Funk Tracy, Frew Patty, Rodgers William, Troxell Megan
Department of Pathology, Oregon Health and Science University, Portland, OR, USA.
Pediatr Dev Pathol. 2006 Jul-Aug;9(4):316-20. doi: 10.2350/06-01-0016.1.
We report a case of fallopian tube and peritoneal lesions resected from a 10-year-old girl with Proteus syndrome. The papillary fallopian tube tumor expressed CA-125 and demonstrated negative staining for CEA, CD15, calretinin, and CK 5/6. Nondilated portions of fallopian tube exhibited evidence of an in situ component. This patient was also found to have peritoneal mesothelial hyperplasia as well as cystic peritoneal lesions of mesothelial origin, with positive calretinin staining. This case provides direct evidence of both Müllerian and mesothelial lesions arising concurrently in a patient with Proteus syndrome and represents the 1st documented fallopian tube neoplasm. Previously reported lesions involving the female and male genital tracts in patients with Proteus syndrome are reviewed.
我们报告了一例从一名患有变形综合征的10岁女孩身上切除的输卵管和腹膜病变。乳头状输卵管肿瘤表达CA-125，而癌胚抗原（CEA）、CD15、钙视网膜蛋白和细胞角蛋白5/6（CK 5/6）染色均为阴性。输卵管未扩张部分显示有原位成分的证据。该患者还被发现有腹膜间皮增生以及间皮起源的囊性腹膜病变，钙视网膜蛋白染色呈阳性。本病例提供了变形综合征患者同时出现苗勒管和间皮病变的直接证据，并且是首例有记录的输卵管肿瘤。本文还对先前报道的变形综合征患者涉及女性和男性生殖道的病变进行了综述。
