Non Peutz-Jegher syndrome associated malignant sex cord stromal tumor with annular tubules
A 32-year-old multiparous lady was admitted for abdominal pain. On pelvic examination, a mass separate from the uterus was felt in the fornices. Ultrasonography showed a well-defined, thickwalled, lobulated, cystic ovarian mass with irregular septae measuring 17 × 16 × 6 cm. As the findings were suggestive of malignant ovarian tumor, she underwent panhysterectomy. Uterus, cervix, and left adnexa were unremarkable. The right ovary was cystic and measured 21 × 18 × 5 cm with a smooth external surface. The cut surface was multiloculated, filled with hemorrhagical fluid with focal solid areas []. Microscopically, the cervix showed diffuse laminar endocervical hyperplasia. No adenoma malignum or mucinous metaplasia of endometrial or tubal epithelium was seen. Right ovary was replaced by a solid, cystic tumor composed of round to oval granulosa cells with vesicular nuclei and an occasional nuclear groove. Cytoplasm was moderate to scant and eosinophilic. Cells were arranged in sheets, nests, micro follicular, macro follicular, and insular pattern []. Simple and complex tubules encircling nodules of hyalinised PAS-positive basement membrane-like material were seen [Figures and ]. Nuclei showed antipodal arrangement with focal sertoliform pattern []. Luteinisation was evident in areas. The adjacent ovarian stroma showed invasion by the tumor and lymphatic emboli []. A diagnosis of malignant SCTAT was rendered after confirmation by immunohistochemistry (IHC) for inhibin and calretinin.