[Low-grade malignant peripheral nerve sheath tumor of the neck soft tissues].
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Klinik für Hals-Nasen-Ohrenheilkunde und Plastische Kopf- und Halschirurgie des Universitätsklinikums Aachen. Justus.Ilgner@post.rwth-aachen.de
J
J
Ilgner
W
W
Rojas
S
S
Biesterfeld
K
K
Schürmann
M
M
Zimny
M
M
Westhofen
D000328:Adult; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D003390:Cranial Nerve Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D009381:Neoplasms, Radiation-Induced / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D014630:Vagus Nerve / Q000473:pathology; D020421:Vagus Nerve Diseases / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery