MCID: INF058
MIFTS: 46

Inflammatory Myofibroblastic Tumor

Categories: Rare diseases, Cancer diseases

Aliases & Classifications for Inflammatory Myofibroblastic Tumor

MalaCards integrated aliases for Inflammatory Myofibroblastic Tumor:

Name: Inflammatory Myofibroblastic Tumor 12 50 56 14 69
Inflammatory Fibrosarcoma 50
Inflammatory Pseudotumor 50
Pseudotumor Inflammatory 52

Classifications:



External Ids:

Disease Ontology 12 DOID:0050905
Orphanet 56 ORPHA178342
UMLS via Orphanet 70 C0334121

Summaries for Inflammatory Myofibroblastic Tumor

NIH Rare Diseases : 50 an inflammatory myofibroblastic tumor (imt) is an uncommon, presumably benign (non-cancerous) tumor made up of cells called myofibroblastic spindle cells. it usually develops in children or young adults, but can affect people of any age. an imt can occur in almost any part of the body but is most commonly found in the lung, orbit (eye socket), peritoneum (lining of the abdominal cavity and internal organs), and mesentery. signs and symptoms vary depending on the site of the tumor. some people with an imt are asymptomatic, while others may have nonspecific respiratory symptoms, fever, or pain. imts may recur, and occasionally become locally invasive and/or spread (metastasize) to other parts of the body. the underlying cause of imts is poorly understood. some cases have been linked to translocations involving the alk gene. treatment involves surgical removal when possible, although there are reports of treatment with oral steroids and radiation therapy. last updated: 2/8/2016

MalaCards based summary : Inflammatory Myofibroblastic Tumor, also known as inflammatory fibrosarcoma, is related to dedifferentiated liposarcoma and gastrointestinal stromal tumor, and has symptoms including fever and pain. An important gene associated with Inflammatory Myofibroblastic Tumor is ALK (ALK Receptor Tyrosine Kinase), and among its related pathways/superpathways are Pathways in cancer and Epstein-Barr virus infection. The drugs Cyclophosphamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include lung, eye and liver, and related phenotypes are cardiovascular system and muscle

Disease Ontology : 12 A mesenchymal cell neoplasm that has material basis in myofibroblastic cells admixed with inflammatory cells.

Related Diseases for Inflammatory Myofibroblastic Tumor

Diseases related to Inflammatory Myofibroblastic Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 212)
id Related Disease Score Top Affiliating Genes
1 dedifferentiated liposarcoma 30.6 KIT S100B
2 gastrointestinal stromal tumor 29.4 ACTC1 DES KIT S100B TP53 VIM
3 histiocytoma 29.1 ACTC1 CCND1 DES KIT TP53 VIM
4 liver inflammatory pseudotumor 12.2
5 focal myositis 11.3
6 desmoid tumor 11.0
7 infantile myofibromatosis 11.0
8 igg4-related hepatopathy 11.0
9 lymphocytic hypophysitis 10.9 ACTC1 VIM
10 igg4-related disease 10.9
11 pulmonary hyalinizing granuloma 10.9
12 infantile scoliosis 10.9 DES VIM
13 gastric signet ring cell adenocarcinoma 10.9 ACTC1 VIM
14 immune-mediated encephalomyelitis 10.9 S100B VIM
15 telo2-related intellectual disability-neurodevelopmental disorder 10.9 ACTC1 DES
16 iridogoniodysgenesis and skeletal anomalies 10.8 ACTC1 VIM
17 acute myeloid leukemia with inv3(p21;q26.2) or t(3;3)(p21;q26.2) 10.8 KIT NPM1
18 benallegue lacete syndrome 10.8 S100B VIM
19 lymphocytic infiltrate of jessner 10.8 DES VIM
20 aortic aneurysm 10.8 ACTC1 DES VIM
21 esophagus leiomyosarcoma 10.8 ACTC1 DES VIM
22 laryngeal neuroendocrine tumor 10.8 ACTC1 DES VIM
23 intellectual disability-polydactyly-uncombable hair syndrome 10.8 DES VIM
24 uniparental disomy of chromosome 11 10.8 ACTC1 DES VIM
25 myxoid leiomyosarcoma 10.8 ACTC1 DES VIM
26 berk-tabatznik syndrome 10.8 ACTC1 DES VIM
27 localized pulmonary fibrosis 10.8 ACTC1 S100B
28 follicular infundibulum tumor 10.8 ACTC1 DES VIM
29 juvenile nasopharyngeal angiofibroma 10.8 ACTC1 S100B
30 papillary glioneuronal tumor 10.8 ACTC1 S100B VIM
31 nephrogenic adenoma of urinary bladder 10.8 ACTC1 DES VIM
32 spleen cancer 10.8 DES S100B
33 small intestine neuroendocrine neoplasm 10.8 DES S100B VIM
34 multiple mitochondrial dysfunctions syndrome 2 10.8 ACTC1 DES VIM
35 vulvar squamous papilloma 10.8 DES S100B VIM
36 marfanoid habitus-inguinal hernia-advanced bone age syndrome 10.7 ACTC1 DES KIT
37 glucocorticoid-induced osteoporosis 10.7 ALK TP53
38 esophagus leiomyoma 10.7 ACTC1 DES VIM
39 skin glomus tumor 10.7 DES S100B VIM
40 hyperpituitarism 10.7 ACTC1 DES VIM
41 mannose-binding lectin protein deficiency 10.7 DES KIT VIM
42 linitis plastica 10.7 ACTC1 DES VIM
43 central breast papilloma 10.7 CR1 CR2
44 punctate palmoplantar keratoderma type 2 10.7 ACTC1 DES KIT
45 liver angiosarcoma 10.7 ACTC1 DES KIT
46 small cell neuroendocrine carcinoma 10.7 ACTC1 DES VIM
47 cerebrum cancer 10.7 ACTC1 DES VIM
48 rectal neoplasm 10.7 DES KIT VIM
49 mitochondrial metabolism disease 10.7 CCND1 S100B
50 malignant adenofibroma 10.7 ACTC1 DES VIM

Graphical network of the top 20 diseases related to Inflammatory Myofibroblastic Tumor:



Diseases related to Inflammatory Myofibroblastic Tumor

Symptoms & Phenotypes for Inflammatory Myofibroblastic Tumor

UMLS symptoms related to Inflammatory Myofibroblastic Tumor:


fever, pain

MGI Mouse Phenotypes related to Inflammatory Myofibroblastic Tumor:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.65 DES KIT MDM2 NPM1 RNF213 TP53
2 muscle MP:0005369 9.23 ACTC1 ACVRL1 DES KIT MDM2 TP53

Drugs & Therapeutics for Inflammatory Myofibroblastic Tumor

Drugs for Inflammatory Myofibroblastic Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 15)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
2
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Mechlorethamine Approved Phase 2, Phase 3 51-75-2 4033
5
Doxil Approved June 1999 Phase 2, Phase 3 31703
6 Alkylating Agents Phase 2, Phase 3
7 Anti-Bacterial Agents Phase 2, Phase 3
8 Antibiotics, Antitubercular Phase 2, Phase 3
9 Isophosphamide mustard Phase 2, Phase 3
10 Topoisomerase Inhibitors Phase 2, Phase 3
11
Crizotinib Approved Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
12 Protein Kinase Inhibitors Phase 2,Phase 1
13
Ceritinib Approved Phase 1 1032900-25-6
14 Antibodies
15 Immunoglobulins

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery Recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride
2 Phase 2 Study Assessing Efficacy and Safety of Crizotinib in Patients Harboring an Alteration on ALK, MET or ROS1 Recruiting NCT02034981 Phase 2 Crizotinib
3 Phase 1/2 Study of X-396, an Oral ALK Inhibitor, in Patients With ALK-positive Non-Small Cell Lung Cancer Recruiting NCT01625234 Phase 1, Phase 2 X-396 (ensartinib)
4 CREATE: Cross-tumoral Phase 2 With Crizotinib Active, not recruiting NCT01524926 Phase 2 Crizotinib (PF-02341066)
5 Phase I Study of LDK378 in Pediatric, Malignancies With a Genetic Alteration in Anaplastic Lymphoma Kinase (ALK) Recruiting NCT01742286 Phase 1 LDK378
6 A Prospective Cohort Study of IgG4RD in China Recruiting NCT01670695
7 Low-dose Protocol for Computed Tomography-guided Lung Biopsy Enrolling by invitation NCT02971176
8 Treatment With Crizotinib Single Patient Expanded Access IND 134375 No longer available NCT03085186 Crizotinib

Search NIH Clinical Center for Inflammatory Myofibroblastic Tumor

Genetic Tests for Inflammatory Myofibroblastic Tumor

Anatomical Context for Inflammatory Myofibroblastic Tumor

MalaCards organs/tissues related to Inflammatory Myofibroblastic Tumor:

39
Lung, Eye, Liver, Bone, Heart, Trachea, Spleen

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 563)
id Title Authors Year
1
Inflammatory myofibroblastic tumor involving liver, gallbladder, pylorus & duodenum: A rare case presentation. ( 28092784 )
2017
2
Central Nervous System Inflammatory Myofibroblastic Tumor Masquerading as Chronic Suppurative Otitis Media. ( 28904584 )
2017
3
A novel fusion of HNRNPA1-ALK in inflammatory myofibroblastic tumor of urinary bladder. ( 28504207 )
2017
4
Robot-assisted Partial Cystectomy for Treatment of Inflammatory Myofibroblastic Tumor of the Bladder. ( 28083481 )
2017
5
Targeting ALK With Crizotinib in Pediatric Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumor: A Children's Oncology Group Study. ( 28787259 )
2017
6
Pulmonary inflammatory myofibroblastic tumor: report of 2 cases with radiologic-pathologic correlation. ( 28491163 )
2017
7
Inflammatory myofibroblastic tumor of the uterus: a clinicopathological, immunohistochemical, and molecular analysis of 13 cases highlighting their broad morphologic spectrum. ( 28664932 )
2017
8
Inflammatory myofibroblastic tumor of the gallbladder: a case report and literature review. ( 28639154 )
2017
9
Inflammatory myofibroblastic tumor of the lacrimal gland: case report of an exceptional location. ( 28824333 )
2017
10
Gastric inflammatory myofibroblastic tumor presenting as fever of unknown origin in a 9-year-old girl. ( 27748672 )
2017
11
Autotransplantation of the Heart for Recurrent Inflammatory Myofibroblastic Tumor. ( 28776353 )
2017
12
Inflammatory myofibroblastic tumor of mandible: A rare case report and review of literature. ( 28479702 )
2017
13
ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis. ( 28923119 )
2017
14
Inflammatory myofibroblastic tumor of the lumbar spinal canal: A Case Report With Literature Review. ( 28658093 )
2017
15
Inflammatory myofibroblastic tumor mimicking a relapse in a patient with Hodgkin's lymphoma: report of an unusual case and review of the literature. ( 28588845 )
2017
16
A case report of tracheal inflammatory myofibroblastic tumor in a 34-week pregnant woman misdiagnosed with asthma. ( 28816991 )
2017
17
Successful Preservation of the Bladder in a Case of Inflammatory Myofibroblastic Tumor with the Diagnostic Efficacy of ALK/p80 Immunohistochemistry and FISH Analysis: Case Report and Review of the Literature. ( 28083479 )
2017
18
Pulmonary inflammatory myofibroblastic tumor versus IgG4-related inflammatory pseudotumor: differential diagnosis based on a case series. ( 28449468 )
2017
19
Gastric inflammatory myofibroblastic tumor identified as ectopic pancreas and treated by endoscopic submucosal dissection. ( 28587772 )
2017
20
Inflammatory myofibroblastic tumor: An unusual hepatic tumor mimicking liver abscess. ( 28089176 )
2017
21
Inflammatory Myofibroblastic Tumor Diagnosed by Transbronchial Needle Aspiration. ( 28645432 )
2017
22
Efficiency of Crizotinib on an ALK-Positive Inflammatory Myofibroblastic Tumor of the Central Nervous System: A Case Report. ( 28409069 )
2017
23
Rare Endobronchial Inflammatory Myofibroblastic Tumor in Pediatric Patient Detected on PET/CT Imaging. ( 28639954 )
2017
24
A Case Report of Paraneoplastic Pemphigus Associated With Retroperitoneal Inflammatory Myofibroblastic Tumor. ( 28724275 )
2017
25
Synchronous Inflammatory Myofibroblastic Tumor in Lung and Brain: A Case Report and Review of Literature. ( 28533999 )
2017
26
Intracranial Inflammatory Myofibroblastic Tumor with Sarcomatous Local Recurrence. ( 27465420 )
2016
27
Inflammatory Myofibroblastic Tumor of Liver Masquerading as Focal Nodular Hyperplasia in a Patient with Non-Cirrhotic Portal Hypertension and Biliary Pancreatitis. ( 27065695 )
2016
28
Radiologic and Clinicopathologic Findings of Inflammatory Myofibroblastic Tumor. ( 27224222 )
2016
29
Febrile Illness in an Infant With an Intracardiac Inflammatory Myofibroblastic Tumor. ( 26798044 )
2016
30
Inflammatory myofibroblastic tumor of the small intestine mimicking acute appendicitis: a case report and review of the literature. ( 27094797 )
2016
31
Inflammatory Myofibroblastic Tumor in the Bladder: A Case Report. ( 27790120 )
2016
32
ALK-rearranged Inflammatory Myofibroblastic Tumor of the Placenta, With Observations on Site of Origin. ( 27636888 )
2016
33
Diagnosing and Treating Inflammatory Myofibroblastic Tumor of the Bladder. ( 27293951 )
2016
34
Inflammatory Myofibroblastic Tumor of the Bladder: 2 Rare Cases Managed with Laparoscopic Partial Cystectomy. ( 27974989 )
2016
35
ETV6-NTRK3 Is Expressed in a Subset of ALK-Negative Inflammatory Myofibroblastic Tumors. ( 27259007 )
2016
36
An isolated inflammatory myofibroblastic tumor of adrenal gland. ( 27806843 )
2016
37
Complete and Repeated Response of a Metastatic ALK-rearranged Inflammatory Myofibroblastic Tumor to Crizotinib in a Teenage Girl. ( 26808369 )
2016
38
Inflammatory myofibroblastic tumor presenting as ileocecal intussusception-A case report. ( 27262346 )
2016
39
Successful sleeve lobectomy of pediatric inflammatory myofibroblastic tumor. ( 27804248 )
2016
40
Anaplastic lymphoma kinase positive inflammatory myofibroblastic tumor of the urachus: A rare neoplasm in an unusual location. ( 26960648 )
2016
41
Inflammatory myofibroblastic tumor with RANBP2 and ALK gene rearrangement with bland cytological features mimicking desmoid-type fibromatosis: A case report and review of the literature. ( 26893756 )
2016
42
Fever,Weight Loss and Early Satiety due to Gastric Inflammatory Myofibroblastic Tumor; Case Report and Literature Review. ( 27252821 )
2016
43
Inflammatory Myofibroblastic Tumor of the Kidney: A Rare Renal Tumor. ( 28050384 )
2016
44
Inflammatory Myofibroblastic Tumor of the Breast Coexisting with Pseudoangiomatous Stromal Hyperplasia. ( 28331757 )
2016
45
Inflammatory myofibroblastic tumor of the small intestine: A case report. ( 27046103 )
2016
46
A Case of Cutaneous Inflammatory Myofibroblastic Tumor. ( 27653445 )
2016
47
Inflammatory myofibroblastic tumor of the left atrium in infant. ( 27526369 )
2016
48
Chromoplectic TPM3-ALK rearrangement in a patient with inflammatory myofibroblastic tumor who responded to ceritinib after progression on crizotinib. ( 27742657 )
2016
49
Remission of ALK-negative primary pulmonary inflammatory myofibroblastic tumor on treatment with clarithromycin: A case report and review of the literature. ( 26998073 )
2016
50
Inflammatory myofibroblastic tumor of carotid artery resulting in recurrent syncope: A case report. ( 27028414 )
2016

Variations for Inflammatory Myofibroblastic Tumor

Expression for Inflammatory Myofibroblastic Tumor

Search GEO for disease gene expression data for Inflammatory Myofibroblastic Tumor.

Pathways for Inflammatory Myofibroblastic Tumor

GO Terms for Inflammatory Myofibroblastic Tumor

Cellular components related to Inflammatory Myofibroblastic Tumor according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 cytoskeleton GO:0005856 9.91 ACTC1 CLTC DES NPM1 TPM3 TPM4
2 cytosol GO:0005829 9.8 ACTC1 CARS CCND1 CLTC DES MDM2
3 focal adhesion GO:0005925 9.65 ACTC1 CLTC NPM1 TPM4 VIM
4 cytoplasm GO:0005737 9.53 ACTC1 CARS CCND1 CLTC DES KIT
5 actin filament GO:0005884 9.5 ACTC1 TPM3 TPM4
6 muscle thin filament tropomyosin GO:0005862 9.16 TPM3 TPM4

Biological processes related to Inflammatory Myofibroblastic Tumor according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.88 CCND1 KIT MDM2 NPM1 S100B
2 positive regulation of gene expression GO:0010628 9.65 ACTC1 KIT MDM2 TP53 VIM
3 movement of cell or subcellular component GO:0006928 9.61 TPM3 TPM4 VIM
4 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 9.5 MDM2 NPM1 TP53
5 viral process GO:0016032 9.5 CR1 CR2 MDM2 NPM1 RANBP2 TP53
6 response to magnesium ion GO:0032026 9.46 CCND1 MDM2
7 complement receptor mediated signaling pathway GO:0002430 9.4 CR1 CR2
8 mitotic G1 DNA damage checkpoint GO:0031571 9.37 CCND1 TP53
9 cellular response to actinomycin D GO:0072717 9.16 MDM2 TP53
10 muscle filament sliding GO:0030049 9.02 ACTC1 DES TPM3 TPM4 VIM

Molecular functions related to Inflammatory Myofibroblastic Tumor according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 protein homodimerization activity GO:0042803 9.65 CARS CR2 KIT NPM1 S100B
2 protein kinase binding GO:0019901 9.55 ACVRL1 CCND1 CLTC NPM1 TP53
3 identical protein binding GO:0042802 9.43 ALK DES MDM2 S100B TP53 VIM
4 disordered domain specific binding GO:0097718 8.8 CLTC MDM2 TP53
5 protein binding GO:0005515 10.22 ACVRL1 ALK CARS CCND1 CLTC DES

Sources for Inflammatory Myofibroblastic Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
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30 HGMD
31 HMDB
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34 ICD10 via Orphanet
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42 MeSH
43 MESH via Orphanet
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46 NCI
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48 NDF-RT
51 NINDS
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60 QIAGEN
65 SNOMED-CT via HPO
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70 UMLS via Orphanet
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