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Case Report

IJHSR. 2016; 6(11): 299-303


Maffucci Syndrome with Composite Hemangioendothelioma: Two Rare Entities Superimposed Upon Each Other.

Ghanshyam Verma, Kirti Makhija, G.R. Tegta, Ajeet Negi, Shikha Sharma.

Abstract
Maffucci syndrome is a rare non hereditary disorder characterized by multiple enchondromas and hemangiomas. A 23 year old female presented to us with multiple nodular swellings over the left hand and a few bright red soft swellings over the left ear and oral cavity. They were found to be enchondromas and hemangiomas respectively. She also presented with a progressively increasing painless swelling over the left foot which on histology showed the features of composite hemangioendothelioma. It is a recently described unusual vascular tumour of low grade malignancy exhibiting a composite of hemangioendothelioma variants.

Key words: Maffucci syndrome, enchondroma, hemangioma, lymphangioma, hemangioendothelioma.



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