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Kaposiform hemangioendothelioma

MedGen UID:
234548
•Concept ID:
C1367420
•
Neoplastic Process
Synonyms: Congenital cutaneous multifocal kaposiform hemangioendothelioma; Kaposiform hemangio-endothelioma
SNOMED CT: Kaposiform hemangioendothelioma (403983000); Kaposiform hemangioendothelioma (66229009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016236
Orphanet: ORPHA2122

Definition

A rare low-grade malignant cutaneous or visceral vascular tumour that may be associated with severe thrombopaenia with consumption coagulopathy (Kasabach-Merritt syndrome) in pediatric patients. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKaposiform hemangioendothelioma
Follow this link to review classifications for Kaposiform hemangioendothelioma in Orphanet.

Professional guidelines

PubMed

Treatment practices and response in kaposiform hemangioendothelioma: A multicenter cohort study.
Borst AJ, Eng W, Griffin M, Ricci KW, Engel E, Adams DM, Dayneka J, Cohen-Cutler SJ, Andreoli SM, Wu MD, Wheeler AP, Heym KM, Crary SE, Nakano TA, Schulte RR, Setty BA, McLean TW, Pahl KS, Intzes S, Pateva I, Teitelbaum M, Zong Z, Li Y, Jeng MR
Pediatr Blood Cancer 2024 Mar;71(3):e30779. Epub 2023 Dec 10 doi: 10.1002/pbc.30779. PMID: 38073018
Vascular Anomalies: Diagnosis of Complicated Anomalies and New Medical Treatment Options.
Adams DM, Ricci KW
Hematol Oncol Clin North Am 2019 Jun;33(3):455-470. doi: 10.1016/j.hoc.2019.01.011. PMID: 31030813
Treatment of kaposiform hemangioendothelioma and tufted angioma.
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149

Recent clinical studies

Etiology

Vascular tumors of intermediate malignancy: An update.
Folpe AL
Hum Pathol 2024 May;147:114-128. Epub 2024 Feb 13 doi: 10.1016/j.humpath.2024.01.014. PMID: 38360216
How we approach the diagnosis of a vascular anomaly.
Heym KM, Masand PM, Margolin JF
Pediatr Blood Cancer 2022 Aug;69 Suppl 3:e29802. Epub 2022 Jun 17 doi: 10.1002/pbc.29802. PMID: 35709330
Kaposiform hemangioendothelioma: current knowledge and future perspectives.
Ji Y, Chen S, Yang K, Xia C, Li L
Orphanet J Rare Dis 2020 Feb 3;15(1):39. doi: 10.1186/s13023-020-1320-1. PMID: 32014025Free PMC Article
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article
Vascular Anomalies.
Johnson AB, Richter GT
Clin Perinatol 2018 Dec;45(4):737-749. Epub 2018 Sep 18 doi: 10.1016/j.clp.2018.07.010. PMID: 30396415

Diagnosis

Vascular Anomalies: Other Vascular Tumors.
Atherton K, Hinen H
Dermatol Clin 2022 Oct;40(4):401-423. Epub 2022 Sep 16 doi: 10.1016/j.det.2022.06.011. PMID: 36243428
Vascular tumors.
Mansfield SA, Williams RF, Iacobas I
Semin Pediatr Surg 2020 Oct;29(5):150975. Epub 2020 Sep 16 doi: 10.1016/j.sempedsurg.2020.150975. PMID: 33069294
Kaposiform hemangioendothelioma: current knowledge and future perspectives.
Ji Y, Chen S, Yang K, Xia C, Li L
Orphanet J Rare Dis 2020 Feb 3;15(1):39. doi: 10.1186/s13023-020-1320-1. PMID: 32014025Free PMC Article
What is new in endothelial neoplasia?
Papke DJ Jr, Hornick JL
Virchows Arch 2020 Jan;476(1):17-28. Epub 2019 Aug 28 doi: 10.1007/s00428-019-02651-4. PMID: 31463731
Congenital Vascular Tumors.
Goss JA, Greene AK
Otolaryngol Clin North Am 2018 Feb;51(1):89-97. doi: 10.1016/j.otc.2017.09.008. PMID: 29217070

Therapy

Progressive kaposiform hemangioendothelioma and sirolimus-related severe thrombocytopenia.
Ying H, Qiao C, Wang L, Lin X
Indian J Dermatol Venereol Leprol 2023 Jan-Frebuary;89(1):54-59. doi: 10.25259/IJDVL_799_19. PMID: 36688881
Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon.
Pati S, Das MK, Rana A, Das E, Sarkar S, Sherpa N, Datta S
Indian J Pediatr 2021 Nov;88(11):1142-1144. Epub 2021 Jun 9 doi: 10.1007/s12098-021-03817-2. PMID: 34106442
Sirolimus as initial therapy for kaposiform hemangioendothelioma and tufted angioma.
Wang H, Guo X, Duan Y, Zheng B, Gao Y
Pediatr Dermatol 2018 Sep;35(5):635-638. Epub 2018 Jul 12 doi: 10.1111/pde.13600. PMID: 29999213
The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma.
Croteau SE, Gupta D
Semin Cutan Med Surg 2016 Sep;35(3):147-52. doi: 10.12788/j.sder.2016.048. PMID: 27607323
Treatment of kaposiform hemangioendothelioma and tufted angioma.
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149

Prognosis

Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article
Vascular Anomalies.
Johnson AB, Richter GT
Clin Perinatol 2018 Dec;45(4):737-749. Epub 2018 Sep 18 doi: 10.1016/j.clp.2018.07.010. PMID: 30396415
The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma.
Croteau SE, Gupta D
Semin Cutan Med Surg 2016 Sep;35(3):147-52. doi: 10.12788/j.sder.2016.048. PMID: 27607323
Kaposiform haemangioendothelioma of the head and neck.
Wong BL, Lee VN, Tikka T, Kim D, Dwivedi RC
Crit Rev Oncol Hematol 2016 Aug;104:156-68. Epub 2016 Jun 16 doi: 10.1016/j.critrevonc.2016.06.005. PMID: 27365122
Vascular Anomalies in Pediatrics.
Foley LS, Kulungowski AM
Adv Pediatr 2015 Aug;62(1):227-55. doi: 10.1016/j.yapd.2015.04.009. PMID: 26205116

Clinical prediction guides

Overexpression Prox1 in HemECs resembles Kaposiform hemangioendothelioma and cytotoxicity of sirolimus in vitro.
Wang J, Han Q, Yan H, Yao W, Wang Z, Li K
J Pediatr Surg 2021 Jul;56(7):1203-1210. Epub 2021 Mar 26 doi: 10.1016/j.jpedsurg.2021.03.023. PMID: 33865602
Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature.
Chundriger Q, Tariq MU, Abdul-Ghafar J, Ahmed A, Din NU
Diagn Pathol 2021 Mar 15;16(1):23. doi: 10.1186/s13000-021-01080-9. PMID: 33722245Free PMC Article
Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and management.
Ji Y, Chen S, Xia C, Zhou J, Jiang X, Xu X, Yang K, Zhang X, Kong F, Lu G, Zhang Y
Orphanet J Rare Dis 2020 Nov 7;15(1):313. doi: 10.1186/s13023-020-01595-2. PMID: 33160383Free PMC Article
A not so harmless mass: Kaposiform hemangioendothelioma complicated by a Kasabach-Merritt phenomenon.
Tribolet S, Hoyoux C, Boon LM, Cheruy C, Demarche M, Jamblin P, Roberti A, Willemaers V, Viellevoye R, Rigo V, Broux I
Arch Pediatr 2019 Sep;26(6):365-369. Epub 2019 Jul 25 doi: 10.1016/j.arcped.2019.06.003. PMID: 31353149
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article

Recent systematic reviews

Infectious complications of vascular anomalies treated with sirolimus: A systematic review.
Kalbfell R, Cohen-Cutler S, Grisham E, Bereitschaft C, Borst AJ, Green AM, Willis DN, Yaeger L, Blatt J, Sisk BA
Pediatr Blood Cancer 2024 Jan;71(1):e30758. Epub 2023 Nov 6 doi: 10.1002/pbc.30758. PMID: 37933207
The use of mTOR inhibitors for the treatment of kaposiform hemangioendothelioma. A systematic review.
Maza-Morales M, Valdés-Loperena S, Durán-McKinster LC, García-Romero MT
Pediatr Dermatol 2023 May-Jun;40(3):440-445. Epub 2023 Jan 30 doi: 10.1111/pde.15262. PMID: 36716766
Vincristine and sirolimus in the treatment of kaposiform haemangioendothelioma.
Peng S, Yang K, Xu Z, Chen S, Ji Y
J Paediatr Child Health 2019 Sep;55(9):1119-1124. Epub 2019 Jan 2 doi: 10.1111/jpc.14370. PMID: 30604513
Comparison of Corticosteroid and Vincristine in Treating Kaposiform Hemangioendothelioma and Tufted Angioma: A Systematic Review and Meta-Analysis.
Yao W, Li K, Wang Z, Pei J, Liu X, Zheng S, Dong K
Eur J Pediatr Surg 2019 Oct;29(5):401-407. Epub 2018 Oct 29 doi: 10.1055/s-0038-1673708. PMID: 30372769
Treatment of kaposiform hemangioendothelioma and tufted angioma.
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149

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