Objective To explore the clinicopathologic features and methods of diagnosis and treatment of the undiffer-entiated carcinoma with osteoclast-like giant cells of pancreas(UCWOGCs). Methods A case of UCWOGCs admitted to and treated in Changhai Hospital Affiliated to Second Military Medical University in December 2011 was reported,and 12 cases in 11 literatures from 1990 to 2013 were also searched. The clinical and pathological features and following up data of the 13 cases were analyzed. Results The patient admitted to our hospital showed upper abdomen discomfort or pain,accompanied with nausea and poor appetite,but showed no jaundice,fever and diarrhea. Physical examination revealed an upper abdominal mass of 16 cm × 15 cm. CT showed space-occupying lesions in abdomen and liver. Distal pancreatectomy,total gastrectomy and splenectomy were performed and pancreas UCWOGCs was confirmed. Among the 13 patients,6 were males and 7 were females. 12 cases showed up-per abdominal discomfort or pain. Physical examination revealed an upper abdominal mass. Ultrasound B and CT examination showed that 11 cases had mass of pancreatic body and tail,and the tumor diameter was 4 cm to 23 cm. All the 13 cases were trea-ted by surgery. Conclusion UCWOGCs is a rare malignant tumor with non -specific symptoms and palpable mass in abdo-men. The origin of the tumor has been controversial. It should be differentiated from malignant fibrous histiocytoma of the pancreas and the giant cell tumor of bone. Surgical resection is the main treatment.%目的：探讨伴有破骨细胞样巨细胞的胰腺未分化癌（ UCWOGCs）的临床病理特征及其诊断与治疗。方法报道第二军医大学附属长海医院2011年12月收治的1例UCWOGCs患者的临床资料，并检索1990-2013年国内文献报道11篇12例患者，对所有13例UCWOGCs患者的临床表现、病理特征及随访资料进行分析。结果本院收治的1例患者3个月前无明显诱因出现上腹部胀痛不适，伴恶心、食欲不振，无黄疸、发热、腹泻等症状，查体剑突下可触及一包块，大小约16 cm ×15 cm；CT示：腹腔内占位，肝内多发占位。行胰体尾+全胃+脾切除术，病理诊断：胰体尾UCWOGCs。13例患者中，男6例，女7例。临床表现上腹部不适或疼痛12例，体检发现上腹部肿块1例。行B超及CT检查，11例提示胰体尾部肿块影。肿瘤直径4~23 cm。13例均行手术治疗。结论 UCWOGCs是一种罕见的恶性肿瘤，临床表现多为非特异性症状，腹部可触及包块，其组织学起源尚有争议，诊断需与胰腺恶性纤维组织细胞瘤、骨巨细胞瘤等鉴别。手术为其主要治疗手段。
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