骨膜骨肉瘤与高度恶性表面骨肉瘤

摘要

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。%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.