John L. Phillips, MD, FACS
Vladimir A. Valera, MD, PhD
BASICS
DESCRIPTION
• Lesions can arise from the paratesticular regions of the scrotum and testes. These soft tissues include
– tunica albugenia, tunica vaginalis, spermatic cord structures and mullerian remnants (eg, appendix testis or appendix epididymis)
– Benign 70%
Lipomas (90% of adult PT tumors)
Adenomatoid tumors 30%
Tunica albugenia cysts
Leiomyomas
Cystadenomas
Adrenal rests
Cysts of the appendix testis
Hamartomas/pseudotumors (rare)
– Malignant 30%
Leiomyosarcoma
Rhabdomyosarcoma (RMS) 24–40%
Liposarcoma
Malignant mesothelioma (MM)
Desmoplastic round cell tumor (DRCT)
EPIDEMIOLOGY
Incidence
• Rare, true incidence unknown
• Europe: 5–7 cases per million
• Adenomatoid tumors typically seen in 4th decade
• Leiomyosarcoma and MM peak in 5th–7th decade
• RMS: Bimodal
– 2–6 yr & 15–19 yr
Most common tumor of lower GU tract in the 1st 2 yr
– 5–7 cases/10 million children
Prevalence
• Reported in 1 of 20–40 orchiectomy specimens
– China: 4.7% of pediatric scrotal tumors over 12 yr (1)
– High in SW Nigeria: 38.5% of adult orchiectomy specimens over 17 yr (2)
RISK FACTORS
• Cryptorchidism not a risk factor for PT tumors
• Asbestos exposure may be risk factor for MM
• Cystadenomas seen in Von Hippel–Lindau (VHL) disease are typically bilateral
• Parental cocaine or THC use seen in pediatric RMS
Genetics
• RMS, embryonal subtype, frequently has monosomy of chromosome 11.
– Alveolar subtypes express t(2;13)(q35;q14) or t(1;13)(p36;q14) and bodes poorly
• DRCT express t(11;22)(p13;p12) fusions of EWS and WT1.
PATHOPHYSIOLOGY
• Arise from epithelial, mesothelial, or mesenchymal tissues
– Epididymis
Adenomatoid tumor
Cystadenoma (1/3 occur in VHL)
– Spermatic cord
Sarcoma (arise from undifferentiated mesoderm)
Adrenal rest (found incidentally during hernia repair)
Lipoma/leiomyoma/liposarc, etc.
– Tunica vaginalis (TV)
MM
ASSOCIATED CONDITIONS
• Germline conditions
– Von Hippel–Lindau disease
Infertility low even in bilateral cases
– Li–Fraumeni syndrome confers predisposition
• Somatic (ie, acquired) conditions
– Ipsilateral hydrocele (eg, reactive)
– Ipsilateral hernia (ie, cause for exploration)
– Pulmonary mesothelioma (and asbestos exposure in MM of the TV)
– Scrotal trauma
– Epididymitis–orchitis
– Infertility (ie, cause for workup or exploration)
GENERAL PREVENTION
No known environmental or occupational risk
DIAGNOSIS
HISTORY
• Slowly growing inguinal or scrotal mass
• Often painless
• Found in workup for hernia
• Found in evaluation of scrotal trauma or inflammatory/infectious scrotal conditions
PHYSICAL EXAM
• Inguinal or scrotal mass
• Testis often discrete and normal
– Adenomatoid tumors may occur more inferiorly on the testis.
– Superior pole tumors may mimic a spermatocele (SC)
• Evaluate for hernia, hydrocele, SC, and varicocele
ALERT
Fixation to inguinal canal or testis suggests malignancy. Prepare for and rule out sarcoma.
• Fixation or involvement of testis and not the chord suggests primary testicular or MM
– Rule out germ cell tumor or MM
• Large size >5 cm suggests RMS
ALERT
• Transillumination does not rule out tumor
• Rule out secondary or, more rare, primary lymphoma.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• Rule out concurrent infection or inflammation
– Urinalysis
– Urine culture
• Rule out concurrent mycobacterial infection (can mimic tumor)
– PPD history
– Chest x-ray (rule out granulomas, tumor)
• Assess testicular germ cell tumor (GCT) markers
– AFP
– β-hCG
– LDH
– PLAP
Imaging
• Scrotal ultrasound critical in evaluation
– Differentiate testicular from PT process
3D or elastosonography not necessary if tumor distinctly separate from testis
– Solid masses require exploration
– Cystic structures such as tunica albuginea cyst may be monitored serially
• CT scan of the chest, abdomen, and pelvis required when malignancy suspected and to rule out metastasis in cases of known malignancy
Diagnostic Procedures/Surgery
• Fine needle aspiration may lead to false negatives or positives
• Solid lesions require inguinal approach
• Benign lesions
– Observation
– Testis-sparing surgery
• Malignant or suspicious lesions
– Radical orchiectomy, inguinal approach
High dissection to internal ring
Early vascular control
Resection of adherent structures
Skin
Fascia
Muscle
Pathologic Findings
• Benign lesions usually need low power H&E
– Adenomatoid tumor
Well circumscribed
May involve tunica albuginea
Benign appearing cords and cystic tubules lined by eosinophilic cells with small nuclei
– Papillary cystadenoma
Well circumscribed
Brown fronds within cystic space
May have clear cells that resemble renal cell carcinoma (commonly associated with VHL)
• Malignant lesions may need electron microscopy
– MM
Friable, multicystic within hydrocele
Epithelioid, papillary, tubulopapillar pattern
Fibrovascular core
– Rhabdomyosarcoma (RMS)
Embryonal in 90%
Alveolar
Mixed pleiomorphic
– Desmoplastic round cell tumor (DRCT)
Firm, white, often near epididymis
Small blue cells in nests and cords
Mitotically active
– Leiomyosarcoma
Intersecting bundles of smooth muscle cells
Atypical and mitotically active
– Liposarcoma
Large, atypical cells, large nuclei
Fibrous septae
Lipoblasts present
DIFFERENTIAL DIAGNOSIS
• Epididymal tumors (rare)
– Adenocarcinoma
– Cystadenoma
• Proliferative folliculitis
– Resemble nodular fasciitis
– Incidental at herniorrhaphy
• Hernia, direct or indirect
• Hydrocele
• Lymphadenopathy
• Metastatic tumor
• Squamous cell carcinoma (scrotal skin)
• Sperm granuloma, usually post-vasectomy
• Testis tumor
• Tunica albuginea cyst
• Vasitis nodosa
TREATMENT
GENERAL MEASURES
• Cystic PT structures are treated with observation or rarely conservative excision
• Solid masses usually require excision
– Benign: use testicle sparing surgery (TSS)
– Malignant
TNM staging
Local excision
Adjuvant chemotherapy for RMS
Adjuvant local radiation for other sarcomas
MEDICATION
First Line
• Malignant rhabsomyosarcoma (RMS)
– Vincristine
– Dactinomycin
– Cyclophosphamide
MESNA for bladder protection
Second Line
N/A
ADDITIONAL TREATMENT
Radiation Therapy
Reserved for local adjuvant or salvage control of malignant PT sarcomas
Additional Therapies
• Secondary resection
– Local recurrence of benign lesions
– Local recurrence of malignant lesions if localized, no evidence of metastatic disease, and radiotherapy not given
Complementary & Alternative Therapies
N/A
ONGOING CARE
PROGNOSIS
• Benign lesions
– Resection is usually curative
– Fertility can be maintained
• Malignant lesions
– RMS
Favorable prognosis in children
Stage I in 60–80%
Unfavorable prognosis in adults and children >10 yr
– DCRT unfavorable prognosis with nodal and pulmonary metastasis
– Mesothelioma. Aggressive; similar to peritoneal mesothelioma
– Lipo-/leiomyosarcoma
Excellent prognosis but may recur locally and require retreatment
Metastases exceedingly rare, reportable
COMPLICATIONS
• General as seen for inguinal surgery:
– Secondary hernia
– Infection
– Hematoma
– Pain
• Loss of testis after testicular sparing surgery (TSS) (rare)
• Infertility (eg, after excision of bilateral cystadenoma or epididymal masses in VHL)
FOLLOW-UP
Patient Monitoring
• Benign lesions
– Physical exam semiannual and self-exam yearly
• Malignant lesions
– Liposarcomas rarely metastasize but can recur locally
– RMS
Interdisciplinary oncologic team (3)
Serial imaging
40% metastasize to retroperitoneum
Role of RPLND in RMS controversial
Patient Resources
• National Cancer Institute
– Childhood Rhabdomyosarcoma Treatment
www.cancer.gov/cancertopics/pdq/treatment/childrhabdomyosarcoma/patient
• Testicular Self-Exam
– www.nlm.nih.gov/medlineplus/ency/article/003909.htm
• Liddy Schriver Sarcoma Initiative
– sarcomahelp.org (Accessed August 22, 2014)
REFERENCES
1. Park SB, Lee WC, Kim JK, et al. Imaging features of benign solid testicular and paratesticular lesions. Eur Radiol. 2011;21:2226–2234.
2. Salako AA, Onakpoya UU, Osasan SA, et al. Testicular and para-testicular tumors in south western Nigeria. Afr Health Sci. 2010;10:14–17.
3. Wiener ES, Anderson JR, Ojimba JI, et al. Controversies in the management of paratesticular rhabdomyosarcoma: Is staging retroperitoneal lymph node dissection necessary for adolescents with resected paratesticular rhabdomyosarcoma? Semin Pediatr Surg. 2001;10:146–152.
ADDITIONAL READING
Vagnoni V, Brunocilla E, Schiavina R, et al. Inguinal canal tumors of adulthood. Anticancer Res. 2013;33(6):2361–2368.
See Also (Topic, Algorithm, Media)
• Epididymis, Mass (Epididymal Tumor and Cysts)
• Paratesticular Tumors
• Rhabdomyosarcoma, Pediatric (Sarcoma Botryoides)
• Scrotum and Testicle, Mass
• Spermatic Cord Mass and Tumors
• Spermatocele
CODES
ICD9
• 214.8 Lipoma of other specified sites
• 239.5 Neoplasm of unspecified nature of other genitourinary organs
• 608.89 Other specified disorders of male genital organs
ICD10
• D49.5 Neoplasm of unspecified behavior of other genitourinary organs
• N44.1 Cyst of tunica albuginea testis
• N50.8 Other specified disorders of male genital organs
CLINICAL/SURGICAL PEARLS
• Most common adult benign paratesticular tumor: Lipoma.
• Most common adult malignant paratesticular tumor: Liposarcoma.
• Most common pediatric PT tumor is malignant RMS (rhabdomyosarcoma).
• Cure rate of RMS with multimodality therapy: 90%.