Kaposiform Hemangioendothelioma

A rare, locally aggressive vascular neoplasm that primarily occurs in childhood. It originates on the skin and is characterized by one or multiple masse; it is often comorbid with Kasabach-Merritt syndrome and lymphangiomatosis. The viscera is usually not affected, but invasion of bone, retroperitoneum, and MEDIASTINUM has occured in some cases. No metastases have been reported.

Also Known As:

Networked: 113 relevant articles (10 outcomes, 6 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Tufted angioma
2.	Kasabach-Merritt Syndrome
3.	Laryngomalacia
4.	Neoplasms (Cancer)
5.	Thrombocytopenia (Thrombopenia)

Experts

1.	Li, Kai: 11 articles (07/2022 - 08/2014)
2.	Yao, Wei: 10 articles (07/2022 - 03/2014)
3.	Wang, Zuopeng: 10 articles (07/2021 - 03/2014)
4.	Zheng, Shan: 8 articles (05/2021 - 08/2014)
5.	Dong, Kuiran: 7 articles (07/2020 - 03/2014)
6.	Ji, Yi: 5 articles (01/2022 - 01/2017)
7.	Chen, Siyuan: 4 articles (01/2022 - 01/2017)
8.	Xiao, Xianmin: 4 articles (04/2015 - 03/2014)
9.	Wang, Jing: 3 articles (07/2022 - 07/2021)
10.	Ozeki, Michio: 3 articles (01/2022 - 08/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Kaposiform Hemangioendothelioma:

1.	Sirolimus (Rapamycin)FDA Link 01/01/2022 - "Bone kaposiform hemangioendothelioma: A rare entity dramatically improved by sirolimus." 03/01/2014 - "Sirolimus, a promising treatment for refractory Kaposiform hemangioendothelioma." 01/01/2018 - "Eventually, multifocal lesions of kaposiform hemangioendothelioma disappeared on the images of magnetic resonance imaging and have remained in remission for 27 months after sirolimus cessation. " 09/01/2019 - "However, a PPK model of sirolimus in Chinese patients with pediatric kaposiform hemangioendothelioma (PKHE) has yet to be established; therefore, this was the purpose of the present study. " 08/01/2023 - "Long-term outcomes of sirolimus treatment for kaposiform hemangioendothelioma: Continuing successes and ongoing challenges."
2.	Vincristine (Oncovin)FDA LinkGeneric 12/23/2019 - "CONCLUSIONS Bifocal tibial kaposiform hemangioendothelioma lesions are unique in pediatric patients and can be successfully treated with vincristine chemotherapy. " 12/23/2019 - "Bifocal Tibial Kaposiform Hemangioendothelioma Responsive to Vincristine Therapy: A Case Report." 03/01/2012 - "Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with embolization and vincristine in two newborns." 08/01/2010 - "Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine." 05/01/2009 - "To report a case of a newborn with a kaposiform hemangioendothelioma (KHE) of the right leg associated with KMS treated successfully with vincristine and oral corticosteroids. "
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 11/01/1998 - "Inoperable kaposiform hemangioendothelioma unresponsive to corticosteroids can be successfully treated with chemotherapy." 01/01/2018 - "We present a patient with a thigh kaposiform hemangioendothelioma successfully treated using a systemic corticosteroid during infancy who was diagnosed with lymphedema in the extremity 9 years later. " 10/01/2012 - "A 7-day-old male infant with Kasabach-Merritt syndrome arising from kaposiform hemangioendothelioma was successfully treated with systemic corticosteroid and surgery. " 10/01/2012 - "Successful treatment of Kasabach-Merritt syndrome arising from kaposiform hemangioendothelioma by systemic corticosteroid therapy and surgery." 11/01/1998 - "The authors introduced a successful trial of a chemotherapy regimen to treat refractory thrombocytopenia and pain caused by a rare kaposiform hemangioendothelioma that had been refractory to corticosteroids. "
4.	TOR Serine-Threonine KinasesIBA 11/01/2019 - "Mammalian target of rapamycin inhibitors have shown promising results in the management of kaposiform hemangioendothelioma (KHE). " 01/01/2018 - "We demonstrated that the AKT/mammalian target of rapamycin signaling pathway played a pivotal role in the kaposiform hemangioendothelioma growth. " 08/01/2023 - "Treatment with sirolimus, an inhibitor of the mammalian target of rapamycin pathway, has improved the prognosis of patients with kaposiform hemangioendothelioma (KHE). " 01/01/2023 - "Sirolimus, a mammalian target of rapamycin inhibitor, has been widely used to treat kaposiform hemangioendothelioma. " 01/01/2020 - "Mammalian target of rapamycin (mTOR) inhibitors have been shown to have excellent effects in the management of kaposiform hemangioendothelioma (KHE); however, the mechanism of action is unclear. "
5.	Ticlopidine (Ticlid)FDA LinkGeneric 10/01/2012 - "Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with vincristine and ticlopidine." 05/01/2009 - "Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine." 09/01/2013 - "This study aimed to clarify the combinatorial treatment effect of agents as aspirin and ticlopidine associated with vincristine in the management of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). "
6.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 01/01/2018 - "Management of Refractory Pediatric Kaposiform Hemangioendothelioma With Sirolimus and Aspirin." 09/01/2013 - "This study aimed to clarify the combinatorial treatment effect of agents as aspirin and ticlopidine associated with vincristine in the management of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenic coagulopathy that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). "
7.	DNA (Deoxyribonucleic Acid)IBA 11/01/2016 - "In this study, we performed exome sequencing using DNA from a Kaposiform hemangioendothelioma patient, and found putative candidates for the responsible mutations. "
8.	Propranolol (Inderal)FDA LinkGeneric 01/01/2022 - "To compare the clinical efficacy and safety of two different doses of propranolol in the treatment of cutaneous kaposiform hemangioendothelioma (KHE). " 01/01/2022 - "Comparison of Effectiveness of Two Different Doses of Propranolol on Kaposiform Hemangioendothelioma." 01/01/2016 - "Successful Propranolol Treatment of a Kaposiform Hemangioendothelioma Apparently Resistant to Propranolol." 01/01/2014 - "We herein reported the successful treatment of mild pediatric KMP with propranolol monotherapy in a case of a five-week-old child with kaposiform hemangioendothelioma with successful treatment of both clinical and hematologic responses. " 05/01/2011 - "Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment."
9.	SteroidsIBA 01/01/2024 - "These were infants with kaposiform hemangioendothelioma (KHE) who were also treated with steroids and did not receive PJP prophylaxis. " 11/01/2015 - "Comment on: Steroid-resistant kaposiform hemangioendothelioma: A retrospective study of 37 patients treated with vincristine and long-term follow-up." 04/01/2015 - "Steroid-resistant kaposiform hemangioendothelioma: a retrospective study of 37 patients treated with vincristine and long-term follow-up." 09/01/2017 - "This report describes a case of kaposiform hemangioendothelioma arising in an adult man during the course of rheumatoid arthritis treated with steroids and methotrexate. "
10.	Prednisolone (Predate)FDA LinkGeneric 01/01/2022 - "Sirolimus plus prednisolone vs sirolimus monotherapy for kaposiform hemangioendothelioma: a randomized clinical trial." 03/01/2015 - "There is currently no consensus on the second-line management of Kaposiform hemangioendothelioma (KHE) that was resistant to prednisolone and vincristine. " 12/01/2018 - "Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in an infant: Successful treatment with prednisolone, vincristine, and addition of sirolimus." 01/01/2018 - "We report the case of a child with kaposiform hemangioendothelioma and the Kasabach-Merritt phenomenon who developed Pneumocystis carinii pneumonia (PCP) while on sirolimus and a prednisolone taper, after lack of adequate response to prednisolone, propranolol, and vincristine. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2020 - "Accordingly, systemic drug therapies have emerged as attractive options for some of the mediastinal vascular tumors, such as kaposiform hemangioendothelioma (KHE) and Kaposi sarcoma (KS). " 12/23/2019 - "CONCLUSIONS Bifocal tibial kaposiform hemangioendothelioma lesions are unique in pediatric patients and can be successfully treated with vincristine chemotherapy. " 11/01/1998 - "Inoperable kaposiform hemangioendothelioma unresponsive to corticosteroids can be successfully treated with chemotherapy." 08/01/2013 - "Paclitaxel-based chemotherapy for aggressive kaposiform hemangioendothelioma of the temporomastoid region: Case report and review of the literature." 01/01/2021 - "Combined Transarterial Sclerotherapy and Embolization with Systemic Chemotherapy Followed by Everolimus in Kaposiform Hemangioendothelioma with Kasabach-Merritt Syndrome."
2.	Therapeutics 12/01/2021 - "This study aimed to analyze the angiographic characteristics of kaposiform hemangioendothelioma (KHE) and investigate the value of transcatheter arterial embolization (TAE) therapy. " 12/01/2021 - "Analysis of angiographic characteristics of kaposiform hemangioendothelioma and investigation of the value of transcatheter arterial embolization therapy." 11/01/2014 - "The therapy of complicated Kaposiform hemangioendothelioma (KHE) is still difficult. " 11/01/2003 - "Successful nonoperative therapy for Kaposiform hemangioendothelioma involving the neck: report of 1 case." 08/01/2023 - "Medical and interventional therapy of Kasabach-Merritt phenomenon associated with Kaposiform hemangioendothelioma: A case report."
3.	Sclerotherapy 01/01/2022 - "A retrospective study was conducted using medical records of all children with a diagnosis of kaposiform hemangioendothelioma (KHE) or tufted angioma (TA) associated with KMP treated with medicine, intralesional laser photocoagulation (ILP), and sclerotherapy between February 2017 and November 2020. " 01/01/2021 - "Combined Transarterial Sclerotherapy and Embolization with Systemic Chemotherapy Followed by Everolimus in Kaposiform Hemangioendothelioma with Kasabach-Merritt Syndrome."
4.	Platelet Transfusion (Blood Platelet Transfusions) 11/01/2021 - "Paradoxical worsening of symptoms with platelet transfusions and radiological evidences led to the diagnosis of a very rare congenital multifocal vascular tumor, kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP). "
5.	Intralesional Injections 11/01/2014 - "Treating kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon by intralesional injection of absolute ethanol."