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