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背景与目的:髓内高分化骨肉瘤发病率低,临床影像及病理检查均易误诊,而因误诊导致的不当治疗对患者预后影响极大。本文旨在探讨髓内高分化骨肉瘤的临床、影像及病理学特点,随访观察其临床规律。方法:收集2000年1月—2012年6月在河北医科大学第三医院就诊并最终经组织学诊断为髓内高分化骨肉瘤的11例病例,另外2例为26年前确诊的病例。回顾性分析该组13例髓内高分化骨肉瘤的临床、影像及病理学资料,并随访患者的预后。随访时间2例为26年,11例为6～101个月,平均37.5个月。结果:13例中男性10例,女性3例。初诊年龄19～52岁,平均32岁。12例病变位于长骨,1例位于髋臼Ⅱ区。除2例病变相对局限,其他病变范围广泛,长骨病变全部涉及干骺端区,可向骨干或骨端延伸。其影像表现虽有一些规律,但缺乏特征性,总体发展缓慢,部分有恶性的征象。该组病变多边界不清,全部有骨皮质破坏、中断,粗大骨嵴常见,骨膜反应和软组织肿块较少见。组织学主要为梭形细胞成分,仅有轻度异型。随访发现,采用截肢及段切治疗者均无复发及转移;采用病灶搔刮治疗者4例,随访过程中3例死亡,其中2例死于肿瘤复发、转移。结论:髓内高分化骨肉瘤好发于长骨干骺端,以股骨远端最为多见。影像表现缺乏特征性。组织学诊断要密切结合临床及影像。病变切除完全者预后良好,病灶刮除或切除不彻底将导致反复复发,并促其转化为高度恶性肿瘤。
BACKGROUND & OBJECTIVE: The incidence of intramedullary well-differentiated osteosarcoma is low. Misdiagnosis of clinical imaging and pathological examination is easily misdiagnosed. Improper treatment due to misdiagnosis has a great impact on the prognosis of patients. This article aims to explore the clinical, imaging and pathological features of intramedullary well-differentiated osteosarcoma, followed up to observe its clinical rules. Methods: From January 2000 to June 2012, 11 cases of advanced intramedullary osteosarcoma were enrolled in the Third Hospital of Hebei Medical University. The other 2 cases were diagnosed 26 years ago. Retrospective analysis of the group of 13 cases of intramedullary well-differentiated osteosarcoma clinical, imaging and pathological data, and follow-up of patients with prognosis. Follow-up time in 2 cases was 26 years, 11 cases were 6 to 101 months, an average of 37.5 months. Results: There were 10 males and 3 females in 13 cases. The first diagnosis of age 19 ~ 52 years old, average 32 years old. Twelve lesions were located in the long bones and one in the acetabular area. In addition to 2 cases of relatively limited disease, other lesions in a wide range of long bone lesions all involve the metaphysis area, can extend to the backbone or bone ends. Although its image performance some laws, but the lack of characteristic, the overall slow development, some of the signs of malignancy. The group of diseased polyborms, all with cortical destruction, disruption, common coarse bone crest, periosteal reaction and soft tissue mass is rare. Histology mainly spindle cell components, only mild atypia. Follow-up found no recurrence and metastasis with amputation and segment cutting. Four patients were treated with lesion scraping and 3 died during the follow-up. Two of them died of tumor recurrence and metastasis. Conclusion: Intramedullary well-differentiated osteosarcoma occurs mainly in the metaphysis of long bones and is the most common distal femur. Image performance is lacking in character. Histological diagnosis should be closely combined with clinical and imaging. Complete excision of the prognosis of a good prognosis, curettage or resection of the lesion will not lead to repeated recurrence and promote its transformation into a highly malignant tumor.