PIGMENTED DERMATOFIBROSARCOMA PROTUBERANS (BEDNAR TUMOR) - A PATHOLOGIC, ULTRASTRUCTURAL, AND IMMUNOHISTOCHEMICAL STUDY
PIGMENTED DERMATOFIBROSARCOMA PROTUBERANS (BEDNAR TUMOR) - A PATHOLOGIC, ULTRASTRUCTURAL, AND IMMUNOHISTOCHEMICAL STUDY
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DOI：
10.1097/00000478-198509000-00002
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发表时间：
1985-01-01
影响因子：
5.6
通讯作者：
WEISS, SW
中科院分区：
文献类型：
--
作者：
DUPREE, WB;LANGLOSS, JM;WEISS, SW
色素性隆起皮肤纤维肉瘤是一种罕见的肿瘤，约占所有隆起皮肤纤维肉瘤(DFSP)病例的1-5%，被Bednar描述为“花冠状神经纤维瘤”。皮损通常表现为真皮或皮下组织的外生性多结节肿瘤。它主要发生在黑人身上。大多数分布在躯干上，其余的大致平均分布在上肢和下肢以及头部和颈部。显微镜下，病变的特点是梭形细胞排列成紧密的条状结构，并夹杂着少量含有黑色素的树突状细胞。树突状细胞是本病区别于传统DFSP的主要特征。通过电子显微镜可以辨认出三种细胞群。大多数细胞类似于成纤维细胞。第二组细胞呈细长的细胞突起，部分或全部被基板包裹。第三类细胞也由基底膜包裹，包含黑素小体和前黑素小体。这种肿瘤的组织起源仍然存在争议。尽管Bednar认为这些病变是神经纤维瘤的变种，但无法识别S-100蛋白，这一发现与对传统神经纤维瘤的描述形成了鲜明对比，传统神经纤维瘤几乎总是含有这种抗原。在9个病例中获得的随访信息表明，这种损害可能在局部复发。虽然在我们的材料中没有观察到明显的转移，但对于这种可能具有中等恶性潜能的肿瘤，建议完全切除并密切跟踪治疗。
Described by Bednar as a "storiform neurofibroma," pigmented dermatofibrosarcoma protuberans is a rare neoplasm accounting for approximately 1-5% of all cases of dermatofibrosarcoma protuberans (DFSP). The lesion commonly presents as an exophytic, multinodular neoplasm of the dermis or subcutaneous tissue. It occurs predominantly in blacks. The majority are located on the trunk, and the remainder are more or less equally distributed in the upper and the lower extremities and the head and neck. Microscopically the lesion is characterized by spindled cells arranged in a tight storiform pattern and admixed wth a small population of melanin-containing dendritic cells. The dendritic cells are the primary feature distinguishing this lesion from conventional DFSP. Three cell populations are identifiable by electron microscopy. The majority of cells resemble fibroblasts. A second population of cells exhibits long slender cell processes partially or completely invested by basal lamina. The third population of cells, also invested by basal lamina, contains both melanosomes and premelanosomes. The histogenesis of this neoplasm remains controversial. Although Bednar considered these lesions as variants of neurofibroma, S-100 protein could not be identified, and this finding contrasts significantly from the description of conventional neurofibroma, which almost always contain this antigen. Follow-up information available in nine cases indicates that this lesion may recur locally. Although distinct metastases were not observed in our material, complete excision in conjunction with close follow-up care is indicated for this neoplasm of probable intermediate malignant potential.