title: Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome.
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.
authors.affiliation: Department of Oncology and Radiotherapy, Rebagliati Hospital, Lima, Peru.
authors.forename: Liliana
authors.initials: L
authors.lastname: Vasquez
authors.affiliation: Department of Oncology and Radiotherapy, Rebagliati Hospital, Lima, Peru.
authors.forename: Mariela
authors.initials: M
authors.lastname: Tello
authors.affiliation: Department of Oncology and Radiotherapy, Rebagliati Hospital, Lima, Peru.
authors.forename: Ivan
authors.initials: I
authors.lastname: Maza
authors.affiliation: Department of Oncology and Radiotherapy, Rebagliati Hospital, Lima, Peru.
authors.forename: Monica
authors.initials: M
authors.lastname: Oscanoa
authors.affiliation: Department of Genetics, Rebagliati Hospital, Lima, Peru.
authors.forename: Milagros
authors.initials: M
authors.lastname: Dueñas
authors.affiliation: Department of Gynecology-Oncology, Rebagliati Hospital, Lima, Peru.
authors.forename: Haydee
authors.initials: H
authors.lastname: Castro
authors.affiliation: Department of Pathology, Rebagliati Hospital, Lima, Peru.
authors.forename: Alan
authors.initials: A
authors.lastname: Latorre