Hilal Lara, Barada Kassem, Mukherji Deborah, Temraz Sally, Shamseddine Ali
Department of Radiation Oncology, Naef K. Basile Cancer Institute, American University of Beirut Medical Center, Beirut, Lebanon.
Gastrointestinal Division, Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
Med Oncol. 2016 Feb;33(2):20. doi: 10.1007/s12032-016-0730-3. Epub 2016 Jan 20.
This review of 76 gastrointestinal (GI) leiomyosarcoma (LMS) cases that include 11 cases from the American University of Beirut Medical Center represents, to our knowledge, the largest number of combined GI LMS cases reported. The age range of GI LMS is variable, and the presentation is non-specific, making pathological diagnosis essential. LMSs usually lack CD117 and CD 34 mutations and are usually positive for smooth muscle cell markers. The review highlights surgery as the mainstay of treatment with negative margins attained most of the times. Adjuvant chemotherapy is used in around 7-27 % of the cases mainly for small intestinal and colorectal LMS. The relatively small number of patients is a limitation on outcome analysis. However, LMS has a risk of recurrence reaching 39-80 % and secondary metastasis reaching 55-71 % in small intestinal and colorectal cases. In light of the high frequency of recurrence and metastasis, enrolling patients in clinical randomized trials to investigate the role of chemotherapy, radiation therapy, and targeted therapy is required for better control of this rare aggressive GI tumor.
据我们所知，这篇对76例胃肠道平滑肌肉瘤（LMS）病例的综述，其中包括来自贝鲁特美国大学医学中心的11例病例，是已报道的胃肠道平滑肌肉瘤合并病例数量最多的。胃肠道平滑肌肉瘤的年龄范围各不相同，临床表现也不具特异性，因此病理诊断至关重要。平滑肌肉瘤通常缺乏CD117和CD34突变，且通常对平滑肌细胞标志物呈阳性反应。该综述强调手术是主要的治疗手段，大多数情况下能实现切缘阴性。约7%-27%的病例使用辅助化疗，主要用于小肠和结肠直肠平滑肌肉瘤。患者数量相对较少限制了疗效分析。然而，在小肠和结肠直肠病例中，平滑肌肉瘤的复发风险达到39%-80%，继发转移风险达到55%-71%。鉴于复发和转移的高频率，需要让患者参与临床随机试验，以研究化疗、放疗和靶向治疗的作用，从而更好地控制这种罕见的侵袭性胃肠道肿瘤。
