Ovarian Sex Cord Tumor with Annular Tubules

**Semantic type:** Neoplastic Process

**Definition:** An ovarian sex cord-stromal tumor characterized by the presence of Sertoli cells forming annular tubules. It may be associated with Peutz-Jeghers syndrome. Cases associated with Peutz-Jeghers syndrome have followed a benign clinical course. Cases which are not associated with Peutz-Jeghers syndrome have been reported having a clinically malignant course.

**Synonyms:** - Ovarian Sertoli Cell Tumor, Annular Tubular Variant - Ovarian Sex Cord Neoplasm with Annular Tubules - Sex cord tumor with annular tubules