Mohamed T. Ismail, MD
Sallyanne M. Fisher, MSN, FNP-C, CUNP
BASICS
DESCRIPTION
• Intrascrotal tumors involving the testicular tunic, epididymis, or cord structures. Can be benign (∼70%) or malignant (∼30%)
• The paratesticular region includes the contents of the spermatic cord, testicular tunics, epididymis, and vestigial remnants (appendices testis and epididymis)
• 90% of extratesticular tumors are found within the spermatic cord:
– Of these, 30% are malignant
– The majority represent benign lipomas
– Mesenchymal tumors of the spermatic cord include rhabdomyosarcoma, leiomyosarcoma, liposarcoma, lipoma, fibrosarcoma, and myxochondrosarcoma
• The most common paratesticular tumor in children is rhabdomyosarcoma, which accounts for ∼24–40% of all paratesticular tumors
• Adenomatoid tumor accounts for 30% of epididymis tumors and are benign:
– Typically seen in 3rd and 4th decades of life
– Rarely arise in testicular tunicae or spermatic cord (1)[C]
• Leiomyosarcoma is the most common type of paratesticular sarcoma in adults:
– Incidence peaks in the 6th and 7th decades
– Can be bilateral
– May accompany a hydrocele or hernia
• Cystadenoma is a benign tumor that involves the epididymis in young adults:
– Two-thirds associated with von Hippel–Lindau syndrome (2)[C]
– Frequently bilateral
• Malignant mesothelioma presents in older patients (55–75 yr) and usually presents in association with a hydrocele
• Malignant lymphoma: Cord structures are frequently invaded by testicular lymphoma, but primary lymphomas do occur rarely
• Epididymal cysts occur in up to 40% of men
– 75% of these are true cysts and contain lymphatic fluid (1)[C]
EPIDEMIOLOGY
Incidence
• The exact incidence of paratesticular soft tissue neoplasms is difficult to estimate
• Rhabdomyosarcoma
– Occurs primarily in children and adolescents during the 1st 2 decades of life
• Racial differential: White > Black (3:1)
• Leiomyosarcoma: Exceedingly rare, ∼110 reported cases in the literature
Prevalence
• Primary malignancies of the epididymis or paratesticular structures in adults extremely rare
• Rhabdomyosarcoma accounts for a large proportion of the paratesticular tumors in the pediatric population.
RISK FACTORS
• Marijuana and cocaine use in the parents is associated with rhabdomyosarcoma.
• Von Hippel–Lindau syndrome is associated with epididymal cystadenomas.
• Equestrians are prone to scrotal injury with up to 77% evidence of scrotal pathology (1)[C].
Genetics
• Partial monosomy of chromosome 11 often leads to embryonal rhabdomyosarcoma.
• Alveolar rhabdomyosarcoma is characterized by translocations t(2;13)(q35;q14) or t(1;13)(p36;q14); this subtype carries a poor prognosis.
PATHOPHYSIOLOGY
• Electron microscopy is very helpful in differentiating the type of sarcoma.
• Subtypes of sarcoma include rhabdomyosarcoma, leiomyosarcoma, liposarcoma, fibrosarcoma, malignant fibrous histiocytoma, and desmoplastic round cell tumor.
• Soft tissue sarcomas tend to infiltrate local tissues widely and have a tendency for local recurrence.
• Rhabdomyosarcoma:
– 97% belong to the favorable histology group of embryonal cell tumors.
ASSOCIATED CONDITIONS
Renal cell carcinoma with von Hippel–Lindau
GENERAL PREVENTION
Testicular self-exam should be performed monthly.
DIAGNOSIS
HISTORY
• Patient complains of mass within his scrotum, distinct from the testicle
– Typically painless
– Delays in presentation due to embarrassment
• Obtain complete history to include accompanying symptoms, duration, and constitutional changes (2)[C]
PHYSICAL EXAM
• Palpation of the testes, epididymis, and cord structures bilaterally including the inguinal region:
– Rhabdomyosarcoma reveals a firm mass that is usually distinct from the testis.
– Adenomatoid tumor appears clinically as small solid lumps and is most commonly found at the head of the epididymis, testicular tunics, or spermatic cord.
– Cystadenoma presents as asymptomatic cystic lumps and are bilateral in up to 1/3 of cases.
– Leiomyosarcoma normally presents as a discrete nodular mass, frequently near the spermatic cord and entirely separate from the testicle.
– Liposarcoma usually presents in an older patient as a large fatty-appearing mass.
– Lymphoma presents as a hard, nontender mass, separate from the testis; seen in young adults. Transillumination suggests a fluid-filled lesion such as a hydrocele.
• Careful exam of the groin is necessary to rule out hernia and to evaluate for lymphadenopathy.
• Masses are occasionally accompanied by hydrocele.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• Urinalysis (midstream) and culture if epididymitis is suspected (1)[C].
• Tumor markers to include β-human chorionic gonadotropin (β-hCG), α-fetoprotein (AFP), or lactic dehydrogenase (LDH) should be sent if the origin of the tumor is in question.
Imaging
• Gold standard: Scrotal ultrasound (US) (1)[C]
– To evaluate location and characteristics of the lesion within the scrotum
– Testicular vs. paratesticular
– Solid vs. cystic (2)[C]
Solid lesions almost always require exploration
Simple cystic lesions are mostly benign
• Computed tomography (CT) of the abdomen and pelvis with and without contrast for staging
– Paratesticular tumors may spread to retroperitoneal lymph nodes or hematogenously depending on the histology of the primary tumor
• Chest radiograph
• Chest CT
– If abdominal or pelvic metastases are seen
• Clinical staging of retroperitoneal lymph nodes
• Radioisotope bone scan:
– Especially for elevated alkaline phosphatase or symptoms with rhabdomyosarcoma
Diagnostic Procedures/Surgery
Surgery is often diagnostic and therapeutic
ALERT
Percutaneous biopsies are contraindicated due to the documented risk of seeding in the scrotal wall with malignancy.
• Bone marrow aspirate:
– Routine part of staging at a time of diagnosis for rhabdomyosarcoma
Pathologic Findings
• Electron microscopy can help differentiate between the different types of sarcoma; these differences can be quite subtle.
• Leiomyosarcoma spreads 1st by lymphatics, then hematogenously, and last by local extension.
DIFFERENTIAL DIAGNOSIS
• Adenomatoid tumors
– Most common benign paratesticular tumor
• Angiomyofibroblastoma
• Cystadenoma of the epididymis
• Epididymal cyst
• Epididymitis
• Fibrous pseudotumor of testicular tunic
• Fibrosarcoma
• Leiomyosarcoma
• Lipoma of the spermatic cord
• Liposarcoma
• Malignant fibrous histiocytoma
• Mesothelioma, benign, testicular tunic
• Mesothelioma, malignant, tunica vaginalis
– Associated with asbestos exposure
• Postoperative changes
– Sperm granuloma after vasectomy
• Spermatocele
• Testicular torsion
• Traumatic injury
• Tunica albuginea lesions
– Cysts, fibrous pseudotumor
• Varicocele
• Hydrocele
• Hydrocele of the spermatic cord
• Inguinal hernia
TREATMENT
GENERAL MEASURES
• US suggests initial management.
• Lesions suggestive of a benign process can be observed with serial exams.
• Remove malignant or potentially malignant structures while minimizing effects on fertility, function, esthetics (2)[C].
• Benign lesions only require intervention if they become massive or cause pain (1)[C].
• Any concern about malignancy and the scrotum should be explored through a high inguinal incision.
• Transscrotal manipulation or biopsy is contraindicated.
• Rhabdomyosarcoma always requires primary surgical excision via inguinal orchiectomy.
• Leiomyosarcoma should also be treated with radical orchiectomy to be followed with adjuvant radiation therapy to reduce local recurrence.
– No survival benefit has been demonstrated from the addition of radical pelvic lymph node dissection (RPLND) to radical orchiectomy.
MEDICATION
First Line
• Chemotherapy for malignant rhabdomyosarcoma
– Vincristine, cyclophosphamide, and dactinomycin, and actinomycin D-based chemotherapy in patients with gross or microscopic residual disease
Second Line
N/A
SURGERY/OTHER PROCEDURES
• Testicular or paratesticular lesions suspected to be malignant should be removed by radical inguinal orchiectomy with high ligation of the spermatic cord.
– Early clamping of the cord limits hematogenous spread in leiomyosarcoma.
• Rhabdomyosarcoma:
– Consider hemiscrotectomy for any degree of scrotal wall involvement.
– The Intergroup rhabdomyosarcoma study group (IRS) recommended radical inguinal orchiectomy and routine RPLND in all males >10 yr and in boys <10 with metastasis noted on imaging.
• Complete surgical excision with a negative margin has significant impact on local recurrence and overall survival in soft tissue sarcomas.
ADDITIONAL TREATMENT
Radiation Therapy
• Rhabdomyosarcoma:
– 4,000–6,000 centigray units of radiation (cGy) over 5 wk
– Dose and port size determined by the tumor’s primary site, patient age, and tumor burden
Additional Therapies
N/A
Complementary & Alternative Therapies
N/A
ONGOING CARE
PROGNOSIS
• Benign lesions—recurrence is rare
• Rhabdomyosarcoma—75% 5-yr survival when multimodal therapy is administered
• Leiomyosarcoma—50–80% survival with microscopic residual disease in 27% of cases
– Adjuvant treatment via radiation is warranted.
• Malignant mesothelioma—high recurrence and mortality
• Larger tumor size, metastasis, higher-grade tumor, and incomplete primary resection lead to poorer overall prognosis.
COMPLICATIONS
• Disease associated death in ∼10% of malignant cases
• Treatment-associated:
– Retrograde ejaculation and intestinal obstruction if RPLND is performed
– Hypogonadism and/or infertility secondary to chemotherapy
– Hemorrhagic cystitis secondary to chemotherapy
– Growth abnormalities secondary to radiation therapy (spinal and renal) in children
FOLLOW-UP
Patient Monitoring
• Serial US for equivocal lesions, especially in the epididymis
• Benign lesions need patient-performed monthly testicular self-exams
• Rhabdomyosarcoma monitoring is provider dependent
– Should be followed closely by a urologist
Patient Resources
N/A
REFERENCES
1. Montgomery JS, Bloom DA. The diagnosis and management of scrotal masses. Med Clin North Am. 2011;95(1):235–244.
2. Rosevear HM, Mishail A, Sheynkin Y, et al. Unusual scrotal pathology: An overview. Nat Rev Urol. 2009;6:491–450.
ADDITIONAL READING
• Ahmed HU, Arya M, Muneer A, et al. Testicular and paratesticular tumours in the prepubertal population. Lancet Oncol. 2010;11(5):476–483.
• Roman Birmingham PI, Navarro Sebastian FJ, Garcia Gonzalez J, et al. Paratesticular tumors. Description of our case series through a period of 25 years. Arch Esp Urol. 2012;65(6):609–615.
See Also (Topic, Algorithm, Media)
• Adenomatoid Tumors (Testis/Tunic/Epididymis)
• Epididymis, Mass (Epididymal Tumor and Cysts)
• Epididymis, Cystadenoma
• Fibrous Pseudotumor of Testicular Tunic
• Hydrocele of the Spermatic Cord
• IRS (Intergroup Rhabdomyosarcoma Study) Clinical Classification
• Mesothelioma, Benign, Testicular Tunic
• Mesothelioma, Malignant, Testicular Tunic
• Paratesticular Tumors Image 
• Rhabdomyosarcoma, Pediatric
• Scrotum and Testicle, Mass
• Spermatic Cord Mass and Tumors
CODES
ICD9
• 187.8 Malignant neoplasm of other specified sites of male genital organs
• 222.8 Benign neoplasm of other specified sites of male genital organs
• 239.5 Neoplasm of unspecified nature of other genitourinary organs
ICD10
• C63.7 Malignant neoplasm of other specified male genital organs
• D29.8 Benign neoplasm of other specified male genital organs
• D49.5 Neoplasm of unspecified behavior of other genitourinary organs
CLINICAL/SURGICAL PEARLS
• It is impossible to distinguish a benign from a malignant tumor based on physical exam.
• Can be indistinguishable from testicular masses.
• Percutaneous biopsies contraindicated due to documented seeding in scrotal wall with malignancy.