Devaney K, Abbondanzo S L, Shekitka K M, Wolov R B, Sweet D E
Department of Pathology, Brown University, Rhode Island Hospital, Providence, USA.
Clin Orthop Relat Res. 1995 Jan(310):176-87.
The diagnosis of Ewing's sarcoma has been based classically in large part on the exclusion of other similar small round-cell tumors by light microscopic and histochemical criteria. This study was undertaken to explore the use of a recently developed immunohistochemical stain directed against the glycoprotein p30/32MIC2 antigen (the gene product of MIC2), as a diagnostic tool and as a probe for the examination of potential interrelationships among the putative members of the family of peripheral primitive neuroectodermal tumors. Fifty-six small round-cell tumors of bone were selected for study from the files of the Armed Forces Institute of Pathology and Rhode Island Hospital; all tissues had been formalin fixed and paraffin embedded. Nine of 10 Ewing's sarcomas were MIC2 positive, as were 2 of 3 atypical Ewing's sarcomas (small round-cell tumors that diverged from the classic pattern of Ewing's sarcoma by exhibiting a greater degree of cytologic atypia and pleomorphism), and 7 of 8 Askin tumors of the thoracopulmonary region. Ten of 11 mesenchymal chondrosarcomas, 1 primitive neuroectodermal tumor of bone, 10 small cell osteosarcomas, 10 malignant lymphomas, and 3 sarcomas of bone (not additionally subclassified) were negative. The finding of MIC2 positivity in the majority of Ewing's sarcomas and Askin tumors provides additional support for earlier proposals (based on a shared cytogenetic abnormality, among other criteria) that these lesions be considered members of the same family, the peripheral primitive neuroectodermal tumors. The present study, drawing on archival and current case material (including decalcified and undecalcified specimens), indicates that neither the specimen age nor the application of any of a variety of decalcification solutions appears to adversely influence MIC2 staining of paraffin-embedded tissues. This suggests that this antibody has use in retrospective and prospective studies. The rare occurrence of false negative (in the case of Ewing's sarcoma) and positive results in tumors other than peripheral primitive neuroectodermal tumors (as in 1 of the mesenchymal chondrosarcomas) suggests that MIC2 staining should not be relied on as the sole criterion for identification or exclusion of Ewing's sarcomas and related tumors.
尤因肉瘤的诊断传统上很大程度上基于通过光学显微镜和组织化学标准排除其他类似的小圆形细胞肿瘤。本研究旨在探索一种最近开发的针对糖蛋白p30/32MIC2抗原（MIC2的基因产物）的免疫组织化学染色方法，将其作为一种诊断工具，并作为一种探针来研究外周原始神经外胚层肿瘤家族假定成员之间潜在的相互关系。从武装部队病理研究所和罗德岛医院的档案中选取了56例骨小圆形细胞肿瘤进行研究；所有组织均经福尔马林固定和石蜡包埋。10例尤因肉瘤中有9例MIC2呈阳性，3例非典型尤因肉瘤（通过表现出更大程度的细胞异型性和多形性而偏离经典尤因肉瘤模式的小圆形细胞肿瘤）中有2例呈阳性，8例胸肺区域的Askin肿瘤中有7例呈阳性。11例间叶性软骨肉瘤中的10例、1例骨原始神经外胚层肿瘤、10例小细胞骨肉瘤、10例恶性淋巴瘤和3例骨肉瘤（未进一步分类）均为阴性。大多数尤因肉瘤和Askin肿瘤中发现MIC2阳性，为早期提议（基于共享的细胞遗传学异常等标准）提供了额外支持，即这些病变应被视为同一家族——外周原始神经外胚层肿瘤的成员。本研究利用存档和当前病例材料（包括脱钙和未脱钙标本）表明，标本年龄以及各种脱钙溶液的应用似乎均未对外周原始神经外胚层肿瘤石蜡包埋组织的MIC2染色产生不利影响。这表明该抗体可用于回顾性和前瞻性研究。尤因肉瘤出现假阴性（罕见）以及外周原始神经外胚层肿瘤以外的肿瘤出现阳性结果（如1例间叶性软骨肉瘤）表明，不应仅依靠MIC2染色作为识别或排除尤因肉瘤及相关肿瘤的唯一标准。
