Extraskeletal osteosarcomas - A clinicopathologic study of 25 cases
Extraskeletal osteosarcomas - A clinicopathologic study of 25 cases
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DOI：
10.1097/00000478-199805000-00010
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发表时间：
1998-05-01
影响因子：
5.6
通讯作者：
Keller, J
中科院分区：
文献类型：
--
作者：
Jensen, ML;Schumacher, B;Keller, J
对1970-1995年在丹麦奥胡斯大学医院骨与软组织肿瘤中心诊断的25例皮肤外骨肉瘤进行了随访研究。使用一组10种抗体对这些肿瘤的免疫组织化学特征进行了评价，并考虑了碱性磷酸酶染色在鉴别诊断中的价值。该研究表明，这种肿瘤是高度恶性的，并且在诊断时影响成年人（中位年龄，67岁;范围，35 - 82岁）。大腿（52%）是最常见的肿瘤位置。7个肿瘤是浅表的，而其余18个是肌内的。两名患有浅表肿瘤的患者先前接受了该区域的放射治疗。9例（36%）患者发生局部复发，15例（60%）患者发生肺部远处转移，这是最常见的部位。中位生存时间为24个月，5年的病因特异性生存率低于25%。13例（52%）肌内型骨肉瘤为成纤维细胞型，常伴少量类骨质。根据碱性磷酸酶强阳性反应可将其与恶性纤维组织细胞瘤区分开来。免疫组化没有显示特征性的特征，因为波形蛋白阳性，偶见结蛋白、肌动蛋白、S-100、上皮膜抗原、细胞角蛋白和p-53阳性，可在许多其他多形性肉瘤中观察到。各种组织病理学因素，如肿瘤大小，肿瘤深度，组织病理学亚型，恶性程度（IIIA与IIIB），MIB-1和p53反应性进行了分析与临床过程。只有MIB增殖与预后相关，MIB-1值小于24%的肿瘤患者的生存期显著延长。我们的研究表明，皮肤外骨肉瘤具有高度侵袭性。碱性磷酸酶染色与免疫组化相比，在与其他多形性肉瘤的鉴别诊断中具有上级优势。
A follow-up investigation of 25 cases of extraskeletal osteosarcomas diagnosed at the Center for Bone and Soft Tissue Tumors, Aarhus University Hospital, Denmark, in the period from 1970-1995 was undertaken. The immunohistochemical profile of these rumors was evaluated using a panel of 10 antibodies, and the value of alkaline phosphatase staining in differential diagnostic situations also was considered. The study revealed that this tumor is high-grade malignant and affects adults (median age, 67 years; range, 35-82 years) at diagnosis. The thigh (52%) was the most common tumor location. Seven tumors were superficial, whereas the remaining 18 were intramuscular. Two patients with superficial tumors previously received radiation to the area. Local recurrences developed in 9 (36%) patients and distant metastases developed in the lungs in 15 (60%) patients as the most common site. Median survival time was 24 months, and the cause-specific survival rate at 5 years was less than 25%. Thirteen (52%) intramuscularly located extraskeletal osteosarcomas were of the fibroblastic subtype, often with sparse amounts of osteoid. They could be separated from malignant fibrous histiocytoma on the basis of a strongly positive alkaline phosphatase reaction. Immunohistochemistry did not reveal characteristic features because positivity for vimentin, occasional positivity for desmin, actin, S-100, epithelial membrane antigen, cytokeratin, and p-53 may be observed in many other pleomorphic sarcomas. Various histopathologic factors, such as tumor size, tumor depth, histopathologic subtype, malignancy grade (IIIA versus IIIB), MIB-1, and p53 reactivity were analyzed in relation to clinical course. Only MIB proliferation was correlated to prognosis, with significantly longer survival in patients with tumors with MIB-1 values less than 24%. Our study has shown extraskeletal osteosarcoma to behave in a highly aggressive fashion. Alkaline phosphatase staining compared with immunohistochemistry proved to be superior in the differentiation from other pleomorphic sarcomas.