non-germ cell tumors leydig cell tumors sertoli cell tumors gonadoblastomas

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; 25-35) 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; 20-45)

– 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 ORCHIECTOMYOrchiectomy - surgical removal of one or both testes