MCID: INF058
MIFTS: 47

Inflammatory Myofibroblastic Tumor malady

Categories: Rare diseases, Cancer diseases

Aliases & Classifications for Inflammatory Myofibroblastic Tumor

Aliases & Descriptions for Inflammatory Myofibroblastic Tumor:

Name: Inflammatory Myofibroblastic Tumor 12 50 56 14 69
Inflammatory Fibrosarcoma 50
Inflammatory Pseudotumor 50
Pseudotumor Inflammatory 52

Classifications:



External Ids:

Disease Ontology 12 DOID:0050905
Orphanet 56 ORPHA178342
UMLS via Orphanet 70 C0334121

Summaries for Inflammatory Myofibroblastic Tumor

NIH Rare Diseases : 50 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 gastrointestinal stromal tumor and fibrosarcoma, 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 Pathways in cancer and Endometrial cancer. The drugs Doxorubicin and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include lung, eye and liver, and related phenotypes are cardiovascular system and homeostasis/metabolism

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 176)
id Related Disease Score Top Affiliating Genes
1 gastrointestinal stromal tumor 29.4 ACTC1 DES KIT S100B TP53 VIM
2 fibrosarcoma 29.4 ALK CLTC KIT RANBP2 TPM3 TPM4
3 liver inflammatory pseudotumor 11.1
4 lymphomatoid papulosis 10.4 ACTC1 VIM
5 chest wall parachordoma 10.4 ACTC1 VIM
6 inflammatory linear verrucous epidermal nevus 10.3 DES VIM
7 oral erosive lichen 10.3 ACTC1 DES
8 congenital myasthenic syndrome 10.3 ACTC1 DES VIM
9 lupus - neurological sequelae 10.3 S100B VIM
10 malignant dermis tumor 10.3 ACTC1 DES VIM
11 urethral cancer 10.3 ACTC1 DES VIM
12 biemond syndrome 10.3 ACTC1 DES VIM
13 intestinal obstruction 10.3 ACTC1 DES VIM
14 male urethral cancer 10.3 ACTC1 DES VIM
15 biliary papillomatosis 10.3 DES S100B VIM
16 lymphoma 10.3
17 hypogonadotropic hypogonadism-retinitis pigmentosa syndrome 10.3 ACTC1 S100B VIM
18 t-cell childhood lymphoblastic lymphoma 10.3 ACTC1 DES VIM
19 carbon monoxide-induced parkinsonism 10.3 ACTC1 DES KIT
20 cavernous sinus meningioma 10.3 DES S100B VIM
21 lung benign neoplasm 10.3 ACTC1 DES VIM
22 vulvar squamous papilloma 10.3 DES S100B VIM
23 marfanoid hypermobility syndrome 10.3 DES KIT VIM
24 skin glomus tumor 10.3 DES S100B VIM
25 orthostatic proteinuria 10.3 ACTC1 DES VIM
26 liver angiosarcoma 10.3 DES KIT VIM
27 pustulosis palmaris et plantaris 10.3 ACTC1 DES KIT
28 scirrhous adenocarcinoma 10.3 ACTC1 DES VIM
29 hyperpituitarism 10.3 ACTC1 DES VIM
30 stomach diverticulosis 10.3 KIT S100B VIM
31 aneurysm of sinus of valsalva 10.3 MDM2 S100B VIM
32 gangliosidosis gm1 10.3 ACTC1 DES KIT
33 acrodermatitis chronica atrophicans 10.3 ALK TP53
34 sengers syndrome 10.3 DES S100B VIM
35 myoma 10.3 ACTC1 DES S100B VIM
36 spleen angiosarcoma 10.3 ACTC1 DES S100B VIM
37 dyslexia 10.3 ACTC1 DES S100B VIM
38 lower clivus meningioma 10.3 CR1 CR2
39 juvenile xanthogranuloma 10.3 ACTC1 DES S100B VIM
40 diabetes persistent mullerian ducts 10.3 ACTC1 DES S100B VIM
41 sebaceous adenoma 10.2 ACTC1 DES KIT VIM
42 pilar sheath acanthoma 10.2 ACTC1 DES KIT VIM
43 fitz-hugh-curtis syndrome 10.2 ACTC1 DES KIT VIM
44 acute generalized exanthematous pustulosis 10.2 ACTC1 S100B
45 infiltrating lipoma 10.2 CCND1 S100B
46 osteomyelitis 10.2 ACTC1 DES MDM2 VIM
47 c-p angle neurinoma 10.2 DES KIT S100B VIM
48 preretinal fibrosis 10.2 ACTC1 DES VIM
49 van regemorter pierquin vamos syndrome 10.2 DES KIT MDM2
50 monostotic fibrous dysplasia 10.2 CR1 CR2 S100B

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

MGI Mouse Phenotypes related to Inflammatory Myofibroblastic Tumor:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.85 ACTC1 ACVRL1 CCND1 DES KIT MDM2
2 homeostasis/metabolism MP:0005376 9.73 ACTC1 ACVRL1 ALK CCND1 CR2 DES
3 muscle MP:0005369 9.23 TPM3 VIM ACTC1 ACVRL1 DES KIT

Drugs & Therapeutics for Inflammatory Myofibroblastic Tumor

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

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

Interventional clinical trials:


id Name Status NCT ID Phase
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
2 Low-dose Protocol for Computed Tomography-guided Lung Biopsy Enrolling by invitation NCT02971176 Phase 3
3 A Phase 1/2 Study of the Oral ALK/EGFR Inhibitor AP26113 Unknown status NCT01449461 Phase 1, Phase 2
4 Phase 2 Study Assessing Efficacy and Safety of Crizotinib in Patients Harboring an Alteration on ALK, MET or ROS1 Recruiting NCT02034981 Phase 2
5 CREATE: Cross-tumoral Phase 2 With Crizotinib Recruiting NCT01524926 Phase 2
6 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
7 Phase I Study of LDK378 in Pediatric, Malignancies With a Genetic Alteration in Anaplastic Lymphoma Kinase (ALK) Recruiting NCT01742286 Phase 1
8 A Prospective Cohort Study of IgG4RD in China Recruiting NCT01670695
9 Treatment With Crizotinib Single Patient Expanded Access IND 134375 No longer available NCT03085186

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:

39
Lung, Eye, Liver, Bone, Trachea, Heart, Spleen

Publications for Inflammatory Myofibroblastic Tumor

Articles related to Inflammatory Myofibroblastic Tumor:

(show top 50) (show all 542)
id Title Authors Year
1
Gastric inflammatory myofibroblastic tumor presenting as fever of unknown origin in a 9-year-old girl. ( 27748672 )
2017
2
Robot-assisted Partial Cystectomy for Treatment of Inflammatory Myofibroblastic Tumor of the Bladder. ( 28083481 )
2017
3
Inflammatory myofibroblastic tumor: An unusual hepatic tumor mimicking liver abscess. ( 28089176 )
2017
4
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
5
Inflammatory myofibroblastic tumor involving liver, gallbladder, pylorus & duodenum: A rare case presentation. ( 28092784 )
2017
6
Myocardial Infarction Secondary to Inflammatory Myofibroblastic Tumor Obstruction of the Left Main: Treated With Primary PCI. ( 27800457 )
2016
7
An isolated inflammatory myofibroblastic tumor of adrenal gland. ( 27806843 )
2016
8
ALK-positive inflammatory myofibroblastic tumor harboring ALK gene rearrangement, occurring after allogeneic stem cell transplant in an adult male. ( 27155927 )
2016
9
A Case of Cutaneous Inflammatory Myofibroblastic Tumor. ( 27653445 )
2016
10
Inflammatory Myofibroblastic Tumor of the Urinary Bladder: A Case Report. ( 27175348 )
2016
11
Febrile Illness in an Infant With an Intracardiac Inflammatory Myofibroblastic Tumor. ( 26798044 )
2016
12
Inflammatory Myofibroblastic Tumor of the Kidney: A Rare Renal Tumor. ( 28050384 )
2016
13
Anaplastic lymphoma kinase positive inflammatory myofibroblastic tumor of the urachus: A rare neoplasm in an unusual location. ( 26960648 )
2016
14
Chromoplectic TPM3-ALK rearrangement in a patient with inflammatory myofibroblastic tumor who responded to ceritinib after progression on crizotinib. ( 27742657 )
2016
15
Radiologic and Clinicopathologic Findings of Inflammatory Myofibroblastic Tumor. ( 27224222 )
2016
16
Laryngeal inflammatory myofibroblastic tumor (IMT): a case report and review of the literature. ( 27339455 )
2016
17
Inflammatory myofibroblastic tumor of the lung in pregnancy mimicking carcinoid tumor. ( 26933315 )
2016
18
Inflammatory myofibroblastic tumor of the small intestine mimicking acute appendicitis: a case report and review of the literature. ( 27094797 )
2016
19
Inflammatory myofibroblastic tumor of the left atrium in infant. ( 27526369 )
2016
20
Primary spinal extradural inflammatory myofibroblastic tumor: A rare cause of paraparesis. ( 27841217 )
2016
21
Complete and Repeated Response of a Metastatic ALK-rearranged Inflammatory Myofibroblastic Tumor to Crizotinib in a Teenage Girl. ( 26808369 )
2016
22
Inflammatory Myofibroblastic Tumor of the Larynx:A Case Report. ( 26878008 )
2016
23
Metastatic Inflammatory Myofibroblastic Tumor of the Spleen: A Case Report and Review of the Literature. ( 28018701 )
2016
24
Appendiceal Inflammatory Myofibroblastic Tumor: A Rare Postoperative Finding. ( 27397035 )
2016
25
Follow-Up Imaging of Inflammatory Myofibroblastic Tumor of the Uterus and Its Spontaneous Regression. ( 27110328 )
2016
26
Neoadjuvant Treatment With Cyclooxygenase-2 Inhibitor and Prednisolone Allows Conservative Surgery for Inflammatory Myofibroblastic Tumor of the Bladder. ( 27322717 )
2016
27
Fever,Weight Loss and Early Satiety due to Gastric Inflammatory Myofibroblastic Tumor; Case Report and Literature Review. ( 27252821 )
2016
28
Aggressive Metastatic Inflammatory Myofibroblastic Tumor After Allogeneic Stem Cell Transplant With Fatal Pulmonary Toxicity From Crizotinib. ( 27271810 )
2016
29
Drastic initial response and subsequent response to two ALK inhibitors in a patient with a highly aggressive ALK-rearranged inflammatory myofibroblastic tumor arising in the pleural cavity. ( 27565932 )
2016
30
Complete resection of a huge hypervascular inflammatory myofibroblastic tumor in right hemithorax after embolization. ( 27286690 )
2016
31
Inflammatory Myofibroblastic Tumor of the Lung. ( 27097710 )
2016
32
Inflammatory myofibroblastic tumor of the small intestine: A case report. ( 27046103 )
2016
33
Inflammatory myofibroblastic tumor with RANBP2 and ALK gene rearrangement with bland cytological features mimicking desmoid-type fibromatosis: A case report and review of the literature. ( 26893756 )
2016
34
Successful sleeve lobectomy of pediatric inflammatory myofibroblastic tumor. ( 27804248 )
2016
35
Inflammatory myofibroblastic tumor: A rare cause of invagination in adults. ( 27022387 )
2016
36
Diagnosing and Treating Inflammatory Myofibroblastic Tumor of the Bladder. ( 27293951 )
2016
37
Inflammatory Myofibroblastic Tumor in the Bladder: A Case Report. ( 27790120 )
2016
38
Inflammatory Myofibroblastic Tumor of Liver Masquerading as Focal Nodular Hyperplasia in a Patient with Non-Cirrhotic Portal Hypertension and Biliary Pancreatitis. ( 27065695 )
2016
39
Inflammatory myofibroblastic tumor in the head of the pancreas with anorexia and vomiting in a 69-year-old man: A case report. ( 27446468 )
2016
40
Inflammatory Myofibroblastic Tumor of the Bladder: 2 Rare Cases Managed with Laparoscopic Partial Cystectomy. ( 27974989 )
2016
41
Sinonasal Inflammatory Myofibroblastic Tumor with Anaplastic Lymphoma Kinase 1 Rearrangement: Case Study and Literature Review. ( 27443585 )
2016
42
Recurrent retroperitoneal inflammatory myofibroblastic tumor: A case report. ( 27446466 )
2016
43
Intracranial Inflammatory Myofibroblastic Tumor with Sarcomatous Local Recurrence. ( 27465420 )
2016
44
Remission of ALK-negative primary pulmonary inflammatory myofibroblastic tumor on treatment with clarithromycin: A case report and review of the literature. ( 26998073 )
2016
45
A case report of inflammatory myofibroblastic tumor of urinary bladder. ( 27453664 )
2016
46
Inflammatory myofibroblastic tumor of carotid artery resulting in recurrent syncope: A case report. ( 27028414 )
2016
47
Inflammatory Myofibroblastic Tumor of the Heart in the Infant: Review of the Literature. ( 26989913 )
2016
48
An inflammatory myofibroblastic tumor of the small intestine in a 15-year-old girl. ( 28007682 )
2016
49
Inflammatory myofibroblastic tumor presenting as ileocecal intussusception-A case report. ( 27262346 )
2016
50
Intraosseous inflammatory myofibroblastic tumor of the mandible with a novel ATIC-ALK fusion mutation: a case report. ( 27846861 )
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

GO Terms for Inflammatory Myofibroblastic Tumor

Cellular components related to Inflammatory Myofibroblastic Tumor according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 cytoskeleton GO:0005856 9.91 ACTC1 CLTC DES NPM1 TPM3 TPM4
2 protein complex GO:0043234 9.8 ALK CLTC MDM2 NPM1 TP53
3 cytosol GO:0005829 9.8 ACTC1 CARS CCND1 CLTC DES MDM2
4 focal adhesion GO:0005925 9.65 ACTC1 CLTC NPM1 TPM4 VIM
5 cytoplasm GO:0005737 9.53 ACTC1 CARS CCND1 CLTC DES KIT
6 actin filament GO:0005884 9.5 ACTC1 TPM3 TPM4
7 muscle thin filament tropomyosin GO:0005862 9.16 TPM3 TPM4

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

id Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.85 CCND1 KIT MDM2 NPM1 S100B
2 positive regulation of gene expression GO:0010628 9.65 ACTC1 KIT MDM2 TP53 VIM
3 movement of cell or subcellular component GO:0006928 9.61 TPM3 TPM4 VIM
4 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 9.5 MDM2 NPM1 TP53
5 viral process GO:0016032 9.5 CR1 CR2 MDM2 NPM1 RANBP2 TP53
6 response to magnesium ion GO:0032026 9.48 CCND1 MDM2
7 complement receptor mediated signaling pathway GO:0002430 9.4 CR1 CR2
8 mitotic G1 DNA damage checkpoint GO:0031571 9.37 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:

id Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.63 ALK DES MDM2 S100B TP53 VIM
2 protein kinase binding GO:0019901 9.35 ACVRL1 CCND1 CLTC NPM1 TP53
3 disordered domain specific binding GO:0097718 8.8 CLTC MDM2 TP53

Sources for Inflammatory Myofibroblastic Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
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31 HMDB
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33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
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42 MeSH
43 MESH via Orphanet
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60 QIAGEN
65 SNOMED-CT via Orphanet
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70 UMLS via Orphanet
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