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食管巨大脂肪肉瘤合并 Li-Fraumeni 样综合征。

Giant liposarcoma of the esophagus with Li-Fraumeni-like syndrome.

机构信息

Department of Thoracic Surgery, Center for Mini-invasive Thoracic Surgery, People's Hospital, Peking University, Beijing, China.

出版信息

Eur J Cardiothorac Surg. 2011 Nov;40(5):1253-5. doi: 10.1016/j.ejcts.2011.02.006. Epub 2011 Mar 31.

Abstract

Primary esophageal liposarcoma is a rare malignancy, with only 25 cases reported in the English literature. In this report, we present a new case of giant esophageal liposarcoma in a 49-year-old woman with a family history of upper gastrointestinal tract cancer. This was a transmural tumor located in the lower esophagus. Subtotal esophagectomy was successfully performed, and histopathology revealed a well-differentiated liposarcoma. The patient was followed-up for 20 months without recurrence. However, asynchronous ovarian serous adenocarcinoma was found 1 year after the esophageal surgery, and Li-Fraumeni-like syndrome was diagnosed.

摘要

原发性食管脂肪肉瘤是一种罕见的恶性肿瘤,英文文献中仅报道了 25 例。本报告介绍了一位 49 岁女性的新病例,该患者有上消化道癌家族史。这是一个位于食管下段的全层肿瘤。成功进行了食管次全切除术,组织病理学显示为分化良好的脂肪肉瘤。患者随访 20 个月无复发。然而,食管手术后 1 年发现卵巢浆液性腺癌,诊断为李-佛美尼样综合征。

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