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Case Reports in Oncological Medicine
Volume 2015, Article ID 392576, 6 pages
http://dx.doi.org/10.1155/2015/392576
Case Report

Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome

1Department of Oncology and Radiotherapy, Rebagliati Hospital, Lima, Peru
2Department of Genetics, Rebagliati Hospital, Lima, Peru
3Department of Gynecology-Oncology, Rebagliati Hospital, Lima, Peru
4Department of Pathology, Rebagliati Hospital, Lima, Peru

Received 21 August 2015; Revised 26 September 2015; Accepted 30 September 2015

Academic Editor: Yoshihito Yokoyama

Copyright © 2015 Liliana Vasquez et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Ovarian and paraovarian neoplasms are uncommon in children, mainly originating from germ cell tumors and, least frequently, epithelial tumors. There is an association between genital tract tumors and Proteus syndrome, a rare, sporadic, and progressive entity, characterized by a postnatal overgrowth in several tissues caused by a mosaic mutation in the AKT1 gene. We describe a 20-month-old asymptomatic infant with Proteus syndrome who developed an endometrioid paraovarian borderline cystic tumor. This is the youngest patient so far reported in the literature with this rare syndrome and an adnexal tumor of borderline malignancy. A total of nine patients have been described with female tract tumors and associated Proteus syndrome, which includes bilateral ovarian cystadenomas and other benign masses. A paraovarian neoplasm is extremely rare in children and could be considered a criterion for Proteus syndrome. Standardized staging and treatment of these tumors are not well established; however, most authors conclude that these neoplasms must be treated as their ovarian counterparts.