MCID: INF058
MIFTS: 45

Inflammatory Myofibroblastic Tumor

Categories: Cancer diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Inflammatory Myofibroblastic Tumor

MalaCards integrated aliases for Inflammatory Myofibroblastic Tumor:

Name: Inflammatory Myofibroblastic Tumor 12 20 58 15 70
Inflammatory Fibrosarcoma 20

Classifications:



External Ids:

Disease Ontology 12 DOID:0050905
UMLS via Orphanet 71 C0334121
Orphanet 58 ORPHA178342
UMLS 70 C0334121

Summaries for Inflammatory Myofibroblastic Tumor

GARD : 20 An inflammatory myofibroblastic tumor (IMT) is an uncommon, usually 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 become locally invasive and/or spread ( metastasize ) to other parts of the body. However, malignant (cancerous) IMTs are rare. 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.

MalaCards based summary : Inflammatory Myofibroblastic Tumor, also known as inflammatory fibrosarcoma, is related to epithelioid inflammatory myofibroblastic sarcoma and rare 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 Endometrial cancer and Measles. The drugs Crizotinib and Tyrosine have been mentioned in the context of this disorder. Affiliated tissues include eye, breast and kidney, and related phenotypes are Decreased cell migration and Decreased cell migration

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 424)
# Related Disease Score Top Affiliating Genes
1 epithelioid inflammatory myofibroblastic sarcoma 32.3 TPM4 TPM3 SEC31A RANBP2 ALK
2 rare tumor 32.0 KIT CR2 ACTC1
3 fasciitis 31.4 VIM TP53 ACTC1
4 fibrosarcoma 31.3 VIM TP53 KIT ALK ACTC1
5 fibrous histiocytoma 31.3 VIM KIT ALK ACTC1
6 pseudosarcomatous fibromatosis 31.3 VIM KIT ALK ACTC1
7 spindle cell sarcoma 31.3 VIM MDM2 KIT ACTC1
8 anaplastic large cell lymphoma 31.3 RNF213 NPM1 ALK ACVRL1
9 gastrointestinal stromal tumor 31.3 VIM TP53 KIT ACTC1
10 leiomyosarcoma 31.1 VIM TP53 MDM2 KIT CDK4 ACTC1
11 liposarcoma 31.1 VIM TP53 MDM2 CDK4
12 soft tissue sarcoma 31.1 TP53 MDM2
13 embryonal rhabdomyosarcoma 31.1 TP53 MDM2 CDK4
14 rhabdomyosarcoma 31.0 VIM TP53 MDM2 KIT CDK4 ALK
15 carcinosarcoma 31.0 VIM TP53 KIT ACTC1
16 retroperitoneal fibrosis 30.9 VIM MDM2
17 malignant fibrous histiocytoma 30.9 VIM TP53 MDM2 CDK4 ACTC1
18 dedifferentiated liposarcoma 30.9 TP53 MDM2 KIT CDK4 ACTC1
19 dendritic cell tumor 30.8 VIM CR2 CR1 ACTC1
20 smooth muscle tumor 30.8 VIM KIT CR2 ACTC1
21 sarcoma 30.8 VIM TP53 NPM1 MDM2 KIT CDK4
22 b-cell lymphoma 30.8 TP53 NPM1 MDM2 CR2 CDK4 ALK
23 adenocarcinoma 30.8 TP53 MDM2 KIT CDK4 ALK
24 endometrial stromal sarcoma 30.8 VIM TP53 KIT ACTC1
25 angiosarcoma 30.8 VIM TP53 MDM2 KIT
26 lymphoma 30.7 TP53 RNF213 NPM1 MDM2 EML4 CR2
27 alk-positive anaplastic large cell lymphoma 30.7 NPM1 ALK
28 inflammatory liposarcoma 30.7 MDM2 ALK
29 congenital fibrosarcoma 30.7 VIM TPM3 ALK ACTC1
30 castleman disease 30.6 CR2 CR1 ACVRL1
31 perineurioma 30.6 VIM KIT ALK ACTC1
32 cystitis 30.6 VIM TP53 KIT
33 ring chromosome 30.6 TP53 MDM2 CDK4
34 hemangiopericytoma, malignant 30.6 VIM MDM2 KIT ACTC1
35 neurofibroma 30.6 VIM TP53 KIT ACTC1
36 hemangioma 30.5 VIM TP53 KIT ACTC1
37 connective tissue benign neoplasm 30.5 TP53 MDM2 KIT CDK4
38 well-differentiated liposarcoma 30.5 TP53 MDM2 KIT CDK4
39 mesenchymal cell neoplasm 30.5 TPM4 TPM3 TP53 RANBP2 NPM1 KIT
40 diffuse large b-cell lymphoma 30.4 TP53 MDM2 CDK4 ALK
41 embryonal sarcoma 30.4 VIM TP53 MDM2 KIT CDK4
42 lymphoma, non-hodgkin, familial 30.4 TP53 NPM1 MDM2 CR2 ALK
43 malignant peripheral nerve sheath tumor 30.4 VIM TP53 MDM2 KIT CDK4 ACTC1
44 ewing sarcoma 30.4 VIM TP53 MDM2 KIT CDK4 ALK
45 neuroblastoma 30.3 TP53 NPM1 MDM2 KIT CDK4 ALK
46 liver inflammatory pseudotumor 11.3
47 infantile myofibromatosis 11.2
48 intussusception 10.6
49 lymphangiectasis 10.6 VIM ACTC1
50 intravascular papillary endothelial hyperplasia 10.6 VIM ACTC1

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

GenomeRNAi Phenotypes related to Inflammatory Myofibroblastic Tumor according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased cell migration GR00055-A-1 9.26 ALK CDK4 VIM
2 Decreased cell migration GR00055-A-3 9.26 TPM4
3 Decreased substrate adherent cell growth GR00193-A-1 9.23 ACVRL1 KIT
4 Decreased substrate adherent cell growth GR00193-A-2 9.23 CDK4 KIT
5 Decreased substrate adherent cell growth GR00193-A-3 9.23 CDK4
6 Decreased substrate adherent cell growth GR00193-A-4 9.23 ACVRL1 CDK4 KIT

MGI Mouse Phenotypes related to Inflammatory Myofibroblastic Tumor:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 muscle MP:0005369 9.56 ACTC1 ACVRL1 CDK4 KIT MDM2 TP53
2 normal MP:0002873 9.28 ACTC1 ACVRL1 ALK CDK4 KIT MDM2

Drugs & Therapeutics for Inflammatory Myofibroblastic Tumor

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Crizotinib Approved Phase 2, Phase 3 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
2
Tyrosine Approved, Investigational, Nutraceutical Phase 2, Phase 3 60-18-4 6057
3
Ceritinib Approved Phase 2 1032900-25-6
4 Protein Kinase Inhibitors Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Targeted Therapy in Children and Adolescents With Recurrent, Progressive and Unresectable Inflammatory Myofibroblastic Tumor With the Inhibitor of Tyrosine Kinase -Crizotinib Unknown status NCT03874273 Phase 2, Phase 3 Crizotinib
2 A Phase 1/2, Open-Label, Safety, Tolerability, Pharmacokinetics, and Anti-Tumor Activity Study of Repotrectinib in Pediatric and Young Adult Subjects With Advanced or Metastatic Malignancies Harboring ALK, ROS1, NTRK1-3 Alterations Recruiting NCT04094610 Phase 1, Phase 2 Oral repotrectinib (TPX-0005)
3 A Phase II, Open Label, Multi-center, Multi-arm Study of Ceritinib in Patients With Advanced Solid Tumors and Hematological Malignancies Characterized by Genetic Abnormalities of Anaplastic Lymphoma Kinase (ALK) Terminated NCT02465528 Phase 2 Ceritinib (LDK378)
4 An Open-Label, Phase 1/2 Dose Escalation and Expansion Trial to Evaluate the Pharmacokinetics, Safety, and Efficacy of Brigatinib as Monotherapy in Pediatric and Young Adult Patients With ALK+ Anaplastic Large Cell Lymphoma, Inflammatory Myofibroblastic Tumors or Other Solid Tumors Withdrawn NCT04260009 Phase 1, Phase 2 Brigatinib;Brigatinib AAF
5 A Phase I, Open-label, Dose Escalation Study of LDK378 in Pediatric Patients With Malignancies That Have a Genetic Alteration in Anaplastic Lymphoma Kinase (ALK) Completed NCT01742286 Phase 1 Ceritinib
6 Study on the Molecular Profile and Molecular Signature of Inflammatory Myofibroblastic Tumor Recruiting NCT04572594

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:

40
Eye, Breast, Kidney, Spleen, Pancreas, Uterus, Trachea

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 1286)
# Title Authors PMID Year
1
Renal inflammatory myofibroblastic tumor: A case report. 61
33732621 2021
2
A rare case of urachal inflammatory myofibroblastic tumor. 61
33537209 2021
3
Inflammatory Myofibroblastic Tumor Presenting as Gross Hematuria in a Pediatric Patient With VACTERL Syndrome Following Bladder Augmentation. 61
33684396 2021
4
Rare Presentation of Inflammatory Myofibroblastic Tumor on a Digit. 61
33754372 2021
5
Sustained complete response on crizotinib in primary lung inflammatory myofibroblastic tumor - Case report and literature review. 61
33794589 2021
6
[Clinicopathological features of inflammatory myofibroblastic tumor]. 61
33677881 2021
7
[Inflammatory myofibroblastic tumor: report of rare cases with a specific immunophenotype]. 61
33677890 2021
8
Colonic Inflammatory Myofibroblastic Tumor. 61
32632730 2021
9
Recurrent infantile inflammatory myofibroblastic tumor of mesentery--Case report and review of imaging findings. 61
33363692 2021
10
Inflammatory Myofibroblastic Tumor After Treatment of Wilms Tumor in a 6-Year-Old Boy: A Case Report and Literature Review. 61
33221418 2021
11
[Dacryocyst inflammatory myofibroblastic tumor: a case report]. 61
33730814 2021
12
Algorithmic Approach to Fibroinflammatory Sinonasal Tract Lesions. 61
33723762 2021
13
Safety and Antitumor Activity of Repeated ASP3026 Administration in Japanese Patients with Solid Tumors: A Phase I Study. 61
33331996 2021
14
Benign skull and subdural lesions in patients with prior medulloblastoma therapy. 61
32876801 2021
15
Mimickers of Urothelial Carcinoma and the Approach to Differential Diagnosis. 61
33668963 2021
16
A novel LRRFIP1-ALK fusion in inflammatory myofibroblastic tumor of hip and response to crizotinib. 61
32915420 2021
17
[Treatment of ALK negative metastatic inflammatory myofibroblastic tumor by combination of pembrolizumab and anlotinib: a case report]. 61
33601493 2021
18
Intracranial inflammatory myofibroblastic tumor: a report of two cases. 61
33517794 2021
19
Pseudosarcomatous myofibroblastic proliferations of the urinary bladder are neoplasms characterized by recurrent FN1-ALK fusions. 61
32908253 2021
20
ALK Immunohistochemistry and Molecular Analysis in Uterine Inflammatory Myofibroblastic Tumor: Proceedings of the ISGyP Companion Society Session at the 2020 USCAP Annual Meeting. 61
33290353 2021
21
A Conservative Approach to Inflammatory Myofibroblastic Tumor of the Bladder: A Case Report and Review of Literature. 61
33763285 2021
22
Inflammatory myofibroblastic tumor of the urinary bladder: A prognostically favorable spindle cell neoplasm. 61
33194556 2021
23
[Casuistics of inflammatory myofibroblastic tumor in a tertiary center]. 61
33495091 2021
24
Inflammatory myofibroblastic tumor successfully treated with metformin: A case report and review of literature. 61
33521112 2021
25
Clinical characteristics and outcomes of 17 cases of inflammatory myofibroblastic tumor at a University Hospital in China. 61
33281962 2021
26
Recurrent pulmonary embolization of inflammatory myofibroblastic tumor: a case report. 61
32858207 2021
27
Common Variable Immunodeficiency with the Concomitant Diagnosis of an Inflammatory Myofibroblastic Tumor. 61
33017039 2021
28
Rechallenge with Multi-Targeted Tyrosine Kinase Inhibitors in Patients with Advanced Soft Tissue Sarcoma: A Single-Center Experience. 61
33776477 2021
29
Inflammatory Myofibroblastic Tumor of the Liver Mimicking Metastasis on 18F-FDG PET/CT. 61
33156048 2021
30
MR imaging findings of unusual leiomyoma and malignant uterine myometrial tumors: what the radiologist should know. 61
33517507 2021
31
Diagnostic Challenge of Sarcomatoid Variant of ALK-Positive Anaplastic Large Cell Lymphoma: Case Report and Updated Systematic Literature Review. 61
33427000 2021
32
Sinonasal IgG4-related disease: a rare and emerging entity broadening the differential diagnosis in the sinonasal universe. 61
33392765 2021
33
Management of bladder tumors in pregnancy: A case of tumor prolapse and avulsion during labor. 61
33304822 2021
34
Prevalence, clinical features and prognosis of malignant solid tumors in infants: a 14-year study. 61
33259778 2020
35
Inflammatory myofibroblastic tumor of ileum with intussusception in adult. 61
32651869 2020
36
Inflammatory Myofibroblastic Tumor of the Bladder With FN1-ALK Gene Fusion: Different Response to ALK Inhibition. 61
33007314 2020
37
[Pinnal inflammatory myofibroblastic tumor: a case report]. 61
33342136 2020
38
Cardiac Inflammatory Myofibroblastic Tumor Causing Pulmonary Artery Obstruction: A Rare Case Report. 61
33273411 2020
39
Inflammatory myofibroblastic tumor in the head and neck-a neoplasm with both tumor features and inflammation. 61
32414698 2020
40
Inflammatory Myofibroblastic Tumor of the Urinary Bladder and Ureter in Children: Experience of a Tertiary Referral Center. 61
32777366 2020
41
Inflammatory Myofibroblastic Tumor of the Uterus: An Immunohistochemical Study of 23 Cases. 61
32568825 2020
42
Inflammatory Myofibroblastic Tumor of Sigmoid Colon: Unusual Cause of Intestinal Obstruction. 61
33409054 2020
43
Local ALK-Positive Histiocytosis With Unusual Morphology and Novel TRIM33-ALK Gene Fusion. 61
33243034 2020
44
Primary Pulmonary Tumors in Pediatric Population: Imaging Markers for Predicting Histology. 61
33487938 2020
45
The Activity of Chemotherapy in Inflammatory Myofibroblastic Tumors: A Multicenter, European Retrospective Case Series Analysis. 61
32584482 2020
46
Inflammatory myofibroblastic tumor arising from soft tissues of extremities harboring a novel CLIP2-ALK fusion. 61
32716129 2020
47
Infantile Inflammatory Myofibroblastic Tumor of Spleen. 61
33095087 2020
48
Adult Mid Ileo-Ileal Intussusception Secondary to Inflammatory Myofibroblastic Tumor (IMT): A Case Report and Literature Review. 61
33194470 2020
49
Clinical characteristics of resected solitary ground-glass opacities: Comparison between benign and malignant nodules. 61
32844603 2020
50
A Case of Simultaneously Diagnosed Lung Adenocarcinoma and Endobronchial Inflammatory Myofibroblastic Tumor with Two Distinct Types of ALK Translocation. 61
33091968 2020

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

Pathways related to Inflammatory Myofibroblastic Tumor according to GeneCards Suite gene sharing:

(show all 18)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.71 TPM3 TP53 MDM2 KIT CDK4
2
Show member pathways
12.64 VIM TP53 MDM2 CR2 CDK4
3
Show member pathways
12.48 TP53 MDM2 EML4 CDK4 ALK
4 12.21 VIM SEC31A EML4 CLTC ACTC1
5 12.13 VIM TP53 NPM1 MDM2 CDK4
6 11.89 TPM3 TP53 MDM2 KIT EML4 CDK4
7 11.75 KIT CR2 CR1
8 11.68 TP53 MDM2 CDK4
9 11.66 TPM4 TPM3 ACTC1
10 11.59 TP53 NPM1 CDK4
11 11.53 TP53 MDM2 CDK4
12
Show member pathways
11.37 TP53 RANBP2 MDM2
13 11.27 TP53 MDM2 CDK4
14 11.22 TP53 MDM2 CDK4
15 11.12 NPM1 EML4 ALK
16 11.11 VIM TPM4 TPM3 ACTC1
17 10.84 TP53 MDM2 CDK4
18 10.57 RANBP2 MDM2

GO Terms for Inflammatory Myofibroblastic Tumor

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

# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 10.34 VIM TPM4 TP53 SEC31A RNF213 RANBP2
2 cytoplasm GO:0005737 10.2 VIM TPM4 TPM3 TP53 SEC31A RNF213
3 cytosol GO:0005829 10 VIM TPM4 TPM3 TP53 SEC31A RNF213
4 protein-containing complex GO:0032991 9.85 TP53 NPM1 MDM2 CLTC CDK4 ALK
5 receptor complex GO:0043235 9.71 KIT CR2 ALK ACVRL1
6 actin filament GO:0005884 9.63 TPM4 TPM3 ACTC1
7 focal adhesion GO:0005925 9.43 VIM TPM4 PPFIBP1 NPM1 CLTC ACTC1
8 cytoskeleton GO:0005856 9.28 VIM TPM4 TPM3 TP53 NPM1 EML4
9 muscle thin filament tropomyosin GO:0005862 9.26 TPM4 TPM3

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.85 NPM1 MDM2 KIT CR1 CDK4
2 positive regulation of gene expression GO:0010628 9.8 VIM TP53 MDM2 KIT ACTC1
3 regulation of cell proliferation GO:0042127 9.62 TP53 KIT CDK4 ALK
4 positive regulation of translation GO:0045727 9.54 VIM NPM1 CDK4
5 viral process GO:0016032 9.5 VIM TP53 RANBP2 NPM1 MDM2 CR2
6 cardiac septum morphogenesis GO:0060411 9.48 TP53 MDM2
7 complement receptor mediated signaling pathway GO:0002430 9.43 CR2 CR1
8 cellular response to UV-C GO:0071494 9.26 TP53 MDM2
9 cellular response to actinomycin D GO:0072717 9.16 TP53 MDM2
10 muscle filament sliding GO:0030049 8.92 VIM TPM4 TPM3 ACTC1

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

# Name GO ID Score Top Affiliating Genes
1 disordered domain specific binding GO:0097718 8.8 TP53 MDM2 CLTC

Sources for Inflammatory Myofibroblastic Tumor

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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