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 Crizotinib have been mentioned in the context of this disorder. Affiliated tissues include lung, liver and eye, 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 429)
# Related Disease Score Top Affiliating Genes
1 epithelioid inflammatory myofibroblastic sarcoma 33.3 TPM4 TPM3 SEC31A RANBP2 ALK
2 rare tumor 32.1 KIT CR2 ACTC1
3 pseudosarcomatous fibromatosis 31.5 VIM ALK ACTC1
4 fibrosarcoma 31.5 VIM TP53 KIT ALK ACTC1
5 anaplastic large cell lymphoma 31.5 RNF213 NPM1 ALK ACVRL1
6 fasciitis 31.4 VIM TP53 ACTC1
7 spindle cell sarcoma 31.4 VIM MDM2 ACTC1
8 fibrous histiocytoma 31.4 VIM TP53 KIT ALK ACTC1
9 leiomyosarcoma 31.3 VIM TP53 MDM2 KIT CDK4 ACTC1
10 gastrointestinal stromal tumor 31.2 VIM TP53 KIT ACTC1
11 liposarcoma 31.2 VIM TP53 MDM2 CDK4
12 carcinosarcoma 31.1 VIM TP53 KIT ACTC1
13 smooth muscle tumor 31.1 VIM KIT CR2 ACTC1
14 rhabdomyosarcoma 31.1 VIM TP53 MDM2 KIT CDK4 ALK
15 embryonal rhabdomyosarcoma 31.1 TP53 MDM2 CDK4 ACTC1
16 retroperitoneal fibrosis 31.1 VIM MDM2
17 soft tissue sarcoma 31.0 TP53 MDM2
18 lymphoma 31.0 TP53 RNF213 NPM1 MDM2 KIT EML4
19 congenital fibrosarcoma 31.0 VIM TPM3 ALK ACTC1
20 dendritic cell tumor 30.9 VIM CR2 CR1 ACTC1
21 dedifferentiated liposarcoma 30.9 TP53 MDM2 KIT CDK4 ACTC1
22 malignant fibrous histiocytoma 30.9 VIM TP53 MDM2 KIT CDK4 ACTC1
23 teratoma 30.9 VIM TP53 KIT ACTC1
24 endometrial stromal sarcoma 30.9 VIM TP53 KIT ACTC1
25 angiosarcoma 30.8 VIM TP53 MDM2 KIT
26 alk-positive anaplastic large cell lymphoma 30.8 NPM1 ALK
27 inflammatory liposarcoma 30.8 MDM2 ALK
28 pneumothorax 30.8 VIM ALK ACTC1
29 b-cell lymphoma 30.8 TP53 NPM1 MDM2 CR2 CDK4 ALK
30 perineurioma 30.8 VIM KIT ALK ACTC1
31 embryonal sarcoma 30.7 VIM TP53 MDM2 KIT CDK4
32 adenocarcinoma 30.7 TP53 MDM2 KIT CDK4 ALK
33 hemangiopericytoma, malignant 30.7 VIM MDM2 KIT ACTC1
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 well-differentiated liposarcoma 30.6 TP53 MDM2 KIT CDK4
38 sarcoma 30.5 VIM TP53 NPM1 MDM2 KIT CDK4
39 mesenchymal cell neoplasm 30.5 TPM4 TPM3 TP53 RANBP2 NPM1 KIT
40 neuroblastoma 30.5 TP53 NPM1 MDM2 KIT EML4 CDK4
41 diffuse large b-cell lymphoma 30.5 TP53 MDM2 CDK4 ALK
42 malignant peripheral nerve sheath tumor 30.4 VIM TP53 MDM2 KIT CDK4 ACTC1
43 ewing sarcoma 30.4 VIM TP53 MDM2 KIT CDK4 ALK
44 lymphoma, non-hodgkin, familial 30.3 TP53 NPM1 MDM2 CR2 ALK
45 lung cancer susceptibility 3 30.3 TP53 MDM2 KIT EML4 CDK4 ALK
46 desmoid tumor 11.7
47 infantile myofibromatosis 11.7
48 liver inflammatory pseudotumor 11.5
49 lymphangiectasis 10.7 VIM ACTC1
50 intravascular papillary endothelial hyperplasia 10.7 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:

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
Crizotinib Approved Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
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 Recruiting 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 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)
4 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
5 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)
6 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
7 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

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, Eye, Bone, Breast, Kidney, Spleen

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 1205)
# Title Authors PMID Year
1
Rare presentation of inflammatory myofibroblastic tumor in the kidney. 61
32577144 2020
2
Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient. 61
32472953 2020
3
Aggressive pelvic angiomyxoma and its mimics: can imaging be the guiding light? 61
32401545 2020
4
Prolapsing inflammatory myofibroblastic tumor of the urinary bladder: A case report and a review of literature. 61
32300533 2020
5
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 2020
6
A Novel ALK-THBS1 Fusion in a Laryngeal Inflammatory Myofibroblastic Tumour: A Case Report and Literature Review. 61
31368077 2020
7
[Inflammatory myofibroblastic tumor with osteoclast-like giant cells of the nasopharynx: report of a case]. 61
32486552 2020
8
Successful treatment of pulmonary inflammatory myofibroblastic tumor with platinum-pemetrexed: The first report of two cases. 61
32495499 2020
9
Inflammatory Myofibroblastic Tumor of the Uterus: An Immunohistochemical Study of 23 Cases. 61
32568825 2020
10
Inflammatory myofibroblastic tumor of maxillary sinus successfully treated with radiotherapy and corticosteroid: report of a rare case. 61
32488371 2020
11
Inflammatory Myofibroblastic Tumor of the Urinary System on Computed Tomography at a High-Volume Institution in China. 61
32526751 2020
12
ALK-1-positive inflammatory myofibroblastic tumor of the thyroid complicated by Hashimoto's thyroiditis: report of a rare case and a literature review. 61
32430041 2020
13
Inflammatory Myofibroblastic Tumor of the Orbit in an 8-Month Old. 61
31880682 2020
14
Inflammatory myofibroblastic tumor in the head and neck-a neoplasm with both tumor features and inflammation. 61
32414698 2020
15
[Clinical and pathological analysis of 64 patients with primary neoplasms of the lacrimal drainage system]. 61
32450669 2020
16
Spontaneous pneumothorax caused by an inflammatory myofibroblastic tumor-like lesion in a 14-year-old girl: a case report. 61
32448976 2020
17
Inflammatory Myofibroblastic Tumor of Spinal Canal: Brief Case Report. 61
31962352 2020
18
Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT). 61
31090942 2020
19
ALK 1 Negative Inflammatory Myofibroblastic Tumor of the Ileum: A Rare Cause of Ileocecal Intussusception. 61
32440556 2020
20
Inflammatory Myofibroblastic Tumor: Findings on 68Ga-DOTA-NOC Positron-emission Tomography-Computed Tomography. 61
32351276 2020
21
Uterine inflammatory myofibroblastic tumor involving the decidua of the extraplacental membranes: report of a case with a TIMP3-ROS1 gene fusion. 61
32247788 2020
22
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
23
Over expression of CDK4 and MDM2 in a patient with recurrent ALK-negative mediastinal inflammatory myofibroblastic tumor: A case report. 61
32195970 2020
24
Carinal Resection and Reconstruction for an Obstructing Inflammatory Myofibroblastic Tumor in a Child. 61
31117919 2020
25
Inflammatory myofibroblastic tumor: The experience of the European pediatric Soft Tissue Sarcoma Study Group (EpSSG). 61
32007712 2020
26
Soft Tissue Special Issue: Fibroblastic and Myofibroblastic Neoplasms of the Head and Neck. 61
31950474 2020
27
Isolated Pure Malignant Rhabdoid Tumor (MRT) of the Bladder: Case Report and Lessons Learned. 61
31733273 2020
28
Acute Intestinal Obstruction Due to Ileocolic Intussusception in an Adult; A Rare Presentation of Inflammatory Myofibroblastic Tumor. 61
32102989 2020
29
Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey 61
31655501 2020
30
Aggressive Inflammatory Myofibroblastic Tumor without Anaplastic Lymphoma Kinase Gene Rearrangement in the Rectum with Liver Metastasis. 61
31588090 2020
31
Unusual subcutaneous dedifferentiated liposarcoma exhibiting coexistence of meningothelial-like whorls and inflammatory myofibroblastic tumor-like structures. 61
31782160 2020
32
Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature. 61
32117841 2020
33
Inflammatory myofibroblastic tumor of the spermatic cord: two cases and review of the literature. 61
32027518 2020
34
An Inflammatory Myofibroblastic Tumor of the Appendix Mimicking an Appendicular Malignant Lesion. 61
32118204 2020
35
Core Biopsy Diagnosis of ALK Positive Inflammatory Myofibroblastic Tumor of Lung: An Interesting Case. 61
30632123 2020
36
Cancer Stem-Like Cells in a Case of an Inflammatory Myofibroblastic Tumor of the Lung. 61
32500024 2020
37
Gastric inflammatory myofibroblastic tumor in a 10-month-old girl: A case report. 61
32172194 2020
38
Inflammatory myofibroblastic tumor of the spermatic cord: two cases and review of the literature. 61
32026712 2020
39
Simultaneous multiple primary cancers with concomitant inflammatory myofibroblastic tumor: a case report. 61
32509097 2020
40
A Rare Case of Inflammatory Myofibroblastic Tumor Causing Left Pulmonary Artery Stenosis and Successfully Treated with Localized Radiotherapy in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibody Vasculitis. 61
32104634 2020
41
Pituitary inflammatory pseudotumor with amenorrhea, polyuria, and impaired vision: case report and review of the literature. 61
32355527 2020
42
Rosai-Dorfman Disease of the Breast With Variable IgG4+ Plasma Cells: A Diagnostic Mimicker of Other Malignant and Reactive Entities. 61
31436555 2019
43
Practical approach to diagnosis of bland-looking spindle cell lesions of the breast. 61
31965112 2019
44
[A Case of Laparoscopic Local Resection of the Stomach for an Inflammatory Myofibroblastic Tumor]. 61
32157057 2019
45
[Canceration of inflammatory myofibroblastic tumor of the larynx:a case report]. 61
31914279 2019
46
IgG4-related Disease at Rectovesical Pouch Mimicking Inflammatory Myofibroblastic Tumor. 61
31884443 2019
47
Successful role of adjuvant radiotherapy in a rare case of tracheal inflammatory myofibroblastic tumor: a case report. 61
30900517 2019
48
Anaplastic lymphoma kinase-negative pulmonary inflammatory myofibroblastic tumor with multiple metastases and its treatment by Apatinib: A case report. 61
31876714 2019
49
Partial Response to Ceritinib in a Patient With Abdominal Inflammatory Myofibroblastic Tumor Carrying a TFG-ROS1 Fusion. 61
31805529 2019
50
Kidney inflammatory myofibroblastic tumor masquerading as metastatic malignancy: A case report and literature review. 61
31911920 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
12.63 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 10.58 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 10.2 VIM TPM4 TPM3 TP53 SEC31A RNF213
2 protein-containing complex GO:0032991 9.85 TP53 NPM1 MDM2 CLTC CDK4 ALK
3 cytosol GO:0005829 9.8 VIM TPM4 TPM3 TP53 SEC31A RNF213
4 cytoskeleton GO:0005856 9.76 VIM TPM4 TPM3 TP53 NPM1 EML4
5 receptor complex GO:0043235 9.71 KIT CR2 ALK ACVRL1
6 actin filament GO:0005884 9.61 TPM4 TPM3 ACTC1
7 muscle thin filament tropomyosin GO:0005862 9.26 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:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.77 VIM TP53 MDM2 KIT ACTC1
2 regulation of cell proliferation GO:0042127 9.62 TP53 KIT CDK4 ALK
3 positive regulation of translation GO:0045727 9.58 VIM NPM1 CDK4
4 viral process GO:0016032 9.5 VIM TP53 RANBP2 NPM1 MDM2 CR2
5 cardiac septum morphogenesis GO:0060411 9.46 TP53 MDM2
6 complement receptor mediated signaling pathway GO:0002430 9.43 CR2 CR1
7 cellular response to UV-C GO:0071494 9.26 TP53 MDM2
8 cellular response to actinomycin D GO:0072717 9.16 TP53 MDM2
9 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
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
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
71 UMLS
72 UMLS via Orphanet
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