MCID: INF058
MIFTS: 50

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 53 59 15 73
Inflammatory Fibrosarcoma 53

Classifications:



External Ids:

Disease Ontology 12 DOID:0050905
Orphanet 59 ORPHA178342
UMLS via Orphanet 74 C0334121
UMLS 73 C0334121

Summaries for Inflammatory Myofibroblastic Tumor

NIH Rare Diseases : 53 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 Cyclophosphamide and Mechlorethamine have been mentioned in the context of this disorder. Affiliated tissues include lung, liver and eye, 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 207)
# Related Disease Score Top Affiliating Genes
1 infantile myofibromatosis 32.2 ACTC1 DES VIM
2 anaplastic large cell lymphoma 30.6 ALK NPM1 RNF213
3 fibrous histiocytoma 30.1 ACTC1 DES VIM
4 spindle cell carcinoma 30.1 ACTC1 DES VIM
5 fibromatosis 30.0 ACTC1 DES KIT VIM
6 dendritic cell tumor 30.0 ACTC1 CR1 CR2 VIM
7 teratoma 30.0 ACTC1 KIT TP53
8 histiocytoma 29.9 ALK KIT MDM2 TP53
9 dedifferentiated liposarcoma 29.9 ACTC1 CDK4 DES MDM2 TP53
10 liposarcoma 29.9 CDK4 DES MDM2 TP53 VIM
11 gastrointestinal stromal tumor 29.8 ACTC1 CCND1 DES KIT TP53 VIM
12 carcinosarcoma 29.8 ACTC1 DES KIT TP53 VIM
13 malignant fibroxanthoma 29.7 ACTC1 CDK4 DES MDM2 VIM
14 rhabdomyosarcoma 29.7 ACTC1 ALK CDK4 DES MDM2 TP53
15 adenocarcinoma 29.6 ALK CCND1 CDK4 KIT TP53
16 liver inflammatory pseudotumor 11.4
17 desmoid tumor 11.2
18 lymphoma 10.6
19 lymphangiectasis 10.3 ACTC1 VIM
20 mesenchymoma 10.3 DES MDM2
21 infantile digital fibromatosis 10.3 DES VIM
22 glomangiomyoma 10.3 ACTC1 VIM
23 fibroblastic rheumatism 10.3 ACTC1 DES
24 lymphangiomatosis 10.3 DES VIM
25 intravascular papillary endothelial hyperplasia 10.3 ACTC1 VIM
26 intussusception 10.3
27 primitive neuroectodermal tumor of the cervix uteri 10.3 DES VIM
28 botryoid rhabdomyosarcoma 10.3 ACTC1 DES
29 fasciitis 10.3 ACTC1 DES VIM
30 parameningeal embryonal rhabdomyosarcoma 10.3 ALK TP53
31 angiolipoma 10.3 ACTC1 DES VIM
32 cutaneous leiomyosarcoma 10.3 ACTC1 DES VIM
33 parachordoma 10.3 ACTC1 DES VIM
34 ossifying fibromyxoid tumor 10.3 ACTC1 DES VIM
35 epithelioid leiomyosarcoma 10.3 ACTC1 DES VIM
36 benign metastasizing leiomyoma 10.3 ACTC1 DES VIM
37 parapharyngeal meningioma 10.3 CR1 CR2
38 mast-cell sarcoma 10.3 KIT VIM
39 cutaneous fibrous histiocytoma 10.3 ACTC1 DES VIM
40 gallbladder sarcoma 10.3 DES KIT
41 ovarian fibrothecoma 10.3 ACTC1 DES KIT
42 perineurioma 10.2 ALK KIT VIM
43 glomangioma 10.2 ACTC1 DES
44 pulmonary vein stenosis 10.2 ACTC1 DES KIT
45 acute myeloblastic leukemia with maturation 10.2 KIT NPM1
46 cystic nephroma 10.2 DES KIT VIM
47 histiocytic sarcoma 10.2 CR1 CR2
48 epulis 10.2 DES VIM
49 anaplastic ependymoma 10.2 MDM2 RNF213 VIM
50 giant cell tumor 10.2 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

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

26
# 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:

46
# 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 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 6055-19-2, 50-18-0 2907
2
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
3
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
4
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
5
Doxil Approved June 1999 Phase 2, Phase 3 31703
6
Isophosphamide mustard Phase 2, Phase 3 0
7 Anti-Bacterial Agents Phase 2, Phase 3
8 Antineoplastic Agents, Alkylating Phase 2, Phase 3
9 Antibiotics, Antitubercular Phase 2, Phase 3
10 Alkylating Agents Phase 2, Phase 3
11 Topoisomerase Inhibitors Phase 2, Phase 3
12
Crizotinib Approved Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
13 Protein Kinase Inhibitors Phase 2,Phase 1
14
Ceritinib Approved Phase 1 1032900-25-6
15 Antibodies
16 Immunologic Factors
17 Immunoglobulins
18 Immunoglobulin G

Interventional clinical trials:


# 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 Suspended NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride
2 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)
3 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
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 Active, not recruiting NCT02971176 Not Applicable
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:

41
Lung, Liver, Eye, Bone, Trachea, Breast, Kidney

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 713)
# Title Authors Year
1
Metastatic inflammatory myofibroblastic tumor of the bladder. ( 30505686 )
2019
2
Inflammatory myofibroblastic tumor of heart in a 4-month-old infant. ( 29058974 )
2018
3
Inflammatory Myofibroblastic Tumor Driven by Novel<i>NUMA1-ALK</i>Fusion Responds to ALK Inhibition. ( 29439172 )
2018
4
The Clinical and Radiological Characteristics of Inflammatory Myofibroblastic Tumor Occurring at Unusual Sites. ( 29888269 )
2018
5
Inflammatory myofibroblastic tumor of the esophagus presenting with hematemesis and melaena: a case report and review of literature. ( 29375843 )
2018
6
An interesting case of inflammatory myofibroblastic tumor presenting as cholangiocarcinoma. ( 29705678 )
2018
7
Anaplastic lymphoma kinase-negative uterine inflammatory myofibroblastic tumor containing the ETV6-NTRK3 fusion gene: a case report. ( 29900760 )
2018
8
Inflammatory Myofibroblastic Tumor Associated With the Placenta: Short Tandem Repeat Genotyping Confirms Uterine Site of Origin. ( 29505427 )
2018
9
Inflammatory myofibroblastic tumor of the orbit: A clinico-pathological study of 25 cases. ( 29755269 )
2018
10
Pulmonary inflammatory myofibroblastic tumor: a case report. ( 29649880 )
2018
11
Inflammatory myofibroblastic tumor mimicking lymphoma on <sup>18</sup>F-FDG PET/CT. Report of a case and review of the literature. ( 29550850 )
2018
12
Neoadjuvant crizotinib in ALK-rearranged inflammatory myofibroblastic tumor of the urinary bladder: A case report. ( 29758320 )
2018
13
An Unusual Case of Inflammatory Myofibroblastic Tumor of the Appendix Masquerading as Acute Appendicitis. ( 29896334 )
2018
14
Uterine metastases originating from a pulmonary inflammatory myofibroblastic tumor. ( 29578184 )
2018
15
A dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features. ( 29446522 )
2018
16
Inflammatory Myofibroblastic Tumor of the Mid Common Bile Duct Masquerading as Cholangiocarcinoma. ( 29453762 )
2018
17
Cytodiagnosis of inflammatory myofibroblastic tumor: A report of three cases in infants. ( 29673102 )
2018
18
Inflammatory Myofibroblastic Tumor Arising in the Cul-De-Sac, Mimicking Ectopic Decidua. ( 29620583 )
2018
19
Inflammatory myofibroblastic tumor of the lung. ( 29490419 )
2018
20
Pulmonary Inflammatory Myofibroblastic Tumor in Children: A Case Report and Brief Review of Literature. ( 29535991 )
2018
21
Inflammatory Myofibroblastic Tumor of the Tongue. Report of a Pediatric Case and Review of the Literature. ( 29336647 )
2018
22
Inflammatory myofibroblastic tumor of the prostate after transurethral resection of the prostate with negative expression of anaplastic lymphoma kinase: a case report. ( 29898011 )
2018
23
Differentiation between inflammatory myofibroblastic tumor and cholangiocarcinoma manifesting as target appearance on gadoxetic acid-enhanced MRI. ( 30515535 )
2018
24
Hepatobiliary and Pancreatic: Inflammatory myofibroblastic tumor of the liver mimicking intrahepatic cholangiocarcinoma with hilar lymph node metastasis. ( 30215848 )
2018
25
Morphologic Overlap Between Inflammatory Myofibroblastic Tumor and IgG4-related Disease: Lessons From Next-generation Sequencing. ( 30451733 )
2018
26
Plaque-like ALK-positive cutaneous inflammatory myofibroblastic tumor. ( 29564876 )
2018
27
Clinical and Histopathologic Correlates and Management Strategies for Inflammatory Myofibroblastic tumor of the lung. A case series and review of the literature. ( 29869302 )
2018
28
Inflammatory Myofibroblastic Tumor Invading the Left Atrium: Report of a Rare Case. ( 29997667 )
2018
29
ALK Is a Specific Diagnostic Marker for Inflammatory Myofibroblastic Tumor of the Uterus. ( 30015720 )
2018
30
Localized Inflammatory Myofibroblastic Tumor Involving the Genitourinary System: Adolescent Case Series and Review. ( 30025866 )
2018
31
Inflammatory myofibroblastic tumor of the larynx. ( 30036428 )
2018
32
Gastric inflammatory myofibroblastic tumor treated with combined laparoscopic and endoscopic gastric wedge resection: a case report. ( 30089486 )
2018
33
A Rare Case of Inflammatory Myofibroblastic Tumor of Meninges. ( 30090151 )
2018
34
Imaging findings of inflammatory myofibroblastic tumor in breast: A case report. ( 30095645 )
2018
35
Inflammatory myofibroblastic tumor of the epiglottis excised with a carbon dioxide laser: Case report and literature review. ( 30138523 )
2018
36
Uncommon Imaging Findings of Inflammatory Myofibroblastic Tumor: Report of a Rare Case With Both Omentum and Mesentery Involvement in the Abdominal Cavity. ( 30153139 )
2018
37
Inflammatory myofibroblastic tumor of the pleura with adjacent chest wall invasion and metastasis to the kidney: a case report. ( 30195334 )
2018
38
Inflammatory Myofibroblastic Tumor of Urinary Bladder. ( 30203811 )
2018
39
Double amplifications of CDK4 and MDM2 in a gastric inflammatory myofibroblastic tumor mimicking cancer with local invasion of the spleen and diaphragm. ( 30252584 )
2018
40
Gastric inverted hyperplastic polyp with inflammatory myofibroblastic tumor-like stroma, mimicking GI stromal tumor. ( 30339952 )
2018
41
Inflammatory myofibroblastic tumor. 18F-FDG PET/CT findings. ( 30366728 )
2018
42
Pediatric inflammatory myofibroblastic tumor of the trachea: Imaging spectrum and review of literature. ( 30381563 )
2018
43
Laser Treatment of a Tracheal Inflammatory Myofibroblastic Tumor. ( 30426959 )
2018
44
Ureteral inflammatory myofibroblastic tumor: A case report and literature review. ( 30431591 )
2018
45
Effectiveness of Crizotinib for Inflammatory Myofibroblastic Tumor with ALK mutation: A Case Report. ( 30449794 )
2018
46
Cardiac inflammatory myofibroblastic tumor in interventricular septum: A rare case report. ( 30508903 )
2018
47
Minimally invasive surgery for inflammatory myofibroblastic tumor of the urinary bladder: Three case reports. ( 30544437 )
2018
48
A Case Report of Paraneoplastic Pemphigus Associated With Retroperitoneal Inflammatory Myofibroblastic Tumor. ( 28724275 )
2017
49
A case report of recurrent thyroid inflammatory myofibroblastic tumor and its metastasis in soft tissue. ( 29137037 )
2017
50
Relapsed and unresectable inflammatory myofibroblastic tumor responded to chemotherapy: A case report and review of the literature. ( 29046787 )
2017

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 33)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.73 CCND1 CDK4 KIT MDM2 TP53 TPM3
2
Show member pathways
12.69 ALK CCND1 CDK4 MDM2 TP53
3 12.59 ALK CCND1 CDK4 KIT MDM2 TP53
4 12.41 ACTC1 CLTC DES SEC31A VIM
5 12.35 CCND1 CDK4 TP53 VIM
6
Show member pathways
12.3 CCND1 CDK4 MDM2 TP53
7 12.28 CCND1 CDK4 MDM2 TP53
8 12.22 CCND1 CDK4 MDM2 TP53
9 12.21 CCND1 CDK4 MDM2 NPM1 TP53 VIM
10
Show member pathways
12.15 CCND1 CDK4 MDM2 TP53
11
Show member pathways
12.13 CCND1 CDK4 MDM2 TP53
12
Show member pathways
12.12 ACTC1 DES TPM3 TPM4
13 12.07 CCND1 CDK4 MDM2 TP53
14 12.06 CCND1 CDK4 MDM2 TP53
15 12.02 CCND1 CDK4 MDM2 TP53
16 11.84 CCND1 CDK4 TP53 VIM
17 11.82 CR1 CR2 KIT
18 11.8 CCND1 CDK4 TP53
19 11.78 CCND1 CDK4 MDM2 TP53
20 11.74 CCND1 CDK4 CR2 MDM2 TP53 VIM
21 11.72 CDK4 NPM1 TP53
22 11.7 ACTC1 TPM3 TPM4
23 11.65 CCND1 CDK4 MDM2 TP53
24 11.53 CCND1 CDK4 MDM2 TP53
25 11.51 CCND1 MDM2 TP53
26
Show member pathways
11.5 MDM2 RANBP2 TP53
27 11.4 CDK4 MDM2 TP53
28
Show member pathways
11.38 DES NPM1 VIM
29 11.18 CCND1 CDK4 TP53
30 11.16 CDK4 MDM2 TP53
31 11.1 CCND1 CDK4 MDM2 TP53
32 10.88 ACTC1 DES TPM3 TPM4 VIM
33 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 9.58 ACTC1 ALK CARS CCND1 CDK4 CLTC
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 negative regulation of apoptotic process GO:0043066 9.8 ACTC1 ALK KIT MDM2 NPM1 TP53
5 positive regulation of translation GO:0045727 9.63 CDK4 NPM1 VIM
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 9.5 ALK CLTC DES MDM2 TP53 TPM4
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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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