Sangüeza O P, Sangüeza P, Valda L R, Meshul C K, Requena L
Department of Dermatology L468, Oregon Health Sciences University, Portland 97201.
J Am Acad Dermatol. 1994 Aug;31(2 Pt 2):356-61. doi: 10.1016/s0190-9622(94)70172-5.
Primitive neuroectodermal tumors are rare, highly aggressive neoplasms that affect both sexes and occur in all age groups. They are a large group of neoplasms with neuroepithelial differentiation, including cutaneous neuroblastomas. Histopathologically they are characterized by a rather uniform population of small, dark cells, with or without Homer Wright rosettes. Immunohistochemically they stain for one or more of the reputed neural markers. Ultrastructurally they show interdigitating processes containing neurosecretory granules, intermediate filaments, and microtubules. We describe a patient with unusual multiple peripheral neuroectodermal tumors localized to the skin, with benign biologic behavior. The patient related the appearance of new lesions for several years without evidence of visceral dissemination or systemic complications. The histopathologic, immunohistochemical, and ultrastructural findings support the diagnosis of a peripheral neuroectodermal tumor.
原始神经外胚层肿瘤是一种罕见的、侵袭性很强的肿瘤，可发生于任何年龄组，男女均可受累。它们是一大类具有神经上皮分化的肿瘤，包括皮肤神经母细胞瘤。组织病理学上，其特征是由相当一致的小而深色的细胞组成，可有或无霍纳·赖特菊形团。免疫组织化学上，它们对一种或多种公认的神经标志物呈阳性染色。超微结构上，它们显示出含有神经分泌颗粒、中间丝和微管的交错突。我们描述了一位患有罕见的多发性皮肤外周神经外胚层肿瘤的患者，其生物学行为为良性。该患者数年来不断出现新病灶，但无内脏播散或全身并发症的证据。组织病理学、免疫组织化学和超微结构检查结果均支持外周神经外胚层肿瘤的诊断。
