ArticleOrphanet journal of rare diseases2019
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Article in Orphanet journal of rare diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 67 citations in OpenAlex.
- Kaposiform vascular tumors with Kasabach-Merritt phenomenon: a case series of KHE and KLA from a tertiary care center in India.European journal of pediatrics · 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
- Lyve1-Driven NrasPediatric blood & cancer · 2025Article
- Case Report: Life-threatening Kasabach-Merritt phenomenon in a 2-month-old child.Frontiers in pediatrics · 2025Article
- Clinical characteristics and risk factors for acute abdomen in patients with abdominal lymphatic malformations.Journal of vascular surgery. Venous and lymphatic disorders · 2025Article
- Hemostasis and thrombosis risks and management in vascular anomalies.Hematology. American Society of Hematology. Education Program · 2024Article
- Extensive retroperitoneal lymphangiectasia presenting as inguinal swelling.BMJ case reports · 2024Article
- Adult-onset multifocal kaposiform hemangioendothelioma in the bone marrow, lung, liver, and brain: a case report.Frontiers in oncology · 2024Article
- Kaposiform hemangioendothelioma presented with raynaud phenomenon: a case report.BMC pediatrics · 2023Article
- Impact of age and tumor size on the development of the Kasabach-Merritt phenomenon in patients with kaposiform hemangioendothelioma: a retrospective cohort study.Precision clinical medicine · 2023Article
- Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment.Pediatric blood & cancer · 2023Review
- Refractory kaposiform lymphangiomatosis relieved by splenectomy.Frontiers in pediatrics · 2023Article
- Management and outcomes of advanced hemangioendothelioma at a medical oncology clinic in an Indian tertiary care center.Future science OA · 2022Article
- Angiopoietin-2-induced lymphatic endothelial cell migration drives lymphangiogenesis via the β1 integrin-RhoA-formin axis.Angiogenesis · 2022Article
- Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients.The American journal of surgical pathology · 2022Article
- Neonatal vascular anomalies manifesting as soft-tissue masses.Pediatric radiology · 2022Review
- Kaposiform lymphangiomatosis presenting with a Group ABMJ case reports · 2022Article
- Sirolimus in the treatment of kaposiform lymphangiomatosis.Orphanet journal of rare diseases · 2021Article
- Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and management.Orphanet journal of rare diseases · 2020Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundKaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) are rare and aggressive vascular disorders. The aim of this study was to examine the clinical features and prognosis of KLA and KHE involving the thorax.
methodsThe clinical features, imaging and pathological findings, treatments and outcome were retrospectively reviewed for 6 patients with KLA and 7 patients with KHE involving the thorax.
resultsThe mean ages at the time of the presentation of signs/symptoms were 26.7 months and 4.1 months for KLA and KHE, respectively. Respiratory symptoms, pericardial and pleural effusion, thrombocytopenia and coagulopathy were common in both KLA and KHE. Diffuse lesions involving the lung and extrathoracic sites were observed in KLA but not in KHE. Histopathologically, all lesions had spindled tumor cells, which were immunoreactive for CD31 and D2-40. In KLA, the spindle cells were distributed in sparse and poorly marginated clusters, whereas the spindle cells formed more defined and confluent vascularized nodules in KHE. Unlike the refractory behavior of KLA, the majority of patients with KHE responded to medical treatments with regression of the lesion and normalization of the hematologic parameters.
conclusionsThe presenting and histological characteristics of KLA can overlap with those of KHE. The presence of diffuse vascular lesions in the mediastinum and lung with refractory thrombocytopenia and coagulopathy should suggest the diagnosis of KLA. Given the rarity and high morbidity and mortality of these disorders, the diagnostic process and therapeutic approach should include a multidisciplinary team review and consensus.
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