title: Non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules: Report of a malignant case.
abstract: A sex cord tumor with annular tubules (SCTAT) is a rare but distinctive subtype of sex cord stromal tumor of the ovary. Its clinical features depend on an association with Peutz-Jeghers syndrome (PJS). SCTAT associated with PJS typically manifests as bilateral, multifocal, and small lesions and is clinically benign. In contrast, SCTAT not associated with PJS often manifests as a unilateral large mass and 20% of such tumors have malignant potential. Most patients with SCTAT are diagnosed at stage I, and metastasis is rare. Here we present a case of malignant SCTAT of stage III A1(ii) (retroperitoneal lymph node metastasis, largest dimension of metastasis >10 mm) in a 14-year-old girl without PJS.
authors.affiliation: Departments of Gynecologic Oncology, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Han
authors.affiliation: Departments of Gynecologic Oncology, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Shumin
authors.initials: S
authors.lastname: Li
authors.affiliation: Departments of Gynecologic Oncology, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Lingying
authors.initials: L
authors.lastname: Wu
authors.affiliation: Department of Pathology, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Xun
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Department of Pathology and Immunology, Washington University School of Medicine in Saint Louis, Missouri, USA.
authors.forename: Dengfeng
authors.initials: D
authors.lastname: Cao
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D009367:Neoplasm Staging; D010051:Ovarian Neoplasms / Q000175:diagnosis* / Q000473:pathology*; D010580:Peutz-Jeghers Syndrome / Q000175:diagnosis* / Q000473:pathology*; D018312:Sex Cord-Gonadal Stromal Tumors / Q000175:diagnosis* / Q000473:pathology*
keywords: Peutz-Jeghers syndrome; behavior; metastasis; sex cord tumor with annular tubules