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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

Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options.
Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B
World J Pediatr 2018 Aug;14(4):322-329. Epub 2018 Jul 27 doi: 10.1007/s12519-018-0171-5. PMID: 30054848
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
Management of hemangiomas and other vascular tumors.
Greene AK
Clin Plast Surg 2011 Jan;38(1):45-63. doi: 10.1016/j.cps.2010.08.001. PMID: 21095471

Recent clinical studies

Etiology

How we approach coagulopathy with vascular anomalies.
Mack JM, Crary SE
Pediatr Blood Cancer 2022 Aug;69 Suppl 3:e29353. doi: 10.1002/pbc.29353. PMID: 36070211
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
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
Kaposiform hemangioendothelioma without cutaneous involvement.
Ji Y, Chen S, Li L, Yang K, Xia C, Li L, Yang G, Kong F, Lu G, Liu X
J Cancer Res Clin Oncol 2018 Dec;144(12):2475-2484. Epub 2018 Oct 6 doi: 10.1007/s00432-018-2759-5. PMID: 30293120

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
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
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
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

A Case of Superficial Kaposiform Hemangioendothelioma Treated with Oral Propranolol Combined with Topical Sirolimus.
Dang N, Ren Y
Vasc Health Risk Manag 2024;20:251-254. Epub 2024 Jun 12 doi: 10.2147/VHRM.S461505. PMID: 38883398Free PMC Article
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
Vascular anomaly cases for the pediatric hematologist oncologists-An interdisciplinary review.
Adams DM, Brandão LR, Peterman CM, Gupta A, Patel M, Fishman S, Trenor CC
Pediatr Blood Cancer 2018 Jan;65(1) Epub 2017 Jul 20 doi: 10.1002/pbc.26716. PMID: 28727248
Management of vascular tumors.
Hochman M
Facial Plast Surg 2012 Dec;28(6):584-9. Epub 2012 Nov 27 doi: 10.1055/s-0032-1329933. PMID: 23188685
Medical management of vascular anomalies.
Blei F
Facial Plast Surg 2012 Dec;28(6):575-83. Epub 2012 Nov 27 doi: 10.1055/s-0032-1329932. PMID: 23188684

Prognosis

Medical Management of Nonmalignant Vascular Tumors of the Head and Neck: Part 1.
Gatts J, Chandra S, Krishnan D, Ricci K
Oral Maxillofac Surg Clin North Am 2024 Feb;36(1):103-113. Epub 2023 Oct 23 doi: 10.1016/j.coms.2023.09.011. PMID: 37875385
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
Kaposiform hemangioendothelioma without cutaneous involvement.
Ji Y, Chen S, Li L, Yang K, Xia C, Li L, Yang G, Kong F, Lu G, Liu X
J Cancer Res Clin Oncol 2018 Dec;144(12):2475-2484. Epub 2018 Oct 6 doi: 10.1007/s00432-018-2759-5. PMID: 30293120
Kaposiform haemangioendothelioma: a review with emphasis on histological differential diagnosis.
Putra J, Gupta A
Pathology 2017 Jun;49(4):356-362. Epub 2017 Apr 21 doi: 10.1016/j.pathol.2017.03.001. PMID: 28438388
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

A Case of Superficial Kaposiform Hemangioendothelioma Treated with Oral Propranolol Combined with Topical Sirolimus.
Dang N, Ren Y
Vasc Health Risk Manag 2024;20:251-254. Epub 2024 Jun 12 doi: 10.2147/VHRM.S461505. PMID: 38883398Free PMC Article
Sirolimus therapy for kaposiform hemangioendothelioma with long-term follow-up.
Wang Z, Yao W, Sun H, Dong K, Ma Y, Chen L, Zheng S, Li K
J Dermatol 2019 Nov;46(11):956-961. Epub 2019 Sep 5 doi: 10.1111/1346-8138.15076. PMID: 31489702
Identical Presentation of Scapular Osteolysis in Two Patients with Thoracic Kaposiform Hemangioendothelioma.
Alonso Arroyo V, Lopez-Gutierrez JC, Martinez R, Fernandez-Pineda I
Pediatr Dermatol 2017 May;34(3):e146-e149. Epub 2017 Apr 6 doi: 10.1111/pde.13114. PMID: 28382716
Vascular congenital dermatofibrosarcoma protuberans: a new histological variant of dermatofibrosarcoma protuberans.
Tantcheva-Poor I, Marathovouniotis N, Kutzner H, Mentzel T
Am J Dermatopathol 2012 Jun;34(4):e46-9. doi: 10.1097/DAD.0b013e318234e704. PMID: 22257899
Tufted angioma with complete regression.
Lam WY, Mac-Moune Lai F, Look CN, Choi PC, Allen PW
J Cutan Pathol 1994 Oct;21(5):461-6. doi: 10.1111/j.1600-0560.1994.tb00290.x. PMID: 7868759

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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