Largest recorded non-invasive true intrathoracic desmoid tumour

Abstract

Intrathoracic desmoid tumours are rare soft-tissue neoplasms arising from fascial or musculo-aponeurotic structures, accounting for less than 0.03% of all neoplasms. Most cases in fact represent intrathoracic extension of chest wall tumours. This case report describes the largest recorded true intrathoracic desmoid tumour without mediastinal or chest wall invasion. The tumour was completely excised through a left thoracotomy with negative tumour margins. Management of the patient will involve radiological surveillance. Desmoids are benign tumours that do not metastasise but have a high rate of recurrence, especially if tumour margins are positive after surgical resection. Complete surgical resection is the treatment of choice. Other treatment options include radiotherapy; non-steroidal inflammatory drugs or antioestrogen therapy (tamoxifen) or a combination thereof; and chemotherapy or targeted drug therapy.