Malignant peripheral nerve sheath tumors (MPNST) in the head and neck region

Introduction:

MPNSTs are extremely rare sarcomas and display a diagnostical challenge as they often appear as a benign tumor. Within the last 15 years 3 patients were diagnosed with MPNSTs in the head and neck region in our department.

Methods:

Retrospective analysis on the basis of different documentary systems of the department.

Results:

In all 3 patients (1 Female, 2 Male) a benign tumor was suspected: a schwannoma of the trigeminal nerve, a vestibular schwannoma and a schwannoma (recurrent tumor) of the vagal nerve. All patients were in their 60's.

Histopathologic examination after resection yielded the immediate diagnosis of a MPNST only in one case, in the two other cases a reference pathologic examination was undertaken.

After complete resection and radiotherapy there was a recurrency in the case of the female patient with the MPNST of the trigeminal nerve. There was a one-year and a three-year recurrence-free survival in the two other cases, before the patients were lost to follow-up.

Conclusions:

MPNSTs should be considered even in benign appearing tumors. Radical resection and adjuvant radiotherapy are the therapy of choice in these aggressive tumors. In the head and neck region radical resection is limited, contributing to the poor prognosis of these rare tumors.

Publication History

Publication Date:
18 April 2018 (online)