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Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients
Antonio R Perez-Atayde1, Larisa Debelenko2, Alyaa Al-Ibraheemi1
1Departments of Pathology.
The American Journal of Surgical Pathology
|April 6, 2022
Summary
Kaposiform lymphangiomatosis, a rare lymphatic anomaly, often presents with respiratory issues and bleeding problems. Genetic mutations in NRAS were found in most patients, contributing to an aggressive disease course and significant mortality.
Area of Science:
- Vascular Anomalies
- Pediatric Pathology
- Molecular Genetics
Background:
- Kaposiform lymphangiomatosis is a rare, aggressive generalized lymphatic anomaly.
- It presents with unique clinical, radiologic, histopathologic, and molecular features.
Purpose of the Study:
- To document the pathology of Kaposiform lymphangiomatosis in a cohort of patients.
- To characterize the clinical, imaging, histopathologic, and molecular findings.
- To assess the clinical course and outcomes.
Main Methods:
- Retrospective review of 43 patients evaluated at Boston Children's Hospital Vascular Anomalies Center (1999-2020).
- Analysis of clinical presentations, imaging findings, histopathology, and molecular data (NRAS variant testing).
Main Results:
- Common presentations included respiratory difficulty, hemostatic abnormalities, and soft tissue masses.
- Imaging frequently showed involvement of mediastinal, pulmonary, pleural, pericardial, splenic, and skeletal compartments.
- Histopathology revealed abnormal lymphatic channels and endothelial cells; activating NRAS variants were found in 90% of tested patients.
- The disease course was aggressive, with a 21% mortality rate due to hemorrhage and thrombocytopenia.
Conclusions:
- Kaposiform lymphangiomatosis is characterized by specific histopathologic features and frequent NRAS mutations.
- The condition follows an aggressive clinical course with high morbidity and mortality.
- Multidisciplinary management is crucial for these complex pediatric vascular anomalies.
