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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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tinguished from Sertoli cell nodules, and<br />

Leydig cell tumours, and rete adenomas.<br />

Sertoli cell nodules, however, are small,<br />

incidentally discovered, non neoplastic<br />

lesions composed of aggregates of small<br />

tubules lined by immature Sertoli cells<br />

and contain prominent basement membrane<br />

deposits. The rete adenomas<br />

occur within the dilated lumens of the<br />

rete testis.<br />

Prognosis<br />

Most Sertoli cell tumours are benign.<br />

Malignant Sertoli cell tumour<br />

ICD-O code 8640/3<br />

Epidemiology<br />

Malignant Sertoli cell tumour not otherwise<br />

specified is rare {1194}. Less than 50<br />

cases have been reported. Age distribution<br />

does not differ from that of the benign<br />

form, occurring from childhood to old age.<br />

Clinical features<br />

Some patients present with metastases;<br />

most commonly to inguinal, retroperitoneal<br />

and/or supraclavicular lymph<br />

nodes. Approximately one third has<br />

gynecomastia at presentation, but apart<br />

from that no specific lesions or syndrome<br />

are known to be associated with malignant<br />

Sertoli cell tumour.<br />

Macroscopy<br />

They tend to be larger than the benign<br />

counterparts {2894}, usually more than 5<br />

cm but range 2 to 18 cm. The macroscopic<br />

appearance may differ from that<br />

of the benign tumour by necrosis and<br />

haemorrhage.<br />

Histopathology<br />

Microscopically, the cellular features and<br />

Fig. 4.75 Sclerosing type of Sertoli cell tumour.<br />

growth patterns are similar to those of the<br />

benign counterpart but tend to be more<br />

variable within the same tumour and<br />

between tumours. The solid, sheet-like<br />

growth pattern is often prominent. The<br />

nuclei may be pleomorphic with one or<br />

more nucleoli, which are usually not very<br />

prominent. Mitotic figures may be numerous,<br />

and necrosis may occur. A fibrous,<br />

hyalinized or myxoid stroma occurs in<br />

varying amounts, but is usually sparse.<br />

Lymphovascular invasion may be seen.<br />

Lymphoplasmacytic infiltration is reported<br />

in some cases varying from sparse to<br />

pronounced and even with secondary<br />

germinal centres.<br />

The most important differential diagnoses<br />

are classical and spermatocytic seminoma<br />

and variants of yolk sac tumour, however<br />

granulomatous reactions and<br />

intratubular germ cell neoplasia are not<br />

present in the surrounding testicular<br />

parenchyma. Endometrioid adenocarcinoma<br />

and metastases, and among the<br />

latter especially adenocarcinomas with<br />

pale or clear cytoplasm, as well as<br />

melanoma should also be considered.<br />

Immunohistochemical staining is helpful<br />

in defining the Sertoli cell nature of the<br />

tumour but not its malignant potential<br />

{1074,1194}. The tumour cells are<br />

cytokeratin and vimentin positive and<br />

they may also be positive for epithelial<br />

membrane antigen. They stain for inhibin<br />

A, but usually not very intensely, and they<br />

may be S100 positive. They are PLAP<br />

and CEA negative.<br />

Granulosa cell tumour group<br />

Definition<br />

Granulosa cell tumours of the testis are<br />

morphologically similar to their ovarian<br />

counterparts. Two variants are distinguished:<br />

adult and juvenile types.<br />

Fig. 4.76 Malignant Sertoli cell tumour.<br />

A<br />

Fig. 4.77 Malignant Sertoli cell tumour. A Solid and tubular components. B Vimentin staining in the tubular<br />

component.<br />

B<br />

Sex cord / gonadal stromal tumours 255

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