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原发性腹膜后脂肪肉瘤:10例临床与组织学分析

Primary retroperitoneal liposarcoma: clinical and histological analysis of ten cases.

作者信息

Fernández-Ruiz Mario, Rodríguez-Gil Yolanda, Guerra-Vales Juan Manuel, Manrique-Municio Alejandro, Moreno-González Enrique, Colina-Ruizdelgado Francisco

机构信息

Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, España.

出版信息

Gastroenterol Hepatol. 2010 May;33(5):370-6. doi: 10.1016/j.gastrohep.2009.12.010. Epub 2010 Mar 4.

Abstract

Retroperitoneal liposarcoma constitutes an uncommon and locally aggressive malignancy. We performed a retrospective analysis of 10 patients (6 males; mean age: 63.2+/-11 years) with histologically proven retroperitoneal liposarcoma seen at our institution between 1999 and 2007. Presence of a palpable abdominal mass was the main symptom at diagnosis. All patients underwent complete surgical resection. Negative microscopic margin was achieved in four cases. Histological analysis revealed the following subtypes: well-differentiated (6 cases), dedifferentiated (two cases), pleomorphic, and myxoid/round cell (one case each). Concomitant resection of adjacent organs was needed in five cases. Half of the patients developed tumor recurrence, mainly limited to the retroperitoneum or abdominal cavity. The mean recurrence-free survival was 43.3 months (95%CI: 25.7-60.8), with 3- and 5-year overall survival rates of 79% and 61%, respectively. Patients undergoing complete resection with clear margins showed a near-significant trend toward increased recurrence-free survival (62.9 vs. 29.3 months; P=0.06).

摘要

腹膜后脂肪肉瘤是一种罕见的局部侵袭性恶性肿瘤。我们对1999年至2007年期间在我院经组织学证实为腹膜后脂肪肉瘤的10例患者(6例男性;平均年龄:63.2±11岁)进行了回顾性分析。诊断时的主要症状是可触及腹部肿块。所有患者均接受了完整的手术切除。4例患者实现了显微镜下切缘阴性。组织学分析显示以下亚型:高分化型(6例)、去分化型(2例)、多形性型以及黏液样/圆形细胞型(各1例)。5例患者需要同时切除相邻器官。半数患者出现肿瘤复发,主要局限于腹膜后或腹腔。无复发生存期的平均时间为43.3个月(95%置信区间:25.7 - 60.8),3年和5年总生存率分别为79%和61%。切缘清晰的完整切除患者的无复发生存期有近乎显著的增加趋势(62.9个月对29.3个月;P = 0.06)。

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