Kaposiform hemangioendothelioma

Common Name(s)

Kaposiform hemangioendothelioma

A Kaposiform hemangioendothelioma (KHE) is a very rare non-cancerous (benign) vascular tumor (affecting the blood vessels) that occurs in children and resembles a birthmark. Most birthmarks go away on their own or cause no medical problems. This type of tumor however requires monitoring and treatment since it involves the vascular system. Signs and symptoms may include: a growing spot (lesion) on the skin, a lesion that swells and becomes painful, and decreased motion in the area around the lesion. KHE tumors are often associated with a more serious condition called Kasabach-Merritt phenomenon. Kasabach-Merritt phenomenon is when blood clots begin to form throughout the body potentially causing organ damage, a loss of platelets (thrombocytopenia) which causes excessive bleeding, and anemia (low levels of iron in the blood). KHE tumors can be diagnosed using MRI tests, ultrasounds, CT scans, blood tests, and by examining the tumor’s cellular appearance. KHE tumors are treatable and medications can be used to treat this kind of tumor. If they are ineffective, additional treatment may be required such as chemotherapy, radiation therapy, or surgery to remove the tumor. Because of the complexity of treatment, multiple specialists may be involved in the care of a KHE tumor.

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Advocacy and Support Organizations

 

Condition Specific Organizations

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Kaposiform hemangioendothelioma" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Kaposiform hemangioendothelioma" returned 17 free, full-text research articles on human participants. First 3 results:

Intestinal obstruction due to kaposiform hemangioendothelioma in a 1-month-old infant: A case report.
 

Author(s): Joong Kee Youn, Shin-Hoo Park, Ji-Won Han, Chaeyoun Oh, Hyun-Young Kim, Sung-Eun Jung

Journal: Medicine (Baltimore). 2017 Sep;96(37):e6974.

 

Kaposiform hemangioendothelioma (KHE) is an aggressive vascular tumor, mainly occurring in infants and young children and previously reported cases were mainly cutaneous or visceral form. Intestinal kaposiform hemangioma was first reported in 2012. Intestinal type KHE showed better ...

Last Updated: 14 Sep 2017

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Exome sequence analysis of Kaposiform hemangioendothelioma: identification of putative driver mutations.
 

Author(s): Sho Egashira, Masatoshi Jinnin, Miho Harada, Shinichi Masuguchi, Satoshi Fukushima, Hironobu Ihn

Journal: An Bras Dermatol. ;91(6):748-753.

 

Kaposiform hemangioendothelioma is a rare, intermediate, malignant tumor. The tumor's etiology remains unknown and there are no specific treatments.

Last Updated: 18 Jan 2017

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Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood.
 

Author(s): Bhagyalakshmi Atla, P V Sudhakar, Nagarjun Rao, Uma Prasad

Journal: Indian J Pathol Microbiol. ;59(3):392-4.

 

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and ...

Last Updated: 11 Aug 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Kaposiform hemangioendothelioma" returned 1 free, full-text review articles on human participants. First 3 results:

Treatment of kaposiform hemangioendothelioma and tufted angioma.
 

Author(s): Xiao Han Liu, Jia Ying Li, Xin Hua Qu, Wei Li Yan, Ling Zhang, Chi Yang, Jia Wei Zheng

Journal: Int. J. Cancer. 2016 Oct;139(7):1658-66.

 

This meta-analysis was to evaluate the efficacy of current treatment modalities for kaposiform hemangioendothelioma and tufted angioma. A systematic review was performed using PubMed (Medline), Web of Science and Embase for clinical studies. The outcome was measured by pooled response ...

Last Updated: 15 Jul 2016

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Sirolimus Versus Sirolimus Plus Prednisolone for Kaposiform Hemangioendothelioma With Kasabach-Merritt Syndrome
 

Status: Recruiting

Condition Summary: Kaposiform Hemangioendothelioma; Kasabach Merritt Phenomenon

 

Last Updated: 15 Jun 2017

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A Study to Compare Vincristine to Sirolimus for Treatment of High Risk Vascular Tumors
 

Status: Recruiting

Condition Summary: Kaposiform Hemangioendothelioma (KHE); Kasabach-Merritt Syndrome; Tufted Angioma

 

Last Updated: 11 Jul 2017

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Identification of Biomarkers for Patients With Vascular Anomalies
 

Status: Recruiting

Condition Summary: Vascular Anomaly; Generalized Lymphatic Anomaly; Kaposiform Hemangioendothelioma; Kaposiform Lymphangiomatosis; Gorham-Stout Disease

 

Last Updated: 26 Apr 2017

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