Ilgner J, Rojas W, Biesterfeld S, Schürmann K, Zimny M, Westhofen M
Klinik für Hals-Nasen-Ohrenheilkunde und Plastische Kopf- und Halschirurgie des Universitätsklinikums Aachen.
Laryngorhinootologie. 2001 Jan;80(1):39-42. doi: 10.1055/s-2001-11034.
Malignant Peripheral Nerve Sheath Tumours (MPNST) either grow sporadically, after radiation or chemotherapy respectively. In many cases they are associated with Neurofibromatosis I. Because of the multiform histologic picture they are often difficult to differentiate from other soft tissue tumours.
We present the case of a sporadic MPNST which developed from the vagus nerve of a 39-year-old patient following radiation of the neck 7 years before. After complete excision there has been no recurrence up to now.
Sporadic MPNST of the head and neck are comparatively rare. With regard to the strong association with Neurofibromatosis I and the difficult differential diagnosis to other soft tissue tumours the emphasis should be put on excluding further manifestations of Neurofibromatosis I and of secondary tumours.
恶性外周神经鞘瘤（MPNST）分别在放疗或化疗后散发性生长。在许多情况下，它们与神经纤维瘤病I相关。由于其组织学表现多样，它们常常难以与其他软组织肿瘤相鉴别。
我们报告一例散发性MPNST病例，该病例发生于一名39岁患者的迷走神经，7年前该患者接受过颈部放疗。完全切除后至今未复发。
头颈部散发性MPNST相对罕见。鉴于其与神经纤维瘤病I的密切关联以及与其他软组织肿瘤的鉴别诊断困难，应着重排除神经纤维瘤病I的其他表现和继发性肿瘤。
