Enrico Martin

238 Chapter 10 Introduction Malignant peripheral nerve sheath tumors (MPNST) are rare and aggressive soft tissue sarcomas (STS) that can occur at any anatomical site. 1 MPNSTs occur more commonly in neurofibromatosis type 1 (NF1) patients, accounting for approximately 25-50% of all patients. 2–5 Surgical resection of these tumors is essential to increase survival, while radiotherapy and chemotherapy mainly increase progression-free survival. 6,7 Despite curative intents of aggressive treatment, local recurrences and distant metastases are common and survival remains poor. 5,6 In general, MPNSTs are treated equally to other STS, and for extremity tumors limb salvage procedures have become standard of care. 8 Combining radiotherapy with limb-sparing surgery has been proven to increase functionality without impairing oncological outcomes. 8,9 For extremity tumors not resectable without morbid surgery or amputation, isolated limb perfusions followed by resection can increase the limb salvation rates. 10 Resecting nerves is sometimes, however, inevitable when operating on any STS and has repeatedly been reported to increase morbidity. 11–13 This is still frequently a reason for amputation in case of major neurovascular involvement. 14,15 The resection of MPNSTs always requires the resection of a nerve, but thus far, postoperative functionality and reconstructions in MPNSTs have had little attention in literature, even though reported rates of motor deficits are as high as 30%. 16 Moreover, functional reconstructions are still not common practice in any STS, both for sensory and motor deficits. 17–19 Aside from functional deficits, neuropathic pain can develop postoperatively also resulting in disability and psychological distress. 20 This phenomenon has not previously been studied in MPNSTs, nor has it widely been studied in sarcoma literature. 21 As neuropathic pain is commonly caused by neuroma formation in transected nerves, 22 MPNST patients may be even more prone to its development. Not only are MPNSTs rare tumors, but they are also operated by different surgical subspecialties due to their tissue of origin. Altogether, more can therefore be learned on surgeons’ operative and reconstructive considerations. This study is not aiming to address the ideal surgical specialty for operating these patients, but aims to investigate considerations for function preservation and reconstruction among these specialties by means of an international survey. Additionally, variation between subspecialties is assessed.