MCID: MST001
MIFTS: 41

Mast-Cell Sarcoma

Categories: Blood diseases, Cancer diseases, Rare diseases

Aliases & Classifications for Mast-Cell Sarcoma

MalaCards integrated aliases for Mast-Cell Sarcoma:

Name: Mast-Cell Sarcoma 12 44 15 70
Mast Cell Sarcoma 58 17

Characteristics:

Orphanet epidemiological data:

58
mast cell sarcoma
Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:355
ICD9CM 34 202.6
MeSH 44 D012515
NCIt 50 C9348
SNOMED-CT 67 118615008
ICD10 32 C96.22
MESH via Orphanet 45 D012515
ICD10 via Orphanet 33 C96.2
UMLS via Orphanet 71 C0036221
Orphanet 58 ORPHA66661
UMLS 70 C0036221

Summaries for Mast-Cell Sarcoma

Disease Ontology : 12 A sarcoma that has material basis in mast cells.

MalaCards based summary : Mast-Cell Sarcoma, also known as mast cell sarcoma, is related to spindle cell sarcoma and hypereosinophilic syndrome. An important gene associated with Mast-Cell Sarcoma is KIT (KIT Proto-Oncogene, Receptor Tyrosine Kinase), and among its related pathways/superpathways are NF-kappaB Signaling and Hematopoietic cell lineage. The drugs Histamine and Cyproheptadine have been mentioned in the context of this disorder. Affiliated tissues include colon, skin and bone marrow, and related phenotypes are sarcoma and mastocytosis

Wikipedia : 73 Mast cell sarcoma is an extremely aggressive form of sarcoma made up of neoplastic mast cells. A sarcoma... more...

Related Diseases for Mast-Cell Sarcoma

Diseases related to Mast-Cell Sarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 91)
# Related Disease Score Top Affiliating Genes
1 spindle cell sarcoma 31.0 VIM KIT
2 hypereosinophilic syndrome 29.9 MPO KIT FIP1L1
3 mastocytosis 29.8 KITLG KIT FIP1L1 CD2
4 mast-cell leukemia 29.8 MPO KITLG KIT
5 systemic mastocytosis 29.5 KITLG KIT FIP1L1 CD2
6 mast cell neoplasm 29.4 KITLG KIT CMA1 CD2
7 extracutaneous mastocytoma 29.2 KITLG KIT FIP1L1 CD2
8 mastocytosis, cutaneous 28.8 KITLG KIT FIP1L1 CMA1 CD2
9 indolent systemic mastocytosis 28.5 KITLG KIT FIP1L1 CMA1 CD2
10 sarcoma 10.6
11 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.2 MPO KIT
12 testicular gonadoblastoma 10.2 VIM KIT
13 liver fibroma 10.2 VIM KIT
14 senile angioma 10.2 VIM KIT
15 aleukemic leukemia cutis 10.2 MPO ANPEP
16 esophageal adenoid cystic carcinoma 10.2 VIM KIT
17 vaginal endometrial stromal sarcoma 10.2 VIM KIT
18 vagina sarcoma 10.2 VIM KIT
19 benign breast phyllodes tumor 10.2 VIM KIT
20 bladder leiomyoma 10.2 VIM KIT
21 embryonal sarcoma 10.2 VIM KIT
22 acute proliferative glomerulonephritis 10.2 MPO C5AR1
23 polymorphous low-grade adenocarcinoma 10.2 VIM KIT
24 benign mastocytoma 10.2 KIT CD2
25 nasal cavity benign neoplasm 10.1 KIT CD2
26 acral lentiginous melanoma 10.1 VIM KIT
27 pleomorphic liposarcoma 10.1 VIM KIT
28 adenosarcoma 10.1 VIM KIT
29 perineurioma 10.1 VIM KIT
30 pericardium disease 10.1 MPO KIT
31 perivascular epithelioid cell tumor 10.1 VIM KIT
32 pseudosarcomatous fibromatosis 10.1 VIM KIT
33 hypersensitivity reaction type iii disease 10.1 MPO C5AR1
34 fibrous histiocytoma 10.1 VIM KIT
35 nephroma 10.1 VIM KIT
36 dendritic cell tumor 10.1 VIM MPO
37 hemangiopericytoma, malignant 10.1 VIM KIT
38 myeloid and lymphoid neoplasms associated with pdgfra rearrangement 10.1 KIT FIP1L1
39 hypereosinophilic syndrome, idiopathic 10.1 KIT FIP1L1
40 mixed phenotype acute leukemia 10.1 MPO ANPEP
41 smooth muscle tumor 10.1 VIM KIT
42 reticulum cell sarcoma 10.1 VIM CD2
43 vascular cancer 10.1 KIT ANPEP
44 urticaria 10.1
45 cutaneous mastocytoma 10.1
46 renal oncocytoma 10.1 VIM KIT
47 cavernous hemangioma 10.1 VIM KIT
48 myeloid sarcoma 10.0 MPO KIT ANPEP
49 acute megakaryocytic leukemia 10.0 MPO KIT ANPEP
50 dysgerminoma 10.0 KITLG KIT

Graphical network of the top 20 diseases related to Mast-Cell Sarcoma:



Diseases related to Mast-Cell Sarcoma

Symptoms & Phenotypes for Mast-Cell Sarcoma

Human phenotypes related to Mast-Cell Sarcoma:

58 31 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 sarcoma 58 31 hallmark (90%) Very frequent (99-80%) HP:0100242
2 mastocytosis 58 31 hallmark (90%) Very frequent (99-80%) HP:0100495
3 splenomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0001744
4 hepatomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0002240
5 fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012378
6 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
7 mediastinal lymphadenopathy 58 31 frequent (33%) Frequent (79-30%) HP:0100721
8 hypoplasia of the ear cartilage 58 31 frequent (33%) Frequent (79-30%) HP:0100720
9 lymphadenopathy 58 Frequent (79-30%)

GenomeRNAi Phenotypes related to Mast-Cell Sarcoma according to GeneCards Suite gene sharing:

26 (show all 14)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-100 9.5 MPO
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-104 9.5 KITLG
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-134 9.5 VIM
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-146 9.5 VIM
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-176 9.5 MPO VIM
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-186 9.5 MPO
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-190 9.5 VIM
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-212 9.5 VIM
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-29 9.5 VIM
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-30 9.5 VIM
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-36 9.5 MPO
12 Decreased shRNA abundance (Z-score < -2) GR00366-A-41 9.5 KITLG
13 Decreased shRNA abundance (Z-score < -2) GR00366-A-85 9.5 VIM
14 Decreased shRNA abundance (Z-score < -2) GR00366-A-91 9.5 VIM

MGI Mouse Phenotypes related to Mast-Cell Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.5 ANPEP C5AR1 CMA1 KIT KITLG MPO
2 immune system MP:0005387 9.17 ANPEP C5AR1 CMA1 KIT KITLG MPO

Drugs & Therapeutics for Mast-Cell Sarcoma

Drugs for Mast-Cell Sarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Histamine Approved, Investigational Phase 3 51-45-6, 75614-87-8 774
2
Cyproheptadine Approved Phase 3 129-03-3 2913
3 Gastrointestinal Agents Phase 3
4 Neurotransmitter Agents Phase 3
5 Dermatologic Agents Phase 3
6
Histamine Phosphate Phase 3 51-74-1 65513
7 Histamine H1 Antagonists Phase 3
8 Histamine Antagonists Phase 3
9 Anti-Allergic Agents Phase 3
10
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
2 Evaluation of Patient Satisfaction With and Acceptance of Their Totally-implanted Central Venous Catheter: Confirmatory Validation of QASICC Questionnaire Terminated NCT01616537

Search NIH Clinical Center for Mast-Cell Sarcoma

Cochrane evidence based reviews: mast-cell sarcoma

Genetic Tests for Mast-Cell Sarcoma

Anatomical Context for Mast-Cell Sarcoma

MalaCards organs/tissues related to Mast-Cell Sarcoma:

40
Colon, Skin, Bone Marrow, Small Intestine, Uterus, Myeloid

Publications for Mast-Cell Sarcoma

Articles related to Mast-Cell Sarcoma:

(show top 50) (show all 57)
# Title Authors PMID Year
1
Mast cell sarcoma with concurrent mast cell leukaemia. 61
32242922 2020
2
[Cutaneous Manifestations in Mastocytosis: Update]. 61
32238242 2020
3
Redistribution, homing and organ-invasion of neoplastic stem cells in myeloid neoplasms. 61
31408723 2020
4
Allogeneic Hematopoietic Stem Cell Transplantation in a Rare Case of Tonsillar Mast Cell Sarcoma. 61
32181155 2020
5
Prevalence of CD30 immunostaining in neoplastic mast cells: A retrospective immunohistochemical study. 61
29794740 2018
6
Mastocytosis - pathogenesis, clinical manifestation and treatment. 61
29314691 2018
7
The first report of human primary thoracic spine mast cell sarcoma: a case report. 61
28992848 2018
8
Immunoreactivity to CYP24A1, but not vitamin D receptor, is increased in mast cells of keratinocyte skin cancers. 61
29165303 2017
9
Natural history and treatment of cutaneous and systemic mastocytosis. 61
28770635 2017
10
Acute mast cell leukemia associated with t(4;5)(q21;q33). 61
28412213 2017
11
R634W KIT Mutation in an Adult With Systemic Mastocytosis. 61
28520972 2017
12
Anaplastic mast cell sarcoma: a unique pathologic entity in mastocytosis. 61
27808598 2017
13
Mast cell sarcoma of the sternum, clonally related to an antecedent germ cell tumor with a novel D579del KIT mutation. 61
28236058 2017
14
Mast cell sarcoma: new cases and literature review. 61
27602777 2016
15
Update on Mastocytosis (Part 2): Categories, Prognosis, and Treatment. 61
26525106 2016
16
FDG-PET/CT findings in systemic mastocytosis: a French multicentre study. 61
26140850 2015
17
Mast cell sarcoma mimicking metastatic colon carcinoma. 61
24141335 2014
18
Mast cell sarcoma: clinical management. 61
24745684 2014
19
Mast cell sarcoma in an infant: a case report and review of the literature. 61
23211696 2013
20
Mast cell sarcoma: a rare and potentially under-recognized diagnostic entity with specific therapeutic implications. 61
23196796 2013
21
Pediatric mast cell sarcoma of temporal bone with novel L799F (2395 C>T) KIT mutation, mimicking histiocytic neoplasm. 61
23388130 2013
22
Mast cell sarcoma: a rare and aggressive entity--report of two cases and review of the literature. 61
23129735 2013
23
Mast cell sarcoma with megakaryocytic differentiation in a calf. 61
22813945 2012
24
Mast cell sarcoma of the scalp: the first sign of undisclosed systemic mastocytosis? 61
22963840 2012
25
Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features. 61
22943012 2012
26
Malignant transformation of mastocytoma developed on skin mastocytosis into cutaneous mast cell sarcoma. 61
22498828 2012
27
Systemic mastocytosis--a systematic review. 61
22381091 2012
28
Systemic mastocytosis. 61
21629042 2011
29
Mast cell sarcoma of the small intestine: a case report. 61
21778298 2011
30
Successful treatment of mast cell sarcoma of the uterus with imatinib. 61
22020400 2011
31
[Diagnosis of hematolymphoid malignancy by using effusion fluid cytology specimens: a study of 33 cases]. 61
20021966 2009
32
Mast-cell sarcoma of the tibia. 61
17405977 2007
33
Pathogenesis, clinical features, and treatment advances in mastocytosis. 61
16781490 2006
34
[Systemic mastocytosis. Classification, symptoms, therapy]. 61
15024485 2004
35
Characterization of novel stem cell factor responsive human mast cell lines LAD 1 and 2 established from a patient with mast cell sarcoma/leukemia; activation following aggregation of FcepsilonRI or FcgammaRI. 61
12801524 2003
36
Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma. 61
12826896 2003
37
Evolution of urticaria pigmentosa into indolent systemic mastocytosis: abnormal immunophenotype of mast cells without evidence of c-kit mutation ASP-816-VAL. 61
12688351 2003
38
Aggressive systemic mastocytosis. 61
12639621 2003
39
Mast cells with bilobed or multilobed nuclei in a nodular lesion of a patient with urticaria pigmentosa. 61
12454601 2002
40
Diagnostic criteria and classification of mastocytosis: a consensus proposal. 61
11377686 2001
41
Temporary response of localized intracranial mast cell sarcoma to combination chemotherapy. 61
11216707 2001
42
Mast cell sarcoma with tissue eosinophilia arising in the ascending colon. 61
10430280 1999
43
[Association of bone marrow mastocytosis with extremely immature extramedullary mast cell sarcoma]. 61
9273545 1997
44
What is your diagnosis? Mast cell sarcoma. 61
9097238 1997
45
Brief communication, Histopathology of a spontaneously developing mast cell sarcoma in a Wistar rat. 61
8736393 1996
46
Involvement of the larynx by hemopoietic neoplasms. An investigation of autopsy cases and review of the literature. 61
7567682 1995
47
[The larynx in lymphoproliferative and myeloproliferative diseases. Part II: Laryngeal autopsy findings and discussion]. 61
7928429 1994
48
[The larynx in lymphoproliferative and myeloproliferative diseases. I: An overview with special reference to primary laryngeal malignant lymphomas and plasmacytomas]. 61
8071093 1994
49
Use of hyperthermia and radiotherapy in treatment of a large mast cell sarcoma in a dog. 61
3145930 1988
50
Mast cell sarcoma of the larynx. 61
3088063 1986

Variations for Mast-Cell Sarcoma

Expression for Mast-Cell Sarcoma

Search GEO for disease gene expression data for Mast-Cell Sarcoma.

Pathways for Mast-Cell Sarcoma

GO Terms for Mast-Cell Sarcoma

Cellular components related to Mast-Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytoplasmic side of plasma membrane GO:0009898 8.62 KIT CD2

Biological processes related to Mast-Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 ovarian follicle development GO:0001541 9.16 KITLG KIT
2 embryonic hemopoiesis GO:0035162 8.96 KITLG KIT
3 ectopic germ cell programmed cell death GO:0035234 8.62 KITLG KIT

Sources for Mast-Cell Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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