胰腺癌肉瘤(Pancreatic carcinosarcoma)是一种极其罕见的疾病,由于相关文献报道及病理数据较少,极易造成误诊。患者最初因梗阻性黄疸入院,入院后完善患者相关辅助检查后发现胰腺钩突占位,结合实验室检查结果诊断为胰腺恶性肿瘤并行手术治疗,术后病理见:胰腺钩突处结节样肉瘤,肿瘤细胞弥漫分布,呈梭形或卵圆形,异型性明显,肿瘤未累及切缘及淋巴结。最初根据病理及免疫组化结果将其诊断为胰腺滑膜肉瘤,后经基因检测并回顾免疫组化结果后确诊为胰腺癌肉瘤,患者术后行化疗并门诊随访6个月,未见复发及转移。Pancreatic carcinosarcoma is an extremely rare disease. Due to the scarcity of case reports and pathological data, it is highly prone to misdiagnosis. The patient initially presented with obstructive jaundice and was admitted to the hospital. Further diagnostic workups revealed a mass in the pancreatic uncinate process. Combining these findings with laboratory test results, the diagnosis of a malignant pancreatic tumor was made, and the patient underwent surgical treatment. Postoperative pathology revealed a nodular sarcoma in the pancreatic uncinate process, characterized by diffusely distributed tumor cells, which were spindle-shaped or oval with significant atypia. The tumor did not involve the surgical margins or lymph nodes. Initially, based on pathological and immunohistochemical findings, the tumor was diagnosed as pancreatic synovial sarcoma. However, genetic testing and a review of the immunohistochemical results ultimately confirmed the diagnosis of pancreatic carcinosarcoma. The patient underwent postoperative chemotherapy and was followed up in outpatient clinics for six months, during which no recurrence or metastasis was observed.