Kempf H G, Becker G, Weber B P, Ruck P, Lenarz T
Department of Otolaryngology, Medizinische Hochschule Hannover, Germany.
ORL J Otorhinolaryngol Relat Spec. 1995 Sep-Oct;57(5):273-8. doi: 10.1159/000276757.
A retrospective analysis of diagnostic procedures and clinical outcome of patients with neurogenic tumours of the head and neck region was performed. There were 25 patients - 16 with neurinoma, 5 with neurofibroma and 4 with neurogenic sarcoma - who presented with a mass in the head and neck area. In 13 patients, the neurinoma originated from the facial nerve, 2 of them were located in the middle ear and mastoid and showed facial palsy. Four patients presented with neurofibromatosis type 1. Twenty-three patients were operated on to remove the tumorous masses. Eighteen tumours were assigned intraoperatively to a cranial nerve main trunk, whereas 7 tumours originated from small branches or showed diffuse growth in neurofibromatosis. All benign neurogenic tumours were removed totally without relapse. One of 2 auricular nerve autografts in middle-ear facial nerve neurinoma resulted in a sufficient muscle tonus of the face. Three patients with malignancies died within 12-24 months showing local recurrencies (n = 1) and/or pulmonary metastasis (n = 2). As expected, neurogenic tumours are unusual neoplasms of the head and neck regions. On the other hand, neurogenic malignancies exhibit diagnostic problems as well as uncertain chances for long-term survival. Tumour staging, determined by extension and critical for treatment decisions, is well evaluated by CT scan and MRI. A multimodal therapy regimen with operation and radiation is recommended for neurogenic sarcoma also when developing in neurofibromatosis. However, in main trunk neurinomas, nerve autografting with interposition is the therapy of choice with a good chance of functional recuperation.
对头部和颈部区域神经源性肿瘤患者的诊断程序和临床结果进行了回顾性分析。共有25例患者，其中16例为神经鞘瘤，5例为神经纤维瘤，4例为神经源性肉瘤，均表现为头颈部肿块。13例神经鞘瘤起源于面神经，其中2例位于中耳和乳突并伴有面瘫。4例患者患有1型神经纤维瘤病。23例患者接受了肿瘤肿块切除术。术中18个肿瘤被确定起源于颅神经主干，而7个肿瘤起源于小分支或在神经纤维瘤病中呈弥漫性生长。所有良性神经源性肿瘤均被完全切除，无复发。中耳面神经神经鞘瘤的2例耳神经自体移植中有1例面部肌肉张力恢复良好。3例恶性肿瘤患者在12 - 24个月内死亡，表现为局部复发（1例）和/或肺转移（2例）。正如预期的那样，神经源性肿瘤是头颈部区域不常见的肿瘤。另一方面，神经源性恶性肿瘤存在诊断问题，长期生存机会也不确定。由肿瘤范围决定且对治疗决策至关重要的肿瘤分期，通过CT扫描和MRI能得到很好的评估。对于神经纤维瘤病中发生的神经源性肉瘤，也推荐采用手术和放疗的多模式治疗方案。然而，对于主干神经鞘瘤，采用神经间置自体移植是首选治疗方法，功能恢复的机会较大。
