Elkiran Emin Tamer, Abali Huseyin, Aksoy Sercan, Altundag Kadri, Erman Mustafa, Kars Ayşe, Turker Alev, Tekuzman Gulten, Ozisik Yavuz
Institute of Oncology, Department of Medical Oncology, Hacettepe University, Sihhiye, Ankara, 06100, Turkey.
Med Oncol. 2007;24(2):197-201. doi: 10.1007/BF02698040.
Primary thymic epithelial neoplasms (PTENs) are uncommon tumors of anterior mediastinum with a broad range of biological characteristics. We retrospectively reviewed 58 consecutive patients with a diagnosis of PTENs that were confirmed pathologically during 28 yr. There were 58 patients, 31 males (53.4%) and 27 females (46.6%), with a mean age of 43.6 +/-13.8 yr (range, 17-73 yr). Twenty-one (36.2%) patients presented at the Masaoka stage I, 13 (22.4%) patient at stage II, 18 (31.0%) patient at stage III, and 6 (10.4%) patients at stage IV. Forty-five (77.7%) patients had myasthenia gravis, 1 (1.7%) immune deficiency, 1 (1.7%) pancytopenia, and 1 (1.7%) nephrotic syndrome. No paraneoplastic syndrome was associated in 10 (17.2%) patients. Complete resection was accomplished in 41 (70.7%) patients, while incomplete resection was performed in 8 (13.8%) patients. In nine (15.5%) patients only biopsy was carried out. Radiotherapy was administered to 19 (32.8%) patients. Eleven (19.0%) out of 58 who presented at advanced stages (at least III) received chemotherapy. Median follow-up period was 59 mo (range, 1-278 mo). During the follow-up period, 17 deaths occurred. Five patients (29.4%) died of tumor-related causes, and the remaining 12 patients died of other causes (cardiovascular diseases [n = 1, 5.9%], sepsis [n = 4, 23.5%], and MG-related respiratory insufficiency [n = 7, 41.2%]). The overall survival rates at 5 yr and 10 yr were 63.9% and 54.2%, respectively. Tumor-related survival rates at 5 yr and 10 yr were 89.0% and 83.2%, respectively. In our series, disease stage, presence or absence of myasthenia gravis, and tumor size did not affect survival (p> 0.05), either. Complete resection of the tumor seems to be the best predictive factor for long-term survival.
原发性胸腺上皮肿瘤(PTENs)是前纵隔少见的肿瘤,具有广泛的生物学特性。我们回顾性分析了连续28年中经病理确诊的58例PTENs患者。58例患者中,男性31例(53.4%),女性27例(46.6%),平均年龄43.6±13.8岁(范围17 - 73岁)。21例(36.2%)患者为Masaoka Ⅰ期,13例(22.4%)为Ⅱ期,18例(31.0%)为Ⅲ期,6例(10.4%)为Ⅳ期。45例(77.7%)患者有重症肌无力,1例(1.7%)有免疫缺陷,1例(1.7%)有全血细胞减少,1例(1.7%)有肾病综合征。10例(17.2%)患者无副肿瘤综合征。41例(70.7%)患者实现了完整切除,8例(13.8%)患者行不完全切除。9例(15.5%)患者仅行活检。19例(32.8%)患者接受了放疗。58例晚期(至少Ⅲ期)患者中有11例(19.0%)接受了化疗。中位随访期为59个月(范围1 - 278个月)。随访期间,17例患者死亡。5例(29.4%)患者死于肿瘤相关原因,其余12例患者死于其他原因(心血管疾病[1例,5.9%]、败血症[4例,23.5%]和重症肌无力相关呼吸功能不全[7例,41.2%])。5年和10年总生存率分别为63.9%和54.2%。5年和10年肿瘤相关生存率分别为89.0%和83.2%。在我们的研究系列中,疾病分期、是否存在重症肌无力及肿瘤大小均不影响生存率(p>0.05)。肿瘤完整切除似乎是长期生存的最佳预测因素。