Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome
| Autor: | Milagros Dueñas, Monica Oscanoa, Ivan Maza, Liliana Vasquez, Haydee Castro, Alan Latorre, Mariela Tello |
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| Rok vydání: | 2015 |
| Předmět: |
Pathology
medicine.medical_specialty Cystic Tumor business.industry Case Report lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens medicine.disease lcsh:RC254-282 Asymptomatic Proteus syndrome Oncology medicine Rare syndrome Neoplasm Postnatal overgrowth Germ cell tumors Akt1 gene medicine.symptom business |
| Zdroj: | Case Reports in Oncological Medicine Case Reports in Oncological Medicine, Vol 2015 (2015) |
| ISSN: | 2090-6714 2090-6706 |
| DOI: | 10.1155/2015/392576 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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