Neurofibrosarcoma in Von Recklinghausen's disease presenting with hypochromic anaemia

A patient with Von Recklinghausen's disease who developed a retroperitoneal neurofibrosarcoma is described. The presenting clinical picture was one of hypochromic anaemia with a large mass palpable in the left hypochondrium. At operation the tumour was found to be invading the small intestine with resultant blood loss. The patient had presented 21 years before with unexplained severe iron deficiency anaemia.