骨旁骨肉瘤23例临床病理分析

Parosteal Osteosarcoma: A Clinicopathological Analysis of 23 Cases

目的 探讨骨旁骨肉瘤的临床病理学特点 ,侧重于诊断和鉴别诊断。方法 回顾性分析 2 3例骨旁骨肉瘤的临床资料、影像学表现、组织学形态及随访结果。结果 男 13例 ,女 10例 ,年龄范围 14～ 5 2岁 ,平均 30岁。临床多表现为下肢肿胀和 /或缓慢性生长的肿块。X线显示为附着于干骺端骨皮质表面生长的广基性蘑菇状致密肿块影 ,部分病例于肿块和其下骨皮质间可见狭窄的透亮带 ,CT证实 6例伴有髓内累及 (2 6 % )。部位以股骨下端后方最多见 (6 5 2 % ) ,其次为胫骨上段 (17 4% )。组织学主要由相对成熟的骨组织和镶嵌于骨组织之间的纤维母细胞样细胞所组成 ,细胞间常伴有明显的胶原化。瘤细胞的异型性不明显 ,大部分为Broder’sⅠ级 ,少数为Ⅱ级。 15例随访结果显示 ,10例局切后复发 (6 6 7% ) ,2例出现肺部转移 (13% ) ,其中 1例于多次局部复发后发生去分化。结论 骨旁骨肉瘤是一种低度恶性的骨肉瘤 ,特征性的X线表现有诊断意义 。

Objective To study the clinical and pathological features of parosteal osteosarcoma, with emphasis on its diagnosis, differential diagnosis and treatment. Method The clinical, radiographic and histopathologic features of 23 cases of the tumor were analyzed. Results There were 13 female and 10 male, with age ranged from 14 to 52 years (mean, 30 years). Clinically, most of the patients complained of swelling or slowly growing mass in the lower extremities. X ray demonstrated a large dense mushroom shaped mass shadow attached to the bone cortex, with a clear lucent zone between the mass and underlying cortex in some cases. CT scanning confirmed the medullary involvement in 6 cases (26%). The tumors occurred most commonly in the distal part of the femur (65.2%), followed by proximal tibia (17.4%). Histologically, the tumor was composed of slender or irregular osseous trabeculae in a relatively mature phase and proliferation of fibroblast-like spindle cells, which showed slight atypia (most Broders' I, a few Broders' II). Follow up information revealed local recurrences in 10 cases (66.7%) and lung metastases in 2 cases (13%), with one case underwent dedifferentiation after repeated local recurrences. Conclusions The radiographic characteristics are very valuable in the diagnosis of paroteal osteosarcoma. The tumor should be differentiated from a variety of benign and malignant lesions. Paroteal osteosarcoma is a low-grade tumor, and inadequate excision almost results in recurrence. The treatment should be adjusted according to the size of the tumor, the state of medullary involvement, local recurrence and dedifferentiation.