阴茎假血管肉瘤样鳞状细胞癌(附1例报告)

Pseudo-angiosarcomatous squamous cell carcinoma of the penis(report of 1 case)

目的探讨阴茎假血管肉瘤样鳞状细胞癌(PASCC)的诊治方法。方法回顾分析1例阴茎PASCC癌患者的临床资料,分析其发病情况、临床病理特征、诊断和治疗。结果首次行阴茎部分切除、双侧腹股沟淋巴结清扫术(T_2N_2M_0),辅以盆腔放疗,术后2个月,因阴茎皮瓣坏死(伴局部复发)再次行阴茎全部切除术。首次术后11个月,因肺部广泛转移死亡。肿瘤组织主要由梭形、多形性的肿瘤细胞和局灶的鳞状细胞癌细胞组成,排列呈血窦状血管样腔隙或网状结构。腹股沟淋巴结仅见普通型高分化鳞状细胞癌细胞(3/9,2/10)。复发肿瘤的结构和形态与原发肿瘤相似。免疫组化染色示肿瘤细胞CK(AE1/AE3)、34βE12、Vimentin(+),EMA呈灶性或片状(+);CD31、CD34、FⅧRAg、HMB45、SMA、Desmin(-)。结论阴茎PASCC是一种罕见的恶性肿瘤,确诊需依赖组织病理学及免疫组织化学检查,早就诊、早诊断和及时恰当的治疗是关键,但预后差。

To discuss the clinical diagnosis and treatment of penile pseudo-angiosarcomatous squamous cell carcinoma（PASCC）. Methods A case of penile PASCC was retrospectively analyzed with literature review including incidence, clinical feature, histopathology, diagnosis and treatment. Results The patient received partial penectomy and bilateral inguinal lymphadenectomy（T2N2Mo）, combining with adjuvant pelvic radiotherapy, two months after operation, the patient with penile flap necrosis and local regional recurrence received total penectomy. Up to 11 months after the first operation, the patient died due to pelvis cavity and lung metastasis, Tumor tissue was compose of spindle cells, polygonal ceils and few typical squamous epithelial cancer cells under microscope. Spindle cells arrayed complex sinuous vessel-liked channels or formation reticularisand space punctuated with small irregular nests. Five of the nineteen examined inguinal lymph nodes contained metastatic tumor that had only some large nests of well-differentiated squamous cells pattern. The recurrent tumor showed the same pseudoangiosarcomatous pattern described above. Immunohistochemically, most tumor cells were strong positive for keratin（AE1/AE3）, focal positivity for EMA. tumor cells in pseudoangiosarcomatous areas were strong positivity for vimentin, typical squamous cells were focal positivity for 34β E12, vimentin. The vessels that proliferated in the tumor were decorated by CD31, CD34 and factor VIII-related antigen, but the tumor cells showed no reaction to them, and were negative for HMB45,SMA, Desmin. Ag staining revealed no vascular channels. Conclusion Penile PASCC was a rare malignant tumor with poor prognosis, which diagnosis depended on histopathological analysis and Immunohistochemistry. Early examine, early diagnosis and early treatment was very important for cure of this disease.