Malcolm Elsa K, Lopes M Beatriz S
Division of Neuropathology, University of Virginia Health Sciences Center, Charlottesville, USA.
Brain Pathol. 2002 Oct;12(4):515-6, 521. doi: 10.1111/j.1750-3639.2002.tb00470.x.
The April 2002 Case of the Month (COM). 35-year-old healthy man developed a mass in the right parotid gland. A superifical parotidectomy was performed for a 4.5 x 1.5 x 1.5 cm mass involving the intraparotid facial nerve. Grossly the tumor was multinodular, smooth and yellow with normal surrounding salivary gland. Microscopically, the tumor showed expanding nodules composed of proliferating fibroblasts, Schwann cells, and perineural-like cells in a myxoid stroma. Normal peripheral nerve twigs were identified in the periphery of the tumor. There was no increased mitotic activity, cellularity or nuclear pleomorphism. S-100 immunohistochemical stain was positive. The tumor was diagnosed as a solitary plexiform neurofibroma. Plexiform neurofibromas in this area have been described in children with von Recklinghausen's disease or neurofibromatosis 1 (NF 1). Plexiform neurofibromas typically involve deep seated nerve trunks and is considered pathognomonic for NF 1. This unusual case represents a solitary variant of plexiform neurofibroma presenting as a parotid mass in an adult patient without a personal stigmata or family history of NF 1.
2002年4月的月度病例(COM)。一名35岁健康男性右侧腮腺出现肿物。对一个大小为4.5×1.5×1.5厘米、累及腮腺内面神经的肿物进行了浅叶腮腺切除术。大体上,肿瘤呈多结节状,表面光滑,黄色,周围唾液腺正常。显微镜下,肿瘤表现为扩张的结节,由增殖的成纤维细胞、施万细胞和类神经束膜细胞组成,位于黏液样基质中。在肿瘤周边发现了正常的外周神经分支。未见有丝分裂活性增加、细胞增多或核异型性。S-100免疫组化染色呈阳性。该肿瘤被诊断为孤立性丛状神经纤维瘤。该区域的丛状神经纤维瘤已在患有冯·雷克林豪森病或神经纤维瘤病1型(NF 1)的儿童中有所描述。丛状神经纤维瘤通常累及深部神经干,被认为是NF 1的特征性表现。这个不寻常的病例代表了丛状神经纤维瘤的一种孤立变异型,表现为成年患者的腮腺肿物,且该患者无NF 1的个人特征或家族史。