MCID: INF058
MIFTS: 45

Inflammatory Myofibroblastic Tumor

Categories: Cancer diseases, Rare diseases

Aliases & Classifications for Inflammatory Myofibroblastic Tumor

MalaCards integrated aliases for Inflammatory Myofibroblastic Tumor:

Name: Inflammatory Myofibroblastic Tumor 12 52 58 15 71
Inflammatory Fibrosarcoma 52

Classifications:



External Ids:

Disease Ontology 12 DOID:0050905
UMLS via Orphanet 72 C0334121
Orphanet 58 ORPHA178342
UMLS 71 C0334121

Summaries for Inflammatory Myofibroblastic Tumor

NIH Rare Diseases : 52 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 Tyrosine and Ceritinib have been mentioned in the context of this disorder. Affiliated tissues include lung, liver and bone, and related phenotypes are Decreased cell migration and Decreased substrate adherent cell growth

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 420)
# Related Disease Score Top Affiliating Genes
1 epithelioid inflammatory myofibroblastic sarcoma 32.9 TPM4 SEC31A RANBP2 CLTC ALK
2 rare tumor 32.1 KIT CR2 ACTC1
3 fibrous histiocytoma 31.6 VIM ALK ACTC1
4 pseudosarcomatous fibromatosis 31.5 VIM ALK ACTC1
5 fibrosarcoma 31.5 VIM TP53 KIT ALK ACTC1
6 fasciitis 31.5 VIM TP53 ACTC1
7 anaplastic large cell lymphoma 31.5 RNF213 NPM1 ALK ACVRL1
8 spindle cell sarcoma 31.4 VIM MDM2 ACTC1
9 gastrointestinal stromal tumor 31.2 VIM TP53 KIT ACTC1
10 leiomyosarcoma 31.2 VIM TP53 MDM2 KIT CDK4 ACTC1
11 liposarcoma 31.2 VIM TP53 MDM2 CDK4
12 embryonal rhabdomyosarcoma 31.1 TP53 MDM2 CDK4
13 carcinosarcoma 31.1 VIM TP53 KIT ACTC1
14 malignant fibroxanthoma 31.1 VIM MDM2 CDK4 ALK ACTC1
15 smooth muscle tumor 31.1 VIM KIT CR2 ACTC1
16 retroperitoneal fibrosis 31.1 VIM MDM2
17 dedifferentiated liposarcoma 31.0 TP53 MDM2 CDK4 ACTC1
18 soft tissue sarcoma 31.0 TP53 MDM2
19 rhabdomyosarcoma 31.0 VIM TP53 MDM2 KIT CDK4 ALK
20 congenital fibrosarcoma 31.0 VIM TPM3 ALK ACTC1
21 lymphoma 30.9 TP53 RNF213 NPM1 MDM2 KIT EML4
22 dendritic cell tumor 30.9 VIM CR2 CR1 ACTC1
23 histiocytoma 30.9 VIM TP53 MDM2 KIT CR1 CDK4
24 teratoma 30.9 VIM TP53 KIT ACTC1
25 endometrial stromal sarcoma 30.8 VIM TP53 KIT ACTC1
26 angiosarcoma 30.8 VIM TP53 MDM2 KIT
27 alk-positive anaplastic large cell lymphoma 30.8 NPM1 ALK
28 inflammatory liposarcoma 30.8 MDM2 ALK
29 perineurioma 30.8 VIM KIT ALK ACTC1
30 b-cell lymphoma 30.7 TP53 NPM1 MDM2 CR2 CDK4 ALK
31 hemangiopericytoma, malignant 30.7 VIM MDM2 KIT ACTC1
32 embryonal sarcoma 30.7 VIM TP53 MDM2 KIT CDK4
33 adenocarcinoma 30.7 TP53 MDM2 KIT CDK4 ALK
34 ring chromosome 30.7 TP53 MDM2 CDK4
35 neurofibroma 30.7 VIM TP53 KIT ACTC1
36 connective tissue benign neoplasm 30.6 TP53 MDM2 KIT CDK4
37 diffuse large b-cell lymphoma 30.5 TP53 MDM2 CDK4 ALK
38 ewing sarcoma 30.4 VIM TP53 MDM2 KIT CDK4
39 malignant peripheral nerve sheath tumor 30.4 VIM TP53 MDM2 KIT CDK4 ACTC1
40 sarcoma 30.4 VIM TP53 NPM1 MDM2 KIT CR2
41 lymphoma, non-hodgkin, familial 30.3 TP53 NPM1 MDM2 CR2 ALK
42 neuroblastoma 30.3 TP53 NPM1 MDM2 KIT EML4 CDK4
43 lung cancer susceptibility 3 30.2 TP53 MDM2 KIT EML4 CDK4 ALK
44 mesenchymal cell neoplasm 30.0 TPM4 TPM3 TP53 SEC31A RANBP2 NPM1
45 desmoid tumor 11.7
46 infantile myofibromatosis 11.7
47 liver inflammatory pseudotumor 11.5
48 lymphangiectasis 10.8 VIM ACTC1
49 intravascular papillary endothelial hyperplasia 10.7 VIM ACTC1
50 ovarian fibrothecoma 10.7 KIT 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 9.35 ACVRL1 ALK CDK4 TPM4 VIM
2 Decreased substrate adherent cell growth GR00193-A-1 9.23 ACVRL1 KIT
3 Decreased substrate adherent cell growth GR00193-A-2 9.23 CDK4 KIT
4 Decreased substrate adherent cell growth GR00193-A-3 9.23 CDK4
5 Decreased substrate adherent cell growth GR00193-A-4 9.23 ACVRL1 CDK4 KIT

MGI Mouse Phenotypes related to Inflammatory Myofibroblastic Tumor:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 muscle MP:0005369 9.56 ACTC1 ACVRL1 CDK4 KIT MDM2 TP53
2 pigmentation MP:0001186 9.02 ALK CDK4 KIT MDM2 TP53

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
Tyrosine Approved, Investigational, Nutraceutical Phase 2, Phase 3 60-18-4 6057
2
Ceritinib Approved Phase 2 1032900-25-6
3
Crizotinib Approved Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
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 Recruiting NCT03874273 Phase 2, Phase 3 Crizotinib
2 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) Completed NCT02465528 Phase 2 Ceritinib (LDK378)
3 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)
4 Cross-tumoral Phase 2 Clinical Trial Exploring Crizotinib (PF-02341066) in Patients With Advanced Tumors Induced by Causal Alterations of ALK and/or MET ("CREATE") Active, not recruiting NCT01524926 Phase 2 Crizotinib (PF-02341066)
5 AcSé CRIZOTINIB : Secured Access to Crizotinib for Patients With Tumors Harboring a Genomic Alteration on One of the Biological Targets of the Drug. Active, not recruiting NCT02034981 Phase 2 Crizotinib
6 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 LDK378

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
Lung, Liver, Bone, Breast, Eye, Heart, Kidney

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 1180)
# Title Authors PMID Year
1
Uterine Inflammatory Myofibroblastic Tumor Showing an Atypical ALK Signal Pattern by FISH and DES-ALK Fusion by RNA Sequencing: A Case Report. 61
30741845 2020
2
Unusual subcutaneous dedifferentiated liposarcoma exhibiting coexistence of meningothelial-like whorls and inflammatory myofibroblastic tumor-like structures. 61
31782160 2020
3
Inflammatory myofibroblastic tumor: The experience of the European pediatric Soft Tissue Sarcoma Study Group (EpSSG). 61
32007712 2020
4
Inflammatory myofibroblastic tumor of the spermatic cord: two cases and review of the literature. 61
32027518 2020
5
Inflammatory myofibroblastic tumor of the spermatic cord: two cases and review of the literature. 61
32026712 2020
6
Soft Tissue Special Issue: Fibroblastic and Myofibroblastic Neoplasms of the Head and Neck. 61
31950474 2020
7
Inflammatory Myofibroblastic Tumor of Spinal Canal: Brief Case Report. 61
31962352 2020
8
Rosai-Dorfman Disease of the Breast With Variable IgG4+ Plasma Cells: A Diagnostic Mimicker of Other Malignant and Reactive Entities. 61
31436555 2019
9
Practical approach to diagnosis of bland-looking spindle cell lesions of the breast. 61
31965112 2019
10
Inflammatory Myofibroblastic Tumor of the Lung: An Extremely Rare Condition in Adults. 61
31970041 2019
11
Inflammatory Myofibroblastic Tumor of the Orbit in an 8-Month Old. 61
31880682 2019
12
Kidney inflammatory myofibroblastic tumor masquerading as metastatic malignancy: A case report and literature review. 61
31911920 2019
13
IgG4-related Disease at Rectovesical Pouch Mimicking Inflammatory Myofibroblastic Tumor. 61
31884443 2019
14
[Canceration of inflammatory myofibroblastic tumor of the larynx:a case report]. 61
31914279 2019
15
Partial Response to Ceritinib in a Patient With Abdominal Inflammatory Myofibroblastic Tumor Carrying a TFG-ROS1 Fusion. 61
31805529 2019
16
Anaplastic lymphoma kinase-negative pulmonary inflammatory myofibroblastic tumor with multiple metastases and its treatment by Apatinib: A case report. 61
31876714 2019
17
Successful role of adjuvant radiotherapy in a rare case of tracheal inflammatory myofibroblastic tumor: a case report. 61
30900517 2019
18
IMFT of the sigmoid colon: a case report. 61
31768246 2019
19
Inflammatory myofibroblastic tumor of the pancreas in a dog. 61
31585512 2019
20
Radical cystectomy for management of inflammatory myofibroblastic tumor of the urinary bladder: A rare case report. 61
31463199 2019
21
Inflammatory Myofibroblastic Tumor After Lung Transplant-A Rare and Aggressive Complication: A Case Report. 61
31611127 2019
22
Isolated Pure Malignant Rhabdoid Tumor (MRT) of the Bladder: Case Report and Lessons Learned. 61
31733273 2019
23
Unusual Nasal Insufficiency in an Infant: What's Behind the Nostril? 61
31204488 2019
24
Inflammatory Myofibroblastic Tumor. 61
31760795 2019
25
Can colonic inflammatory polyp with numerous immunoglobulin G4-positive plasma cells represent a colonic manifestation of immunoglobulin G4-related disease? A case report. 61
30895472 2019
26
Diagnostic utility of pan-Trk immunohistochemistry for inflammatory myofibroblastic tumors. 61
31578734 2019
27
The differential diagnoses of uterine leiomyomas and leiomyosarcomas using DNA and RNA sequencing. 61
31121144 2019
28
Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey. 61
31655501 2019
29
Inflammatory myofibroblastic tumor arising from the ascending aorta mimicking a thymoma. 61
31641928 2019
30
A newborn case of jejunal ALK-negative inflammatory myofibroblastic tumor with ETV6-NTRK3 fusion. 61
31250485 2019
31
[Primary Pulmonary Tumor in an Adolescent]. 61
31958395 2019
32
Aggressive Inflammatory Myofibroblastic Tumor without Anaplastic Lymphoma Kinase Gene Rearrangement in the Rectum with Liver Metastasis: A Case Report. 61
31588090 2019
33
How long should we continue crizotinib in ALK translocation-positive inflammatory myofibroblastic tumors? Long-term complete response with crizotinib and review of the literature. 61
31615346 2019
34
A Pancreatic Inflammatory Myofibroblastic Tumor with Spontaneous Remission: A Case Report with a Literature Review. 61
31627359 2019
35
Inflammatory myofibroblastic tumor of bone harboring an ALK gene amplification. 61
31326196 2019
36
Clinical Utility of In Situ Hybridization Assays in Head and Neck Neoplasms. 61
30467669 2019
37
Cardiac Inflammatory Myofibroblastic Tumor. 61
31500449 2019
38
Laryngeal Inflammatory Myofibroblastic Tumor With Anemia and Thrombocytosis. 61
31569971 2019
39
Inflammatory Myofibroblastic Tumor of the Mid Common Bile Duct Masquerading as Cholangiocarcinoma. 61
29453762 2019
40
Calcifying fibrous tumor and inflammatory myofibroblastic tumor are epigenetically related: A comparative genome-wide methylation study. 61
31202195 2019
41
Durable response to the ALK inhibitor alectinib in inflammatory myofibroblastic tumor of the head and neck with a novel SQSTM1-ALK fusion: a case report. 61
30790150 2019
42
A case report: Pharmacology and resistance patterns of three generations of ALK inhibitors in metastatic inflammatory myofibroblastic sarcoma. 61
29925295 2019
43
Successful treatment based on molecular biological assessment of invasive anaplastic lymphoma kinase-positive inflammatory myofibroblastic tumor of the lung. 61
31342196 2019
44
A Novel ALK-THBS1 Fusion in a Laryngeal Inflammatory Myofibroblastic Tumour: A Case Report and Literature Review. 61
31368077 2019
45
Inflammatory myofibroblastic tumor of the oral cavity: A case report and literature review. 61
31169252 2019
46
Inflammatory myofibroblastic tumor: A demographic, clinical and therapeutic study of 92 cases. 61
31698588 2019
47
Successful rigid bronchoscopic resection of recurrent pulmonary inflammatory myofibroblastic tumor after complete surgical resection. 61
31384203 2019
48
PD-L1 Expression and Tumor-infiltrating Lymphocytes in Uterine Smooth Muscle Tumors: Implications for Immunotherapy. 61
31009388 2019
49
Uterine Mesenchymal Tumors: Update on Classification, Staging, and Molecular Features. 61
31097109 2019
50
[Inflammatory myofibroblastic tumor: Variable presentation of the same pathology]. 61
31344194 2019

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
12.47 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 cytoplasm GO:0005737 10.13 VIM TPM4 TPM3 TP53 SEC31A RNF213
2 cytoskeleton GO:0005856 9.98 VIM TPM4 TPM3 NPM1 EML4 CLTC
3 protein-containing complex GO:0032991 9.8 TP53 NPM1 MDM2 CLTC CDK4 ALK
4 cytosol GO:0005829 9.8 VIM TPM4 TPM3 TP53 SEC31A RNF213
5 receptor complex GO:0043235 9.67 KIT CR2 ALK ACVRL1
6 actin filament GO:0005884 9.58 TPM4 TPM3 ACTC1
7 muscle thin filament tropomyosin GO:0005862 9.16 TPM4 TPM3
8 focal adhesion GO:0005925 9.1 VIM TPM4 PPFIBP1 NPM1 CLTC ACTC1

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.8 VIM TP53 MDM2 KIT ACTC1
2 regulation of cell proliferation GO:0042127 9.67 TP53 KIT CDK4 ALK
3 positive regulation of translation GO:0045727 9.61 VIM NPM1 CDK4
4 viral process GO:0016032 9.5 VIM TP53 RANBP2 NPM1 MDM2 CR2
5 response to hyperoxia GO:0055093 9.49 TP53 CDK4
6 cardiac septum morphogenesis GO:0060411 9.48 TP53 MDM2
7 complement receptor mediated signaling pathway GO:0002430 9.46 CR2 CR1
8 cellular response to UV-C GO:0071494 9.32 TP53 MDM2
9 regulation of DNA damage response, signal transduction by p53 class mediator GO:0043516 9.26 TP53 NPM1
10 cellular response to actinomycin D GO:0072717 9.16 TP53 MDM2
11 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 protein homodimerization activity GO:0042803 9.43 TPM4 TP53 NPM1 KIT CR2 CARS1
2 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
28 GO
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30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
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43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
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50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
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72 UMLS via Orphanet
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