MCID: INF058
MIFTS: 49

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 49 55 14 69
Inflammatory Fibrosarcoma 49
Inflammatory Pseudotumor 49
Pseudotumor Inflammatory 51

Classifications:



External Ids:

Disease Ontology 12 DOID:0050905
Orphanet 55 ORPHA178342
UMLS via Orphanet 70 C0334121
UMLS 69 C0334121

Summaries for Inflammatory Myofibroblastic Tumor

NIH Rare Diseases : 49 An inflammatory myofibroblastic tumor (IMT) is an uncommon, presumably 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 occasionally become locally invasive and/or spread (metastasize) to other parts of the body. 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. Last updated: 2/8/2016

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 pain and fever. An important gene associated with Inflammatory Myofibroblastic Tumor is ALK (ALK Receptor Tyrosine Kinase), and among its related pathways/superpathways are Endometrial cancer and Pathways in cancer. The drugs Cyclophosphamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include lung, eye and liver.

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 208)
# Related Disease Score Top Affiliating Genes
1 infantile myofibromatosis 32.4 ACTC1 DES VIM
2 anaplastic large cell lymphoma 30.6 ALK NPM1 RNF213
3 spindle cell carcinoma 30.3 ACTC1 DES VIM
4 fibrous histiocytoma 30.3 ACTC1 DES VIM
5 fibromatosis 30.1 ACTC1 DES KIT VIM
6 dedifferentiated liposarcoma 30.0 ACTC1 DES MDM2 TP53
7 liposarcoma 30.0 DES MDM2 TP53 VIM
8 rhabdomyosarcoma 30.0 ALK DES MDM2 TP53
9 dendritic cell tumor 29.8 ACTC1 CR1 CR2 S100B VIM
10 histiocytoma 29.8 ALK KIT MDM2 TP53
11 gastrointestinal stromal tumor 29.0 ACTC1 CCND1 DES KIT S100B TP53
12 liver inflammatory pseudotumor 11.2
13 desmoid tumor 11.1
14 lymphangiectasis 10.6 ACTC1 VIM
15 skin glomus tumor 10.6 DES VIM
16 infantile digital fibromatosis 10.6 DES VIM
17 glomangiomyoma 10.6 ACTC1 VIM
18 hydromyelia 10.6 S100B VIM
19 fibroblastic rheumatism 10.6 ACTC1 DES
20 lymphangiomatosis 10.6 DES VIM
21 angiolipoma 10.5 ACTC1 DES VIM
22 cutaneous leiomyosarcoma 10.5 ACTC1 DES VIM
23 intravascular papillary endothelial hyperplasia 10.5 ACTC1 VIM
24 parachordoma 10.5 ACTC1 DES VIM
25 epithelioid leiomyosarcoma 10.5 ACTC1 DES VIM
26 benign metastasizing leiomyoma 10.5 ACTC1 DES VIM
27 congenital fibrosarcoma 10.5 ACTC1 DES VIM
28 bednar tumor 10.5 S100B VIM
29 primitive neuroectodermal tumor of the cervix uteri 10.5 DES VIM
30 angiocentric glioma 10.5 ACTC1 S100B VIM
31 pseudosarcomatous fibromatosis 10.5 ACTC1 DES VIM
32 reticular perineurioma 10.5 KIT S100B
33 malignant giant cell tumor of soft parts 10.5 ACTC1 S100B
34 syringoma 10.5 DES S100B VIM
35 juvenile xanthogranuloma 10.5 DES S100B VIM
36 ovarian fibrothecoma 10.5 ACTC1 DES KIT
37 parameningeal embryonal rhabdomyosarcoma 10.5 ALK TP53
38 fasciitis 10.5 ACTC1 DES VIM
39 granular cell tumor 10.5 DES S100B VIM
40 glomangioma 10.5 ACTC1 DES VIM
41 malignant triton tumor 10.5 DES S100B
42 pulmonary vein stenosis 10.5 ACTC1 DES KIT
43 cystic nephroma 10.5 ACTC1 DES KIT
44 myoepithelioma 10.5 ACTC1 S100B VIM
45 parapharyngeal meningioma 10.5 CR1 CR2
46 acute myeloblastic leukemia with maturation 10.5 KIT NPM1
47 rhabdoid cancer 10.5 ACTC1 DES VIM
48 malignant iris melanoma 10.5 CCND1 S100B
49 perineurioma 10.5 KIT S100B VIM
50 chordoma 10.5 DES S100B 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:


pain, fever

Drugs & Therapeutics for Inflammatory Myofibroblastic Tumor

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

(show all 15)
# 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 Isophosphamide mustard Phase 2, Phase 3 0
10 Topoisomerase Inhibitors Phase 2, Phase 3
11
Crizotinib Approved Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
12 Protein Kinase Inhibitors Phase 2,Phase 1
13
Ceritinib Approved Phase 1 1032900-25-6
14 Antibodies
15 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 2 Study Assessing Efficacy and Safety of Crizotinib in Patients Harboring an Alteration on ALK, MET or ROS1 Recruiting NCT02034981 Phase 2 Crizotinib
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 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 Enrolling by invitation NCT02971176
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:

38
Lung, Eye, Liver, Bone, Heart, Trachea, Breast

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 584)
# Title Authors Year
1
Inflammatory Myofibroblastic Tumor of the Tongue. Report of a Pediatric Case and Review of the Literature. ( 29336647 )
2018
2
Inflammatory myofibroblastic tumor of the esophagus presenting with hematemesis and melaena: a case report and review of literature. ( 29375843 )
2018
3
A dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features. ( 29446522 )
2018
4
Inflammatory myofibroblastic tumor of heart in a 4-month-old infant. ( 29058974 )
2018
5
Inflammatory Myofibroblastic Tumor Driven by Novel<i>NUMA1-ALK</i>Fusion Responds to ALK Inhibition. ( 29439172 )
2018
6
Dramatic response to alectinib in inflammatory myofibroblastic tumor with anaplastic lymphoma kinase fusion gene. ( 28977547 )
2017
7
Robot-assisted Partial Cystectomy for Treatment of Inflammatory Myofibroblastic Tumor of the Bladder. ( 28083481 )
2017
8
Inflammatory myofibroblastic tumor of the lacrimal gland: case report of an exceptional location. ( 28824333 )
2017
9
Pulmonary inflammatory myofibroblastic tumor versus IgG4-related inflammatory pseudotumor: differential diagnosis based on a case series. ( 28449468 )
2017
10
Pulmonary inflammatory Myofibroblastic tumor indistinguishable from tuberculosis: a case report in a five-year-old child with hemoptysis. ( 29216875 )
2017
11
A case report of recurrent thyroid inflammatory myofibroblastic tumor and its metastasis in soft tissue. ( 29137037 )
2017
12
Inflammatory myofibroblastic tumor of kidney together with ipsilateral perinephric and periureteric fibrosis: A case report and literature review. ( 29245241 )
2017
13
Inflammatory myofibroblastic tumor of the gallbladder: a case report and literature review. ( 28639154 )
2017
14
Synchronous Inflammatory Myofibroblastic Tumor in Lung and Brain: A Case Report and Review of Literature. ( 28533999 )
2017
15
Pulmonary inflammatory myofibroblastic tumor with TPM4-ALK translocation. ( 29268561 )
2017
16
Inflammatory myofibroblastic tumor mimicking a relapse in a patient with Hodgkin's lymphoma: report of an unusual case and review of the literature. ( 28588845 )
2017
17
Inflammatory myofibroblastic tumor: An unusual hepatic tumor mimicking liver abscess. ( 28089176 )
2017
18
Pulmonary inflammatory myofibroblastic tumor: report of 2 cases with radiologic-pathologic correlation. ( 28491163 )
2017
19
An inflammatory myofibroblastic tumor in the transplanted liver displaying quick wash-in and wash-out on contrast-enhanced ultrasound: A case report. ( 29245287 )
2017
20
Inflammatory Myofibroblastic Tumor: Rare Manifestation in Face. ( 29023301 )
2017
21
Inflammatory myofibroblastic tumor of the lumbar spinal canal: A Case Report With Literature Review. ( 28658093 )
2017
22
Inflammatory myofibroblastic tumor involving liver, gallbladder, pylorus & duodenum: A rare case presentation. ( 28092784 )
2017
23
A novel fusion of HNRNPA1-ALK in inflammatory myofibroblastic tumor of urinary bladder. ( 28504207 )
2017
24
Rare Endobronchial Inflammatory Myofibroblastic Tumor in Pediatric Patient Detected on PET/CT Imaging. ( 28639954 )
2017
25
Inflammatory myofibroblastic tumor of the uterus: a clinicopathological, immunohistochemical, and molecular analysis of 13 cases highlighting their broad morphologic spectrum. ( 28664932 )
2017
26
Inflammatory myofibroblastic tumor of mandible: A rare case report and review of literature. ( 28479702 )
2017
27
ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis. ( 28923119 )
2017
28
Inflammatory Myofibroblastic Tumor of the Liver: Challenges in the Preoperative Diagnosis and Treatment. ( 29235003 )
2017
29
Inflammatory Myofibroblastic Tumor of the Breast Mimicking Malignancy in an Elderly Male. ( 29026362 )
2017
30
Paraneoplastic Pemphigus with Underlying Retroperitoneal Inflammatory Myofibroblastic Tumor: A Case Report and Review of the Literature. ( 29204394 )
2017
31
Inflammatory myofibroblastic tumors of the lung carrying a chimeric A2M-ALK gene: report of 2 infantile cases and review of the differential diagnosis of infantile pulmonary lesions. ( 28705706 )
2017
32
Imaging findings of inflammatory myofibroblastic tumor from the greater omentum: One case report. ( 29019904 )
2017
33
Targeting ALK With Crizotinib in Pediatric Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumor: A Children's Oncology Group Study. ( 28787259 )
2017
34
Central Nervous System Inflammatory Myofibroblastic Tumor Masquerading as Chronic Suppurative Otitis Media. ( 28904584 )
2017
35
Uterine inflammatory myofibroblastic tumor: more common than expected: Case report and review. ( 29310405 )
2017
36
Efficiency of Crizotinib on an ALK-Positive Inflammatory Myofibroblastic Tumor of the Central Nervous System: A Case Report. ( 28409069 )
2017
37
A case report of tracheal inflammatory myofibroblastic tumor in a 34-week pregnant woman misdiagnosed with asthma. ( 28816991 )
2017
38
A Case Report of Paraneoplastic Pemphigus Associated With Retroperitoneal Inflammatory Myofibroblastic Tumor. ( 28724275 )
2017
39
Relapsed and unresectable inflammatory myofibroblastic tumor responded to chemotherapy: A case report and review of the literature. ( 29046787 )
2017
40
Gastric inflammatory myofibroblastic tumor identified as ectopic pancreas and treated by endoscopic submucosal dissection. ( 28587772 )
2017
41
Gastric inflammatory myofibroblastic tumor presenting as fever of unknown origin in a 9-year-old girl. ( 27748672 )
2017
42
Testicular Inflammatory Myofibroblastic Tumor: A Known Entity at a Very Rare Site. ( 29430318 )
2017
43
Autotransplantation of the Heart for Recurrent Inflammatory Myofibroblastic Tumor. ( 28776353 )
2017
44
Inflammatory Myofibroblastic Tumor Diagnosed by Transbronchial Needle Aspiration. ( 28645432 )
2017
45
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
46
Inflammatory myofibroblastic tumor of the small intestine: A case report. ( 27046103 )
2016
47
A Case of Cutaneous Inflammatory Myofibroblastic Tumor. ( 27653445 )
2016
48
Recurrent retroperitoneal inflammatory myofibroblastic tumor: A case report. ( 27446466 )
2016
49
Appendiceal Inflammatory Myofibroblastic Tumor: A Rare Postoperative Finding. ( 27397035 )
2016
50
Inflammatory myofibroblastic tumor of the lung in pregnancy mimicking carcinoid tumor. ( 26933315 )
2016

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 15)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.85 ALK CCND1 KIT MDM2 TP53 TPM3
2 12.16 ALK CCND1 KIT MDM2 TP53 TPM3
3 12.14 ACTC1 CLTC DES SEC31A VIM
4 12.09 CR2 MDM2 TP53 VIM
5 12.02 CCND1 MDM2 NPM1 TP53 VIM
6
Show member pathways
11.94 ACTC1 DES TPM3 TPM4
7 11.67 CCND1 MDM2 TP53
8 11.59 ACTC1 TPM3 TPM4
9 11.55 CCND1 MDM2 TP53
10 11.37 CCND1 MDM2 TP53
11
Show member pathways
11.34 MDM2 RANBP2 TP53
12
Show member pathways
11.2 DES NPM1 VIM
13 11.14 CCND1 MDM2 TP53
14 10.88 ACTC1 DES TPM3 TPM4 VIM
15 10.58 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 9.55 ACTC1 CARS CCND1 CLTC DES HNRNPA1
5 actin filament GO:0005884 9.5 ACTC1 TPM3 TPM4
6 muscle thin filament tropomyosin GO:0005862 9.16 TPM3 TPM4
7 extracellular exosome GO:0070062 10.07 ACTC1 ALK CLTC CR1 CR2 DES

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 cell proliferation GO:0008284 9.89 CCND1 KIT MDM2 NPM1 S100B
2 positive regulation of gene expression GO:0010628 9.72 ACTC1 KIT MDM2 TP53 VIM
3 movement of cell or subcellular component GO:0006928 9.63 TPM3 TPM4 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 9.48 CCND1 MDM2
7 complement receptor mediated signaling pathway GO:0002430 9.43 CR1 CR2
8 positive regulation of cell cycle GO:0045787 9.43 CCND1 MDM2 TP53
9 mitotic G1 DNA damage checkpoint GO:0031571 9.4 CCND1 TP53
10 cellular response to actinomycin D GO:0072717 9.16 MDM2 TP53
11 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 9.73 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 10.09 ALK CARS CCND1 CLTC CR2 DES

Sources for Inflammatory Myofibroblastic Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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