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J Obstet Gynaecol Res. 2016 Feb;42(2):224-7. doi: 10.1111/jog.12883. Epub 2015 Dec 29.

Non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules: Report of a malignant case.

Author information

1
Departments of Gynecologic Oncology, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
2
Department of Pathology, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
3
Department of Pathology and Immunology, Washington University School of Medicine in Saint Louis, Missouri, USA.

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.

KEYWORDS:

Peutz-Jeghers syndrome; behavior; metastasis; sex cord tumor with annular tubules

PMID:
26807963
DOI:
10.1111/jog.12883
[Indexed for MEDLINE]

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