Peydró-Olaya Amando, Llombart-Bosch Antonio, Carda-Batalla Carmen, Lopez-Guerrero Jose Antonio
Department of Pathology, Medical School and Hospital Clinico, University of Valencia, Valencia, Spain.
Semin Diagn Pathol. 2003 Feb;20(1):25-45.
The current study disusses a new approach to the group of small round cell tumors (SRCTs) independently of their primary anatomical location. We perform this analysis supported mainly by morphological means and particularly with the help of immunohistochemistry and electron microscopy; the last of which continues to play a decisive role in their differential diagnosis. The microscopical similarity of many of these tumors often makes the diagnosis in routine histology extremely difficult, due to the varying degree of heterogeneity present, and may have important therapeutic and prognostic implications. Thus a correct final diagnosis is mandatory for the clinic. Within the group of tumors that express a dominant or occasional small round cell pattern "SRCT" (neoplasms of the Central Nervous System excluded) are included: Ewing's sarcoma and peripheral neuroectodermal tumor (Es/pPNET) comprising its varieties, neuroblastoma, desmoplastic small round cell tumor, rhabdomyosarcoma, alveolar, solid and embryonal, small cell osteosarcoma, chondrosarcoma, myxoid and mesenchymal, round cell and myxoid liposarcoma, synovial sarcoma (monophasic undiffentiated), primitive malignant peripheral nerve sheath tumor (malignant small cell schwannoma), malignant non-Hogdkin lymphoma, Merkel cell tumor of the skin (small cell carcinoma including neuroendocrine carcinoma). This study discusses in each case not only the histology, supported by immunohistochemistry, but also the main ultrastructural characteristics. We are conscious that in some cases further cytogenetic or molecular biology support may be necessary, when considering the limits of morphology today. Thus, short references on molecular genetics, complementing the structural findings, are given.
本研究探讨了一种针对小圆形细胞肿瘤（SRCTs）的新方法，而不考虑其原发解剖位置。我们主要通过形态学方法进行此项分析，特别是借助免疫组织化学和电子显微镜；其中电子显微镜在其鉴别诊断中继续发挥决定性作用。由于存在不同程度的异质性，这些肿瘤中有许多在显微镜下的相似性常常使得常规组织学诊断极为困难，并且可能具有重要的治疗和预后意义。因此，正确的最终诊断对临床来说是必不可少的。在表现为主要或偶尔小圆形细胞模式的“SRCT”肿瘤组（不包括中枢神经系统肿瘤）中包括：尤因肉瘤和外周神经外胚层肿瘤（Es/pPNET）及其变种、神经母细胞瘤、促结缔组织增生性小圆形细胞肿瘤、横纹肌肉瘤（肺泡型、实体型和胚胎型）、小细胞骨肉瘤、软骨肉瘤（黏液样和间叶型）、圆形细胞和黏液样脂肪肉瘤、滑膜肉瘤（单相未分化型）、原始恶性外周神经鞘瘤（恶性小细胞神经鞘瘤）、恶性非霍奇金淋巴瘤、皮肤默克尔细胞肿瘤（包括神经内分泌癌的小细胞癌）。本研究在每种情况下不仅讨论了免疫组织化学支持下的组织学，还讨论了主要的超微结构特征。我们意识到，考虑到当今形态学的局限性，在某些情况下可能需要进一步的细胞遗传学或分子生物学支持。因此，给出了关于分子遗传学的简短参考文献，以补充结构研究结果。
