Experience of a single center in the treatment of rare malignant peripheral nerve sheath tumors during the years 1991–2021

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare sarcoma with a poor prognosis, as the aggressive types of this cancer tend to grow rapidly and metastasize frequently. MPNST is associated with neurofibromatosis type 1 gene mutation. The minority of cases arise secondary to radiation therapy or sporadically. The primary treatment for MPNST is early surgical resection of the tumor. The aim of this study was to retrospectively evaluate the outcome of the treatment of MPNST in Helsinki University Hospital from the years 1991 to 2021. Fourteen MPNST cases were evaluated in this study retrospectively. Descriptive statistical analysis was performed on the collected patient data. Marginal resection was completed in nine cases, wide margins were achieved in three cases, and in two cases the final histological examination of the specimen revealed intralesional removal. During the follow-up time of 36.7 ± 12.1 months, all patients who underwent wide margin resection were alive. One patient died 22 months after intralesional resection and six within 38.3 ± 30.9 months of marginal resection. Seventy-one percent of tumor surgeries resulted in Clavien-Dindo class 3b complications, reflecting the complexity of the surgeries. The aggressive nature of MPNST and the large size of these tumors requires extensive surgery, which can lead to complications. The prognosis of MPNST needs improvement.