Malignant peripheral nerve sheath tumour of the paranasal sinuses and the anterior skull base.
BACKGROUND
Malignant peripheral nerve sheath tumours (MPNSTs) are highly aggressive neoplasms with a marked propensity for local recurrence and metastatic spread. The management of MPNSTs continues to challenge pathologists and surgeons. As MPNSTs of the paranasal sinuses and the skull base are rare, prognostic factors and treatment modalities have not been consistently identified.


METHODS
We present a case of MPNST of the anterior skull base and provide an overview of all MPNSTs reported since 1970, in which the tumour location was the anterior skull base or the paranasal sinuses.


RESULTS
Literature review revealed 33 well-documented cases of MPNSTs in this anatomic location. These cases were analysed with emphasis on age, gender, affected site, therapy, outcome, presence of neurofibromatosis, local recurrence and metastases.


CONCLUSIONS
Despite multimodal therapy and advances in surgical techniques, the prognosis of MPNST located in the paranasal sinuses and the anterior skull base remains dismal. Outcome is mainly a function of local control by surgical resection. Adjuvant radiochemotherapy has shown no benefit. It may therefore be advisable to abstain from radiochemotherapy in order to improve chances for surgical intervention in case of recurrent disease. Close follow-up investigations are indispensable.
Pfeiffer
Jens
J
Department of Otorhinolaryngology - Head and Neck Surgery, University Medical School Freiburg, Germany. pfeiffer@hno.ukl.uni-freiburg.de
Arapakis
Iakovos
I
Boedeker
Carsten C
CC
Ridder
Gerd J
GJ
D000328:Adult; D006801:Humans; D008297:Male; D018317:Nerve Sheath Neoplasms / Q000473:pathology* / Q000601:surgery; D009669:Nose Neoplasms / Q000473:pathology* / Q000601:surgery; D010255:Paranasal Sinus Neoplasms / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D019292:Skull Base Neoplasms / Q000473:pathology* / Q000601:surgery