d'Amore E S, Ninfo V
Istituto di Anatomia Patologica dell' Universitá di Padua, Italy.
Semin Diagn Pathol. 1996 Aug;13(3):184-203.
Soft tissue small round cell tumors (SRCTs) comprise a heterogeneous group of neoplasms that predominate in childhood and adolescence and share similar morphological features, consisting of dense cellular proliferation of small round cells with a primitive appearance. Rhabdomyosarcomas, peripheral neuroepitheliomas, Ewing's sarcomas, and lymphomas/leukemias are the prototypic SRCT; other recently described tumors that should be added to the list are the desmoplastic SRCT and the rhabdoid tumor of soft tissues. In addition, several other primary soft tissue neoplasms and metastatic tumors have occasionally been considered in the differential diagnosis of SRCT. The precise identification of a given SRCT is important because of its clinical relevance. However, it may be difficult because the diagnostic criteria are sometimes subtle and several histologic and immunohistochemical features are not specific and/or may be simulated by different tumor types. We discuss the morphological clues that in our opinion are most useful for their diagnosis, the criteria for distinguishing between peripheral neuroepithelioma and Ewing's sarcoma, and the main diagnostic pitfalls.
软组织小圆细胞肿瘤（SRCTs）是一组异质性肿瘤，在儿童和青少年中占主导地位，具有相似的形态学特征，由外观原始的小圆形细胞密集增殖组成。横纹肌肉瘤、外周神经上皮瘤、尤因肉瘤和淋巴瘤/白血病是典型的SRCT；其他最近描述的应列入该列表的肿瘤是促纤维组织增生性SRCT和软组织横纹肌样瘤。此外，在SRCT的鉴别诊断中偶尔也会考虑其他几种原发性软组织肿瘤和转移性肿瘤。鉴于其临床相关性，准确识别特定的SRCT很重要。然而，这可能很困难，因为诊断标准有时很细微，而且一些组织学和免疫组化特征不具有特异性和/或可能被不同的肿瘤类型模拟。我们讨论了我们认为对其诊断最有用的形态学线索、区分外周神经上皮瘤和尤因肉瘤的标准以及主要的诊断陷阱。
