Ileal haemangiopericytoma and von Recklinghausen's disease

Abstract

Haemangiopericytoma is an uncommon tumour of vascular pericytes which usually arises in deep subcutaneous tissues or the retroperitoneum. There are few reports in the English literature of haemangiopericytoma arising in the ileum. Von Recklinghausen's disease, a condition of autosomal dominant inheritance with a prevalence of 1 in 3000, is often associated with tumours of neural origin. Tumours of non-neural origin also occur in Von Recklinghausen's disease but haemangiopericytoma has not been described. We report on a patient with Von Recklinghausen's disease presenting with massive haemorrhage from an ileal haemangiopericytoma.