Levy Bencheton A, Mallet S, Rojat Habib M-C, Figarella-Branger D, Sigaudy S, Grob J-J, Richard M-A
Service de dermatologie, hôpital Sainte-Marguerite, université de la Méditerranée Aix Marseille II, 270, boulevard de Sainte-Marguerite, 13009 Marseille, France.
Ann Dermatol Venereol. 2010 Apr;137(4):301-4. doi: 10.1016/j.annder.2010.02.018. Epub 2010 Mar 30.
Plexiform neurofibroma (NFP) is a benign nervous tumour typically involving the head and neck region due to the rich innervation of the latter. It is considered pathognomonic of neurofibromatosis type 1(NF1). This report describes an unusual case of neurofibroma and discusses its singular presentation, namely an isolated cutaneous tumour of late onset and with myofibroblastic histology.
A 85-year-old man presented swelling of the cutaneous part of the lower palpebral region which had been present for several months. The lesion was relapsing after repeated incomplete excisions and had grown slowly to become firm and suspect. It was decided to perform a large excision with a frontal rotation flap. Initial histological examinations performed on each excision suggested fibrosis and scarring, leading to diagnosis of fibrocytic change and post-surgical neuroma. The final histological analysis indicated diffuse plexiform neurofibroma with a myofibroblastic component. This was a solitary lesion in a patient without any stigmata or familial history of NF1.
This case is original in terms of its characteristics: a single cutaneous tumour of late onset in a patient with no stigmata of NF1 (in most cases of plexiform neurofibroma, NF1 is either multiple or else isolated at a mucous site). The histological findings for this tumour with a myofibroblastic component have never previously been described. Plexiform neurofibroma classically involves the head-and-neck region as in our case, with deep invasion of subcutaneous tissues making excision difficult and leading to frequent recurrence.
丛状神经纤维瘤（NFP）是一种良性神经肿瘤，由于头颈部神经分布丰富，该肿瘤通常累及头颈部区域。它被认为是1型神经纤维瘤病（NF1）的特征性表现。本报告描述了一例不寻常的神经纤维瘤病例，并讨论了其独特的表现，即一种迟发性孤立性皮肤肿瘤，组织学表现为肌成纤维细胞。
一名85岁男性患者，下睑区域皮肤肿胀数月。该病变在多次不完全切除后复发，生长缓慢，质地变硬且可疑。决定采用额部旋转皮瓣进行大面积切除。对每次切除标本进行的初步组织学检查提示纤维化和瘢痕形成，诊断为纤维性改变和术后神经瘤。最终组织学分析显示为弥漫性丛状神经纤维瘤，伴有肌成纤维细胞成分。这是一例孤立性病变，患者无任何NF1的体征或家族史。
该病例在特征方面具有独特性：一名无NF1体征的患者出现迟发性单发皮肤肿瘤（在大多数丛状神经纤维瘤病例中，NF1为多发或孤立于黏膜部位）。此前从未描述过这种具有肌成纤维细胞成分肿瘤的组织学表现。如我们的病例所示，丛状神经纤维瘤通常累及头颈部区域，皮下组织深层受侵使得切除困难，且导致频繁复发。
