Case report Long-term survival of a patient with aggressive sporadic malignant peripheral nerve sheath tumour presenting as haemothorax

mour (MPNST) belongs to the group of rare soft tissue sarcomas. High his­ tological grade is a common feature, explaining its poor prognosis irrespec­ tive of the aggressiveness of local treat­ ment. Standard therapy of inoperable local relapses or distant metastases has not been established, as phase II or III clinical trials are lacking and treatment strategies are based on an­ ecdotal reports on the effectiveness of standard chemotherapy or different novel agents. Here we present a long­ term survivor of the sporadic type of MPNST – a giant thoracic tumour with an uncommon presentation of haemo­ thorax. Despite radical excision of the tumour, followed by high­dose ra­ diotherapy to the tumour bed, the patient was diagnosed with multiple metastases located in the postpneu­ monectomy thoracic cavity and the right lung. First­line doxorubicin fol low­ ed by somatostatin analogue resulted in a six­month PFS, while second­line ifosfamide­based chemo therapy result­ ed in good and long lasting symptom palliation.