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Generalized lymphangiomatosis presenting as cardiomegaly
Yi-Ling Chen1, Chin-Cheng Lee, Ming-Lun Yeh
1Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.
Summary
Generalized lymphangiomatosis in an infant was treated with surgery and interferon (IFN) alpha-2b. This approach showed no recurrence over two years, suggesting IFN alpha-2b as a potential treatment.
Area of Science:
- Pediatric Oncology
- Vascular Anomalies
- Immunotherapy
Background:
- Lymphangioma is a benign proliferation of lymphatic tissue.
- Generalized lymphangiomatosis is a rare condition affecting multiple organs.
Observation:
- An 8-month-old infant presented with tachypnea, fever, and a mediastinal mass causing cardiomegaly.
- The mass extended to the thymus, lung, and spleen, diagnosed as lymphangiomatosis via biopsy.
- Initial treatments including fluid drainage and parenteral nutrition were ineffective.
Findings:
- Surgical resection of the mediastinal mass and spleen was performed.
- Subcutaneous interferon (IFN) alpha-2b was administered for 3 months post-surgery.
- No recurrence was observed during a 2-year follow-up period.
Implications:
- Interferon (IFN) alpha-2b shows promise as an alternative treatment for generalized lymphangiomatosis.
- This case highlights a successful multimodal approach for a rare pediatric condition.
- Further research into immunotherapy for lymphangiomatosis is warranted.
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