Bruner J M
Section of Neuropathology, University of Texas System Cancer Center, M. D. Anderson Hospital and Tumor Institute, Houston 77030.
Semin Diagn Pathol. 1987 May;4(2):136-49.
The head and neck region is a common location for benign peripheral nerve sheath tumors (PNST) and a rare site for malignant PNST. The diagnostic distinction between schwannoma (neurilemmoma) and benign neurofibroma remains clinically and prognostically important. Most benign PNST are schwannomas, and these do not have a recognized malignant potential. Malignant PNST arise de novo or from benign neurofibromas. The definitive diagnosis of malignant PNST may be difficult or impossible using only routine light microscopy. Electron microscopy and immunohistochemistry are special techniques that may be helpful. The prognosis in patients with malignant PNST depends heavily on the extent of surgical excision, size of the primary tumor, and presence or absence of von Recklinghausen's neurofibromatosis.
头颈部是良性周围神经鞘瘤（PNST）的常见发生部位，而恶性PNST则较为罕见。神经鞘瘤（神经膜瘤）与良性神经纤维瘤之间的诊断区分在临床和预后方面都很重要。大多数良性PNST是神经鞘瘤，这些肿瘤没有公认的恶变潜能。恶性PNST可原发产生或由良性神经纤维瘤恶变而来。仅使用常规光学显微镜可能难以或无法确诊恶性PNST。电子显微镜检查和免疫组织化学是可能有帮助的特殊技术。恶性PNST患者的预后在很大程度上取决于手术切除范围、原发肿瘤大小以及是否存在冯雷克林霍增氏神经纤维瘤病。
