Klippel-Trenaunay syndrome and cerebral haemangiopericytoma: a potential association.

Abstract

Klippel-Trenaunay syndrome (KTS) a rare mesodermal phakomatosis consisting of capillary malformations, varicose veins, and limb hypertrophy, often associated with vascular malformations and benign tumours. A 33-year-old male presented with headaches secondary to a previously diagnosed intracranial tumour. He had a large blanching port-wine stain and hypertrophy of the left side of his body and limbs partial syndactyly of the 2(nd) and 3(rd) digits in all four extremities. The lesion was surgically resected and histology showed a Haemangiopericytoma. Thus KTS may be associated with intracranial Haemangiopericytomas, a malignant vascular tumour.