Retiform Sertoli-Leydig cell tumour of the ovary

Epidemiology

This is a rare variant of Sertoli-Leydig cell tumour of the ovary. It presents at a relatively young age and has a lower incidence of virilisation.

Macroscopic appearances

These tumours are variably solid and cystic with spongy haemorrhagic areas.

Histopathology

The various components consist of:

• retiform or tubulopapillary areas. Spaces vary form slit-like to microcystic, containing eosinophilic fluid. Hyaline globules may be seen. The sex cord element may be confluent with the retiform component.

• sex cord component consisting of tubules lined by cells resembling Sertoli cells.

• gonadal stroma, varying form blastematous to hypocellular or resembling the mature ovarial cortex.

• Leydig cells, usually within hypocellular stroma.

• heterologous elements including mucinous epithelium, hepatocytes (cytokeratin and AFP positive), skeletal muscle

Immunohistochemistry, based on 19 cases1:

Hepatocytes, resembling Leydig cells, were demonstrated in 5 of 8 cases and showed weak inhibin positivity as well as cytokeratin and AFP positivity. One case showed mucinous heterologous epithelium, which was moderately positive for inhibin.

Differential diagnosis

• papillary serous adenocarcinoma: the epithelium should be negative for inhibin, although associated stroma may be positive.

• yolk sac (endodermal sinus) tumour; usually associated with a raised serum AFP. Sertoli-Leydig cell tumour may also result in a mildly raised serum AFP. The gross appearances of haemorrhage and necrosis may be similar. A papillary pattern may be seen in both.

• undifferentiated carcinoma

• sertoliform endometrioid carcinoma

• carcinosarcoma

• FATWO are also positive for keratins, inhibin (but usually patchy and weak) and vimentin with variable ER/PR positivity. They lack heterologous elements.

• carcinoid tumour

• struma ovarii

• endometrial stromal sarcoma

Prognosis

About 25% of cases show malignant behaviour. Mesenchymal heterologous elements are an indicator of malignancy.

References

1Mooney, E. E., Nogales, F. F., Bergeron, C., Tavassoli, F. A. Retiform Sertoli-Leydig cell tumours: clinical, morphological and immunohistochemical findings. Histopathology 2002;41:110-117.

This page last revised 4.12.2002.

©SMUHT/PW Bishop