Ovarian sex cord tumor with annular tubules

A pathologic study was done on four cases of ovarian sex cord tumor with annular tubules. All four tumors occurred in young women (11-24 years of age) and were not associated with the Peutz-Jeghers syndrome. Two patients had evidence of hyperestrinism. One patient who had metastasis to the retroperitoneum, left supraclavicular lymph node, and liver confirmed the malignant potential of this tumor. Gross examination revealed tumors that were solid, yellowish, and unilateral, with varying degrees of cystic degeneration. Microscopic examination showed simple or complex annular tubules with prominent basement membranes. Many tumor cells contained lipid in the cytoplasm. Ultrastructural study showed Charcot-Bottcher filaments in all four cases, indicating Sertoli cell differentiation. True lumens and microvilli were identified in one case. The classification of the sex cord tumor with annular tubules as a Sertoli cell tumor, annular tubular type was proposed on the basis of ultrastructural findings.