Solitary fibrous tumour along with non-small-cell lung cancer and Doege-Potter syndrome

Solitary fibrous tumour (SFT) is a mesenchymal tumour usually arising from the pleura. It accounts for less than 5% of all primary pleural tumours and less than 2% of all soft tissue tumours [1]. This tumour occurs equally in both men and women and is diagnosed most frequently in the 6th and 7th decades of life [1]. Usually SFTs are well circumscribed (well defined) and benign. Malignant SFTs with a higher recurrence rate and distant metastatic progression are rarely seen (10–20%) [1]. These tumours should be diagnosed by the use of combined pathologic and immunohistochemical analysis. Malignant tumours present with increased pleomorphism and cellularity, increased mitotic activity (> 4 mitoses per high power field), extensive necrosis and haemorrhage. In more than 50% of patients the disease is asymptomatic and pulmonary lesions are diagnosed incidentally during follow-up examinations. The remaining patients usually present with dyspnoea and chest pain. Paraneoplastic syndromes are rarely seen. The most commonly reported paraneoplastic syndromes are Pierre-Marie-Bamberg syndrome and Doege-Potter syndrome, which is characterized by a large tumour with increased excretion of insulin-like growth factor 2 (IGF-2) causing hypoglycaemia [1].
A 69-year old woman with the diagnosis of non-small-cell lung cancer (NSCLC) of the right lung was admitted to the hospital in May 2009. Chest computed tomography (CT) revealed a well-circumscribed, polycyclic lesion in the frontal part of the left lung apex and irregular infiltration (20 mm diameter) in the 2nd segment of the right lung. No other lesions within both lungs were detected. The examination did not reveal enlarged lymph nodes or presence of fluid in the pleural cavity. Bronchofibroscopy did not reveal intrabronchial changes. Fine-needle biopsy of the left lung apex lesion was performed, but it was non-diagnostic. The biopsy of the right lung lesion was performed and adenocarcinoma was diagnosed. Positron emission tomography-CT (PET-CT) was performed to assess the clinical stage of the disease. The result indicated an active, metabolic proliferative process in the right lung (nodule of 16 × 24 mm in size, 18-fluorodeoxyglucose maximum standardized uptake value – 18F-FDG SUV(max) = 9.9). The polycyclic tissue lesion in the left lung apex (36 × 36 × 30 mm), adjacent to the pleura, did not accumulate 18F-FDG (18F-FDG SUV(max) = 1.0). No lymph node enlargement or increased 18F-FDG accumulation was found and...


View full text...