Non-Syndromic Ovarian Sex Cord Tumor with Annular Tubules: A Case Report

Sex Cord Tumors with Annular Tubules (SCTAT) are a very rare type of non-epithelial ovarian cancer, in one third of cases associated with Peutz-Jegher Syndrome (PJS). The diagnosis is histopathological and challenging due to their rarity. Surgery is the mainstay of treatment. Although there is a high rate of recurrence, the prognosis is relatively favorable.

34 year-old female, presenting with acute abdominal pain, increased abdominal volume and a one year long history of amenorrhea. Computed Tomography (CT) showed a voluminous left adnexal tumor and para-aortic, iliac and mediastinal lymph node enlargement. The intraoperative pathology examination was compatible with a sex cord tumor, apparently confined to the left ovary, so conservative surgery was performed with peritoneal staging. The histopathological examination of the specimen was diagnostic of SCTAT.

SCTAT are a very rare ovarian neoplasm, which makes diagnosis and treatment difficult, due to the lack of evidence. Some retrospective studies, based on case reports, have been performed, but there is still a need for further investigations for optimal care.