: Adult genitalia
• Breast development (girls)
– Stage 1: Prepubertal
– Stage 2: Breast bud stage with elevation of breast and papilla; enlargement of areola
– Stage 3: Further enlargement of breast and areola; no separation of their contour
– Stage 4: Areola and papilla form a secondary mound above level of breast
– Stage 5: Mature stage: Projection of papilla only, related to recession of areola
REFERENCES
Marshall WA, Tanner JM. Variations in pattern of pubertal changes in girls. Arch Dis Child. 1969;4423(5):291–303.
Marshall WA, Tanner JM. Variations in the pattern of pubertal changes in boys. Arch Dis Child. 1970;4523(9):13–23.
TERATOMA, SACROCOCCYGEAL, UROLOGIC CONSIDERATIONS
DESCRIPTION Sacrococcygeal tumors are usually diagnosed in the neonate (1 in 40,000 births) and less frequently in infants or adults. Females are more affected than males. Clinical presentation is usually in the form of palpable mass, skin discoloration, or hairy nevus. Related diseases include bilateral hydronephrosis, neurologic deficit, bladder or bowel dysfunction (obstruction or incontinence), high-output cardiac failure, or fet al hydrops. Sacrococcygeal tumors of the newborn are generally benign, whereas those discovered later have a 50% chance of being malignant. Wide, local excision of the tumor is primary management.
REFERENCE
Okada T, Sasaki F, Cho K, et al. Management and outcome in prenatally diagnosed sacrococcygeal teratomas. Pediatr Int. 2008;50(4):576–580.
TESTICULAR FEMINIZATION SYNDROME
DESCRIPTION Now commonly described as complete androgen insensitivity syndrome, this syndrome involves phenotypic women with a 46, XY karyotype and normal development of testes in utero, but with a genetic defect in the androgen receptor that leads to insensitivity to androgens. (See also Section II: “Androgen Insensitivity Syndrome [AIS or Androgen Resistance Syndrome], Complete and Partial.”)
REFERENCE
Conn J, Gillam L, Conway GS. Revealing the diagnosis of androgen insensitivity syndrome in adulthood. BMJ. 2005;331(7517):628–630.
TESTICULAR PROSTHESIS
DESCRIPTION Prosthetic device implanted in the scrotum to help cope with the psychological distress of losing a testicle from torsion, malignancy, trauma, or agenesis. Studies have shown that the improved cosmetic result increases patient self-esteem, body image, and sexual function. Implants were typically made of silicone rubber filled with silicone gel until 1995, when they were taken off the US market due to concerns about the association of silicone and connective tissue disease. Currently, in the United States, the testicular prosthetic of choice is comprised of a silicone rubber shell filled with saline to achieve desired size and consistency. Implantation is fairly simple and well tolerated. It is most commonly placed in a subdartos, subcapsular, or subtunical position. The most common major complication reported was extrusion of the implant, occurring in 2% and the most common minor complication was postoperative discomfort or pain, occurring in 9% of patients (Image 
).
REFERENCES
Bush NC, Bagrodia A. Initial results for combined orchiectomy and prosthesis exchange for unsalvageable testicular torsion in adolescents: Description of intravaginal prosthesis placement at orchiectomy. J Urol. 2012;188(4):1424–1428.
Turek PJ, Master VA, and the Testicular Prosthesis Study Group. Safety and effectiveness of a new saline filled testicular prosthesis. J Urol. 2004;172(4):1427–1430.
TESTIS BIOPSY, INDICATIONS
DESCRIPTION Testicular biopsy was once considered a cornerstone in the diagnosis of unexplained male infertility and azoospermia, but its current indications are now limited to a specific subgroup of patients. Current practice guidelines limit biopsy to those with obstructive azoospermia, those who require TESE (testicular sperm extraction), and to confirm the diagnosis of carcinoma in situ (CIS) of the testis. In men with obstructive azoospermia confirmation of the presence of normal spermatogenesis is usually helpful before surgical correction of the obstruction. TESE often requires multiple testicular biopsies for sperm harvesting with part of the specimen to be used for histologic exam. In special situations where ultrasonographic abnormalities indicate the potential presence of CIS, namely testicular microlithiasis, inhomogeneity, or a solid testicular mass, a testicular biopsy may prove to be helpful before orchiectomy.
REFERENCE
Dohle GR, Elzanaty S, van Casteren NJ. Testicular biopsy: clinical practice and interpretation. Asian J Androl. 2012:14:88–93.
TESTIS, CARCINOID
DESCRIPTION Carcinoid tumors of the testis are rare neoplasms that originate from neuroendocrine cells. Most carcinoid tumors are found in the GI tract, particularly in the ileum or appendix. Because of their neuroendocrine precursors, some tumors elicit endocrine activity; 88% secrete 5-hydroxyindoleacetic acid (5-HIAA), a metabolite of serotonin. Carcinoid syndrome results from the vasoactive substances secreted from the tumor and can cause symptoms such as flushing, sweating, wheezing, diarrhea, abdominal pain, and fibrosis of cardiac valves. Carcinoid tumors of the testis can be divided into 3 groups: Primary, metastasis from a primary site, or carcinoid tumor originating from a testicular teratoma. Most patients present with a painless scrotal mass; unlike germ cell tumor (GCT), there is no predilection for a particular age group. ∼16% of patients have carcinoid syndrome. Tumors localized to the testis have an excellent prognosis; however long-term follow-up is needed, due to risk of late metastases.
As with all testicular neoplasms, treatment begins with radical orchiectomy, with high ligation of the spermatic cord. Chemotherapy or radiation therapy has been reported, but these treatment modalities have poor efficacy. Octreotide analogs have been reported to stabilize disease progression, as well as help relieve symptoms of carcinoid syndrome. Once discovered, other extratesticular sources of carcinoid tumor should be determined. 5-HIAA can be measured in the urine and can be a useful adjunct. GI endoscopy, CT scan, MIBG scintigraphy, or somatostatin receptor scintigraphy can be used to search for GI sources of carcinoid tumor.
REFERENCE
Stroosma OB, Delaere KP. Carcinoid tumours of the testis. BJU Int. 2008;101(9):1101–1105.
TESTIS, CARCINOMA IN SITU (CIS)/INTRATUBULAR GERM CELL NEOPLASIA (ITGCN)
DESCRIPTION Testicular CIS is considered to be the precursor to all GCT, except for yolk sac tumors and spermatocytic seminoma. CIS may be present during infancy; however, lesions do not proliferate until adolescence, when hormonal influences come into play. Studies have shown that 50% of males with CIS developed invasive tumor growth within 5 yr; virtually all patients with CIS will progress to testicular cancer. Risk factors for testicular CIS are cryptorchidism, contralateral testis cancer, extragonadal GCT, infertility, and intersex patients with a Y chromosome. There is controversy concerning the screening, management, and treatment of testicular CIS. Diagnosis is made through testis biopsy. Treatment options involve surveillance, radiation, and orchiectomy. Cisplatin-based chemotherapy has been associated with incomplete treatment and recurrence. High-risk patients may be offered testicular biopsy and subsequent treatment, although some physicians would advocate surveillance alone. Surveillance is a viable treatment option due to the long, protracted nature of CIS, the morbidity of treatment options, and the fact that effective treatment of GCT exist. Radiation (14–20 Gy) may be performed in the patient with a testicular tumor and contralateral CIS, or in the patient with bilateral CIS. Orchiectomy is offered to the patient with unilateral CIS and a normal contralateral testis. Extensive counseling regarding the risks and benefits of surveillance vs. biopsy and subsequent treatment is needed. The option to cryopreserve sperm should be offered as well. (See also Section II: “Testis, Microlithiasis” and (Image ).)
REFERENCE
Hoei-Hansen CE, Rajpert-De Meyts E, Daugaard G, et al. Carcinoma in situ testis, the progenitor of testicular germ cell tumours: A clinical review. Ann Oncol. 2005;16(6):863–868.
TESTIS, CYSTIC LYMPHANGIOMAS
DESCRIPTION Testicular cystic lymphangiomas, benign tumors of unknown etiology, are often misdiagnosed as other conditions like hernia, hydrocele, hematocele, varicocele, or possible torsion in children. Lymphangiomas are characterized by an extensive overgrowth of lymphatic vessels; 50% are present at birth, 90% are evident by the age of 2 yr. Gradual painless scrotal swelling is the common symptom. On palpation, the mass may be soft and spongy or firm and tense, usually not separable from the scrotal skin. Skin showing bluish/purplish discoloration or erythematous changes is often present. The ultrasound exam usually shows a complex multilocular septated cystic mass without perfusion. Often, these lesions are more extensive than expected according the preoperative investigations, with deep perineal, inguinal, pelvic, or retroperitoneal involvement. Complete excision is mandatory to prevent recurrence.
REFERENCE
Liniger B, Fleischmann A, Zachariou Z. Benign cystic lesions in the testis of children. J Pediatr Urol. 2012;8(3):226–233.
TESTIS, CYSTS
DESCRIPTION Intratesticular cysts, once considered rare, are being reported with an increasing prevalence as a result of the wider use of scrotal ultrasound (8–9.8% incidence in men undergoing ultrasound). Nonneoplastic testicular cysts include hydatid, epidermoid, simple, and cystic dysplasia. Hydatid cysts are rarely seen, except in the Middle East. These are very rare lesions and are generally clinically interpreted as neoplastic, until proven otherwise at the time of postorchiectomy pathology. Treatment has included enucleation and even radical orchiectomy over concern for malignancy. A more conservative approach with serial ultrasound scanning has been advocated if a clear distinction can be made between neoplastic and nonneoplastic testicular cysts. Selected cases can potentially be managed by intraoperative frozen section to demonstrate no malignancy and enucleation of the tumor performed.
In children, testicular tumors are rare and the majority are benign, especially before puberty. Pediatric simple testicular cysts are very rare, as most cysts occur in men >40 yr. Most can be treated with testicle-sparing surgery. The differential diagnosis of testis cysts in the pediatric population:
• Infancy: Juvenile granulosa cell tumor, simple testicular cyst.
• Early childhood: Prepubertal teratoma, cystic lymphangioma, cystic dysplasia of the rete testis. Teratomas can present as solid, cystic, or mixed solid and cystic lesions and are always almost benign in children.
• In the older child/young adult epidermoid and dermoid cysts predominate (Image ).
REFERENCES
Kang SM, Hwang DS, Lee JW, et al. Multiple Intratesticular Cysts. World J Mens Health. 2013;31(1):79–82.
Liniger B, Fleischmann A, Zachariou Z. Benign cystic lesions in the testis of children. J Pediatr Urol. 2012;8(3):226–233.
Mahdavi-Zafarghandi R, Shakiba B, Ameli M. Testis-sparing surgery in testicular mass: Testicular epidermoid cysts. Can Urol Assoc J. 2014;8(1–2):E101–E103
TESTIS, DERMOID CYST
DESCRIPTION A primary non-germ cell tumor (GCT) representing 1% of testis tumors, with 1/2 of the patients presenting in their 20s. Grossly, the lesions present as encapsulated intratesticular nodules, which are round and sharply circumscribed, with firm consistency. The cut surface reveals a grayish white, cheesy, amorphous mass. The microscopic picture is that of dense fibrous tissue lined by keratinized stratified squamous epithelium with degeneration and macrocalcification. The benign behavior of these tumors is the rule. These are categorized separately from a mature testicular teratoma because of the malignant nature of the latter in postpubertal patients. Testicular ultrasound may aid in this tumor’s differentiation from GCT. Most cases have been managed by radical orchiectomy, although local excision has been equally successful in a small number of patients.
REFERENCE
Ulbright TM, Srigley JR. Dermoid cyst of the testis: A study of five postpubertal cases, including a pilomatrixoma-like variant, with evidence supporting its separate classification from mature testicular teratoma. Am J Surg Pathol. 2001;25(6):788–793.
TESTIS, HEMANGIOMA
DESCRIPTION Testicular hemangioma is a very rare neoplasm; only 25 cases are in the English literature. Intratesticular tumors of vascular origin are extremely rare. Intratesticular hemangioma can mimic malignant testicular tumors on presentation and imaging. Testicular hemangiomas histologically comprise 3 types: Cavernous, capillary, and epithelioid. Patients usually present with testicular enlargement with or without tenderness. Although it is impossible to differentiate a hemangioma from a seminoma preoperatively, intraoperative frozen study may be helpful in the differential diagnosis. Frozen section must be performed if the neoplasm has significant vascular proliferation identified by Doppler sonography. Since the lesion is benign, conservative surgical treatment by means of tumor enucleation with preservation of the testis is possible if intraoperative frozen section exam can be performed.
REFERENCE
Kryvenko ON, Epstein JI. Testicular hemangioma: A series of 8 cases. Am J Surg Pathol. 2013;37(6):860–866.
TESTIS, LEUKEMIA
DESCRIPTION Of children with acute lymphocytic leukemia (ALL), 5% will develop testicular disease at initial presentation or at 1st relapse. The testis is a common site of primary relapse (8%) after treatment of ALL. Children with T-cell lymphoblastic leukemia and/or a high initial leukemia cell burden are at higher risk of initial testicular involvement, as well as of testicular relapse. The condition commonly presents as either unilateral or bilateral testicular painless mass or swelling. Diagnosis is made with testicular biopsy.
Irradiation of both testes with 18–24 Gy plus systemic chemotherapy is the standard recommended treatment. Orchiectomy may be considered, depending on the extent of infiltration.
REFERENCE
Gutjahr P, Humpl T. Testicular lymphoblastic leukemia/lymphoma. World J Urol. 1995;13(4):230–232.
TESTIS, LYMPHOMA
DESCRIPTION Most common testicular malignancy in men >60, and the most common secondary neoplasms of the testis. Initial presentation is usually painless testicular swelling. Diagnosis is made through orchiectomy. Bilateral involvement occurs in ∼50% of cases (10% are synchronous). Treatment is multimodal; orchiectomy is performed, followed by systemic chemotherapy (doxorubicin), CNS prophylaxis, and local radiation. (See also Section II: “Lymphoma, Urologic Considerations" and (Image ).)
REFERENCE
Vitolo U, Ferreri AJ, Zucca E. Primary testicular lymphoma. Crit Rev Oncol Hematol. 2008;65(2):183–189.
TESTIS, METASTASIS TO
DESCRIPTION Due to the nature of the disease, this metastasis usually presents in men >50. Spread to the testis may be hematogenous, lymphatic, or by direct extension. Most common primary malignancies are prostate, lung, GI tract, melanoma, and kidney cancers (Image ).
REFERENCE
Rosevear HM, Mishail A, Sheynkin Y, et al. Unusual Scrotal pathology: An overview. Nat Rev Urol. 2009;6(9):491–500.
TESTIS, MICROLITHIASIS
DESCRIPTION Numerous and diffuse calcifications throughout the entire testicle seen on transscrotal ultrasound. Advances in ultrasound technology have led to an increased detection of testicular microlithiasis. It is reported in undescended testicles (0.3% incidence), prepubertal Klinefelter syndrome, and male pseudohermaphroditism, and is slightly more common in prepubertal males. Infertility and malignancy have been reported to be associated with the condition, and some consider it possibly premalignant. Both seminomas and nonsemino-matous GCT have been described in association with microlithiasis. Others suggest an association with carcinoma in situ of the testicle, but this is not settled. Recent studies have a prevalence of 2% in boys who undergo scrotal US, most commonly bilateral, with increased risk of malignancy. Most advocate close surveillance of patients with testicular microlithiasis, such as yearly testicular ultrasound, physical exam, and judicious tumor marker determinations (Image ).
REFERENCES
Cooper ML, Kaefer M, Fan R, et al. Testicular microlithiasis in children and associated testicular cancer. Radiology. 2014;270(3):857–863.
Heller HT, Oliff MC, Doubilet PM, et al. Testicular microlithiasis: Prevalence and association with primary testicular neoplasm. J Clin Ultrasound. 2014 Feb 28. [Epub ahead of print]
TESTIS, NORMAL SIZE
DESCRIPTION Testis size measurements can be made by ultrasound (US) (most accurate) or Prader orchidometer. At birth, testicular length is ∼1.5 cm with a volume of 1.1 cm3. In prepubertal boys, testicular length is usually <2 cm and volume <2 cm3. Normal testicular size after puberty in an adult is about 4.5–5.5 cm with a volume ranging from 15–30 cm3.
REFERENCES
Keefer JR. Endocrinology. In: Siberry GK, Iannone R, eds. Harriet Lane Handbook. 15th ed. St. Louis, MO: Mosby; 2000.
Krone KD, Carroll BA. Scrotal ultrasound. Radiol Clin North Am. 1985;23:121–139.
TESTIS, RETRACTILE
DESCRIPTION A testicle that can ride high in the scrotum or near the external inguinal ring; caused by a brisk cremasteric reflex. The testicle is able to be gently manipulated into the scrotum. Usually considered a variant of normally descended testes, 32% of retractile testes may ultimately become undescended (ascending or acquired undescended), and this is seen more frequently in boys <7 yo. Boys with retractile testes should be followed annually until the outcome of descent or nondescent is clear, which in many cases won’t be until puberty.
REFERENCE
Agarwal PK, Diaz M, Elder JS. Retractile testis: Is it really a normal variant? J Urol. 2006;175(4):1496–1499.
TESTIS, SEX CORD STROMAL TUMORS
DESCRIPTION These tumors arise from the supporting structures of the testis and not the germ cells. They usually present as a mass, are rarely hormonally active, and do not produce tumor markers such as hCG. Leydig cell tumors comprise about 3% of testicular neoplasms. They occur in both adults (80% of cases) and children. Sertoli cell tumors (<1% of testicular tumors) can be found in children and middle-aged adults, and ∼10% can be malignant. Granulosa cell tumor is usually found in older men and can present with gynecomastia. The testicular juvenile granulosa cell tumor is the most common neoplasm of the testis in the 1st 6 mo of life (yolk sac tumors peak after 6 mo). Other tumors sometimes placed in this category include malignant mesothelioma of the tunica vaginalis, paratesticular rhabdomyosarcoma, and adenocarcinoma of the rete testis.
REFERENCE
Young RH. Sex cord-stromal tumors of the ovary and testis: Their similarities and differences with consideration of selected problems. Mod Pathol. 2005;18:S81–S98.
TESTIS, TERATOMA, EXTRAGONADAL
DESCRIPTION Primary tumors of extragonadal origin are rare. In a decreasing order of frequency, the most common sites are the mediastinum, retroperitoneum, sacrococcygeal region, and pineal gland. Theories include a displacement of primitive germ cells that takes place during early embryonic migration from the yolk sac endoderm, and pluripotential cells that persist in sequestered primitive rests during early somatic development. Histologically, all types of GCT are represented, with pure seminoma accounting for 1/2 of mediastinal and retroperitoneal tumors. Clinically, males are affected more often than females, with the exception of sacrococcygeal tumors, where females predominate. The majority of adults present with advanced local disease and distant metastasis. Patients with mediastinal extragonadal tumors are usually diagnosed in their 20s, with or without symptoms of chest pain, cough, or dyspnea. Patients with primary retroperitoneal tumors may present with abdominal or back pain, a palpable mass, or vascular obstruction. Tumors of the pineal gland usually present in children and young adults, with symptoms of increased intracranial pressure, oculomotor dysfunction, hearing loss, hypopituitarism, or hypothalamic disturbances. Fine needle aspiration (FNA) can be used to assist in the diagnosis, but carries the risk of insufficient cellular return. When suspicion for teratoma exists, it is important to differentiate between mature and immature teratoma as immature carries the risk of malignant transformation.
TREATMENT
• Intensive chemotherapy regimens have shown some success in primary retroperitoneal seminoma.
• The nonseminomatous version has done poorly despite surgery, radiotherapy, and chemotherapy.
• Primary radiation therapy has been much favored for pineal tumors (a CSF shunt may be required).
REFERENCES
Garnick MB, Canellos GP, Richie JP, et al. Treatment and surgical staging of testicular and primary extragonadal germ cell cancer. JAMA. 1983; 250:1733–1741.
Gupta R, Mathur SR, Arora VK, et al. Cytologic features of extragonadal germ cell tumors. Cancer. 2008;114(6):504–511.
TESTIS, VASOCONGESTION FROM SEXUAL AROUSAL WITHOUT EJACULATION (“BLUE BALLS”)
DESCRIPTION “Blue balls” is a colloquialism describing scrotal pain after sustained sexual arousal unrelieved because of lack of orgasm and ejaculation. This term has only been referred to in the pediatric literature. It is likely due to vasocongestion of the external genitalia associated with the physiology of sexual arousal but no formal studies are available in the peer-reviewed literature. As noted by Chalett and Neremberg “The medical literature completely lacks acknowledgment of condition.” This is suspected to be more common among young male adults and should be considered in the differential diagnosis of acute testicular/scrotal pain in such patients.
REFERENCES
Chalett JM, Nerenberg LT. Blue balls: A diagnostic consideration in testiculoscrotal pain in young adults: A case report and discussion. Pediatrics. 2000;106:843–844.
Rockney R, Alario AJ. Blue Balls. Pediatrics. 2001;108(5):1233–1234.
TESTOSTERONE (FREE AND TOTAL) SERUM
DESCRIPTION Measurement of serum testosterone is most commonly used as the initial test for hypogonadism in males; it is less commonly used in the evaluation of virilism and hirsutism in females. Testosterone assays should be collected from 8–11 AM, due to higher morning plasma levels from the typical male circadian rhythm. In males, increased testosterone levels can be found in complete androgen resistance (testicular feminization syndromes). Decreased levels occur in hypogonadism, surgical orchiectomy, estrogen or LHRH analog or antagonist therapy, Klinefelter syndrome, hypopituitarism, and hepatic cirrhosis:
• Generally accepted normal ranges total testosterone (may vary by lab):
– Prepubertal children: 5–20 ng/dL (0.17–0.7 nmol/L)
– Men (≥17 yr): 300–1,000 ng/dL (10.4–34.7 nmol/L)
– Women: 20–70 ng/dL (0.7–2.6 nmol/L)
• A total testosterone level (free plus protein bound) of <200 ng/dL (or 6.9 nmol/L) (American Association of Clinical Endocrinologists) or <300 ng/dL (or 10 nmol/L) (FDA) is associated with hypogonadism and warrants further workup in an adult.
• Free testosterone (adult male range 8.8–27 pg/mL) is a useful diagnostic test as well, as elevated or decreased sex hormone binding globulin (SHBG) changes the bioavailability of testosterone. It can be used as an adjunct to the patient with low total testosterone levels. For example, obesity is characterized by reduced total testosterone and normal free testosterone due to reduced protein binding.
• Serum SHBG concentrations increase with age. With increasing age, less of the total testosterone is free or biologically active, as SHBG binds testosterone with high affinity.
• Serum T peaks early morning and then declines over the course of the day until the nadir in the evening (15% lower than morning values, but may vary up to 50% in younger men).
• Testosterone replacement monitoring: For men on transdermal T preparations measure at any time; for men on injectable T measure T midway between injections. Target T should generally be in the range of 400–700 ng/dL (13.9–27.7 nmol/L). If higher, the dose should be reduced.
• Methods used to measure T: Radioimmunoassay (RIA), Enzyme-linked Immunoassay (EIA) and Liquid Chromatography-Mass Spectroscopy (LC-MS) with the AUA and other organizations working to establish standardization of assay methods.
REFERENCES
Miner MM, Sadovsky R. Evolving issues in male hypogonadism: Evaluation, management, and related comorbidities. Cleve Clin J Med. 2007;74(Suppl 3):S38–S46.
Paduch D, et al. The Laboratory Diagnosis of Testosterone Deficiency: AUA White Paper available at http://www.auanet.org/common/pdf/education/clinical-guidance/Testosterone-Deficiency-WhitePaper.pdfAccessed March 27, 2014.
TESTOSTERONE REPLACEMENT FOLLOWING LOCALIZED PROSTATE CANCER THERAPY
DESCRIPTION Both prostate cancer and hypogonadism become more prevalent as men age; up to 20% of men who undergo radical prostatectomy (RP) are found to be hypogonadal and many men who receive combined hormonal ablation and radiation may not recover testosterone to pre treatment levels. Patients with hypogonadism may have decreased muscle mass, ED, osteopenia, decreased libido, depression, and possibly increase cardiovascular risk. Studies have shown that TRT does not increase the risk of prostate cancer development in men with both normal and increased risk. In addition, in limited series, postprostatectomy testosterone replacement does not correlate with biochemical recurrence or increases in PSA. TRT may help to preserve erectile function and improve quality of life, but the supplementation of testosterone in hypogonadal men after treatment for prostate cancer remains controversial. All testosterone replacements in the United States contain warnings concern the use with a history of prostate cancer. The 2012 European Association of Urology (EAU) Guidelines are as follows:
• Following RP and without evidence of disease with symptoms of testosterone deficiency man can be cautiously considered for TRT
• Approach is considered “off label”
• Restrict to low risk: Preop Gleason <8; pT1-2; PSA <10 ng/mL
• Wait 1 yr postop
• With radiation therapy for prostate cancer if low-risk and hypogonadism, use TRT cautiously with monitoring (See also Section I: “Testosterone Replacement Therapy, General Principles.”)
REFERENCES
Dohle GR, et al. Guidelines on Male Hypogonadism http://www.uroweb.org/gls/pdf/16_Male_Hypogonadism_LR%20II.pdf
Khera M, Lipshultz LI. The role of testosterone replacement therapy following radical prostatectomy. Urol Clin N Am. 2007;34(4):549–553.
TESTOSTERONE REPLACEMENT THERAPY, PROSTATE CANCER RISK
DESCRIPTION Multiple published review articles have shown that testosterone replacement therapy (TRT) does not cause prostate cancer or place patients at increased risk for the development of prostate cancer. A recent audit of the UK Androgen Study showed that initiating testosterone treatment had no statistically significant effect on the incidence of prostate cancer. These findings from this study show that, with careful monitoring, men with symptoms of testosterone deficiency can be treated safely with TRT without an increased risk of developing or progressing already present prostate cancer. (See also Section I: “Testosterone Replacement Therapy, General Principles.”)
REFERENCE
Feneley MR, Carruthers M. Is testosterone treatment good for the prostate? Study of safety during long- term treatment. J Sex Med. 2012;9:2138–2149.
TETHERED CORD
DESCRIPTION Tethered cord is an acquired or congenital occurrence that results from tissue attachments causing restriction of the normal movement of the spinal cord. It is most often seen in patients with spina bifida or meningomyelocele who have undergone surgical closure of the spine. This may result in scar tissue formation that adheres to the spinal cord. Patients may also present with a congenital tethered cord, mostly seen in patients with spina bifida occulta. As the child moves or grows, the tethered spinal cord is stretched, causing direct trauma to the cord, as well as compromising blood flow by stretching blood vessels. Tethering may also occur after spinal cord injury (SCI) when scar tissue prevents fluid flow around the cord. This causes pressure to build up, as well as cyst formation. The degree of tethering and strain placed on the spinal cord is correlated to severity and time of presentation of symptoms. The constellation of symptoms caused by a tethered cord is called tethered cord syndrome. (See also Section I: “Myelodysplasia [Spinal Dysraphism], Urologic Considerations” and Section II: “Tethered Cord Syndrome” and “Spina Bifida/Spina Bifida Occulta, Urologic Considerations.”)
REFERENCE
Agawalla PK, Dunn IF, Scott RM, et al. Tethered cord syndrome. Neurosurg Clin N Am. 2007;18(3):531–547.
TETHERED CORD SYNDROME
DESCRIPTION Late sequelae of spinal dysraphism, in which fixation or scarring of the spinal cord and conus medullaris, due to prior spinal surgery, prevents normal cephalad migration of spinal cord with childhood growth and causes spinal cord ischemia. Usually manifests with changes in voiding pattern, or with neurologic or musculoskelet al deficits. Urodynamic evaluation typically reveals detrusor hyperreflexia or detrusor areflexia. Detrusor-external sphincter dyssynergia or poor detrusor compliance with elevated bladder pressure can also be seen and warrant aggressive intervention. MRI is usually diagnostic. (See also Section I: “Myelodysplasia [Spinal Dysraphism], Urologic Considerations” and Section II: “Tethered Cord” and “Spina Bifida/Spina Bifida Occulta, Urologic Considerations.”)
TREATMENT
• Surgery to untether spinal cord
• Urologic intervention, based on urodynamic findings
REFERENCE
Palmer LS, Richards I, Kaplan WE. Subclinical changes in bladder function in children presenting with nonurologic symptoms of the tethered cord syndrome. J Urol. 1998;159(1):231–234.
THIERSCH-DUPLAY HYPOSPADIAS REPAIR
DESCRIPTION The distal urethral plate is tubularized to advance the meatus. The glans is reapproximated over the repair. Thiersch urethroplasty is the most commonly performed technique after surgical correction of penoscrotal transposition.
REFERENCE
Sunay M, Emir L, Karabulut A, et al. Our 21-year experience with the Thiersch-Duplay technique following surgical correction of penoscrotal transposition. Urol Int. 2009;82(1):28–31.
THOMPSON PYELOPLASTY
DESCRIPTION A surgical procedure initially introduced in 1969 that is used when insufficient renal pelvis is available to close lesions due to trauma or scarring. A triangular flap of renal capsule is sharply developed, then flipped over onto the renal pelvic opening and closed with 5-0 chromic sutures.
REFERENCES
Kay R. Procedures for ureteropelvic junction obstruction. In: Novick AC, Streem SB, Pontes JE, eds. Stewart’s Operative Urology. Baltimore, MD: Williams & Wilkins; 1989:220–233.
Thompson IM, Baker J, Robards VL Jr, et al. Clinical experience with renal capsule flap pyeloplasty. J Urology. 1969:101:487–490.
THORACIC KIDNEY
DESCRIPTION A type of renal ectopia in which the kidney is found in the posterior mediastinum, partially or completely above the level of the diaphragm. The diaphragm is thin, yet envelops the protruding kidney, keeping it separate from the pleural cavity. Aside from the ectopic location, the renal anatomy is essentially normal; the kidney is usually not malrotated. The ureter may be longer due to its higher position, however it inserts into the bladder orthotopically. Similarly, the renal vessels usually arise from their normal origins, although in some cases they may insert in a position more cranially. The vast majority of patients are asymptomatic. (See also Section I: “Renal Ectopia.”)
REFERENCE
Donat SM, Donat PE. Intrathoracic kidney: A case report with a review of the world literature. J Urol. 1988;140(1):131–133.
TINEA CRURIS (JOCK ITCH)
DESCRIPTION Dermatophytic infection of the crural areas of the genitalia. Caused by dermatophytes Trichophyton rubrum, Trichophyton mentagrophytes, and Epidermophyton floccosum. Clinically, reddish brown lesions with an elevated red border can be identified in the crural area, inner thigh, and scrotum. Penis involvement is rare. Postinflammatory hyperpigmentation may occur as a result of chronic or recurrent disease. Culture or KOH exam is necessary to confirm diagnosis. Scraping should be performed on the active border of the lesion and reveals branching septate hyphae. Differential diagnosis includes erythrasma, psoriasis, and seborrheic dermatitis. Recurrent disease is not unusual, and treatment should be aimed toward active disease rather than postinflammatory hyperpigmentation. (See also Section II: “Pruritus, External Genitalia, Male.”)
TREATMENT
• Prevent skin maceration by keeping skin dry.
• Apply antifungal agents on overt lesions. Agents include Lotrimin, Mycelex, Loprox, Spectazole, Lamisil, and others for up to 14 days.
• Rarely, oral agents may be needed if topical agents fail: Ketoconazole (Nizoral) for 14 days (requires baseline lab monitoring CBC, LFTs).
REFERENCE
Geer DL. An overview of common dermatophytes. J Am Acad Dermatol. 1994;31:S112.
TMPRSS2-ERG GENE FUSION, PROSTATE CANCER
DESCRIPTION Fusion of the transmembrane protease, serine 2 (TMPRESS2) and erythroblastosis virus E26 (ERG) genes is known as the TMPRSS2-ERG gene fusion. It is present in up to 80% of human prostate cancers. This gene fusion contributes to development of androgen-independence in prostate cancer through disruption of androgen receptor signaling. TMPRSS2:ERG, in combination with urine prostate cancer antigen 3 (PCA3), improved the performance of Prostate Cancer Prevention Trial risk calculator in predicting cancer on biopsy. The men were stratified into 3 groups based upon the levels of TMPRSS2:ERG and PCA3 in their urine: low, intermediate and high. Prostate cancer was diagnosed in each of the groups, respectively: 21%, 43%, and 69%. High-grade prostate cancer (>Gleason score), was diagnosed 7%, 20%, and 40% in each group, respectively. TMPRSS2:ERG and PCA3 urine analysis appears to enhance the utility of serum PSA for predicting prostate cancer risk and clinically relevant cancer on biopsy. (See also Section II: “PCA3 [Prostate Cancer Gene 3] Urine Assay.”)
REFERENCE
Tomlins SA, et al. Urine TMPRSS2:ERG Fusion Transcript Stratifies Prostate Cancer Risk in Men with Elevated Serum PSA Sci Transl Med 3 August 2011: Vol. 3, Issue 94, p. 94.
TOILETING PROGRAMS
DESCRIPTION Type of behavioral training to treat urinary incontinence. The patient is instructed to establish a routine voiding schedule regardless of the sensation to void. Initially, the patient is told to void at frequent intervals (eg, every 1 hr); the time between voids is then slowly increased, usually until he or she establishes an acceptable period (usually 2–4 hr) of continence.
REFERENCE
Wallace SA, Roe B, Williams K, et al. Bladder training for urinary incontinence in adults. Cochrane Database Syst Rev. 2004;(1):CD001308.
TRANSESOPHAGEAL ECHOCARDIOGRAM (TEE), UROLOGIC CONSIDERATIONS
DESCRIPTION A useful diagnostic tool in the management of renal tumors with tumor thrombus; the TEE is used to identify the extent of tumor thrombus involvement in the inferior vena cava. This may be used for preoperative surgical planning or may be used intraoperatively as well.
REFERENCE
Zini L, Haulon S, Decoene C, et al. Renal cell carcinoma associated with tumor thrombus in the inferior vena cava: Surgical strategies. Ann Vasc Surg. 2005;19(4):522–528.
TRANSURETEROURETEROSTOMY, TECHNIQUE AND INDICATIONS
DESCRIPTION Transureteroureterostomy (TUU) is an alternative surgical technique used to treat distal ureteral strictures that are not amenable to more conventional reconstruction techniques. It is useful in patients with a history of prior pelvic radiation or in those with an obstructing pelvic malignancy. There must be adequate length of diseased ureter proximal to the strictured segment to allow a tension free anastomosis. Relative contraindications include patients with upper tract urothelial carcinoma, nephrolithiasis, retroperitoneal fibrosis, chronic pyelonephritis, and bladder pathology—including reduced bladder capacity, thickened bladder wall, or invasion carcinoma. In addition, reflux to the recipient ureter needs to be identified preoperatively with a voiding cystourethrogram and treated, if present.
TUU is performed through a midline incision where the diseased ureter is mobilized and divided just proximal to the strictured segment. A tunnel is then created under the mesentery of the sigmoid colon and the recipient ureter is incised anteriomedially. The spatulated donor ureter is then anastomosed to the recipient in a tension free end-to-side fashion over an indwelling double J ureteral stent.
REFERENCES
Iwaszko MR, Krambeck AE, Chow GK, et al. Transureteroureterostomy Revisited: Long-term surgical outcomes. J Urol. 2010;183(3):1055–1059.
Nakada SY, Hsu THS. Management of upper urinary tract obstruction. In: Wein AJ, et al., eds. Campbell-Walsh Urology. 10th ed. Philadelphia, PA: Saunders; 2011.
TRANSSEXUALISM, UROLOGIC CONSIDERATIONS
DESCRIPTION Transsexualism, also known as gender identity disorder, is the strong desire to be and also identify with the opposite sex, which includes the desire to change the body hormonally and/or surgically to be similar to the aspired sex. This disorder causes severe impairment and distress. Treatment is multidisciplinary, and starts with an evaluation with an experienced mental health professional to discuss initiating hormonal and/or surgical procedures that are long-term and irreversible. Consultation with plastic surgeons, urologists, and gynecologists in high-volume centers is essential. Genital sex reassignment surgeries are available for male-to-female patients, and involve clitoroplasty, vulvuloplasty, and vaginoplasty. However, no consensus operative standard has been agreed upon regarding female-to-male reassignments, particularly regarding neophallus creation.
REFERENCE
Sohn M, Bosinski HA. Gender identity disorders: Diagnosis and surgical aspects. J Sex Med. 2007;4(5):1193–1207.
TRI-MIX
DESCRIPTION A custom-compounded formulation for intracorporal injection therapy of erectile dysfunction (ED). Typically consists of prostaglandin E1 (5.88 g/mL), papaverine (18 mg/mL), and phentolamine (0.6 mg/mL), but no dosage is considered standardized. It is usually administered to patients who cannot receive prostaglandin E1 injection alone due to lack of response.
REFERENCE
Seyam R, Mohamed K, Akhras AA, et al. A prospective randomized study to optimize the dosage of trimix ingredients and compare its efficacy and safety with prostaglandin E1. Int J Impot Res. 2005;17:246–353.
TRICHOMONIASIS
DESCRIPTION Sexually transmitted infection caused by the protozoan Trichomonas vaginalis. It is a rare cause of nongonococcal urethritis in men, common cause of vaginitis in women (from 4–35%). It is associated with a high prevalence of coinfection with other STDs. Signs and symptoms include urethral discharge, dysuria, and the presence of neutrophils in urethral secretions. Some women have symptoms characterized by a diffuse, malodorous, yellow-green vaginal discharge with vulvar irritation. However, many women have minimal or no symptoms. Because of the high prevalence of trichomoniasis in clinical and non-clinical settings, testing for T. vaginalis should be performed in women seeking care for vaginal discharge. T. vaginalis has, if any, only minor influence on male fertility. In women, it can cause premature rupture of the membranes and preterm delivery, as well as tubal infertility and cervical neoplasia. Females may have an elevated vaginal pH of >4.5. A positive diagnosis is made by identification of the protozoan on wet mount (must be examined in <10–20 min). Culture on Diamond’s medium may take up to 7 days; a more rapid commercial culture method is available (InPouchT. vaginalis culture system, with results in 3 days). FDA-cleared tests for trichomoniasis in women include OSOM Trichomonas Rapid Test (Genzyme Diagnostics, Cambridge, Massachusetts), an immunochromatographic capillary flow dipstick technology, and the Affirm VP III (Becton Dickenson, San Jose, California), a nucleic acid probe test that evaluates forT. vaginalis, G. vaginalis, and C. albicans. Each of these tests, which are performed on vaginal secretions, have a sensitivity of >83% and a specificity of >97%. Both tests are considered point-of-care diagnostics. Treatment is metronidazole 2 g PO or Tinidazole 2 g orally in a single dose for patient and sexual partners. It is not considered mandatory to identify the organism in partners before treating (CDC Expedited partner therapy). Metronidazole gel is considerably less efficacious for the treatment of trichomoniasis (<50%) than oral preparations of metronidazole. Topically applied antimicrobials (eg, metronidazole gel) are unlikely to achieve therapeutic levels in the urethra or perivaginal glands; therefore, use of this gel is not recommended. (See also Section I: “Sexually Transmitted Infections [STIs]; Sexually Transmitted Diseases [STDs], General”; Section II: “Vaginal Discharge, Urologic Considerations” and “Vaginosis” and (Image ).)
REFERENCE
Centers for Disease Control and Prevention. Sexually Transmitted Diseases Treatment Guidelines, 2010. MMWR Recomm Rep. 2010;59(No. RR-12):1–110.
TRICHOTEMNOMANIA, PUBIC
DESCRIPTION Trichotemnomania is the obsessive compulsive habit of cutting or shaving hair that often includes the pubic area. It is considered a willful self-induced type of isolated alopecia. It is a distinct disorder that should not be confused with trichotillomania (pulling hair). Behavioral counseling is indicated. (See also Section II: “Trichotillomania, Pubic.”)
REFERENCE
Happle R. Trichotemnomania: Obsessive-compulsive habit of cutting or shaving the hair. J Am Acad Dermatol. 2005;52(1):157–159.
TRICHOTILLOMANIA, PUBIC
DESCRIPTION Trichotillomania is defined by the Diagnostics and Statistic Manual of Mental Disorders, 4th edition (DSM-IV) as hair loss from a patient’s repetitive self-pulling of hair leading to a noticeable loss of hair. Any body area, including the pubic region, can be involved. The disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. Most individuals start pulling their hair during childhood or adolescence and may also consume the hair. Anxiety, depression and obsessive–compulsive disorders are frequently encountered in patients with trichotillomania. Treatment is a combination of education, behavior therapy and if necessary medication such as antidepressants. (See also Section II: “Trichotemnomania, Pubic.”)
REFERENCE
Sah DE, Koo J, Price VH. Trichotillomania. Dermatol Ther. 2008;21(1):13–21.
TRIGONITIS
DESCRIPTION Sometimes referred to as Pseudomembranous trigonitis, this is a common cause of microscopic hematuria in women. Nonkeratinized squamous epithelium is commonly seen on the trigone and bladder neck in up to 86% of women of reproductive age and in up to 75% after menopause. Considered a normal finding and distinct from squamous metaplasia, which is considered a premalignant lesion. Cystoscopically, these are pale white-gray areas with irregular borders. This condition is not seen in men, except for some reports in men receiving estrogens for prostate cancer. Treatment is not necessary. (See also Section II: “Squamous Metaplasia, Genitourinary.”)
REFERENCE
Stephenson TJ, Henry L, Harris SC, et al. Pseudomembranous trigonitis of the bladder: Hormonal aetiology. J Clin Pathol. 1989;42(9):922–926.
TRISOMY 4 P
DESCRIPTION This trisomy features hypertelorism and GU anomalies in the form of hydronephrosis, hypospadias, and undescended testes.
REFERENCE
Barakat AY, Seikaly MG, Der Kaloustian VM. Urogenital abnormalities in genetic disease. J Urol. 1986;136:778–785.
TRISOMY 8
DESCRIPTION A trisomy associated with a large, square head; a prominent forehead; widely spaced eyes; a slender body; and GU anomalies in the form of hydronephrosis, horseshoe kidney, reflux, hypospadias, and undescended testis. Males are more frequently affected than females. In the absence of serious problems, life expectancy is normal. Complete trisomy 8 is lethal and often results in miscarriage during the 1st trimester.
REFERENCE
Hale NE, Keane JF Jr. Piecing together a picture of trisomy 8 mosaicism syndrome. J Am Osteopath Assoc. 2010;110(1):21–23.
TRISOMY 9
DESCRIPTION This trisomy is characterized by a small cranium and GU anomalies in the form of renal hypoplasia and hypospadias.
REFERENCE
Barakat AY, Seikaly MG, Derkaloustian VM. Urogenital abnormalities in genetic disease. J Urol. 1986;136:778.
TRISOMY 9 P
DESCRIPTION This trisomy produces strabismus and GU anomalies in the form of a pancake kidney and undescended testis.
REFERENCE
Barakat AY, Seikaly MG, Der Kaloustian VM. Urogenital abnormalities in genetic disease. J Urol. 1986;136:778–785.
TRISOMY 10 Q
DESCRIPTION A trisomy characterized by oval, flat face and GU anomalies in the form of hydronephrosis and small penis.
REFERENCE
Mininberg D. The genetic basis of urologic disease. AUA Update Series. 1992;9:218.
TRISOMY 11 Q
DESCRIPTION A trisomy producing flat nose, wide glabella, cleft lip/palate, and micropenis.
REFERENCE
Mininberg D. The genetic basis of urologic disease. AUA Update Series. 1992;9:218.
TRISOMY 13
DESCRIPTION This trisomy is associated with polydactyly, congenital heart disease, and cystic kidney.
REFERENCE
Barakat AY, Seikaly MG, Der Kaloustian VM. Urogenital abnormalities in genetic disease. J Urol. 1986;136:778–785.
TRISOMY 18 (EDWARDS SYNDROME)
DESCRIPTION A trisomy producing hypertonia and GU anomalies in the form of hydronephrosis and small penis. Hypoplasia of the labia majora may cause a false impression of a large clitoris.
REFERENCE
Barakat AY, Seikaly MG, Der Kaloustian VM. Urogenital abnormalities in genetic disease. J Urol. 1986;136:778–785.
TRISOMY 20 P
DESCRIPTION A trisomy characterized by short nose, dental abnormalities, vertebral abnormalities, and polycystic kidney.
REFERENCE
Mininberg D. The genetic basis of urologic disease. AUA Update Series. 1992;9:218.
TRISOMY 21
DESCRIPTION See Section II: “Down Syndrome, Urologic Considerations.”
TRISOMY 22
DESCRIPTION A trisomy producing microcephaly, low-set ears, cleft palate, beaked nose, and microphallus.
REFERENCE
Mininberg D. The genetic basis of urologic disease. AUA Update Series. 1992;9:218.
TRISOMY SYNDROME
DESCRIPTION A congenital condition characterized by the presence of 3 instead of the normal pair of homologous or sex chromosomes; these disorders often result in a wide range of phenotypical expressions, including GU anomalies. Examples include trisomy 13 and 18, which produce cryptorchidism; trisomy 4p and 9p, which produce micropenis; and trisomy 7, which is associated with sporadic papillary RCC.
REFERENCE
Kliegman RM. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders; 2007.
TRUE HERMAPHRODITISM (OVO-TESTICULAR DISORDER OF SEXUAL DIFFERENTIATION [OVO-DSD])
DESCRIPTION True hermaphrodite or ovo-testicular disorder of sexual differentiation (OVO-DSD) is 1 of the rarest variety of all disorders of sexual differentiation. Up to 90% have 46, XX karyotype with infrequent 46, XY/46, XX mosaicism and 46, XY. The gonads are asymmetrical having both ovarian and testicular differentiation on either sides separately or combined as ovotestis. In ovotestis, testis is always central and ovary polar in location. Testosterone and müllerian inhibitory substance (MIS) are either normal or low. However, for final diagnosis there must be histologic documentation of both types of gonadal epithelium. On the basis of location of gonads and histology these patients are classified as:
• Lateral: Testis and contralateral ovary (30%).
• Bilateral: Testicular and ovarian tissue identified on both sides, usually as ovotestis (50%).
• Unilateral: Ovotestis on 1 side and testis or ovary on other side (20%).
The commonest presentation is an abnormal external genitalia ranging from normal male to normal female. In many of these cases such distinction may not be present and chordee, hypospadias and cryptorchidism may be noted. Proper gender assignment to a neonate born with DSD is a social emergency of the newborn period. A few cases of malignancies like dysgerminoma and gonadoblastoma have been reported. (See also Section I: “Disorders of Sexual Development [DSD].”)
REFERENCE
Iqbal MZ, Jam MR, Saleem M, et al. True Hermaphrodite: A Case Report. APSP J Case Rep. 2011;2(2):16.
TUBERCULOSIS, BLADDER AND URETHRA
DESCRIPTION The hematogenous dissemination of TB can affect the entire urinary system. Bladder TB is caused by a descending infection from renal TB. Urethral TB is rare and can present as periurethral fistulas and abscess. Symptoms include urinary frequency, urgency, and dysuria, with mucosal ulcerations and thickening, and diminished bladder capacity. (See also Section I: “Tuberculosis, Genitourinary.”)
DIAGNOSIS
• Urine culture of acid-fast bacilli: Typically 1st morning void, requires multiple sequential cultures.
• Urethral TB is typically diagnosed on biopsy.
• Imaging such as IVU and CT may be normal, but can aid in diagnosis.
TREATMENT
• Combination antituberculous treatment: Typical regimen: 6 mo of rifampicin, isoniazid, pyrazinamide, and ethambutol
• Urethral TB may require suprapubic cystostomy tube drainage and urethral dilation for stricture.
REFERENCE
Raghavaiah NV. Tuberculosis of the male urethra. J Urol. 1979;122(3):417–418.
TUBERCULOSIS, MALE EXTERNAL GENITALIA
DESCRIPTION Rare manifestation of TB that may present as a papulonecrotic ulcer, tubercular cavernositis, or a solid nodule, which may be clinically indistinguishable from malignant disease. Diagnosis is confirmed on biopsy. Treatment is systemic antituberculous medication. (See also Section I: “Tuberculosis, Genitourinary.”)
REFERENCE
Wu WC, Chen SC, Hsieh JT, et al. Penile tuberculosis associated with monoclonal gammopathy of undetermined significance. J Formos Med Assoc. 2006;105(9):753–755.
TUBERCULOSIS, PROSTATE AND EPIDIDYMIS
DESCRIPTION A recent case study of an HIV and tuberculosis (TB) population noted that 10% of genitourinary tuberculosis involves the testicle or prostate. Tuberculous prostatitis can present with an elevated PSA, irritative voiding symptoms, and a hard, nodular prostate. Tissue exam can distinguish it from carcinoma. Prostate involvement is thought to spread hematogenously. Another case report of post mortem tuberculosis patients showed that 10–14% of patients who died from TB had asymptomatic tuberculous prostatitis. Tuberculous epididymitis can be diagnosed with a urine culture positive for acid fast bacilli, a swollen, painful epididymis, and/or ultrasound findings showing edema predominantly in the tail of the epididymis with marked heterogeneity. The presence of pulmonary or renal disease provides indirect evidence for the diagnosis. Spread of TB to the epididymis is controversial but is thought to occur either hematogenously, through lymphatics, retrograde from the urethra, or by direct extension. Conjugal transmission has been reported as well. (See also Section I: “Tuberculosis, Genitourinary, General Considerations.”)
REFERENCE
Wise GJ, Shteynshlyuger A. An update on lower urinary tract tuberculosis. Current Urology Reports. 2008(9):305–313.
TUBEROUS SCLEROSIS
DESCRIPTION An autosomal dominant disease mapped to chromosome 16 or 9 that clinically presents with a classic triad of epilepsy, mental retardation, and adenoma sebaceum. Named for the tubers or periventricular calcifications seen on CT scan. Adenoma sebaceum describes the facial angiofibromas around nasolabial regions and cheeks. The renal system may be free of anomalies or may display cysts (10%), angiomyolipomas (AML) in (40–80%), or both. AML can bleed, with increasing risk at 3.5 cm. Renal failure is a result of parenchymal compression by expanding cysts. Hypertension may also occur. Renal cell carcinoma (RCC) is present in 2% of patients. (See also Section I: “Renal Angiomyolipoma.”)
SYNONYMS
• Tuberous sclerosis complex
• Bourneville disease
TREATMENT
• Absolute indications for intervention include suspicion of malignancy, hemorrhage with significant hypotension or symptoms, persistent hematuria.
• Symptomatic AML or lesion >4 cm: Selective arterial embolization or nephron-sparing surgery.
• Surveillance best for lesions <4 cm.
• Embolization is 1st-line treatment for acute hemorrhage from AML.
• mTOR inhibitors are under study.
REFERENCE
Wong IY, Shortliffe LD. The management of renal angiomyolipomas in a patient with tuberous sclerosis. Nat Clin Pract Urol. 2009;6(3):168–172.
TUMOR LYSIS SYNDROME (TLS)
DESCRIPTION A syndrome associated with chemotherapy, radiation, and other treatments in which massive tumor lysis occurs, with subsequent release of large amounts of potassium, phosphate, and nucleic acids that are converted to massive amounts of uric acid. More commonly associated with chemotherapy of lymphomas and leukemias, it can be seen with any tumor type. Extensive metastatic tumor, pretreatment renal impairment, and markedly elevated LDH concentrations have been reported to be serious risk factors in developing TLS. Lab investigations would reveal hyperkalemia, hypocalcemia, hyperphosphatemia, and concurrent metabolic acidosis, as well as severe hyperuricemia with eventual renal failure. If these patients are being treated with allopurinol, they are at risk of ARF and xanthine stone formation (See also Section II: “Nephropathy, Urate and Xanthine”; “Urolithiasis [Xanthinuria].”)
TREATMENT
• Prevention with adequate hydration (urine output of >80–100 mL/m2/h).
• Cautious use of rasburicase; some recommend allopurinol.
• Urinary alkalinization should not be done, as it may cause further precipitation of calcium phosphate in the kidney and other tissues.
REFERENCE
Coiffier B, Altman A, Pui CH, et al. Guidelines for the management of pediatric and adult tumor lysis syndrome: An evidence-based review. J Clin Oncol. 2008;26(16):2767–2778.
TUNICA VAGINALIS TUMORS
DESCRIPTION Malignant mesothelioma of the tunica vaginalis is rare (0.3–5% of all malignant mesotheliomas) and often fatal. However, increased frequency has been reported since 1980. Most of the patients are 40–79 yr of age, with prior exposure to asbestos being reported in some. Microscopically, mesothelioma may be epithelial, fibrous, or a combination of both. The malignant nature of the disease is indicated by its frequent mitosis, nuclear atypia with prominent nucleoli, and invasion of adjacent structures or lymphatics. Positive staining with keratin and failure to stain with CEA indicate mesothelioma. In addition, an immunoperoxidase stain has been reported to be specific for the tumor. CT and aspiration cytology may aid in preoperative diagnosis. The most important differential diagnostic considerations include mesothelial hyperplasia, adenomatoid tumor, carcinoma of the rete testis, and serous papillary tumors. The prognosis for this entity is grave, with a median survival of 23 mo and aggressive therapy with radical orchiectomy remains the mainstay of treatment. Adjunct chemotherapy may be tried. However, its value has not been established. (See also Section I:”Paratesticular Tumors” and “Scrotum and Testicle, Mass.”)
REFERENCE
Chekol SS, Sun CC. Malignant mesothelioma of the tunica vaginalis testis: Diagnostic studies and differential diagnosis. Arch Pathol Lab Med. 2012;136(1):113–117.
TURNER SYNDROME (XO SYNDROME)
DESCRIPTION A sex chromosome abnormality with a 46, XO karyotype. It is the most common sex chromosome abnormality with an incidence of 1:10,000 newborn females. Neonates may have lymphedema. Clinically, patients present with short stature, primary amenorrhea, webbed neck, shield-like chest, streak gonads, hypertension, and coarctation of the aorta. GU anomalies include horseshoe kidney, anomalous blood supply to the kidney, and infantile genitalia. The external genitalia are female but immature, and most women with Turner syndrome are infertile. Karyotype analysis should be performed to confirm the diagnosis. Any patient with Turner syndrome who presents with virilization should also be evaluated for Y-chromosome mosaicism, as these individuals are at increased risk of gonadoblastoma or germ cell tumor.
TREATMENT
• Echocardiography at diagnosis to detect cardiovascular anomalies (coarctation of the aorta or a bicuspid aortic valve are most common causes of morbidity and mortality)
• Renal US to determine if GU abnormalities are present
• Hormonal therapy in the form of growth hormone, estrogen, and medroxyprogesterone, aimed toward maximizing final height, induction of secondary sexual characteristics, and menarche
REFERENCE
Stochholm K, Juul S, Juel K, et al. Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab. 2006;91(10):3897–3902.
TURNER–WARWICK INLAY URETHROPLASTY
DESCRIPTION Through a midline scrotal incision, the urethral stricture is opened and scarred tissue is removed, leaving a strip of urethra in place. The edges of the scrotal incision are sutured to the urethral strip. In a 2nd stage, the mature marsupialized urethra is tubularized with the surrounding scrotal skin and closed.
REFERENCE
Devine CJ, Devine PC. Operations for urethral stricture. In: Novick AC, Streem SB, Pontes JE, eds. Stewart’s Operative Urology. Baltimore, MD: Williams & Wilkins; 1989:550–680.