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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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ICD-O codes<br />

Granulosa cell tumour 8620/1<br />

Adult type granulosa cell<br />

tumour 8620/1<br />

Juvenile type granulosa<br />

cell tumour 8622/1<br />

Adult type granulosa cell tumour<br />

Incidence and clinical features<br />

This tumour is rare {1,477,1443,1705,1<br />

812,2567}, grows slowly and only two<br />

dozen cases have been reported {1901}.<br />

Some are incidental. About 25% of<br />

patients have gynecomastia. The average<br />

age at presentation is 44 years (range, 16-<br />

76 years). Patients have elevated serum<br />

levels of both inhibin, as occurs in other<br />

sex cord-stromal tumours {1781}, and<br />

Müllerian-inhibiting hormone, as occurs in<br />

similar ovarian tumours {1433}.<br />

Macroscopy<br />

These tumours are circumscribed, sometimes<br />

encapsulated, have a firm consistency<br />

and vary from yellow to beige.<br />

They vary from microscopic to 13 cm in<br />

diameter. The tumour surface may show<br />

cysts from 1-3 mm in diameter. Necrosis<br />

or haemorrhage are unusual.<br />

Histopathology<br />

Several patterns occur: macrofollicular,<br />

microfollicular, insular, trabecular, gyriform,<br />

solid and pseudosarcomatous.<br />

The microfollicular pattern is the most<br />

frequent. Microfollicles consist of palisading<br />

cells, which surround an<br />

eosinophilic material (Call-Exner bodies).<br />

Tumour cells are round to ovoid with<br />

grooved nuclei (coffee-bean nuclei) with<br />

one to two large peripheral nucleoli.<br />

Cellular pleomorphism and mitotic figures<br />

are infrequent, except for those<br />

areas showing fusiform cell pattern. The<br />

tumour may intermingle with seminiferous<br />

tubules and infiltrate the tunica<br />

albuginea. Some show focal theca cell<br />

differentiation, or have smooth muscle or<br />

osteoid {46}.<br />

Tumour cells are immunoreactive for<br />

vimentin, smooth muscle actin, inhibin,<br />

MIC2 (013-Ewing sarcoma marker), and<br />

focally cytokeratins.<br />

Fig. 4.78 Granulosa cell tumour, adult type.<br />

Juvenile type granulosa cell<br />

tumour<br />

This tumour is multicystic and its structure<br />

resembles that of Graafian follicles.<br />

Although it is rare, it is the most frequent<br />

congenital testicular neoplasm {1022,<br />

2528}, comprising 6.6% of all prepubertal<br />

testicular tumours {1275}.<br />

Clinical features<br />

The tumour presents as a scrotal or<br />

abdominal asymptomatic mass, preferentially<br />

located in the left testis {1896}. It<br />

involves an abdominal testis in about<br />

30% of cases. The contralateral testis is<br />

often undescended too. Most of the<br />

tumours are observed in the perinatal<br />

period, and presentation after the first<br />

year of life is exceptional. External genitalia<br />

are ambiguous in 20% and the most<br />

frequent associated anomaly is mixed<br />

gonadal dysgenesis, followed by<br />

hypospadias. In all cases with ambiguous<br />

genitalia the karyotype is abnormal:<br />

45X / 46XY mosaicism or structural<br />

anomalies of Y chromosome. Neither<br />

recurrences nor metastases have been<br />

observed {400,2092,2136,2576,2895}.<br />

Neither gynecomastia nor endocrine disorders<br />

appeared associated.<br />

Macroscopy<br />

These tumours are usually cystic, with<br />

solid areas and partially encapsulated.<br />

The tumour size varies from 0.8 to 5 cm<br />

in size {1453}. Haemorrhage secondary<br />

to a torsion or trauma may make diagnosis<br />

difficult {407}.<br />

Histopathology<br />

Cysts are lined by several cell layers,<br />

depending on the degree of cystic dilation.<br />

The inner cells are similar to granulosa<br />

cells, while the outer cells resemble<br />

theca cells. Granulosa-like cells are<br />

Prognosis<br />

The tumour metastasizes in 20% or more<br />

of patients, even several years after the<br />

presentation {1223,1647}.<br />

Fig. 4.79 Juvenile granulosa cell tumour. Note prominent cysts.<br />

256 Tumours of the testis and paratesticular

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