A rare case of malignant solitary fibrous tumour of pleura with Doege-Potter syndrome

Abstract

Solitary fibrous tumour of pleura (SFTP) is a rare tumor contributing to less than 5% of all pleural tumours and about 10% - 30% of these tumours show features of malignancy. Occurrence of hypoglycaemia with solitary fibrous tumour is an unusual paraneoplastic syndrome designated as Doege-Potter syndrome which can occur both in benign and malignant tumours. Very few cases of solitary fibrous tumour with Doege-Potter syndrome are documented in world literature and only a couple of cases are documented in Indian literature so far. We, report here an interesting case of 40-year-old male who presented with a short history of weakness in left upper and lower limbs following an ischaemic stroke; breathlessness, productive cough, seizures and repeated episodes of loss of consciousness. Laboratory investigation revealed normal serum C-peptide level (1.04 ng/mL) and his blood sugar level was observed to be low varying from 25 mg/dL to 47 mg/dL. Computed tomography (CT) of lung and pleura showed a large mass in right hemithorax for which he underwent right hemithoracotomy. Histopathological examination of the resected thoracotomy specimen along with immunohistochemical analysis confirmed the diagnosis of malignant solitary fibrous tumour of pleura. Basing on the clinical, histopathological and immunohistochemical examination findings, the case was finally diagnosed as malignant solitary fibrous tumour of pleura with Doege-Potter syndrome. The clinico-pathological features and therapeutic management of such rare syndrome are being discussed here.

Keywords

• Solitary fibrous tumour
• Carcinoma
• Hypoglycaemia