MCID: INF058
MIFTS: 48

Inflammatory Myofibroblastic Tumor

Categories: Rare diseases, Cancer 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 Innate Immune System and Endometrial cancer. The drugs Cyclophosphamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include lung, eye and liver, and related phenotype is Reduced mammosphere formation.

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 212)
# Related Disease Score Top Affiliating Genes
1 infantile myofibromatosis 32.9 ACTC1 DES VIM
2 anaplastic large cell lymphoma 31.0 ALK NPM1 RNF213
3 spindle cell carcinoma 30.7 ACTC1 DES VIM
4 fibrous histiocytoma 30.5 ACTC1 DES S100B VIM
5 fibromatosis 30.4 ACTC1 DES KIT VIM
6 dedifferentiated liposarcoma 30.3 ACTC1 DES MDM2 TP53
7 liposarcoma 30.2 DES MDM2 TP53 VIM
8 histiocytoma 30.0 ALK KIT MDM2 TP53
9 dendritic cell tumor 29.9 ACTC1 CR1 CR2 S100B VIM
10 malignant fibroxanthoma 29.7 ACTC1 ALK DES MDM2 S100B VIM
11 rhabdomyosarcoma 29.6 ACTC1 ALK DES MDM2 TP53 VIM
12 gastrointestinal stromal tumor 28.7 ACTC1 CCND1 DES KIT S100B TP53
13 liver inflammatory pseudotumor 11.2
14 desmoid tumor 11.1
15 lymphangiectasis 11.0 ACTC1 VIM
16 infantile digital fibromatosis 11.0 DES VIM
17 glomangiomyoma 11.0 ACTC1 VIM
18 fibroblastic rheumatism 11.0 ACTC1 DES
19 hydromyelia 11.0 S100B VIM
20 lymphangiomatosis 11.0 DES VIM
21 intravascular papillary endothelial hyperplasia 10.9 ACTC1 VIM
22 cutaneous leiomyosarcoma 10.9 ACTC1 DES
23 bednar tumor 10.9 S100B VIM
24 primitive neuroectodermal tumor of the cervix uteri 10.9 DES VIM
25 malignant giant cell tumor of soft parts 10.9 ACTC1 S100B
26 botryoid rhabdomyosarcoma 10.9 ACTC1 DES
27 malignant triton tumor 10.9 DES S100B
28 reticular perineurioma 10.9 KIT S100B
29 angiolipoma 10.9 ACTC1 DES VIM
30 parachordoma 10.9 ACTC1 DES VIM
31 epithelioid leiomyosarcoma 10.9 ACTC1 DES VIM
32 parameningeal embryonal rhabdomyosarcoma 10.9 ALK TP53
33 benign metastasizing leiomyoma 10.9 ACTC1 DES VIM
34 congenital fibrosarcoma 10.8 ACTC1 DES VIM
35 angiocentric glioma 10.8 ACTC1 S100B VIM
36 rhabdoid cancer 10.8 ACTC1 DES VIM
37 primary hepatic neuroendocrine carcinoma 10.8 S100B VIM
38 syringoma 10.8 DES S100B VIM
39 fasciitis 10.8 ACTC1 DES VIM
40 mast-cell sarcoma 10.8 KIT VIM
41 juvenile xanthogranuloma 10.8 DES S100B VIM
42 parapharyngeal meningioma 10.8 CR1 CR2
43 glomangioma 10.8 ACTC1 DES VIM
44 myopericytoma 10.8 ACTC1 S100B
45 ovarian fibrothecoma 10.8 ACTC1 DES KIT
46 granular cell tumor 10.8 DES S100B VIM
47 gallbladder sarcoma 10.8 DES KIT
48 myoepithelioma 10.8 ACTC1 S100B VIM
49 acute myeloblastic leukemia with maturation 10.8 KIT NPM1
50 pulmonary vein stenosis 10.8 ACTC1 DES KIT

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 9.17 CARS CCND1 CLTC DES MDM2 RANBP2

Drugs & Therapeutics for Inflammatory Myofibroblastic Tumor

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

(show all 17)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
2
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
5
Doxil Approved June 1999 Phase 2, Phase 3 31703
6 Alkylating Agents Phase 2, Phase 3
7 Anti-Bacterial Agents Phase 2, Phase 3
8 Antibiotics, Antitubercular Phase 2, Phase 3
9 Antineoplastic Agents, Alkylating Phase 2, Phase 3
10
Isophosphamide mustard Phase 2, Phase 3 0
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 Immunoglobulin G
17 Immunoglobulins

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 Recruiting 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, Eye, Liver, Bone, Heart, Trachea, Spleen

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 603)
# Title Authors Year
1
Inflammatory myofibroblastic tumor of heart in a 4-month-old infant. ( 29058974 )
2018
2
Inflammatory Myofibroblastic Tumor Driven by Novel<i>NUMA1-ALK</i>Fusion Responds to ALK Inhibition. ( 29439172 )
2018
3
The Clinical and Radiological Characteristics of Inflammatory Myofibroblastic Tumor Occurring at Unusual Sites. ( 29888269 )
2018
4
Inflammatory myofibroblastic tumor of the esophagus presenting with hematemesis and melaena: a case report and review of literature. ( 29375843 )
2018
5
An interesting case of inflammatory myofibroblastic tumor presenting as cholangiocarcinoma. ( 29705678 )
2018
6
Anaplastic lymphoma kinase-negative uterine inflammatory myofibroblastic tumor containing the ETV6-NTRK3 fusion gene: a case report. ( 29900760 )
2018
7
Inflammatory Myofibroblastic Tumor Associated With the Placenta: Short Tandem Repeat Genotyping Confirms Uterine Site of Origin. ( 29505427 )
2018
8
Inflammatory myofibroblastic tumor of the orbit: A clinico-pathological study of 25 cases. ( 29755269 )
2018
9
Pulmonary inflammatory myofibroblastic tumor: a case report. ( 29649880 )
2018
10
Inflammatory myofibroblastic tumor mimicking lymphoma on <sup>18</sup>F-FDG PET/CT. Report of a case and review of the literature. ( 29550850 )
2018
11
Neoadjuvant crizotinib in ALK-rearranged inflammatory myofibroblastic tumor of the urinary bladder: A case report. ( 29758320 )
2018
12
An Unusual Case of Inflammatory Myofibroblastic Tumor of the Appendix Masquerading as Acute Appendicitis. ( 29896334 )
2018
13
Uterine metastases originating from a pulmonary inflammatory myofibroblastic tumor. ( 29578184 )
2018
14
A dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features. ( 29446522 )
2018
15
Inflammatory Myofibroblastic Tumor of the Mid Common Bile Duct Masquerading as Cholangiocarcinoma. ( 29453762 )
2018
16
Cytodiagnosis of inflammatory myofibroblastic tumor: A report of three cases in infants. ( 29673102 )
2018
17
Inflammatory Myofibroblastic Tumor Arising in the Cul-De-Sac, Mimicking Ectopic Decidua. ( 29620583 )
2018
18
Inflammatory myofibroblastic tumor of the lung. ( 29490419 )
2018
19
Pulmonary Inflammatory Myofibroblastic Tumor in Children: A Case Report and Brief Review of Literature. ( 29535991 )
2018
20
Inflammatory Myofibroblastic Tumor of the Tongue. Report of a Pediatric Case and Review of the Literature. ( 29336647 )
2018
21
Inflammatory myofibroblastic tumor of the prostate after transurethral resection of the prostate with negative expression of anaplastic lymphoma kinase: a case report. ( 29898011 )
2018
22
Plaque-like ALK-positive cutaneous inflammatory myofibroblastic tumor. ( 29564876 )
2018
23
Clinical and Histopathologic Correlates and Management Strategies for Inflammatory Myofibroblastic tumor of the lung. A case series and review of the literature. ( 29869302 )
2018
24
A Case Report of Paraneoplastic Pemphigus Associated With Retroperitoneal Inflammatory Myofibroblastic Tumor. ( 28724275 )
2017
25
A case report of recurrent thyroid inflammatory myofibroblastic tumor and its metastasis in soft tissue. ( 29137037 )
2017
26
Relapsed and unresectable inflammatory myofibroblastic tumor responded to chemotherapy: A case report and review of the literature. ( 29046787 )
2017
27
Gastric inflammatory myofibroblastic tumor presenting as fever of unknown origin in a 9-year-old girl. ( 27748672 )
2017
28
A novel fusion of HNRNPA1-ALK in inflammatory myofibroblastic tumor of urinary bladder. ( 28504207 )
2017
29
A case report of tracheal inflammatory myofibroblastic tumor in a 34-week pregnant woman misdiagnosed with asthma. ( 28816991 )
2017
30
Inflammatory myofibroblastic tumor of the lacrimal gland: case report of an exceptional location. ( 28824333 )
2017
31
ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis. ( 28923119 )
2017
32
Inflammatory Myofibroblastic Tumor Diagnosed by Transbronchial Needle Aspiration. ( 28645432 )
2017
33
Inflammatory myofibroblastic tumor involving liver, gallbladder, pylorus &amp;amp; duodenum: A rare case presentation. ( 28092784 )
2017
34
Synchronous Inflammatory Myofibroblastic Tumor in Lung and Brain: A Case Report and Review of Literature. ( 28533999 )
2017
35
Imaging findings of inflammatory myofibroblastic tumor from the greater omentum: One case report. ( 29019904 )
2017
36
Inflammatory Myofibroblastic Tumor of the Breast Mimicking Malignancy in an Elderly Male. ( 29026362 )
2017
37
Inflammatory myofibroblastic tumor: An unusual hepatic tumor mimicking liver abscess. ( 28089176 )
2017
38
Efficiency of Crizotinib on an ALK-Positive Inflammatory Myofibroblastic Tumor of the Central Nervous System: A Case Report. ( 28409069 )
2017
39
Gastric inflammatory myofibroblastic tumor identified as ectopic pancreas and treated by endoscopic submucosal dissection. ( 28587772 )
2017
40
Inflammatory myofibroblastic tumor of mandible: A rare case report and review of literature. ( 28479702 )
2017
41
Targeting ALK With Crizotinib in Pediatric Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumor: A Children's Oncology Group Study. ( 28787259 )
2017
42
Inflammatory myofibroblastic tumor of kidney together with ipsilateral perinephric and periureteric fibrosis: A case report and literature review. ( 29245241 )
2017
43
Inflammatory myofibroblastic tumor of the lumbar spinal canal: A Case Report With Literature Review. ( 28658093 )
2017
44
Central Nervous System Inflammatory Myofibroblastic Tumor Masquerading as Chronic Suppurative Otitis Media. ( 28904584 )
2017
45
Dramatic response to alectinib in inflammatory myofibroblastic tumor with anaplastic lymphoma kinase fusion gene. ( 28977547 )
2017
46
Rare Endobronchial Inflammatory Myofibroblastic Tumor in Pediatric Patient Detected on PET/CT Imaging. ( 28639954 )
2017
47
Pulmonary inflammatory myofibroblastic tumor: report of 2 cases with radiologic-pathologic correlation. ( 28491163 )
2017
48
Successful Preservation of the Bladder in a Case of Inflammatory Myofibroblastic Tumor with the Diagnostic Efficacy of ALK/p80 Immunohistochemistry and FISH Analysis: Case Report and Review of the Literature. ( 28083479 )
2017
49
Pulmonary inflammatory Myofibroblastic tumor indistinguishable from tuberculosis: a case report in a five-year-old child with hemoptysis. ( 29216875 )
2017
50
Inflammatory Myofibroblastic Tumor of the Liver: Challenges in the Preoperative Diagnosis and Treatment. ( 29235003 )
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 17)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.88 CCND1 CLTC CR1 KIT MDM2 RANBP2
2
Show member pathways
12.71 CCND1 KIT MDM2 TP53 TPM3
3 12.15 ALK CCND1 KIT MDM2 TP53 TPM3
4 12.14 ACTC1 CLTC DES SEC31A VIM
5 12.08 CR2 MDM2 TP53 VIM
6 12.02 CCND1 MDM2 NPM1 TP53 VIM
7
Show member pathways
11.94 ACTC1 DES TPM3 TPM4
8 11.72 CR1 CR2 KIT
9 11.66 CCND1 MDM2 TP53
10 11.58 ACTC1 TPM3 TPM4
11 11.53 CCND1 MDM2 TP53
12 11.37 CCND1 MDM2 TP53
13
Show member pathways
11.34 MDM2 RANBP2 TP53
14
Show member pathways
11.2 DES NPM1 VIM
15 11.14 CCND1 MDM2 TP53
16 10.88 ACTC1 DES TPM3 TPM4 VIM
17 10.57 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 cytoskeleton GO:0005856 9.95 ACTC1 CLTC DES NPM1 TPM3 TPM4
2 cytosol GO:0005829 9.8 ACTC1 CARS CCND1 CLTC DES MDM2
3 focal adhesion GO:0005925 9.65 ACTC1 CLTC NPM1 TPM4 VIM
4 cytoplasm GO:0005737 9.58 ACTC1 ALK CARS CCND1 CLTC DES
5 actin filament GO:0005884 9.54 ACTC1 TPM3 TPM4
6 muscle thin filament tropomyosin GO:0005862 9.16 TPM3 TPM4
7 extracellular exosome GO:0070062 10.02 ACTC1 ALK CLTC CR1 CR2 DES

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 9.88 CCND1 KIT MDM2 NPM1 S100B
2 negative regulation of apoptotic process GO:0043066 9.73 ACTC1 ALK KIT MDM2 NPM1 TP53
3 positive regulation of gene expression GO:0010628 9.72 ACTC1 KIT MDM2 TP53 VIM
4 viral process GO:0016032 9.56 CR1 CR2 HNRNPA1 MDM2 NPM1 RANBP2
5 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 9.54 MDM2 NPM1 TP53
6 response to magnesium ion GO:0032026 9.46 CCND1 MDM2
7 complement receptor mediated signaling pathway GO:0002430 9.43 CR1 CR2
8 mitotic G1 DNA damage checkpoint GO:0031571 9.4 CCND1 TP53
9 cellular response to actinomycin D GO:0072717 9.16 MDM2 TP53
10 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 protein homodimerization activity GO:0042803 9.63 CARS CR2 KIT NPM1 S100B TPM4
2 identical protein binding GO:0042802 9.5 ALK DES MDM2 S100B TP53 TPM4
3 disordered domain specific binding GO:0097718 8.8 CLTC MDM2 TP53
4 protein binding GO:0005515 10.19 ALK CARS CCND1 CLTC CR2 DES

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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