Vasquez Liliana, Tello Mariela, Maza Ivan, Oscanoa Monica, Dueñas Milagros, Castro Haydee, Latorre Alan
Department of Oncology and Radiotherapy, Rebagliati Hospital, Lima, Peru.
Department of Genetics, Rebagliati Hospital, Lima, Peru.
Case Rep Oncol Med. 2015;2015:392576. doi: 10.1155/2015/392576. Epub 2015 Oct 19.
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.
卵巢及卵巢旁肿瘤在儿童中并不常见，主要起源于生殖细胞肿瘤，上皮性肿瘤则最为少见。生殖道肿瘤与普洛提斯综合征之间存在关联，普洛提斯综合征是一种罕见的、散发性的进行性疾病，其特征是由AKT1基因的镶嵌突变导致多个组织在出生后过度生长。我们报告了一名20个月大的无症状婴儿，患有普洛提斯综合征，该患儿发生了子宫内膜样卵巢旁交界性囊性肿瘤。这是迄今为止文献报道的患有这种罕见综合征且伴有附件交界性恶性肿瘤的最年轻患者。共有9例女性生殖道肿瘤合并普洛提斯综合征的病例被描述，其中包括双侧卵巢囊腺瘤及其他良性肿块。卵巢旁肿瘤在儿童中极为罕见，可被视为普洛提斯综合征的一个标准。这些肿瘤的标准化分期和治疗方法尚未完全确立；然而，大多数作者认为这些肿瘤必须按照卵巢肿瘤的治疗方法进行处理。
