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Sex cord stromal tumors

• Make up 0.4-4% testis tumors, 90% benign, can only prove malignant if metastatic disease present
• Consider testis-sparing surgery if mass < 3cm
• Leydig cell tumor: majority (75-80%) stromal tumors, present with mass/pain, gynecomastia, impotence, decreased libido, infertility, check hormone labs (T, LH, FSH, estrogen), manage with orchiectomy, if 2+ malignant features then manage with RPLND (usually chemo/XRT resistant), 40% require T supplementation post-orchiectomy
• Sertoli cell tumor: may secrete estrogen/tesosterone, may present with gynecomastia, manage similar to Leydig cell tumors
• Granulosa cell tumor: rare, 7% prepubertal testis tumors (75% diagnosed within 1mo birth), usually cured with orchiectomy
• Gonadoblastoma: occur almost always in patients with dysgenic gonads and DSD, 40% risk for bilateral tumors, bilateral orchiectomy recommended

Other testis tumors

• Dermoid/epidermoid cyst: onion peel appearance without flow on ultrasound, unlike teratoma will not have ITGCN in surrounding tissue
• Rete testis adenocarcinoma: rare, presents with mass + hydrocele, 50% have metastatic disease, median survival 1yr, RPLND may be curative but chemo/XRT are not useful
• Adrenal rests: remnant adrenal tissue seen with CAH, usually presents with bilateral testis masses, steroid therapy causes regression/stabilization
• Lymphoma: testis primary seen in 1-2% lymphomas, more commonly a site of spread, usually presents age 50-60+, 35% have bilateral involvement, 25% have systemic symptoms, 10% have CNS involvement, manage with orchiectomy, require further chemotherapy, usually poor prognosis due to systemic disease
• ALL: testicle is site for relapse after chemotherapy, can diagnose with biopsy, orchiectomy not needed, manage with XRT 20Gy and include contralateral testis, poor prognosis due to associated systemic disease

Adnexal tumors

• Adenomatoid tumor: most common paratesticular tumor, 75% involve epididymis, managed with inguinal resection
• Cystadenoma: associated with VHL syndrome, multicystic lesions, may be bilateral
• Mesothelioma: painless mass with hydrocele, manage with radical inguinal orchiectomy and hemiscrotectomy, consider node dissection, poor survival for malignant disease (median < 2yrs)
• Sarcoma: explore any solid non-testicular scrotal mass via inguinal approach and perform biopsy, perform wide excision including orchiectomy, usually recur locally, consider XRT for liposarcoma, RPLND for non-liposarcoma, chemotherapy for RP metastasis