长骨造釉细胞瘤的手术治疗及预后16例分析

目的分析长骨造釉细胞瘤的手术治疗效果及预后。方法回顾分析我科1999年11月至2014年3月,收治的随访5年以上的长骨造釉细胞瘤患者,分析其手术治疗方法及预后。结果 16例入选,男9例,女7例,年龄14~42岁,中位年龄21.5岁。病理诊断11例为经典型造釉细胞瘤,5例诊断为骨性纤维结构不良(osteofibrous dysplasia,OFD)样造釉细胞瘤。经典型造釉细胞瘤中9例行瘤段切除术,1例行皮质切除术,1例因术前穿刺为OFD而行刮除术。OFD样造釉细胞瘤中3例行瘤段截除术,2例行刮除术。术后随访65~184个月,中位随访109个月。局部复发4例,总复发率为25%(4/16),其中经典型造釉细胞瘤复发3例,复发率为27.3%(3/11),OFD样造釉细胞瘤复发1例,复发率为20%(1/5)。发生肺转移2例,总转移率为12.5%(2/16),均为经典型造釉细胞瘤。2例经典型造釉细胞瘤死于肺转移,OFD样造釉细胞瘤均存活,11例经典型造釉细胞瘤的5年生存率为100%,10年生存率为88.9%,15年生存率为71.1%。结论长骨造釉细胞瘤是一种罕见的原发骨肿瘤,须长期随访,经典型造釉细胞瘤表现出低度恶性特征,需要手术广泛切除,OFD样造釉细胞瘤表现出中间性肿瘤的特点,扩大刮除能得到很好的局部控制。

Surgical treatment and prognosis of adamantinoma of long bones

Objective To detect surgical treatment methods and prognosis of admantinoma (AD) of long bones.Methods We retrospectively reviewed adamantinoma of long bones between November 1999 and March 2014 with at least 5 years’ follow-up,and analyzed the surgical treatment and prognosis.Results This study enrolled 16 cases including 9 males and 7 females.The mean age was 21.5 years (range:14-42 years).Eleven patients with classic AD and five with osteofibrous dysplasia-like (OFD-like) AD were analyzed.Nine patients underwent segmental resection,1 patient underwent cortical resection and 1 patient underwent curettage due to the biopsy indicating the OFD-like AD among classic AD patients.Three patients underwent segmental resection and 2 patients underwent curettage among OFD-like AD patients.All patients were followed up for 65-184 months with a mean of 109 months.Local recurrence (LR) occurred in 4 cases with the total LR rate of 25% (4/16),including 3 classic ADs (27.3%,3/11) and 1 OFD-like AD (20%,1/5).Lung metastasis occurred in 2 classic AD cases with the metastasis rate of 12.5% (2/16).Two classic ADs died of the disease and all the OFD-like ADs survived.The 5-year,10-year,15-year survival rates of classic AD were 100%,88.9% and 71.1%,respectively.Conclusions Adamantinoma,as a rare primary bone tumor,needs long-time follow-up.Radical surgery with wide margin is recommended for classic admantinoma with low malignancy,while extensive curettage is advised for OFD-like admantinoma by its intermediate tumor features.