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Year : 2016  |  Volume : 3  |  Issue : 2  |  Page : 97-100

Upfront radiotherapy for diffuse lymphangiomatosis in a child

1 Department of Radiation Oncology, Army Hospital Research and Referral, New Delhi, India
2 Department of Pediatrics, Army Hospital Research and Referral, New Delhi, India
3 Department of Pathology and Molecular Sciences, Army Hospital Research and Referral, New Delhi, India

Correspondence Address:
Abhishek Purkayastha
Department of Radiation Oncology, Army Hospital Research and Referral, Dhaula Kuan, Delhi Cantonment - 110 010, Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2349-4220.195938

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Lymphangiomatosis is an extremely rare multisystem disorder resulting from congenital malformations of lymphatic development mainly affecting the pulmonary and skeletal systems without a preset therapeutic approach. We hereby present a unique case of diffuse lymphangiomatosis in a 2-year-old male child who presented with massive right pleural effusion, ascites, right hydrocele, and lytic lesions in right hemipelvis, vertebrae, and femur. Biopsy from right thigh revealed lymphangiomatosis. The child was treated with upfront conformal radiotherapy (RT) to the affected sites with fractionated doses of 18–20 Gy which he tolerated well. Post-therapy evaluation by imaging showed a significant response to radiation with excellent symptomatic relief. Presently, he is on follow-up for over 1 year with a Karnofsky performance status of 90% without any evidence of progression of the disease. The aim of reporting this case is to sensitize the medical fraternity about RT as an effective, noninvasive, definitive therapeutic modality for treating this uncommon disease.

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