单中心原发骶骨肿瘤790例的流行病学分析

Epidemiological study of 790 consecutive primary sacral tumors treated in a single center

中文关键词:  原发骶骨肿瘤  流行病学分析

英文关键词:Primary sacral tumors  Epidemiological analysis

中文摘要:

  【摘要】 目的：总结原发骶骨肿瘤的流行病学特点、手术方式及治疗效果。方法：2000年7月~2013年12月在北京大学人民医院骨与软组织肿瘤中心接受手术治疗的骶骨原发肿瘤患者790例，其中男416例，女374例。发病年龄5~78岁，平均44.8岁。病理类型：脊索瘤193例，骨巨细胞瘤141例，神经纤维瘤83例，神经鞘瘤48例，恶性外周神经鞘瘤19例，骨髓瘤39例，骨肉瘤26例，软骨肉瘤49例，尤文肉瘤/PNET 28例，畸胎瘤37例，其他肿瘤127例。随访期3~5年。分析上述主要病理类型病例的男女比例、年龄特点、病灶部位及生长特点、手术方式、局部复发及生存率。结果：①脊索瘤193例，占24.4%。男120例，女73例，平均年龄56.7岁（21~75岁）。89例累及S3以下的患者接受了广泛或边缘性切除；70例累及S2以下的患者接受了整块切除，其中21例整块切除后肿瘤上缘骶骨有残留，进行了补充切除；34例肿瘤累及全骶骨的患者中，19例接受了全骶骨整块切除手术，余患者行分块切除术。远处转移19例（9.8%），包括肺转移10例，骨转移6例，肝转移3例。151例首次手术的患者中57例局部复发（37.7%）。术后5年无病生存率45.1%，5年总生存率87.7%。②骨巨细胞瘤141例，占17.8%。其中男69例，女72例，平均年龄34.2岁（16~61岁）。132例行切刮术，3例行全骶骨切除术，6例因肿瘤巨大仅行多次血管栓塞术。术后共25例复发（18.9%），13例为采用术中腹主动脉球囊临时阻断者，12例为未采用腹主动脉球囊临时阻断者。③神经源性肿瘤150例，占骶骨原发肿瘤的19%。分别为良性神经源性肿瘤131例（神经纤维瘤83例、神经鞘瘤48例）和恶性神经鞘瘤19例。131例良性神经源性肿瘤中，男62例，女69例，平均年龄为42.3岁（17~67岁）；均行边缘性切除，17例（12.9%）术后复发。④骨肉瘤26例，占3.3%。男15例，女11例，平均年龄25.8岁（12~58岁）。16例行术前化疗，21例行术后化疗。13例行整块切除术或全骶骨切除术，13例行分块切除术。12例（46%）手术后复发。术后3年及5年生存率分别为43.5%和21.8%。⑤尤文肉瘤/PNET 28例，占3.5%。男13例，女15例，平均年龄22.3岁（5~50岁）。21例行术前化疗，27例行术后放化疗。14例行整块切除术或全骶骨切除术，14例行分块切除术。15例（53.6%）手术后复发。术后3年及5年生存率分别为39.1%和19.6%。⑥软骨肉瘤49例，占6.2%。男26例，女23例，平均年龄42.5岁（17~69岁）。29例行整块切除术或全骶骨切除术，余行分块切除术。22例（44.9%）手术后复发。术后2年及5年总生存率分别为58.7%和47.0%；术后2年及5年无病生存率分别为42.3%和31.8%。结论：最常见的原发骶骨肿瘤为脊索瘤、骨巨细胞瘤、神经源性肿瘤、骨肉瘤、尤文肉瘤、软骨肉瘤和骨髓瘤。各肿瘤的好发年龄、性别比例、部位及生长方式各有特点。不同的病理类型应选择不同的手术方式。骶骨脊索瘤肿瘤整块切除的术后复发率远低于病灶内手术；骶骨巨细胞瘤治疗最大的难点和关键点是控制术中出血；骶骨原发高度恶性肿瘤如骨肉瘤、尤文肉瘤等边缘或病灶内手术后复发率极高，应行肿瘤整块广泛切除。

英文摘要:

  【Abstract】 Objectives: To summarize the epidemiology, surgical treatment and clinical outcome of patients with primary sacral tumor. Methods: From July 2000 to December 2013, 790 patients with primary sacral tumor underwent surgeries in the department of musculoskeletal tumor, Peking University People′s Hospital. There were 416 males and 374 females with a mean age of 44.8 years(ranged from 5 to 78 years). Major pathological diagnosis in the series included 193 chrondromas, 141 giant cell tumors, 83 neurofibromas, 48 schwannomas, 19 malignant schwannomas, 39 myelomas, 16 lymphomas, 26 osteosarcomas, 49 chondrosarcomas, 28 Ewing′s sarcomas/PNETs, 37 teratomas and 127 other tumors. The follow-up period ranged 3-5 years. Pathological diagnosis, dermography, lesion location, surgical treatment, survival and recurrence of tumors were analyzed retrospectively. Results: ①A total of 193 sacral chordoma was enrolled, representing 24.4% of all 790 cases. There were 120 males and 73 females with an average age of 56.7 years(21-75 years), 89 cases with tumors lower than S3 underwent wide or marginal resection, 70 cases with tumors lower than S2 received en bloc resection(21 cases needed re-excision for removing residual lesions on the upper margin of the tumors). In 34 cases with tumors invading the whole sacrum, 19 cases received total en bloc sacrectomy, while piecemeal resection was performed on the remaining cases. 19 cases(9.8%) had metastasis, including lung metastases in 10 patients, bone metastasis in 6 cases and liver metastasis in 3 cases. In 151 cases undergoing their first operations in our department, 57 cases(37.7%) experienced local recurrence. Postoperative progressive free survival rate was 45.1%, 5-year overall survival rate was 87.7%. ②There were 141 sacral giant cell tumor patients, representing 17.8% of all primary sacral tumors. There were 69 males and 72 females with an average age of 34.2 years(16-61 years), 132 cases underwent intraleisional curettage, 3 cases underwent total en bloc sacrectomy. In 6 cases with huge metastases and unavailable for surgery received multiple embolization. 25 cases(18.9%) were noted local recurrence, which included 12 cases without abdominal aorta occlusion. ③There were 150 neurogenic tumors, with 131 benign neurogenic tumors(83 neurofibromas and 48 schwannomas) and 19 malignant schwannomas, which accounted for 19% of all primary sacral tumors. Among 131 benign neurogenic tumors, there were 62 males and 69 females with an average age of 42.3 years(17-67 years). All cases experienced marginal excision and postoperative recurrence occurred in 17(12.9%). ④A total of 26 sacral osteosarcoma was enrolled, which accounted for 3.3% of the whole series. There were 15 males and 11 females with an average age of 25.8years(12-58 years), among them, 16 cases accepted neo-adjuvant chemotherapy, while 21 cases underwent postoperative chemotherapy. 13 cases underwent en bloc resection or total sacrectomy while the other 13 cases underwent piecemeal resection. 12 cases（46%） experienced postoperative recurrence. Three-year survival rate was 43.5% and 5-year overall survival rate was 21.8%. ⑤28 Ewing′s sarcomas/PNETs accounted for 3.5% of all primary sacral tumors. There were 13 males and 15 females with an average age of 22.3 years(5-50 years). 21 cases accepted neo-adjuvant chemotherapy while 27 cases received postoperative chemotherapy and radiotherapy. 14 cases underwent en bloc resection or total sacrectomy while 14 cases underwent piecemeal resection. 15 cases(53.6%) were noted postoperative recurrence. Three-year overall survival rate was 39.1% and 5-year overall survival rate was 19.6%. ⑥A total of 49 sacral chondrosarcomas accounted for 6.2% of all cases. There were 26 males and 23 females with an average age of 42.5 years(17-69 years). Among these 49 paients, 29 cases underwent en bloc resection or total sacrectomy while piecemeal resection was performed on the others. 22 cases(44.9%) were noted postoperative recurrence. The overall survival rate at 2 years and 5 years was 58.7% and 47.0%, respectively. The disease free survival rate at 2 years and 5 years was 42.3% and 31.8%, respectively. Conclusions: Chordoma, giant cell tumor, neurogenic tumor, osteosarcoma, Ewing′s sarcoma, chondrosarcoma and myeloma are the most common primary sacral tumors. These tumors vary in age, sex ratio, location and growth pattern. The resection margin is determined by pathological diagnosis. The local recurrence rate of sacral chordoma after en bloc resection is much lower than that of those undergoing intralesional surgery. The challenges in sacral giant cell tumor surgery are massive intraoperative bleeding and high local recurrence rate. Consequently, a vital measurement to lowering recurrence rate is to control intraoperative bleeding. High grade malignant tumors, such as osteosarcoma and Ewing′s sarcoma, are of very high tendency to recur locally after marginal or intralesional resection. Thus, en bloc resection with wide margin is necessary for these tumors.