A small proportion of tumours can undergo malignant transformation. We report a case series of five patients diagnosed with giant solitary fibrous tumours of the pleura. These cases highlight the unpredictable nature of this disease process, with significant variability in clinical course observed, from indolence to aggressive progression. Three patients were found to have malignant disease on explant, with two of these having preoperative imaging and histology suggesting benign pathology. This finding emphasises that accurately differentiating between benign and malignant disease on imaging and/or biopsy has low specificity and sensitivity and cannot be relied upon in guiding the management of these tumours. Patients with solitary fibrous tumours of the pleura should be managed cautiously, owing to the unpredictable and potentially aggressive clinical course. We would advocate the position that all patients with solitary fibrous tumours of the pleura should be managed as if they have malignant disease. Prolonged follow-up is required due to the risk of disease recurrence, even in patients with benign disease.
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