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目的探讨可能来源于Wolff管的女性附件肿瘤(FATWO)的临床病理学特征、鉴别诊断要点与治疗进展。方法对2例FATWO进行临床病理分析及免疫组化染色观察,并结合文献复习。结果 1例为输卵管系膜原发FATWO,术后7年发生盆腔广泛转移;另1例为输卵管系膜原发FATWO。原发病变及复发转移病变均表现为境界清楚的实性肿块,镜下可见管状、网状及实性结构。免疫组化:肿瘤细胞CKpan、CK7、vimentin、CD10和calretinin(+),α-inhibin局灶弱(+),EMA、CEA和CK20(-)。结论 FATWO是一种罕见的起源于残留中肾管的肿瘤,其诊断和鉴别诊断主要依靠组织学形态和免疫组化。对于原发性FATWO首选治疗方式是完整肿块切除加子宫及双附件切除;对于复发病例常规化疗或放疗的效果并不确切,针对CD117阳性的复发或转移FATWO病例,格列卫有望成为一种可供选择的新型化疗药物。
Objective To investigate the clinicopathological characteristics of female accessory tumor (WAVF) that may originate from Wolff tube, and to differentiate the diagnosis points and treatment progress. Methods Two cases of FATWO were analyzed by clinicopathological and immunohistochemical staining combined with literature review. Results One case was oviduct mesenteric primary fat tissue, extensive pelvic metastasis occurred 7 years after operation. The other case was oviduct mesenteric primary fat tissue. The primary lesions and recurrence and metastasis lesions showed clear real solid tumors, microscopic tubular, reticular and solid structure. Immunohistochemistry: The tumor cells CKpan, CK7, vimentin, CD10 and calretinin (+), α-inhibin focal weak (+), EMA, CEA and CK20 (-). Conclusions FATWO is a rare tumor originated from the residual renal tube. The diagnosis and differential diagnosis of FATWO rely mainly on histological morphology and immunohistochemistry. The preferred treatment for primary FATWO is a complete lump resection plus resection of the uterus and double attachments; the efficacy of conventional chemotherapy or radiotherapy for recurrent cases is not clear, and Gleevec is expected to become a potential target for CD117-positive cases of recurrent or metastatic FATWO Alternative chemotherapy drugs.