Ustuner Zeki, Basaran Mert, Dizdar Yavuz, Agaoglu Fulya Yaman, Bilgic Bilge, Sakar Burak, Basaran Gul Atalay, Darendeliler Emin, Ozger Harzem, Onat Haluk, Bavbek Sevil
Department of Medical Oncology, Istanbul University Institute of Oncology, Istanbul, Turkey.
Tohoku J Exp Med. 2007 Nov;213(3):221-9. doi: 10.1620/tjem.213.221.
Rhabdomyosarcoma (RMS) is rare disease in adults (age >or= 16 years). The data from randomized prospective trials are scarce; the clinical outcome of these patients seems poor with the currently available treatment strategies. In this study, we report a single institution's experience in the treatment of adult RMS. We reviewed the medical records of patients with RMS who were >or= 16 years and have been treated in our institution between 1988 and 2003 retrospectively. We analyzed the survival outcome of these patients and the prognostic impact of clinical/pathological factors on their survival. In total, 23 patients with RMS were identified. Median age was 26 years (range, 16-72 years). Majority of patients were male (n: 17, 73.9%), and had large tumors (>or= 5 cm, n: 13, 56.5%), localized disease (N0, M0, n: 12, 52.2%), and embryonal histology (n: 10, 43.5%). Median overall survival was 31.3 months, and the 3-year progression-free survival and overall survival rates were 19.9% and 34.94%, respectively. Patients with smaller tumors (< 5 cm) (p < 0.04), local disease (p < 0.01), and normal lactic dehydrogenase (LDH) level (p < 0.01) at the time of diagnosis were found to have better survival outcome. The tumor size, serum LDH level, and metastatic disease at the time of diagnosis are potential predictors of outcome in patients with adult RMS. Adult RMS is an aggressive disease with poor survival despite treatment. The data from prospective, randomized multicenter trials are necessary in order to improve the clinical outcome of adult RMS patients.
横纹肌肉瘤（RMS）在成人（年龄≥16岁）中是一种罕见疾病。来自随机前瞻性试验的数据稀少；采用目前可用的治疗策略，这些患者的临床结局似乎较差。在本研究中，我们报告了一家机构治疗成人RMS的经验。我们回顾性分析了1988年至2003年间在我们机构接受治疗的年龄≥16岁的RMS患者的病历。我们分析了这些患者的生存结局以及临床/病理因素对其生存的预后影响。总共确定了23例RMS患者。中位年龄为26岁（范围16 - 72岁）。大多数患者为男性（n = 17，73.9%），患有大肿瘤（≥5 cm，n = 13，56.5%），疾病局限（N0，M0，n = 12，52.2%），组织学类型为胚胎型（n = 10，43.5%）。中位总生存期为31.3个月，3年无进展生存率和总生存率分别为19.9%和34.94%。诊断时肿瘤较小（<5 cm）（p < 0.04）、疾病局限（p < 0.01）以及乳酸脱氢酶（LDH）水平正常（p < 0.01）的患者生存结局较好。诊断时的肿瘤大小、血清LDH水平和转移性疾病是成人RMS患者预后的潜在预测因素。成人RMS是一种侵袭性疾病，尽管接受了治疗，生存率仍较低。为了改善成人RMS患者的临床结局，有必要开展前瞻性、随机多中心试验。
