MCID: KPS002
MIFTS: 35

Kaposiform Hemangioendothelioma malady

Rare diseases, Cardiovascular diseases, Fetal diseases, Cancer diseases categories

Aliases & Classifications for Kaposiform Hemangioendothelioma

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Sources:
42NIH Rare Diseases, 48Orphanet, 61UMLS, 34MESH via Orphanet, 26ICD10 via Orphanet, 62UMLS via Orphanet
See all sources

Aliases & Descriptions for Kaposiform Hemangioendothelioma:

Name: Kaposiform Hemangioendothelioma 42 48 61
Congenital Cutaneous Multifocal Kaposiform Hemangioendothelioma 42
Kaposiform Hemangio-Endothelioma 42
 
Khe 42
Kh 42


Classifications:



Characteristics (Orphanet epidemiological data):

48
kaposiform hemangioendothelioma:
Inheritance: Not applicable; Age of onset: All ages


External Ids:

Orphanet48 2122
MESH via Orphanet34 C537007
ICD10 via Orphanet26 D18.0
UMLS via Orphanet62 C1367420

Summaries for Kaposiform Hemangioendothelioma

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MalaCards based summary: Kaposiform Hemangioendothelioma, also known as congenital cutaneous multifocal kaposiform hemangioendothelioma, is related to hemangioendothelioma and lymphedema. An important gene associated with Kaposiform Hemangioendothelioma is FLT4 (fms-related tyrosine kinase 4), and among its related pathways is Lymphocyte Signaling. The compounds paraffin and vegf have been mentioned in the context of this disorder. Affiliated tissues include skin, endothelial and bone, and related mouse phenotypes are cardiovascular system and liver/biliary system.

Related Diseases for Kaposiform Hemangioendothelioma

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Diseases related to Kaposiform Hemangioendothelioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 56)
idRelated DiseaseScoreTop Affiliating Genes
1hemangioendothelioma31.5CD34, FLT4, FLI1
2lymphedema30.5FLT4, PROX1
3hemangioma30.1CD34, PROX1, FLT4, FLI1
4lymphangioma30.0FLT4, PROX1, CD34
5sarcoma30.0FLI1, FLT4, CD34
6acute leukemia29.5FLT4, CD34
7leukemia29.5CD34, FLT4, FLI1
8tufted angioma10.7
9mediastinitis10.5
10lymphangiomatosis10.5
11endotheliitis10.4
12lymphangiosarcoma10.4PROX1
13fragile x syndrome10.3
14intussusception10.2
15tonsillitis10.2
16duodenitis10.2
17hereditary lymphedema10.2
18cervicitis10.2
19duodenal obstruction10.2
20laryngitis10.2
21pancreatitis10.2
22pericardial effusion10.2
23scoliosis10.2
24hemophilia10.2
25hemangioma thrombocytopenia syndrome10.2
26soft tissue sarcoma10.2
27classic kaposi sarcoma10.2CD34
28hiv-110.0
29cutaneous leishmaniasis10.0
30spinal muscular atrophy10.0
31waldenstrom macroglobulinemia10.0
32neuronitis10.0
33presbyopia10.0
34muscular atrophy10.0
35macroglobulinemia10.0
36amyloidosis10.0
37brain stem infarction10.0
38influenza10.0
39inflammatory bowel disease10.0
40sarcoma, synovial10.0FLI1, CD34
41liposarcoma10.0CD34, FLI1
42epithelioid hemangioendothelioma10.0CD34, FLT4
43pyogenic granuloma10.0FLT4, CD34
44thrombocytopenia9.9FLI1, CD34
45factor v leiden thrombophilia9.9CD34, FLI1
46endometrial cancer9.8CD34, FLT4
47lymphoma, non-hodgkin9.8CD34, FLI1
48stomach cancer9.8CD34, FLT4
49vascular cancer9.8FLI1, FLT4, CD34
50angiosarcoma9.8CD34, FLT4, FLI1

Graphical network of the top 20 diseases related to Kaposiform Hemangioendothelioma:



Diseases related to kaposiform hemangioendothelioma

Symptoms for Kaposiform Hemangioendothelioma

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Drugs & Therapeutics for Kaposiform Hemangioendothelioma

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FDA approved drugs:

id Drug Name Active Ingredient(s)13 Pharmaceutical Company Approval Date
1
Gilotrif13 38 AFATINIB DIMALEATE Boehringer Ingelheim Approved July 2013
FDA Label: Gilotrif
Malady that Drug Treats: metastatic non-small cell lung cancer with EGFR mutations
Indications and Usage:13 GILOTRIF is a kinase inhibitor indicated for the first-line treatment of; patients with metastatic non-small cell lung cancer (NSCLC) whose tumors; have epidermal growth factor receptor (EGFR) exon 19 deletions or exon 21; (L858R) substitution mutations as detected by an FDA-approved test (1); Limitation of Use: Safety and efficacy of GILOTRIF have not been; established in patients whose tumors have other EGFR mutations (1)
DrugBank Targets:11 1. Epidermal growth factor receptor; 2. Receptor tyrosine-protein kinase erbB-2; 3. Receptor tyrosine-protein kinase erbB-4
Mechanism of Action:13 
Target: tyrosine kinase autophosphorylation
Action: inhibitor
FDA: Afatinib covalently binds to the kinase domains of EGFR (ErbB1), HER2 (ErbB2), and HER4 (ErbB4) and; irreversibly inhibits tyrosine kinase autophosphorylation, resulting in downregulation of ErbB signaling.; Afatinib demonstrated inhibition of autophosphorylation and in vitro proliferation of cell lines expressing wildtype; EGFR or those expressing selected EGFR exon 19 deletion mutations or exon 21 L858R mutations,; including some with a secondary T790M mutation, at afatinib concentrations achieved, at least transiently, in; patients. In addition, afatinib inhibited in vitro proliferation of cell lines overexpressing HER2.; Treatment with afatinib resulted in inhibition of tumor growth in nude mice implanted with tumors either; overexpressing wild type EGFR or HER2 or in an EGFR L858R/T790M double mutant model.
2
Treanda13 38 BENDAMUSTINE HYDROCHLORIDE Cephalon Approved October 2008
FDA Label: Treanda
Malady that Drug Treats: Chronic lymphocytic leukemia and B-cell non-Hodgkin s lymphoma
Indications and Usage:13 TREANDA is an alkylating drug indicated for treatment of patients with:; Chronic lymphocytic leukemia (CLL). Efficacy relative to first line; therapies other than chlorambucil has not been established. (1.1); Indolent B-cell non-Hodgkin lymphoma (NHL) that has progressed during; or within six months of treatment with rituximab or a rituximab-containing; regimen. (1.2)
DrugBank Targets: -
Mechanism of Action:13 
Target: -
Action: -
FDA: Bendamustine is a bifunctional mechlorethamine derivative containing a purine-like benzimidazole ring.; Mechlorethamine and its derivatives form electrophilic alkyl groups. These groups form covalent bonds with electronrich; nucleophilic moieties, resulting in interstrand DNA crosslinks. The bifunctional covalent linkage can lead to cell; death via several pathways. Bendamustine is active against both quiescent and dividing cells. The exact mechanism of; action of bendamustine remains unknown.

Drug clinical trials:

Search ClinicalTrials for Kaposiform Hemangioendothelioma

Search NIH Clinical Center for Kaposiform Hemangioendothelioma

Genetic Tests for Kaposiform Hemangioendothelioma

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Anatomical Context for Kaposiform Hemangioendothelioma

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MalaCards organs/tissues related to Kaposiform Hemangioendothelioma:

31
Skin, Endothelial, Bone, Spleen, Thymus, Kidney, Breast, Cervix, Tonsil, Tongue

Animal Models for Kaposiform Hemangioendothelioma or affiliated genes

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MGI Mouse Phenotypes related to Kaposiform Hemangioendothelioma:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.8PROX1, FLT4, FLI1
2MP:00053708.5FLI1, FLT4, PROX1
3MP:00053877.9CD34, PROX1, FLT4, FLI1

Publications for Kaposiform Hemangioendothelioma

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Articles related to Kaposiform Hemangioendothelioma:

(show top 50)    (show all 103)
idTitleAuthorsYear
1
The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon. (25692091)
2015
2
Kaposiform hemangioendothelioma: therapeutic efficacy for an enigmatic diagnosis. (25559734)
2015
3
CyberKnife Treatment for Kaposiform Hemangioendothelioma of the Ilium in an adult: case report and review of the literature. (25138302)
2014
4
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome mistaken for child abuse in a newborn. (24738105)
2014
5
Infantile hemangioma or kaposiform hemangioendothelioma? (24745913)
2014
6
A rare case of kaposiform hemangioendothelioma presenting as intussusception in a 4-month-old child without Kasabach-Merrit syndrome: A case report. (25336808)
2014
7
Multifocal kaposiform hemangioendothelioma of soft tissue with bilateral pulmonary involvement in an adolescent. (25550706)
2014
8
Successful treatment of kaposiform hemangioendothelioma with everolimus. (25306933)
2014
9
Multifocal kaposiform hemangioendothelioma in multiple visceral organs: an autopsy of 9-day-old female baby. (24931465)
2014
10
Paclitaxel-based chemotherapy for aggressive kaposiform hemangioendothelioma of the temporomastoid region: Case report and review of the literature. (22907922)
2013
11
Microscopic Kaposiform hemangioendothelioma with extensive lymphangiomatosis: an extraordinary example of an unusual entity. (23129838)
2013
12
Congenital Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon Successfully Treated with Low-Dose Radiation Therapy. (23458157)
2013
13
Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: ultrastructural observation and immunohistochemistry staining reveal the trapping of blood components. (24134598)
2013
14
Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. (22871490)
2013
15
Kaposiform hemangioendothelioma in the uterine cervix of a 5-year girl. (22432747)
2012
16
Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon. (22648868)
2012
17
Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: from vincristine to sirolimus. (23070861)
2012
18
Capillary-lymphatic malformation, kaposiform hemangioendothelioma and delayed Kasabach-Merritt phenomenon. (20403115)
2011
19
Massive congenital kaposiform hemangioendothelioma of the eyelid in a neonate. (22134318)
2011
20
Kaposiform hemangioendothelioma of the breast in an adult female. (21570000)
2011
21
Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report. (21605441)
2011
22
Kaposiform hemangioendothelioma of the spleen in an adult: an initial case report. (21190143)
2011
23
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment. (21516018)
2011
24
Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. (20975337)
2010
25
Treatment of childhood kaposiform hemangioendothelioma with sirolimus. (20730884)
2010
26
Kaposiform hemangioendothelioma of the choledochus. (20850638)
2010
27
Kaposiform hemangioendothelioma of the kidney: an unusual presentation of a rare vascular neoplasm. (20588198)
2010
28
Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma. (19426625)
2009
29
Mediastinal and neck kaposiform hemangioendothelioma: report of three cases. (19706099)
2009
30
Adult-onset kaposiform hemangioendothelioma in a posttraumatic site. (19325356)
2009
31
Kaposiform hemangioendothelioma of the tongue in an adult. (19687407)
2009
32
Kaposiform hemangioendothelioma (with Kasabach Merritt syndrome) of the head and neck: case report and review of the literature. (19643504)
2009
33
Kaposiform hemangioendothelioma: a rare cause of spontaneous hemothorax in infancy. Review of the literature. (18206444)
2008
34
Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult! (18629354)
2008
35
Kaposiform hemangioendothelioma of the mediastinum in a 7-month-old boy: a case report. (16863862)
2006
36
Kaposiform hemangioendothelioma arising from the internal auditory canal. (16611794)
2006
37
Usefulness of D2-40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma. (16872472)
2006
38
Kaposiform hemangioendothelioma in an adult with an unusual presentation. (16684311)
2006
39
D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma. (15920541)
2005
40
Pathology quiz case 2. Diagnosis: Kaposiform hemangioendothelioma (KHE). (15313873)
2004
41
Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. (15163230)
2004
42
Transformation between Kaposiform hemangioendothelioma and tufted angioma. (12771476)
2003
43
Kaposiform hemangioendothelioma associated with Milroy's disease (primary hereditary lymphedema). (12861592)
2003
44
Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome. (14507321)
2003
45
Successful nonoperative therapy for Kaposiform hemangioendothelioma involving the neck: report of 1 case. (14595290)
2003
46
Kaposiform hemangioendothelioma of the external auditory canal in an adult. (12112561)
2002
47
A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging. (11727024)
2001
48
Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. (9591789)
1998
49
Successful multimodal therapy for kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: case report and review of the literature. (9658430)
1998
50
Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. (9385948)
1997

Variations for Kaposiform Hemangioendothelioma

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Expression for genes affiliated with Kaposiform Hemangioendothelioma

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Search GEO for disease gene expression data for Kaposiform Hemangioendothelioma.

Pathways for genes affiliated with Kaposiform Hemangioendothelioma

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Pathways related to Kaposiform Hemangioendothelioma according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.1CD34, FLI1

Compounds for genes affiliated with Kaposiform Hemangioendothelioma

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Sources:
44Novoseek
See all sources

Compounds related to Kaposiform Hemangioendothelioma according to GeneCards Suite gene sharing:

idCompoundScoreTop Affiliating Genes
1paraffin449.1CD34, FLT4
2vegf448.2CD34, PROX1, FLT4

GO Terms for genes affiliated with Kaposiform Hemangioendothelioma

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Biological processes related to Kaposiform Hemangioendothelioma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1lymphangiogenesisGO:00019469.3PROX1, FLT4
2positive regulation of cell proliferationGO:00082849.3PROX1, FLT4
3positive regulation of endothelial cell migrationGO:00105959.2PROX1, FLT4
4positive regulation of endothelial cell proliferationGO:00019389.0PROX1, FLT4

Sources for Kaposiform Hemangioendothelioma

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2CDC
12ExPASy
13FDA
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet