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原发性肝-carciinosarcoma 由肝细胞癌、胆管细胞癌、骨肉瘤和横纹肌肉瘤组成,预后不良。

Primary Hepatic Carcinosarcoma Composed of Hepatocellular Carcinoma, Cholangiocarcinoma, Osteosarcoma and Rhabdomyosarcoma With Poor Prognosis.

机构信息

Department of Pathology, Mercy Medical Center, Baltimore, MD, U.S.A.

University of Maryland, College Park, MD, U.S.A.

出版信息

Anticancer Res. 2020 Apr;40(4):2225-2229. doi: 10.21873/anticanres.14184.

Abstract

BACKGROUND

Primary hepatic carcinosarcoma is a rare subtype of liver malignancy, with only a small number of cases described in the English literature.

CASE REPORT

We report the case of a 72-year-old man with a history of hepatitis C, who presented with complaints of abdominal pain. The patient's alpha fetoprotein (AFP) level was highly elevated at 7,406 ng/ml. His albumin, total bilirubin, aspartate aminotransferase, alanine aminotransferase, and alkaline phosphatase levels were within normal ranges. Computer tomographic scans discovered a 12×9×8 cm mass in the left lobe of the liver, extending to the anterior gastric wall. A partial hepatectomy of segments 2 and 3 with en bloc distal gastrectomy and omentectomy, a Roux-en-Y gastrojejunostomy, and a cholecystectomy were performed. Pathology revealed the mass to be a hepatic carcinosarcoma composed of collision tumor of four malignant components: hepatocellular carcinoma, cholangiocarcinoma, osteosarcoma and rhabdomyosarcoma. One and half month post-surgery, the patient was found to have a mass confirmed by biopsy as hepatocellular carcinoma in the right lobe, nodules in his lung and bone, and his AFP level elevated to 51,027.6 ng/ml. He died after two months during hospice care.

CONCLUSION

To the best of our knowledge, this is the first documented case of primary hepatic carcinosarcoma with collision tumor of four malignant entities (hepatocellular carcinoma, cholangiocarcinoma, osteosarcoma and rhabdomyosarcoma). The pathogenesis, diagnosis, treatment and prognosis of this disease are discussed.

摘要

背景

原发性肝肉瘤是一种罕见的肝恶性肿瘤亚型,仅在英文文献中有少量病例描述。

病例报告

我们报告了一例 72 岁男性患者,有丙型肝炎病史,因腹痛就诊。患者的甲胎蛋白(AFP)水平显著升高,达到 7406ng/ml。他的白蛋白、总胆红素、天门冬氨酸氨基转移酶、丙氨酸氨基转移酶和碱性磷酸酶水平均在正常范围内。计算机断层扫描发现肝脏左叶有一个 12×9×8cm 的肿块,延伸至胃前壁。进行了部分肝切除术 2 和 3 段,包括整块远端胃切除术和网膜切除术、Roux-en-Y 胃空肠吻合术和胆囊切除术。病理学显示肿块由四个恶性成分的碰撞肿瘤组成:肝细胞癌、胆管癌、骨肉瘤和横纹肌肉瘤。手术后一个半月,患者被发现右叶有一个经活检证实为肝细胞癌的肿块,肺部和骨骼有结节,AFP 水平升高至 51027.6ng/ml。他在两个月的临终关怀期间去世。

结论

据我们所知,这是首例记录在案的原发性肝肉瘤伴四种恶性实体(肝细胞癌、胆管癌、骨肉瘤和横纹肌肉瘤)碰撞肿瘤的病例。讨论了该疾病的发病机制、诊断、治疗和预后。

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