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表现为头皮结节的恶性原始神经外胚层肿瘤。

Malignant primitive neuroectodermal tumor presenting as a scalp nodule.

作者信息

Jacinto C M, Grant-Kels J M, Knibbs D R, Daman L A, Piorkowski R J

机构信息

Department of Medicine, Hartford Hospital, Connecticut.

出版信息

Am J Dermatopathol. 1991 Feb;13(1):63-70. doi: 10.1097/00000372-199102000-00011.

Abstract

We report a case of a 20-year-old woman who presented with a 3-year history of a stable cystic nodule on the scalp. Light microscopy of the excised nodule demonstrated a malignant small round cell undifferentiated neoplasm. Immunohistochemical studies suggested a neural crest origin, while ultrastructural examination revealed characteristics of schwannian differentiation. Both of these special techniques were essential in establishing the diagnosis of a malignant epithelioid schwannoma of superficial tissue (neurotropic melanoma, desmoplastic melanoma). We discuss the differential diagnoses and describe the immunohistochemical and ultrastructural characteristics of this tumor. This case illustrates the difficulty of diagnosing this rare malignant tumor, which masqueraded as a benign-appearing scalp lesion.

摘要

我们报告一例20岁女性,其头皮出现一个稳定的囊性结节,病史3年。切除结节的光镜检查显示为恶性小圆形细胞未分化肿瘤。免疫组化研究提示神经嵴起源,而超微结构检查显示有施万细胞分化特征。这两种特殊技术对于确诊浅表组织恶性上皮样施万瘤(亲神经性黑色素瘤、促纤维增生性黑色素瘤)均至关重要。我们讨论了鉴别诊断,并描述了该肿瘤的免疫组化和超微结构特征。该病例说明了诊断这种罕见恶性肿瘤的困难,它伪装成一个看似良性的头皮病变。

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