Taungjaruwinai Wirach M, Goldberg Lynne J
Department of Dermatology, Boston University School of Medicine, MA, USA.
J Cutan Pathol. 2009 Nov;36(11):1164-7. doi: 10.1111/j.1600-0560.2009.01249.x.
A 54-year-old African-American male patient underwent removal of several cutaneous neurofibromas. Histopathologic examination revealed a nonencapsulated, haphazardly arranged proliferation of slender spindle cells in a myxoid stroma consistent with neurofibroma. Interestingly, each specimen exhibited numerous, large, multinucleate giant cells with nuclei arranged in a wreath-like or linear pattern. Immunoperoxidase staining revealed these cells to be negative with S-100 and CD-34. It was subsequently learned that this patient has neurofibromatosis type 1 (NF1).
A retrospective review of all lesions coded as neurofibroma from our institution between 1 June 2006 and 28 August 2006 was performed.
Biopsies of 53 cutaneous neurofibromas from 51 patients were reviewed. In these, multinucleate giant cells were present in only three (5.7%), all in patients with single lesions.
Although the incidence of floret-like giant cells in neurofibromas of patients with NF1 is unknown, our findings suggest that the presence of these cells might be a clue to the presence of NF1.
一名54岁的非裔美国男性患者接受了多处皮肤神经纤维瘤切除术。组织病理学检查显示，在黏液样基质中存在一种无包膜、排列杂乱的细长梭形细胞增殖，符合神经纤维瘤的表现。有趣的是，每个标本中都有大量大的多核巨细胞，其细胞核呈花环样或线性排列。免疫过氧化物酶染色显示这些细胞S-100和CD-34均为阴性。随后得知该患者患有1型神经纤维瘤病（NF1）。
对2006年6月1日至2006年8月28日期间我院编码为神经纤维瘤的所有病变进行回顾性研究。
对51例患者的53处皮肤神经纤维瘤活检标本进行了复查。其中，仅3例（5.7%）出现多核巨细胞，且均为单发病变患者。
虽然NF1患者神经纤维瘤中花环状巨细胞的发生率尚不清楚，但我们的研究结果表明，这些细胞的存在可能是NF1存在的一个线索。
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