non-germ cell tumors
DESCRIPTION
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 PresentationTRANSCRIPT
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