Case Report
Year: 2016 | Month: November | Volume: 6 | Issue: 11 | Pages: 299-303
Maffucci Syndrome with Composite Hemangioendothelioma: Two Rare Entities Superimposed Upon Each Other
Ghanshyam Verma1, Kirti Makhija2, G.R. Tegta3, Ajeet Negi4, Shikha Sharma2
1Associate Professor, 2Junior Resident, 3Professor and Head, 4Senior Resident,
Department of Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla.
Corresponding Author: Ghanshyam Verma
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.