Gonzalez-Crussi F, Mangkornkanok M, Hsueh W
Am J Surg Pathol. 1987 Jan;11(1):59-65. doi: 10.1097/00000478-198701000-00008.
The present report describes the difficulties attending the establishment of a correct diagnosis in an adolescent patient with a large retroperitoneal tumor. It is known that clinical and pathologic findings may sometimes be insufficient to effect a distinction between germ-cell and lymphomatous origin of a large-celled neoplasm. Our purpose is to demonstrate that these difficulties may persist even when current methods of morphologic study, including electron microscopy, are complemented with the application of many antibodies that are commonly available for immunohistologic use. In the young, the differential diagnosis of large-celled neoplasm must include "rhabdoid" tumor, and the poorly characterized nature of this neoplasm compounds the unusually difficult diagnostic dilemma. Our problem case expressed none of many lymphoid tissue markers used, was positive for epithelial membrane antigen (EMA), and was comprised of so-called "anemone" cells visible by electron microscopy, which appearance may correspond to that of carcinomas or lymphomas. Study of gene rearrangement provided additional evidence that the neoplasm was of lymphomatous origin, by showing rearrangement of genes for immunoglobulins. This valuable technique has an important place in diagnostic surgical pathology.
本报告描述了一名患有巨大腹膜后肿瘤的青少年患者在确立正确诊断时所遇到的困难。众所周知，临床和病理检查结果有时可能不足以区分大细胞肿瘤的生殖细胞起源和淋巴瘤起源。我们的目的是证明，即使在当前的形态学研究方法（包括电子显微镜检查）辅以多种常用于免疫组织学的抗体的情况下，这些困难仍可能存在。对于年轻人，大细胞肿瘤的鉴别诊断必须包括“横纹肌样”肿瘤，而这种肿瘤特征不明确的性质使异常困难的诊断困境更加复杂。我们的疑难病例未表达所使用的多种淋巴组织标志物，上皮膜抗原（EMA）呈阳性，并且由电子显微镜下可见的所谓“海葵”细胞组成，其外观可能与癌或淋巴瘤的外观相符。基因重排研究通过显示免疫球蛋白基因的重排，提供了额外的证据表明该肿瘤起源于淋巴瘤。这项有价值的技术在诊断性外科病理学中具有重要地位。
