Ramiro J. Madden-Fuentes, MD
Judd W. Moul, MD, FACS
BASICS
DESCRIPTION
• Small, discernible growth anywhere along the epididymis
• Frequently asymptomatic, discovered on routine genital exam or incidentally by the patient
• Pain may be presenting symptom
EPIDEMIOLOGY
Incidence
• Not well defined
• Epididymal cysts are usually asymptomatic and may occur in up to 30% of asymptomatic males
• Cysts more common with advancing age
• Adenomatoid tumors—benign and most common (1)
Prevalence
Not well defined
RISK FACTORS
• Aging
• Von Hippel–Lindau disease associated with cystadenoma
• DES exposure in utero: Epididymal cysts
• Prior vasectomy
Genetics
Epididymal cystadenoma associated with Von Hippel–Lindau syndrome (hereditary, autosomal dominant)
PATHOPHYSIOLOGY
• Most solid lesions benign (such as adenomatoid tumors)
• Malignant lesions uncommon
• Metastatic disease is rare but reported
ASSOCIATED CONDITIONS
• Von Hippel–Lindau disease
• Young syndrome
GENERAL PREVENTION
• Routine self-exam for identification of scrotal content masses
• Routine genital exam by physician
DIAGNOSIS
HISTORY
• Age: Cystic lesions increase with age
• Timing of identification
• Interval growth
• Associated pain
• Dysuria, hematuria, frequency, urgency, tenderness—consider epididymitis
• Exposure to tuberculosis (TB)
• History of sarcoidosis, histoplasmosis
• History of vasectomy
• History of urinary tract infection (UTI) or sexually transmitted infection
– History of anal insertive intercourse increases risk of coliform or STD infection
• Recent GU manipulation
– Bacillus Calmette–Guérin (BCG) instillation
– Catheterization
– Transurethral procedure
PHYSICAL EXAM
• Scrotal exam
– Identify location of mass—single or multiple
– Compare with contralateral scrotal contents
– Evaluate if fixed, mobile, indurated, or encroaching on other structures
– Identify spermatic cord/vas deferens
– Scars from vasectomy—sperm granuloma, epidermal inclusion cyst
– Examine testicle for associated masses
• Inguinal exam
– Evaluate for lymphadenopathy
– Hernia
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• Urinalysis: to evaluate for UTI. Include urine culture if suspicious for infection.
• Tumor markers if any concern for testicular mass
– α-Fetoprotein (AFP), β-human chorionic gonadotropin (β-HCG), lactate dehydrogenase (LDH)
• Purified protein derivative (PPD) if TB suspected
Imaging
• Scrotal ultrasound
– Solid vs. cystic
– Location—testicular or paratesticular
– Vascular or avascular
– Cannot reliably differentiate between malignant or benign
• Chest x-ray if TB suspected
• If rhabdomyosarcoma and >10 yr old—CT scan of the abdomen and pelvic with contrast to evaluate for retroperitoneal nodes (2)[A]
Diagnostic Procedures/Surgery
• Rarely needed for epididymal lesions
• Inguinal approach
• Frozen section for pathology; proceed to orchiectomy with high cord ligation if malignant
Pathologic Findings
• Benign
– Adenomatoid
– Epididymal cystadenoma/papillary cystadenoma
– Spermatocele
• Malignant
– Rhabdosarcoma
– Leiomyosarcoma
– Fibrosarcoma
– Metastatic carcinoma
DIFFERENTIAL DIAGNOSIS
• Adenomatoid tumor of the epididymis:
– Most common solid tumor of the epididymis
• Ectopic tissues:
– Adrenal cortical rests
– Splenogonadal fusion
• Epidermoid cyst
• Epididymal calcinosis
• Epididymal cystadenoma/papillary cystadenoma:
– 2/3 associated with von Hippel–Lindau syndrome
– 1/3 of all epididymal tumors
– 2/3 associated with VHL syndrome
– On US, most common appearance is 15–20-mm solid mass with small cystic components.
• Epididymitis:
– Acute; very tender on exam
– Chronic; may have secondary calcification
– Common cause of epididymal pain
• Fibroma of epididymis
• Fibrous pseudotumor
• Funiculitis
• Granulomas: Sarcoidosis, TB, histoplasmosis
• Hernia
• Hydrocele
• Hydrocele of the cord
• Leiomyoma
• Malignant epididymal tumor:
– Primary (very rare): Liposarcoma, rhabdomyosarcoma (high on differential in children), leiomyosarcoma, adenocarcinoma, lymphoma
– Metastatic: Prostate, kidney, stomach most common
• Papillary cystadenoma
• Polyorchidism
• Sarcoid
• Sperm granuloma:
– Seen in 40% postvasectomy or 2.5% idiopathic in general population
– Granulomatous lesion with few giant cells
– Consequence of extravasation of spermatozoa generally postvasectomy (of vasectomized men and of general population)
• Testicular tumor
• TB of the epididymis
• Varicocele
• Vasitis and vasitis nodosa (usually associated with epididymitis)
• Young syndrome (obstructive azoospermia, sinusitis, bronchiectasis)
TREATMENT
GENERAL MEASURES
• As most epididymal lesions are benign, observation for asymptomatic cystic lesions
• Epididymitis
– Consider sexually transmitted infection as source in young men and treat accordingly (See “Sexually Transmitted Infections [STIs] (Sexually Transmitted Diseases [STDs]), General)”
– Older men more likely to be infected by enteric organisms Escherichia coli, other coliforms, and Pseudomonas
MEDICATION
First Line
• Epididymitis (3)[A]
– <35 year old: Consider gonorrhea and chlamydia
Ceftriaxone IM 500 mg × 1 AND
Azithromycin 1 g PO × 1
– >35 year old: Enteric organisms
Levofloxacin 500 mg PO daily × 10 days
• TB: Treat according to current CDC guidelines (http://www.cdc.gov/tb/)
Second Line
• Epididymitis (3)[A]
– Doxycycline 100 mg PO BID × 10 days in lieu of azithromycin
SURGERY/OTHER PROCEDURES
• Excision of suspicious lesion via inguinal approach
• Frozen section
• If positive for malignancy, radical orchiectomy
• Further surgical therapy guided by pathology but may include retroperitoneal lymph node dissection if rhabdomyosarcoma
ADDITIONAL TREATMENT
Radiation Therapy
Use of radiation for local control of rhabdomyosarcoma in young patient is controversial
Additional Therapies
• Chemotherapy
– Vincristine, cyclophosphamide, dactinomycin may have a role in rhabdomyosarcoma depending on extent of disease and oncologist recommendations (4)[A]
Complementary & Alternative Therapies
N/A
ONGOING CARE
PROGNOSIS
• Adenomatoid tumors
– Benign, excellent prognosis
• Rhabdomyosarcoma
– In children with low stage disease survival may be as high as 90%. Worst stage (IV), survival is ∼5.2% (4)[A]
COMPLICATIONS
• Untreated epididymitis can cause severe systemic illness.
– More advanced infections can present with testicular swelling and pain (epididymo-orchitis).
• If radiation or chemotherapy needed:
– infertility, higher risk for secondary neoplasms including lymphoma, leukemia, soft tissue sarcomas
FOLLOW-UP
Patient Monitoring
• Oncologic follow-up if malignant disease
• Teach patient testicular self-exam
Patient Resources
National Cancer Institute. http://www.cancer.gov/cancertopics/pdq/treatment/childrhabdomyosarcoma/Patient
REFERENCES
1. Montgomery JS, Blood DA. The diagnosis and management of scrotal masses. Med Clin North Am. 2011;95(1):235–244.
2. Grimsby GM, Ritchey ML. Pediatric urologic oncology. Pediatr Clin North Am. 2012;59(4):947–959.
3. Workowski KA1, Berman S; Centers for Disease Control and Prevention. Sexually transmitted diseases treatment guidelines, 2010. MMWR. 2010;59(No. RR-12):1–110.
4. Oberlin O, Rey A, Sanchez de Toledo J, et al. Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: Long-term results from the international society of pediatric oncology MMT95 study. J Clin Oncol. 2012;30(20):2457–2465.
ADDITIONAL READING
Rubenstein RA, Dogra VS, Seftel AD, et al. Benign intrascrotal lesions. J Urol. 2004;171(5):1765–1772.
See Also (Topic, Algorithm, Media)
• Adenomatoid Tumors, Testicular and Paratesticular
• Epididymal Cystadenoma/Papillary Cystadenoma
• Epididymis, Mass (Epididymal Tumor and Cysts) Images 
• Epididymis, Metastasis to
• Epididymitis
• Hydrocele
• Paratesticular tumors
• Scrotum and Testicle, Mass
• Sexually Transmitted Infections (STIs) (Sexually Transmitted Diseases [STDs]), General
• Sperm Granuloma
• Spermatocele
• Von Hippel–Lindau Disease
CODES
ICD9
• 222.3 Benign neoplasm of epididymis
• 608.89 Other specified disorders of male genital organs
ICD10
• D29.30 Benign neoplasm of unspecified epididymis
• D29.31 Benign neoplasm of right epididymis
• N50.8 Other specified disorders of male genital organs
CLINICAL/SURGICAL PEARLS
• Most epididymal lesions are benign and should be followed serially.
• Treatment for epididymitis is guided by risk of STIs as a source.
• Ultrasound is important to delineate a testicular vs. paratesticular origin of the mass.
• Ultrasound cannot reliably differentiate malignant solid tumors from benign tumors.
• Rhabdomyosarcoma predominantly occurs in children.