Self Regressing Epitheloid Haemangioendothelioma of Tibia in an Infant-A rare case report and review of literature.

Abstract

Introduction:Epitheloid hemangioendothelioma is a rare vascular soft tissue tumour of intermediate malignant potential. The tumor affects any age group, and liver is the commonest internal organ affected. Bones as calvarium, spine, tibia and femur may also be affected. The lesion can be multifocal in the liver or the same bone itself and can metastasise to lungs. Here we report a rare case of epithelioid hemangioendothelioma involving tibia in a male infant, which regressed by itself without any treatment, after incisional biopsy over a period of one and half years.Case Report:A 7 month old male baby was presented with incessant cry, fever and swelling in left upper leg of 2 weeks duration. X-ray of leg showed a well demarcated lytic lesion in the meta diaphysial region of left tibia. Magnetic resonance imaging showed an irregular lytic lesion with intramedullary extension. Incisional biopsy showed a vascular tumor, epithelioid hemangioendothelioma grade 1. Meanwhile the patient became asymptomatic and subsequent follow ups showed regression in the size of the tumor and complete disappearance after one and half years. This points towards the need of a wait and watch policy in such intermediate grade vascular tumours even though the lesion is so extensive. This is the first case report of an extensive self regressing epithelioid hemangioendothelioma of tibia in an infant to our knowledge.Conclusion:Intermediate grade vascular tumors can undergo spontaneous regression which points towards the need of a wait and watch policy in such tumors thus avoiding extensive surgeries, especially in young patients.