Vater壶腹部原发性癌肉瘤临床病理分析并文献复习

Primary Carcinosarcoma of the Ampulla of Vater: Clinicopathological Analysis and Literature Review

目的:探讨Vater壶腹部原发性癌肉瘤的临床病理学特征。方法:回顾性分析2例Vater壶腹部原发性癌肉瘤的临床病理学特征,免疫表型,诊断及鉴别诊断并复习相关文献。结果:两例病人均因上腹痛不适入院,上腹部CT动态增强扫描均提示壶腹区占位。光镜下均可见癌和肉瘤两种成分,癌成分均为腺癌,其中一例腺癌成分伴肠母细胞分化;肉瘤成分均为多形性未分化肉瘤并伴有异源性成分,分别见骨肉瘤和软骨肉瘤分化。癌成分均表达CK,不表达Vimentin;肉瘤成分均表达Vimentin,不表达CK;伴肠母细胞分化的腺癌成分表达SALL4、GPC-3和AFP。结论:Vater壶腹部原发性癌肉瘤罕见,其中腺癌成分伴肠母细胞分化国内外尚未见报道。癌肉瘤主要与肉瘤样癌相鉴别,明确诊断需要病理广泛取材及免疫组织化学检测。

Objective: To investigate the primary carcinosarcoma of the ampulla of vater clinicopathological features. Methods: A retrospective analysis was performed for the clinical features, morphological features, immunophenotype, diagnosis and differential diagnosis of two patients with primary carcinosarcoma of the ampulla of vater, and related articles were reviewed. Results: Both patients were admitted to hospital due to epigastric pain, and Upper-Abdomen Routine Enhanced Scan of CT showed space occupying lesion in the ampulla. Two components of carcinoma and sarcoma were observed under the microscope in both cases. The components of carcinoma were adenocarcinoma, and one adenocarcinoma was accompanied by enteroblastic differentiation. The components of sarcoma were undifferentiated pleomorphic sarcomas with heterologous differentiation, osteosarcoma and chondrosarcoma differentiation respectively. The components of carcinoma were immunopositive for CK, losing of Vimentin protein expression. The components of sarcoma were immunopositive for Vimentin, losing of CK protein expression. The components of adenocarcinoma with enteroblastic differentiation were positive for SALL4, GPC-3 and AFP. Conclusion: The primary carcinosarcoma of the ampulla of vater with enteroblastic differentiation is a rare tumor, which hasn’t been reported so far. Carcinosarcoma mainly needs to be differentiated from sarcomatoid carcinoma, and the definite diagnosis requires careful pathological sampling and immunohistochemical (IHC) detection.