Department of Dermatology, Venereology and Andrology, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Indian J Dermatol Venereol Leprol. 2011 Sep-Oct;77(5):594-6. doi: 10.4103/0378-6323.84068.
Plexiform schwannoma is a rare neurogenic tumor, arising from skin and subcutaneous tissue. The presence of multiple schwannomas suggests a possible association with neurofibromatosis type 2 (NF2). A 50-year old male patient presented with multiple papulo-nodular cutaneous lesions on both arms and forearms. Histopathological examination revealed a dermal multinodular pattern of well-circumscribed masses of closely packed cells, with peripheral myxoid tissue, well-encapsulated in a thin collagenous capsule. S-100 immunohistochemical staining was diffusely and strongly positive. Neuron-specific enolase was positive, confirming a neural tissue tumor. An audiogram and Magnetic Resonance Imaging (MRI) of cerebro-pontine angle showed no detected abnormality, excluding acoustic neuroma. Thus, we present a case of multiple bilateral isolated cutaneous plexiform schwannomas, not associated with NF2. Multiple plexiform schwannomas is a very rare entity, distinct from neurofibromatosis (NF), and being confined to the dermis is even more rarely reported.
丛状神经鞘瘤是一种罕见的神经源性肿瘤，起源于皮肤和皮下组织。多发性神经鞘瘤的存在提示可能与神经纤维瘤病 2 型（NF2）有关。一位 50 岁男性患者双侧手臂和前臂出现多发性丘疹性结节性皮肤病变。组织病理学检查显示真皮多结节模式，由紧密排列的细胞组成的界限清楚的肿块，外周黏液样组织，被薄的胶原囊包裹。S-100 免疫组化染色弥漫且强阳性。神经元特异性烯醇化酶阳性，证实为神经组织肿瘤。听阈和桥脑小脑角磁共振成像（MRI）显示无异常，排除听神经瘤。因此，我们报告一例双侧多发性孤立性皮肤丛状神经鞘瘤，不伴 NF2。多发性丛状神经鞘瘤非常罕见，与神经纤维瘤病（NF）不同，而且局限于真皮的更为罕见。
