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

Malignant peripheral nerve sheath tumour (MPNST) belongs to the group of rare soft tissue sarcomas. High histological grade is a common feature, explaining its poor prognosis irrespective of the aggressiveness of local treatment. 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 anecdotal 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 haemothorax. Despite radical excision of the tumour, followed by high-dose radiotherapy to the tumour bed, the patient was diagnosed with multiple metastases located in the postpneumonectomy thoracic cavity and the right lung. First-line doxorubicin followed by somatostatin analogue resulted in a six-month PFS, while second-line ifosfamide-based chemotherapy resulted in good and long lasting symptom palliation.