Solitary fibrous tumour of the pleura

A 68-year-old man with a medical history of chronic myeloid leukaemia was referred to the radiology department for an ultrasound of the abdomen due to aberrant liver values. The patient was asymptomatic.

Ultrasound showed no intra-abdominal abnormalities. However, a large heterogeneous solid and vascularised mass in the right lung base, reaching up to the liver, was noted (Figure 1). Computed tomography (CT) of the chest confirmed a right-sided thoracic mass (15 x 14 x 13 cm) with a broad pleural base, internal vascularisation, and adjacent atelectasis (Figure 2). There was no invasion of osseous structures or other suspicious lung lesions. CT-guided core-needle biopsy of the lesion was performed, which confirmed a solitary fibrous tumour of the pleura.

Three months later, the chest CT was repeated because of increasing chest pain. This showed a further increase in the volume of the mass, together with the onset of a significant amount of pleural fluid (Figure 3). The patient was referred for surgery, and the mass was resected using a right anterior thoracotomy (Figure 4).

The pleura is a two-layered serous membrane from which benign and malignant masses can form. Solitary fibrous tumour (SFT) or pleural fibroma are rare mesenchymal tumours. Their aetiology is still unknown, but they occur most frequently between the 6th and 7th decade of life [1]. The majority of SFTs are benign, slow-growing lesions arising from the visceral pleura, but 20% may be malignant and tend to arise from the parietal pleura [1]. SFTs may metastasise, most commonly affecting the pleura, chest/abdominal wall, lung, peritoneum and liver [2].

Solitary fibrous tumours are often asymptomatic and an incidental finding on imaging. If symptomatic, they can be accompanied by cough, chest pain, or dyspnoea. More pathognomic for SFT is the presentation of hyperglycaemia due to the production of insulin-like growth factor 2 (Doege–Potter syndrome) or hypertrophic pulmonary osteoarthropathy due to the production of hyaluronic acid (Pierre Marie–Bamberger syndrome) [3].

CT is the preferred imaging technique, but it is often nonspecific and difficult to differentiate. CT will show a soft tissue pleural-based neoplasm, often with areas of necrosis, haemorrhage and cystic changes, which will enhance heterogeneously post-contrast [2]. The presence of calcifications or wall invasion increases the likelihood of malignancy. Additional MRI can provide further information about the fibrous nature of the tumour. The definitive diagnosis is made histologically.

There are no established guidelines for follow-up, but recurrence can occur in around 8% of treated patients [4].

Resection of the lesion is the treatment of choice. In case of malignancy or metastatic disease, therapies such as radiation and chemotherapy may be considered.

All patient data have been completely anonymised throughout the entire manuscript and related files.