A 29-year-old female patient goes to the emergency room due to tremor in lower limbs since 72 hours. She suffers from habitual migraine headaches as the only precedent of interest.
A chest X-ray was performed and it was completed with a enhanced chest computed tomography. Subsequently a MRI was performed for the best characterisation of the lesion.
An initial posteroanterior and lateral chest X-ray, showed in the left lower lobe a well-defined lesion of obtuse edges with the thoracic wall, suggestive of pleural or extrapleural origin.
Enhanced chest CT confirms the presence of an extrapulmonary well-defined and hypervascular lesion. It loses the obtuse angle with the pleural surface and erodes the adjacent costal arch.
Dorsal column MRI showed a polylobulated and well-defined lesion, hyperintense on T2 weighted sequences and hypointense on T1-weighted sequences, showing ample contact with the pleural surface.
Solitary fibrous tumours of the pleura (SFTP) are rare neoplasms originating from submesothelial primitive mesenchymal cells. Although usually benign, approximately 10%–20% are malignant or locally aggressive [2, 5]. There is no known aetiologic agent and no link to tobacco or asbestos [1-5].
Most cases are identified in middle-aged adults (especially in the 6-7 decade) with no sex preponderante. However in some series there is predilection for the female sex. [2, 3, 4]
Patients are usually asymptomatic and are diagnosed frequently as an incidental finding. Symptomatic cases usually start with cough, dyspnoea, haemoptysis or chest pain. Paraneoplastic syndromes are uncommon (hypoglycaemia, acropachy and osteoarthropathy). [3, 4].
Most of the tumours present as pleural-based masses, but have also been reported in other locations: mediastinum, lung, and extrathoracic sites. Most originate in the visceral pleura (80%) and have predilection towards the mid-to-lower zones of the chest [3, 4, 6].
On chest X-ray, it presents as a pleural based mass, circumscribed and sometimes lobulated. It often forms an in obtuse angle with the chest wall. Tumours may grow to a large size. Pedunculated lesions can change position - appearance with respiration or with a change in position. Calcification, rib destruction, and pleural effusions are uncommon [2, 6].
The chest CT is the key image test, shows the size and location of the tumour and allows a correct surgical planning. On contrast-enhanced scans shows relatively homogenous intense background enhancement (from rich vascularisation). Non-enhancing areas may be present (necrosis, myxoid degeneration, or haemorrhage) [5, 6].
On T1-weighted sequence shows a low-intermediate signal. On T2-weighted sequence presents low signal and may be having hyperintense foci (corresponds to the hypodense areas of CT), as is this case. Usually presents an intense enhancement with gadolinium administration [2, 3, 4].
The usual radiographic appearance and clinical presentation gives the surgeon the
preoperative clinical suspicion that an SFTP is present. In experienced hands, prompt surgical treatment can be carried out safely without a preoperative diagnosis . However, the final diagnosis is anatomopathological. Immunohistochemically expresses CD34, vimentin, bcl-2 and CD99 .
The treatment of choice is complete resection. Approximately 80% of the forms are benign and cured. Malignant variety has a high rate of recurrence and mortality. Thus, aggressive surgery and close follow-up are needed in the postoperative period. Recurrence can occur 20 years after its presentation and is more common in the same hemithorax [1, 5].
Written informed patient consent for publication has been obtained.
Differential Diagnosis List
Fibrous tumour of the pleura.
Bone myeloma / plasmocytoma
Primary bone tumour (for example: Sarcoma of Ewing)
Fibrous tumour of the pleura.