NON-GERM CELL TUMORS Leydig Cell Tumors Sertoli Cell Tumors Gonadoblastomas

NON-GERM CELL TUMORS Leydig Cell Tumors Sertoli Cell Tumors Gonadoblastomas

LEYDIG CELL TUMORS Epidemiology and Pathology – Most common non- germ cell tumor (1–3% of all testicular tumors) – Bimodal age distribution (5-9; )  25% occurs in childhood – No association w/ cryptorchidism unlike germ cell tumor

Gross Pathology Pathologic examination reveals a small, yellow, well- circumscribed lesion devoid of hemorrhage or necrosis.

Histopathology hexagonal; granular, eosinophilic cytoplasm w/ lipid vacuoles; Reinke crystals (fusiform cytoplasmic inclusions; pathognomonic)

Clinical Findings Virilization (prepubertal)  tumors are benign Asymptomatic (adults); 20-25% w/ gynecomastia 10% tumors in adults are malignant Increased serum and urinary 17-ketosteroids and estrogens

Treatment and Prognosis Radical orchiectomy - initial treatment Clinical staging is similar to that for germ cell tumors levels of the 17-ketosteroids – distinguishing between benign and malignant lesions – Elevations of 10–30 times normal are typical of malignancy. RPLND is recommended for malignant lesions Prognosis is excellent for benign lesions Poor for patients with disseminated disease.

NON-GERM CELL TUMORS Leydig Cell Tumors Sertoli Cell Tumors Gonadoblastomas

SERTOLI CELL TUMORS Epidemiology and Pathology – Rare (less than 1% of all testicular tumors) – Bimodal age distribution (<1; ) – 10% are malignant

Histopathology – Gross: yellow or gray-white w/ cystic components; benign: well-circumscribed; malignant: ill-defined borders – Microscopic: epithelial and stromal components; large nucleus; solitary nucleolus; vacuolated cytoplasm

Clinical Findings Testicular mass- most common presentation Virilization (children) Gynecomastia (30% in adults) Treatment – Radical orchiectomy- initial procedure of choice – Malignancy: RPLND is indicated

NON-GERM CELL TUMORS Leydig Cell Tumors Sertoli Cell Tumors Gonadoblastomas

GONADOBLASTOMAS Epidemiology – 0.5% of all testicular – Almost exclusively seen in patients w/ gonadal dysgenesis – <30 y/o (age distribution ranges from infancy to beyond 70 years)

Histopathology Gross examination reveals a yellow or gray-white lesion that can vary in size from microscopic to greater than 20 cm and may exhibit calcifications. Microscopically, 3 cell types are seen: Sertoli cells, interstitial cells, and germ cells.

Sagittal US – well circumscribed heterogenous echotexture

Clinical Findings 4/5 of patients with gonadoblastomas are pheno- typic females Males typically have cryptorchidism or hypospadias. Treatment and Prognosis – Radical orchiectomy – In the presence of gonadal dysgenesis, a contralateral gonadectomy is recommended because the tumor tends to be bilateral in 50% of case – Excellent prognosis

Germ Cell Tumors Non- Germ Cell Tumors Secondary Tumors

SECONDARY TUMORS Lymphoma Leukemic infiltration Metastatic

SECONDARY TUMORS Lymphoma Leukemic infiltration Metastatic

LYMPHOMA Epidemiology and Pathology – Most common tumor in a patient >50 y/o – Most common secondary neoplasm (5% of all testicular tumors) – Seen in 3 clinical settings Late manifestation of widespread lymphoma Initial presentation of clinically occult disease Primary extranodal disease

Histopathology Gross: bulging, gray or pink, ill-defined margins; hemorrhage & necrosis are common Microscopic: diffuse histiocytic lymphoma

Clinical Findings Painless enlargement of testis Bilateral in 50%; asynchronous Treatment and Prognosis – FNA: patients with a known or suspected diagnosis of lymphoma – Radical orchiectomy is reserved for those with suspected primary lymphoma of the testicle – Further staging and treatment should be handled in conjunction with the medical oncologist – Prognosis related to stage – Adjuvant chemotherapy for primary testicular lymphoma: 93% survival rate

SECONDARY TUMORS Lymphoma Leukemic infiltration Metastatic

LEUKEMIC INFILTRATION Testis: relapse site for children w/ ALL Bilateral (50% of cases) Testis biopsy rather than orchiectomy is the diagnostic procedure of choice Bilateral irradiation (20Gy) & adjuvant chemotherapy- treatment of choice Prognosis remains guarded.

SECONDARY TUMORS Lymphoma Leukemic infiltration Metastatic

METASTATIC TUMORS Metastasis to the testis is rare Prostate: most common primary site Other sites: lung, GI tract, melanoma, kidney Pathology: neoplastic cells in the interstitium w/ relative sparing of the seminiferous tubules

RADICAL ORCHIECTOMY Orchiectomy - surgical removal of one or both testes