Maurer J, Mann W
Univ.-HNO-Klinik Freiburg.
Laryngorhinootologie. 1989 Aug;68(8):433-6. doi: 10.1055/s-2007-998370.
Malignant schwannomas are uncommon in the head and neck. They are derived from Schwann cells of cranial, cervical or sympathetic nerves and occur as solitary tumours or in association with neurofibromatosis (von Recklinghausen's disease). We present a patient of 30 years of age with a tumour of the right nasal and paranasal sinus system and a 68-year-old woman with the first described intratracheal malignant epitheloid Schwannoma. After the diagnosis of a malignant Schwannoma wide surgical excision is the recommended treatment, depending on the individual case, eventually followed by radiation therapy. As the tumour is more aggressive in patients with neurofibromatosis, the prognosis is better for patients without neurofibromatosis.
恶性神经鞘瘤在头颈部并不常见。它们起源于颅神经、颈神经或交感神经的施万细胞，可表现为孤立性肿瘤，或与神经纤维瘤病（冯雷克林霍增氏病）相关。我们报告了一名30岁患有右侧鼻腔和鼻窦系统肿瘤的患者，以及一名68岁患有首例经描述的气管内恶性上皮样神经鞘瘤的女性患者。诊断为恶性神经鞘瘤后，根据具体病例情况，推荐进行广泛的手术切除，最终可能需要进行放射治疗。由于该肿瘤在神经纤维瘤病患者中更具侵袭性，因此无神经纤维瘤病的患者预后较好。
