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

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...

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Autores principales: K. Nair, Lekha, Das, Anitha, Kumar A.S, Arun, Ramachandran, V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Indian Orthopaedic Research Group 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4719350/
https://www.ncbi.nlm.nih.gov/pubmed/27299017
http://dx.doi.org/10.13107/jocr.2250-0685.251
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author K. Nair, Lekha
Das, Anitha
Kumar A.S, Arun
Ramachandran, V.
author_facet K. Nair, Lekha
Das, Anitha
Kumar A.S, Arun
Ramachandran, V.
author_sort K. Nair, Lekha
collection PubMed
description 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.
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spelling pubmed-47193502016-06-13 Self Regressing Epitheloid Haemangioendothelioma of Tibia in an Infant-A rare case report and review of literature K. Nair, Lekha Das, Anitha Kumar A.S, Arun Ramachandran, V. J Orthop Case Rep Case Report 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. Indian Orthopaedic Research Group 2015 /pmc/articles/PMC4719350/ /pubmed/27299017 http://dx.doi.org/10.13107/jocr.2250-0685.251 Text en Copyright: © Indian Orthopaedic Research Group http://creativecommons.org/licenses/by-nc-sa/3.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc-sa/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
K. Nair, Lekha
Das, Anitha
Kumar A.S, Arun
Ramachandran, V.
Self Regressing Epitheloid Haemangioendothelioma of Tibia in an Infant-A rare case report and review of literature
title Self Regressing Epitheloid Haemangioendothelioma of Tibia in an Infant-A rare case report and review of literature
title_full Self Regressing Epitheloid Haemangioendothelioma of Tibia in an Infant-A rare case report and review of literature
title_fullStr Self Regressing Epitheloid Haemangioendothelioma of Tibia in an Infant-A rare case report and review of literature
title_full_unstemmed Self Regressing Epitheloid Haemangioendothelioma of Tibia in an Infant-A rare case report and review of literature
title_short Self Regressing Epitheloid Haemangioendothelioma of Tibia in an Infant-A rare case report and review of literature
title_sort self regressing epitheloid haemangioendothelioma of tibia in an infant-a rare case report and review of literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4719350/
https://www.ncbi.nlm.nih.gov/pubmed/27299017
http://dx.doi.org/10.13107/jocr.2250-0685.251
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