non-germ cell tumors

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

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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) - PowerPoint PPT Presentation

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Page 1: NON-GERM CELL TUMORS

NON-GERM CELL TUMORS

• Leydig Cell Tumors• Sertoli Cell Tumors• Gonadoblastomas

Page 2: NON-GERM CELL TUMORS

NON-GERM CELL TUMORS

• Leydig Cell Tumors• Sertoli Cell Tumors• Gonadoblastomas

Page 3: NON-GERM CELL TUMORS

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

Page 4: NON-GERM CELL TUMORS

Gross Pathology

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

Page 5: NON-GERM CELL TUMORS

Histopathology

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

Page 6: NON-GERM CELL TUMORS

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

Page 7: NON-GERM CELL TUMORS

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.

Page 8: NON-GERM CELL TUMORS

NON-GERM CELL TUMORS

• Leydig Cell Tumors• Sertoli Cell Tumors• Gonadoblastomas

Page 9: NON-GERM CELL TUMORS

SERTOLI CELL TUMORS

• Epidemiology and Pathology– Rare (less than 1% of

all testicular tumors)– Bimodal age

distribution (<1; 20-45)

– 10% are malignant

Page 10: NON-GERM CELL TUMORS

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

Page 11: NON-GERM CELL TUMORS

Clinical Findings

• Testicular mass- most common presentation• Virilization (children)• Gynecomastia (30% in adults)

• Treatment– Radical orchiectomy- initial procedure of choice– Malignancy: RPLND is indicated

Page 12: NON-GERM CELL TUMORS

NON-GERM CELL TUMORS

• Leydig Cell Tumors• Sertoli Cell Tumors• Gonadoblastomas

Page 13: NON-GERM CELL TUMORS

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)

Page 14: NON-GERM CELL TUMORS

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.

Page 15: NON-GERM CELL TUMORS

Sagittal US – well circumscribed heterogenous echotexture

Page 16: NON-GERM CELL TUMORS

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

Page 17: NON-GERM CELL TUMORS

• Germ Cell Tumors• Non- Germ Cell Tumors• Secondary Tumors

Page 18: NON-GERM CELL TUMORS

SECONDARY TUMORS

• Lymphoma• Leukemic infiltration• Metastatic

Page 19: NON-GERM CELL TUMORS

SECONDARY TUMORS

• Lymphoma• Leukemic infiltration• Metastatic

Page 20: NON-GERM CELL TUMORS

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

Page 21: NON-GERM CELL TUMORS

Histopathology

• Gross: bulging, gray or pink, ill-defined margins;

• hemorrhage & necrosis are common

• Microscopic: diffuse histiocytic lymphoma

Page 22: NON-GERM CELL TUMORS

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

Page 23: NON-GERM CELL TUMORS

SECONDARY TUMORS

• Lymphoma• Leukemic infiltration• Metastatic

Page 24: NON-GERM CELL TUMORS

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.

Page 25: NON-GERM CELL TUMORS

SECONDARY TUMORS

• Lymphoma• Leukemic infiltration• Metastatic

Page 26: NON-GERM CELL TUMORS

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

Page 27: NON-GERM CELL TUMORS

RADICAL ORCHIECTOMYOrchiectomy - surgical removal of one or both testes