可能来源于Wolff管的女性附件肿瘤临床病理特征分析

Clinicopathologic features of female adnexal tumor of probable Wolffian origin

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。

Obvjective To study the clinical and pathological features,differential diagnosis and new treatment of female adnexal tumor of probable Wolffian origin （FATWO）.Methods Light microscopy and immunohistochemistry methods were utilized to study the features of the histopathology of two cases of FATWO and the literature was reviewed.Results One case had recurrence in pelvic cavity 7 years after her primary tumor was diagnosed and excised.Another case had primary FATWO in the mesosalpinx.They showed varying morphologies with solid,tubular,and retiform features.Immunohistochemical staining was positive for CKpan,CK7,vimentin,CD10 and calretinin,partially weak positive for αinhibin,but negative for EMA,CEA and CK20.Conclusions FATWO is a rare neoplasm arising from the remnants of mesonephric/Wolffian ducts.Surgical debulking with hysterectomy and bilateral adnexectomy appears as the most successful initial treatment for FATWO.However,the role of chemotherapy and radiotherapy in recurrent disease is unknown.The administration of Gleevec （STI 571） in recurrent,C-kit-positive FATWO could be considered.