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Thoracic lymphangiomatosis in a child
Ofelia A Alvarez1, Ingrid Kjellin, Craig W Zuppan
1Department of Pediatrics, University of Miami, Miami, Florida 33101,USA. oalvarez2@med.miami.edu
Journal of Pediatric Hematology/Oncology
|February 10, 2004
Summary
Thoracic lymphangiomatosis is a rare condition presenting as a mediastinal mass with lung involvement. This review highlights its varied symptoms and poor prognosis in children, emphasizing its importance in differential diagnosis.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Medical Pathology
Background:
- Lymphangiomatosis is a rare congenital malformation of lymphatic vessels.
- Thoracic involvement, characterized by mediastinal masses and pulmonary infiltrates, presents a significant diagnostic and therapeutic challenge.
- Disseminated intravascular coagulation (DIC) is a severe complication associated with advanced disease.
Observation:
- A case of an 8-year-old boy with mediastinal mass, pulmonary infiltrates, and DIC diagnosed as lymphangiomatosis is presented.
- Review of 52 additional cases reveals common presentations including chylothorax (49%), mediastinal mass (47%), and pulmonary infiltrates (45%).
- Pediatric patients (<16 years) exhibit a significantly worse prognosis (39% mortality) compared to adults.
Findings:
- Thoracic lymphangiomatosis presents with a spectrum of clinical manifestations, including bone and splenic lesions.
- Mortality is strongly associated with parenchymal lung involvement or pleural effusion.
- Disseminated intravascular coagulation (DIC) occurs in 9% of cases, indicating systemic disease progression.
Implications:
- Thoracic lymphangiomatosis must be considered in the differential diagnosis of pediatric mediastinal masses with pulmonary findings.
- Early recognition and multidisciplinary management are crucial for improving outcomes in pediatric patients.
- Further research into targeted therapies for thoracic lymphangiomatosis is warranted to improve survival rates.