骨膜骨肉瘤与高度恶性表面骨肉瘤

目的报告表面骨肉瘤的两种少见亚型，即骨膜骨肉瘤和高度恶性表面骨肉瘤的诊治经验。方法回顾 5例骨膜骨肉瘤和 4例高度恶性表面骨肉瘤患者的影像学和病理学特点，并对临床治疗结果进行分析。 5例骨膜骨肉瘤患者，男 1例，女 4例；年龄 28～ 42岁，平均 35岁；肿瘤位于胫骨上段者 4例，股骨下段者 1例。 4例高度恶性表面骨肉瘤患者，男 3例，女 1例；年龄 17～ 23岁，平均 19.25岁； 4例患者肿瘤均位于股骨下段。结果 5例骨膜骨肉瘤均予以广泛切除，除 1例因局部复发行截肢术，现无瘤生存 1年 3个月外，余 4例已无瘤生存 3～ 9年，平均 5年 9个月。 4例高度恶性表面骨肉瘤患者，虽经积极综合治疗，但仅 1例无瘤生存 7年； 1例术后 2年 5个月复发而截肢，现无瘤生存 1年 9个月； 1例于术后 2年 4个月死于肺转移；另 1例在确诊后 3个月死亡。结论骨膜骨肉瘤和高度恶性表面骨肉瘤各具鲜明的影像学和病理学特点，骨膜骨肉瘤恶性程度较低，应采用以局部广泛切除为主的手术治疗，预后相对较好；而高度恶性表面骨肉瘤的生物学行为则与经典的髓内骨肉瘤相似，预后较差，必须采用手术与化疗相结合的综合治疗方法。

Periosteal osteosarcoma and high grade surface osteosarcoma

Objective To report the clinical experience of treatment for periosteal osteosarcoma (PERI) and high grade surface osteosarcoma (HGS), which are two rare subtypes of osteosarcoma arising from the surface of bone. Methods The clinical, radiographic and histologic features of 5 cases with PERI and 4 cases with HGS were reviewed. The ages of the 5 patients with PERI(1 male and 4 females) ranged from 28 to 42 years (mean 35 years). Four of five patients with PERI had involvement of the upper tibial shaft, the other one had the lesion situated on the lower femur. Of the 4 cases with HGS, 3 were male and 1 was female. The average age of the 4 patients with HGS was 19.25 years (ranged from 17 to 23 years). All cases with HGS had involvement over the distal femoral shaft. Results All patients with PERI received wide resection, of which four patients remained continuously disease free(ranged from 3 to 9 years, mean 5 years and 9 months). The remaining one has remained disease free for one year and three months after secondary amputation for local recurrence. Of the three patients with HGS who had received wide excision and effective chemotherapy initially, one survived for 1 year and 9 months after amputation for recurrence, one died with pulmonary metastasis in 2 years and 4 months after operation, and one had tumor free survival for 7 years. The remaining one died in three months after diagnostic biopsy. Conclusion PERI is a less aggressive tumor than conventional osteosarcoma. Simple wide resection is the treatment of choice. HGS is a rare subtype of surface osteosarcoma with a prognosis similar to that of conventional osteosarcoma. Wide excision and effective systemic chemotherapy are associated with better clinical results.