13例髓内高分化骨肉瘤的临床、影像及病理学观察

Clinical,radiologic and pathologic features of intramedullary well-differentiated osteosarcoma:a retrospective review of 13 cases

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 and purpose： Intramedullary well-differentiated osteosarcomas are rare and commonly misdiagnosed, both pathologically and radiologically, as a benign lesion. Misdiagnosis can lead to inappropriate treatment, which obviously influence the prognosis of patient. This study aimed to explore the clinical, radiological and pathological characteristics of intramedullary well-differentiated osteosarcoma and follow up their treatment outcomes. Methods： Thirteen cases of intramedullary well-differentiated osteosarcoma confirmed by pathology in Hebei Medical University Third Hospital （11 cases from Jun. 2000 to Jun. 2012 and 2 cases from 26 years ago）, were retrospectively analyzed and the outcomes after surgical treatment were followed up. Follow-up times in 2 cases were more than 26 years, and in 11 cases, the median were 37.5 months, ranged from 6 to 101 months. Results： Twelve cases located in long bone, 1 case located in lI area of acetabula. In long bone lesions, 8 cases were in femur, 6 of them were in the distal end. Ten patients were male and 3 were female. Age ranged from 19 to 52 years, average age was 32. All of lesions were large and extensive, except 2 cases with relative limitation. All of the long bone lesions were involving metaphyseal areas, extending to the diaphysis and （or） epiphysis. The imaging signs of the lesions were lack of characteristics, with a wide range of findings from well-circumscribed to large, poorly marginated lesions with periosteal reactions, cortical destruction, and soft-tissue invasion. In the series, all cases had a cortical break.lntralesional trabeculation is a common finding. Periosteal new bone formation and soft tissue extension were rare. A frequent pattern was a slow-growing large fibro-osseous lesion with varying amounts of ossification associated with focal areas of aggression. Histologically, the tumors were composed of spindle cells with only minimal atypia. After amputations and wide excision of the lesions, neither recurrence nor metastasis occurred. After curettage in 4 cases, 3 cases died during follow-up, in which 2 cases died of tumor recurrence and metastasis. Conclusion： Long bone metaphysis, especially the distal end of the femur is the most common site for intramedullary well-differentiated osteosarcoma. The original histological and radiological diagnosis were easily misdiagnosed, therefore it is vital to combine both radiological and histological findings. Wide excision was almost never followed by recurrence, curettage or marginal excision of lesions can lead to repeated recurrence, curettage may be a stimulating factor to promote the tumors further malignant transformation.