MCID: INF058
MIFTS: 51

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 54 60 15 74
Inflammatory Fibrosarcoma 54

Classifications:



External Ids:

Disease Ontology 12 DOID:0050905
UMLS via Orphanet 75 C0334121
Orphanet 60 ORPHA178342
UMLS 74 C0334121

Summaries for Inflammatory Myofibroblastic Tumor

NIH Rare Diseases : 54 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 infantile myofibromatosis and anaplastic large cell lymphoma, 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 Glioma. The drugs Crizotinib and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include lung, liver and bone, and related phenotypes are Reduced mammosphere formation and cardiovascular system

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 226)
# Related Disease Score Top Affiliating Genes
1 infantile myofibromatosis 32.5 ACTC1 DES VIM
2 anaplastic large cell lymphoma 30.8 ALK NPM1 RNF213
3 spindle cell carcinoma 30.6 ACTC1 DES VIM
4 fibrous histiocytoma 30.4 ACTC1 DES VIM
5 fibromatosis 30.2 ACTC1 DES KIT VIM
6 dendritic cell tumor 30.1 ACTC1 CR1 CR2 VIM
7 teratoma 30.1 ACTC1 KIT TP53
8 fibrosarcoma of bone 30.0 CDK4 KIT MDM2
9 histiocytoma 30.0 ALK KIT MDM2 TP53
10 sarcoma 30.0 CDK4 KIT MDM2 TP53 VIM
11 carcinosarcoma 29.9 ACTC1 DES KIT TP53 VIM
12 dedifferentiated liposarcoma 29.9 ACTC1 CDK4 DES MDM2 TP53
13 liposarcoma 29.9 CDK4 DES MDM2 TP53 VIM
14 gastrointestinal stromal tumor 29.8 ACTC1 CCND1 DES KIT TP53 VIM
15 malignant fibroxanthoma 29.8 ACTC1 CDK4 DES MDM2 VIM
16 rhabdomyosarcoma 29.6 ACTC1 ALK CDK4 DES MDM2 TP53
17 adenocarcinoma 29.5 ALK CCND1 CDK4 KIT TP53
18 epithelioid inflammatory myofibroblastic sarcoma 11.6
19 liver inflammatory pseudotumor 11.4
20 desmoid tumor 11.3
21 lymphoma 10.6
22 lymphangiectasis 10.5 ACTC1 VIM
23 mesenchymoma 10.5 DES MDM2
24 infantile digital fibromatosis 10.5 DES VIM
25 glomangiomyoma 10.5 ACTC1 VIM
26 fibroblastic rheumatism 10.5 ACTC1 DES
27 lymphangiomatosis 10.5 DES VIM
28 intravascular papillary endothelial hyperplasia 10.4 ACTC1 VIM
29 cutaneous leiomyosarcoma 10.4 ACTC1 DES
30 primitive neuroectodermal tumor of the cervix uteri 10.4 DES VIM
31 botryoid rhabdomyosarcoma 10.4 ACTC1 DES
32 parameningeal embryonal rhabdomyosarcoma 10.4 ALK TP53
33 angiolipoma 10.4 ACTC1 DES VIM
34 parachordoma 10.4 ACTC1 DES VIM
35 syringocystadenoma papilliferum 10.4 ACTC1 DES VIM
36 ossifying fibromyxoid tumor 10.4 ACTC1 DES VIM
37 epithelioid leiomyosarcoma 10.4 ACTC1 DES VIM
38 mast-cell sarcoma 10.4 KIT VIM
39 benign metastasizing leiomyoma 10.4 ACTC1 DES VIM
40 parapharyngeal meningioma 10.4 CR1 CR2
41 gallbladder sarcoma 10.4 DES KIT
42 cutaneous fibrous histiocytoma 10.4 ACTC1 DES VIM
43 ovarian fibrothecoma 10.4 ACTC1 DES KIT
44 perineurioma 10.4 ALK KIT VIM
45 glomangioma 10.4 ACTC1 DES
46 pulmonary vein stenosis 10.4 ACTC1 DES KIT
47 epulis 10.4 DES VIM
48 acute myeloblastic leukemia with maturation 10.4 KIT NPM1
49 cystic nephroma 10.4 DES KIT VIM
50 anaplastic ependymoma 10.4 MDM2 RNF213 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

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

27
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.23 CARS CCND1 CDK4 CLTC DES MDM2

MGI Mouse Phenotypes related to Inflammatory Myofibroblastic Tumor:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.96 ACTC1 CCND1 CDK4 DES KIT MDM2
2 muscle MP:0005369 9.76 ACTC1 CDK4 DES KIT MDM2 TP53
3 neoplasm MP:0002006 9.5 ALK CCND1 CDK4 KIT MDM2 NPM1
4 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):

(show all 21)
# 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
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
3
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
4
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
5
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
6
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
7
Tyrosine Approved, Investigational, Nutraceutical Phase 2, Phase 3 60-18-4 6057
8
Doxil Approved June 1999 Phase 2, Phase 3 31703
9 Protein Kinase Inhibitors Phase 2, Phase 3,Phase 1
10 Topoisomerase Inhibitors Phase 2, Phase 3
11 Antineoplastic Agents, Alkylating Phase 2, Phase 3
12 Anti-Bacterial Agents Phase 2, Phase 3
13 Antibiotics, Antitubercular Phase 2, Phase 3
14 Alkylating Agents Phase 2, Phase 3
15
Isophosphamide mustard Phase 2, Phase 3 0
16 Ensartinib Investigational Phase 1, Phase 2 1370651-20-9
17
Ceritinib Approved Phase 1 1032900-25-6
18 Immunologic Factors
19 Antibodies
20 Immunoglobulin G
21 Immunoglobulins

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Study of Crizotinib in Children and Adolescents With Myofibroblastic Tumors Recruiting NCT03874273 Phase 2, Phase 3 Crizotinib
2 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 Active, not recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin;Doxorubicin Hydrochloride;Ifosfamide;Pazopanib;Pazopanib Hydrochloride
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 Phase 2 Study Assessing Efficacy and Safety of Crizotinib in Patients Harboring an Alteration on ALK, MET or ROS1 Active, not recruiting NCT02034981 Phase 2 Crizotinib
5 CREATE: Cross-tumoral Phase 2 With Crizotinib Active, not recruiting NCT01524926 Phase 2 Crizotinib (PF-02341066)
6 Phase I Study of LDK378 in Pediatric, Malignancies With a Genetic Alteration in Anaplastic Lymphoma Kinase (ALK) Completed NCT01742286 Phase 1 LDK378
7 A Prospective Cohort Study of IgG4RD in China Recruiting NCT01670695
8 Low-dose Protocol for Computed Tomography-guided Lung Biopsy Active, not recruiting NCT02971176 Not Applicable
9 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:

42
Lung, Liver, Bone, Eye, Kidney, Trachea, Heart

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 736)
# Title Authors Year
1
Differentiation between inflammatory myofibroblastic tumor and cholangiocarcinoma manifesting as target appearance on gadoxetic acid-enhanced MRI. ( 30515535 )
2019
2
Hepatobiliary and Pancreatic: Inflammatory myofibroblastic tumor of the liver mimicking intrahepatic cholangiocarcinoma with hilar lymph node metastasis. ( 30215848 )
2019
3
Morphologic Overlap Between Inflammatory Myofibroblastic Tumor and IgG4-related Disease: Lessons From Next-generation Sequencing. ( 30451733 )
2019
4
Effectiveness of Crizotinib for Inflammatory Myofibroblastic Tumor with ALK mutation. ( 30449794 )
2019
5
Metastatic inflammatory myofibroblastic tumor of the bladder. ( 30505686 )
2019
6
Inflammatory myofibroblastic tumor of urinary bladder with severe hematuria: A Case report and literature review. ( 30608442 )
2019
7
A Surgical Case of Inflammatory Myofibroblastic Tumor of the Liver: Potentially Characteristic Gross Features. ( 30799968 )
2019
8
A Rare Presentation of Inflammatory Myofibroblastic Tumor in the Nasolabial Fold. ( 30809407 )
2019
9
Inflammatory Myofibroblastic Tumor of the Retroperitoneum Including Chronic Granulomatous Inflammation Suggesting Tuberculosis: A Case Report. ( 30889946 )
2019
10
Inflammatory myofibroblastic tumor. 18F-FDG PET/CT findings. ( 30366728 )
2019
11
Gastric inverted hyperplastic polyp with inflammatory myofibroblastic tumor-like stroma, mimicking GI stromal tumor. ( 30339952 )
2019
12
Inflammatory Myofibroblastic Tumor in Female Genital Tract. ( 29965784 )
2019
13
Inflammatory Myofibroblastic Tumor of the Stomach Presenting as an Exophytic Mass - A Diagnostic Dilemma. ( 28272683 )
2019
14
ALK Negative Inflammatory Myofibroblastic Tumor Synchronous with Classical Hodgkin Lymphoma. ( 31043949 )
2019
15
Letter in Reply: Appendiceal Inflammatory Myofibroblastic Tumor and HIV Infection, An Association Not To Be Missed. ( 30918618 )
2019
16
Appendiceal Inflammatory Myofibroblastic Tumor and HIV Infection, An Association Not To Be Missed. ( 30918617 )
2019
17
Successful role of adjuvant radiotherapy in a rare case of tracheal inflammatory myofibroblastic tumor: a case report. ( 30900517 )
2019
18
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. ( 30790150 )
2019
19
Spectral Computed Tomography Imaging in the Differential Diagnosis of Lung Cancer and Inflammatory Myofibroblastic Tumor. ( 30762653 )
2019
20
Uterine Inflammatory Myofibroblastic Tumor Showing an Atypical ALK Signal Pattern by FISH and DES-ALK Fusion by RNA Sequencing: A Case Report. ( 30741845 )
2019
21
Intracranial inflammatory myofibroblastic tumor with negative expression of anaplastic lymphoma kinase: a case report and review of the literature. ( 30735872 )
2019
22
ALK inhibition in two emblematic cases of pediatric inflammatory myofibroblastic tumor: Efficacy and side effects. ( 30697903 )
2019
23
ALK-negative urachal inflammatory myofibroblastic tumor in an elderly female: A case report: Erratum. ( 30653181 )
2019
24
Case report: Crizotinib is effective in a patient with ROS1-rearranged pulmonary inflammatory myofibroblastic tumor. ( 30642440 )
2019
25
Core Biopsy Diagnosis of ALK Positive Inflammatory Myofibroblastic Tumor of Lung: An Interesting Case. ( 30632123 )
2019
26
An Unusual Case of Inflammatory Myofibroblastic Tumor of the Appendix Masquerading as Acute Appendicitis. ( 29896334 )
2018
27
An interesting case of inflammatory myofibroblastic tumor presenting as cholangiocarcinoma. ( 29705678 )
2018
28
Inflammatory Myofibroblastic Tumor of the Mid Common Bile Duct Masquerading as Cholangiocarcinoma. ( 29453762 )
2018
29
A dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features. ( 29446522 )
2018
30
Pediatric inflammatory myofibroblastic tumor of the trachea: Imaging spectrum and review of literature. ( 30381563 )
2018
31
Laser Treatment of a Tracheal Inflammatory Myofibroblastic Tumor. ( 30426959 )
2018
32
Ureteral inflammatory myofibroblastic tumor: A case report and literature review. ( 30431591 )
2018
33
Cardiac inflammatory myofibroblastic tumor in interventricular septum: A rare case report. ( 30508903 )
2018
34
Minimally invasive surgery for inflammatory myofibroblastic tumor of the urinary bladder: Three case reports. ( 30544437 )
2018
35
Primary gastric inflammatory myofibroblastic tumor: A case report. ( 30557996 )
2018
36
ALK-negative urachal inflammatory myofibroblastic tumor in an elderly female: A case report. ( 30572472 )
2018
37
Inflammatory Myofibroblastic Tumor of the Liver: Challenges in the Preoperative Diagnosis and Treatment. ( 29235003 )
2018
38
Inflammatory Myofibroblastic Tumor of the Tongue. Report of a Pediatric Case and Review of the Literature. ( 29336647 )
2018
39
Inflammatory myofibroblastic tumor of the esophagus presenting with hematemesis and melaena: a case report and review of literature. ( 29375843 )
2018
40
Inflammatory Myofibroblastic Tumor Driven by Novel NUMA1-ALK Fusion Responds to ALK Inhibition. ( 29439172 )
2018
41
Inflammatory myofibroblastic tumor of the lung. ( 29490419 )
2018
42
Double amplifications of CDK4 and MDM2 in a gastric inflammatory myofibroblastic tumor mimicking cancer with local invasion of the spleen and diaphragm. ( 30252584 )
2018
43
Inflammatory Myofibroblastic Tumor of Urinary Bladder. ( 30203811 )
2018
44
Inflammatory myofibroblastic tumor of the pleura with adjacent chest wall invasion and metastasis to the kidney: a case report. ( 30195334 )
2018
45
Uncommon Imaging Findings of Inflammatory Myofibroblastic Tumor: Report of a Rare Case With Both Omentum and Mesentery Involvement in the Abdominal Cavity. ( 30153139 )
2018
46
Inflammatory myofibroblastic tumor of the epiglottis excised with a carbon dioxide laser: Case report and literature review. ( 30138523 )
2018
47
Imaging findings of inflammatory myofibroblastic tumor in breast: A case report. ( 30095645 )
2018
48
A Rare Case of Inflammatory Myofibroblastic Tumor of Meninges. ( 30090151 )
2018
49
Gastric inflammatory myofibroblastic tumor treated with combined laparoscopic and endoscopic gastric wedge resection: a case report. ( 30089486 )
2018
50
Inflammatory myofibroblastic tumor of the larynx. ( 30036428 )
2018

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 32)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.72 CCND1 CDK4 KIT MDM2 TP53 TPM3
2
Show member pathways
12.6 ALK CCND1 CDK4 MDM2 TP53
3 12.53 ALK CCND1 CDK4 KIT MDM2 TP53
4 12.4 ACTC1 CLTC DES SEC31A VIM
5 12.35 CCND1 CDK4 TP53 VIM
6
Show member pathways
12.29 CCND1 CDK4 MDM2 TP53
7 12.28 CCND1 CDK4 MDM2 TP53
8 12.21 CCND1 CDK4 MDM2 TP53
9
Show member pathways
12.14 CCND1 CDK4 MDM2 TP53
10
Show member pathways
12.13 CCND1 CDK4 MDM2 TP53
11
Show member pathways
12.12 ACTC1 DES TPM3 TPM4
12 12.1 CCND1 CDK4 MDM2 NPM1 TP53 VIM
13 12.06 CCND1 CDK4 MDM2 TP53
14 12.05 CCND1 CDK4 MDM2 TP53
15 12.01 CCND1 CDK4 MDM2 TP53
16 11.82 CR1 CR2 KIT
17 11.81 CCND1 CDK4 TP53 VIM
18 11.79 CCND1 CDK4 TP53
19 11.75 CCND1 CDK4 MDM2 TP53
20 11.72 CDK4 NPM1 TP53
21 11.7 ACTC1 TPM3 TPM4
22 11.61 CCND1 CDK4 MDM2 TP53
23 11.5 CCND1 MDM2 TP53
24
Show member pathways
11.49 MDM2 RANBP2 TP53
25 11.49 CCND1 CDK4 MDM2 TP53
26 11.39 CDK4 MDM2 TP53
27
Show member pathways
11.37 DES NPM1 VIM
28 11.16 CCND1 CDK4 TP53
29 11.14 CDK4 MDM2 TP53
30 10.9 CCND1 CDK4 MDM2 TP53
31 10.88 ACTC1 DES TPM3 TPM4 VIM
32 10.68 MDM2 RANBP2

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 protein-containing complex GO:0032991 9.85 ALK CDK4 CLTC MDM2 NPM1 TP53
2 extracellular exosome GO:0070062 9.85 ACTC1 ALK CLTC CR1 CR2 DES
3 cytosol GO:0005829 9.83 ACTC1 CARS CCND1 CDK4 CLTC DES
4 focal adhesion GO:0005925 9.8 ACTC1 CLTC NPM1 TPM4 VIM
5 actin filament GO:0005884 9.61 ACTC1 TPM3 TPM4
6 cytoplasm GO:0005737 9.55 ACTC1 CARS CCND1 CDK4 CLTC DES
7 muscle thin filament tropomyosin GO:0005862 9.26 TPM3 TPM4
8 membrane GO:0016020 10.22 ACTC1 ALK CCND1 CDK4 CLTC CR1
9 cytoskeleton GO:0005856 10 ACTC1 CLTC DES NPM1 TPM3 TPM4

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.91 CCND1 CDK4 KIT MDM2 NPM1
2 cytokine-mediated signaling pathway GO:0019221 9.83 CCND1 KIT TP53 VIM
3 positive regulation of gene expression GO:0010628 9.8 ACTC1 KIT MDM2 TP53 VIM
4 positive regulation of translation GO:0045727 9.63 CDK4 NPM1 VIM
5 negative regulation of apoptotic process GO:0043066 9.62 ACTC1 MDM2 NPM1 TP53
6 viral process GO:0016032 9.56 CR1 CR2 HNRNPA1 MDM2 NPM1 RANBP2
7 positive regulation of cell cycle GO:0045787 9.54 CCND1 CDK4 MDM2
8 complement receptor mediated signaling pathway GO:0002430 9.49 CR1 CR2
9 response to magnesium ion GO:0032026 9.48 CCND1 MDM2
10 mitotic G1 DNA damage checkpoint GO:0031571 9.43 CCND1 TP53
11 negative regulation of cell cycle arrest GO:0071157 9.33 CCND1 CDK4 MDM2
12 cellular response to actinomycin D GO:0072717 9.26 MDM2 TP53
13 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:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.43 ALK DES MDM2 TP53 TPM4 VIM
2 disordered domain specific binding GO:0097718 8.8 CLTC MDM2 TP53
3 protein binding GO:0005515 10.09 ALK CARS CCND1 CDK4 CLTC CR2

Sources for Inflammatory Myofibroblastic Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....