Surgical management of malignant peripheral nerve sheath tumors.
OBJECTIVE
The aim of this study was to describe the presentation of patients harboring soft tissue sarcomas involving the nerves, most of which were malignant peripheral nerve sheath tumors (MPNSTs), and provide an algorithm for their treatment.


METHODS
The authors retrospectively analyzed data on 43 surgically treated soft tissue sarcomas involving the nerves, 34 (79%) of which were MPNSTs. Tumor classifications are presented, together with patient numbers, locations of MPNSTs, surgical techniques, and adjunctive treatments.


RESULTS
The 34 MPNSTs were surgically treated during a period of 40 years. Most of these lesions (19 MPNSTs [56%]) were located in the brachial plexus, whereas the rest were located on other major nerves. Neurofibromatosis Type 1-associated tumors (12 lesions) represented 35% of the total number of MPNSTs. Although the main goal of surgery was complete excision, it was successful in only 16 patients (47%). Of the available records, 18 patients (53%) died due to disease progression.


CONCLUSIONS
Malignant peripheral nerve sheath tumors are rare. Surgical removal with margins when possible coupled by adjuvant radiotherapy offers the best chance of survival. The role of chemotherapy is still being defined.
Gachiani
John
J
Department of Neurosurgery, Louisiana State University Health Sciences Center, New Orleans, Louisiana, USA.
Kim
Daniel
D
Nelson
Adriane
A
Kline
David
D
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D019468:Disease Management; D006801:Humans; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery*; D012189:Retrospective Studies
D000970:Antineoplastic Agents