ArticleThe American journal of surgical pathology2022
Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients.
Article in The American journal of surgical pathology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- Vascular malformations: from genetics to therapeutics.EMBO molecular medicine · 2026Review
- Long-term low-dose sirolimus therapy and successful discontinuation in an adult with kaposiform lymphangiomatosis and disseminated intravascular coagulation: a case report.Frontiers in medicine · 2026Article
- Kaposiform lymphangiomatosis-the effects of long-term treatment with sirolimus: case series study and review of the literature.Frontiers in medicine · 2026Article
- Trametinib normalizes angiopoietin-2 levels and successfully treats kaposiform lymphangiomatosis.Journal of vascular anomalies · 2025Article
- Perioperative Considerations for a Patient with Juvenile Idiopathic Scoliosis and Kaposiform Lymphangiomatosis Undergoing Spinal Fusion: A Case Report.Journal of orthopaedic case reports · 2025Article
- Case Report: Life-threatening Kasabach-Merritt phenomenon in a 2-month-old child.Frontiers in pediatrics · 2025Article
- A Unique Case of Intra-Abdominal Diffuse Lymphangiomatosis Mimicking a Pseudomyxoma Peritonei.Surgical case reports · 2025Article
- Central conducting lymphatic anomaly: from bench to bedside.The Journal of clinical investigation · 2024Review
- Challenging diagnosis of Kaposiform lymphangiomatosis: Unveiling a rare primary lymphatic disorder.Radiology case reports · 2024Article
- Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment.Pediatric blood & cancer · 2023Review
- Refractory kaposiform lymphangiomatosis relieved by splenectomy.Frontiers in pediatrics · 2023Article
- Kaposiform Lymphangiomatosis in a Male Adolescent: A Clinical Challenge and the Role of Genetics.Journal of investigative medicine high impact case reportsArticle
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20 authors.
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Abstract
Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with distinctive clinical, radiologic, histopathologic, and molecular findings. Herein, we document the pathology in 43 patients evaluated by the Boston Children's Hospital Vascular Anomalies Center from 1999 to 2020. The most frequent presentations were respiratory difficulty, hemostatic abnormalities, and a soft tissue mass. Imaging commonly revealed involvement of some combination of mediastinal, pulmonary, pleural, and pericardial compartments and most often included spleen and skeleton. Histopathology was characterized by dilated, redundant, and abnormally configured lymphatic channels typically accompanied by dispersed clusters of variably canalized, and often hemosiderotic, spindled lymphatic endothelial cells that were immunopositive for D2-40, PROX1, and CD31. An activating lesional NRAS variant was documented in 9 of 10 patients. The clinical course was typically aggressive, marked by hemorrhage, thrombocytopenia, diminished fibrinogen levels, and a mortality rate of 21%.
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