Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome
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...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2015/392576 |
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| author | Liliana Vasquez Mariela Tello Ivan Maza Monica Oscanoa Milagros Dueñas Haydee Castro Alan Latorre |
| author_facet | Liliana Vasquez Mariela Tello Ivan Maza Monica Oscanoa Milagros Dueñas Haydee Castro Alan Latorre |
| author_sort | Liliana Vasquez |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-793b11221034454b97a094ed92e12284 |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-793b11221034454b97a094ed92e122842025-02-03T05:58:34ZengWileyCase Reports in Oncological Medicine2090-67062090-67142015-01-01201510.1155/2015/392576392576Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus SyndromeLiliana Vasquez0Mariela Tello1Ivan Maza2Monica Oscanoa3Milagros Dueñas4Haydee Castro5Alan Latorre6Department of Oncology and Radiotherapy, Rebagliati Hospital, Lima, PeruDepartment of Oncology and Radiotherapy, Rebagliati Hospital, Lima, PeruDepartment of Oncology and Radiotherapy, Rebagliati Hospital, Lima, PeruDepartment of Oncology and Radiotherapy, Rebagliati Hospital, Lima, PeruDepartment of Genetics, Rebagliati Hospital, Lima, PeruDepartment of Gynecology-Oncology, Rebagliati Hospital, Lima, PeruDepartment of Pathology, Rebagliati Hospital, Lima, PeruOvarian 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.http://dx.doi.org/10.1155/2015/392576 |
| spellingShingle | Liliana Vasquez Mariela Tello Ivan Maza Monica Oscanoa Milagros Dueñas Haydee Castro Alan Latorre Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome Case Reports in Oncological Medicine |
| title | Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome |
| title_full | Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome |
| title_fullStr | Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome |
| title_full_unstemmed | Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome |
| title_short | Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome |
| title_sort | endometrioid paraovarian borderline cystic tumor in an infant with proteus syndrome |
| url | http://dx.doi.org/10.1155/2015/392576 |
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