Functional Status in Malignant Peripheral Nerve Sheath Tumors

Malignant peripheral nerve sheath tumors (MPNST) are rare in the population. Because of its aggressive behavior, an MPNST is considered to be part of the group of soft tissue sarcomas (STS). They generally harbor poor prognosis with high rates of local recurrence and metastases. Median survival varies between 5 and 8 years [1–3]. In STS literature, the focus of research has generally remained on improving oncological outcomes and diminishing postoperative complications. However, MPNSTs are generally large tumors requiring major resection of soft tissues, in turn resulting in significant morbidity. To date, STS arising in extremities are primarily treated by limb-sparing surgery. Nevertheless, amputations are not uncommon and although their indications differ, major neurovascular involvement is still often seen as one [4, 5]. Resection of any type of nerve has been shown to affect function and quality of life after resection of an STS [6, 7]. As MPNST have their origin in nerve tissue, they are among the STS with the highest risk for postoperative function loss. Yet functional outcomes still have earned little attention in literature.