MCID: SPN035
MIFTS: 47

Spindle Cell Sarcoma

Categories: Cancer diseases

Aliases & Classifications for Spindle Cell Sarcoma

MalaCards integrated aliases for Spindle Cell Sarcoma:

Name: Spindle Cell Sarcoma 12 15
Sarcoma 44 73
Sarcoma, Spindle Cell 73
Sarcoma Spindle Cell 55
Sarcoma - Category 73

Classifications:



External Ids:

Disease Ontology 12 DOID:4235
MeSH 44 D012509
NCIt 50 C27005
SNOMED-CT 68 9801004

Summaries for Spindle Cell Sarcoma

Disease Ontology : 12 A sarcoma that results in cells that are spindle-shaped. They are usually resistant to radiation therapy.

MalaCards based summary : Spindle Cell Sarcoma, also known as sarcoma, is related to ewing sarcoma and sarcoma, synovial. An important gene associated with Spindle Cell Sarcoma is VIM (Vimentin), and among its related pathways/superpathways are ERK Signaling and Dilated cardiomyopathy (DCM). The drugs Doxil and Fusilev have been mentioned in the context of this disorder. Affiliated tissues include lung, liver and kidney, and related phenotypes are Decreased focal adhesion (FA) area, decreased FA length, decreased FA mean intensity, increased number of small and round FAs, increased FA abundance and cardiovascular system

Wikipedia : 76 Spindle cell sarcoma is a type of connective tissue cancer in which the cells are spindle-shaped when... more...

Related Diseases for Spindle Cell Sarcoma

Diseases related to Spindle Cell Sarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 247)
# Related Disease Score Top Affiliating Genes
1 ewing sarcoma 32.0 DES IL6 MDM2 S100B VEGFA VIM
2 sarcoma, synovial 30.8 DES KRT7 MDM2 MUC1 VIM
3 extraosseous osteosarcoma 30.6 KRT7 MDM2
4 soft tissue sarcoma 30.5 MDM2 VEGFA
5 endodermal sinus tumor 30.4 KRT7 VIM
6 fibrosarcoma 30.0 DES NTRK3 VIM
7 giant cell tumor 29.6 DES MUC1 VIM
8 cholangiocarcinoma 28.8 IL6 KRT7 MUC1 VEGFA
9 rhabdomyosarcoma 28.7 ACTC1 DES MDM2 VIM
10 fibrous histiocytoma 28.6 ACTC1 DES S100B VIM
11 fasciitis 27.6 ACTC1 DES IL6 MUC1 VIM
12 malignant fibroxanthoma 26.9 ACTC1 DES MDM2 MUC1 S100B VIM
13 kaposi sarcoma 12.6
14 spindle cell synovial sarcoma 11.3
15 retroperitoneal fibrosis 10.9 MDM2 VIM
16 sarcoma 10.8
17 infiltrating angiolipoma 10.7 MDM2 VEGFA
18 adenoid squamous cell carcinoma 10.7 KRT7 VIM
19 infantile digital fibromatosis 10.7 DES VIM
20 papillary adenoma 10.7 KRT7 VIM
21 lymphangiomatosis 10.7 DES VIM
22 critical limb ischemia 10.7 IL6 VEGFA
23 primitive neuroectodermal tumor of the cervix uteri 10.7 DES VIM
24 medulloepithelioma 10.7 KRT7 VIM
25 epulis 10.7 DES VIM
26 metanephric adenoma 10.7 KRT7 VIM
27 spinal disease 10.7 IL6 VEGFA
28 adenomatoid tumor 10.7 KRT7 VIM
29 mesenchymoma 10.6 DES MDM2
30 hydromyelia 10.6 S100B VIM
31 poems syndrome 10.6 IL6 VEGFA
32 chondroid lipoma 10.6 KRT7 MDM2
33 bednar tumor 10.6 S100B VIM
34 primary hepatic neuroendocrine carcinoma 10.6 S100B VIM
35 malignant fibrous histiocytoma of bone 10.6 DES MDM2
36 endocervical adenocarcinoma 10.6 KRT7 VIM
37 pancreatic serous cystadenoma 10.6 KRT7 VEGFA
38 primary effusion lymphoma 10.6 IL6 VEGFA
39 castleman disease 10.6 DES IL6
40 melanotic neuroectodermal tumor 10.5 S100B VIM
41 renal pelvis carcinoma 10.5 KRT7 VEGFA
42 macular retinal edema 10.5 IL6 VEGFA
43 ovarian benign neoplasm 10.5 DES KRT7
44 malignant peritoneal mesothelioma 10.5 MUC1 VIM
45 horner's syndrome 10.5 DES KRT7
46 papillary tumor of the pineal region 10.5 MUC1 VIM
47 central retinal vein occlusion 10.5 IL6 VEGFA
48 endometrial mucinous adenocarcinoma 10.4 MUC1 VIM
49 eales disease 10.4 IL6 VEGFA
50 congenital epulis 10.4 DES VIM

Graphical network of the top 20 diseases related to Spindle Cell Sarcoma:



Diseases related to Spindle Cell Sarcoma

Symptoms & Phenotypes for Spindle Cell Sarcoma

GenomeRNAi Phenotypes related to Spindle Cell Sarcoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased focal adhesion (FA) area, decreased FA length, decreased FA mean intensity, increased number of small and round FAs, increased FA abundance GR00210-A 8.92 MUC1 NTRK3 VEGFA VIM

MGI Mouse Phenotypes related to Spindle Cell Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.92 MDM2 NTRK3 VEGFA ACTC1 VIM DES
2 cellular MP:0005384 9.86 KRT7 MDM2 VEGFA ACTC1 VIM DES
3 homeostasis/metabolism MP:0005376 9.81 KRT7 MDM2 S100B VEGFA ACTC1 VIM
4 muscle MP:0005369 9.43 MDM2 VEGFA ACTC1 VIM DES IL6
5 normal MP:0002873 9.1 KMT2B MDM2 NTRK3 S100B VEGFA ACTC1

Drugs & Therapeutics for Spindle Cell Sarcoma

FDA approved drugs:

# Drug Name Active Ingredient(s) 18 Company Approval Date
1
Doxil 18 49 DOXORUBICIN HYDROCHLORIDE Alza June 1999
2
Fusilev 18 LEVOLEUCOVORIN CALCIUM Spectrum Pharmaceuticals March of 2008
3
Intron A 18 49 INTERFERON ALFA-2B Schering-Plough December 1997/ December 1995/ March 1997
4
Taxol 18 49 PACLITAXEL Bristol-Myers Squibb August 1997
5
Votrient 18 49 PAZOPANIB HYDROCHLORIDE GlaxoSmithKline April 2012/ October of 2009

Drugs for Spindle Cell Sarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 36)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 1, Phase 2 23214-92-8 31703
2
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
3
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
4
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177
5
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 5284616 6436030 46835353
6
Levoleucovorin Approved, Investigational Phase 2 68538-85-2
7
leucovorin Approved, Nutraceutical Phase 2 58-05-9 143 6006
8
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
9
Doxil Approved June 1999 Phase 1, Phase 2 31703
10 Folic Acid Antagonists Phase 2
11 Topoisomerase Inhibitors Phase 1, Phase 2
12 Dermatologic Agents Phase 2
13 Nucleic Acid Synthesis Inhibitors Phase 2
14 Anti-Bacterial Agents Phase 1, Phase 2
15 Vitamin B Complex Phase 2
16 Antibiotics, Antitubercular Phase 1, Phase 2
17 Immunosuppressive Agents Phase 1, Phase 2,Phase 2
18 Anti-Infective Agents Phase 1, Phase 2
19 Antifungal Agents Phase 1, Phase 2
20 Antirheumatic Agents Phase 2
21 Antimetabolites Phase 2
22 Antimetabolites, Antineoplastic Phase 2
23 Micronutrients Phase 2
24 Hematinics Phase 2
25 Trace Elements Phase 2
26 Vitamins Phase 2
27 Antidotes Phase 2
28 Protective Agents Phase 2
29 Folate Nutraceutical Phase 2
30 Vitamin B9 Nutraceutical Phase 2
31
Cisplatin Approved 15663-27-1 84093 441203 2767
32
Mechlorethamine Approved, Investigational 51-75-2 4033
33
Ifosfamide Approved 3778-73-2 3690
34
Isophosphamide mustard 0
35 Alkylating Agents
36 Antineoplastic Agents, Alkylating

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Safety and Efficacy Study of Torisel and Liposomal Doxorubicin for Patients With Recurrent Sarcoma Completed NCT00949325 Phase 1, Phase 2 temsirolimus (Torisel) plus liposomal doxorubicin (Doxil)
2 Will Glucarpidase After Methotrexate Treatment for Bone Sarcoma Lead to Fewer Side Effects and Reduce Chemotherapy Delays? Terminated NCT02022358 Phase 2 Glucarpidase;Methotrexate;Folinic Acid
3 European Study in Bone Sarcoma Patients Over 40 Years Completed NCT02986503 Doxorubicin+cisplatin+ifosfamide;Doxorubicin+cisplatin+ifosfamide+methotrexate

Search NIH Clinical Center for Spindle Cell Sarcoma

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Cochrane evidence based reviews: sarcoma

Genetic Tests for Spindle Cell Sarcoma

Anatomical Context for Spindle Cell Sarcoma

MalaCards organs/tissues related to Spindle Cell Sarcoma:

41
Lung, Liver, Kidney, Skin, Endothelial, Bone, T Cells

Publications for Spindle Cell Sarcoma

Articles related to Spindle Cell Sarcoma:

(show top 50) (show all 144)
# Title Authors Year
1
Recombinant methioninase in combination with doxorubicin (DOX) overcomes first-line DOX resistance in a patient-derived orthotopic xenograft nude-mouse model of undifferentiated spindle-cell sarcoma. ( 29306021 )
2018
2
Mutational analysis of high-grade spindle cell sarcoma of the femur in Mazabraud's syndrome. ( 29804209 )
2018
3
Spindle cell sarcoma: a SEER population-based analysis. ( 29568070 )
2018
4
Primary intracranial spindle cell sarcoma with rhabdomyosarcoma-like features share a highly distinct methylation profile and DICER1 mutations. ( 29881993 )
2018
5
Temozolomide regresses a doxorubicin-resistant undifferentiated spindle-cell sarcoma patient-derived orthotopic xenograft (PDOX): precision-oncology nude-mouse model matching the patient with effective therapy. ( 29737543 )
2018
6
Growth of doxorubicin-resistant undifferentiated spindle-cell sarcoma PDOX is arrested by metabolic targeting with recombinant methioninase. ( 29143983 )
2017
7
Oral Spindle Cell Sarcoma: A Rare Case Report and Review of Literature. ( 28571292 )
2017
8
Surgical Management of Primary Spindle Cell Sarcoma of Prostate. ( 28411101 )
2017
9
Spindle Cell Sarcoma of the Iliac Vein on 18 F-FDG PET/CT. ( 28134693 )
2017
10
Spindle cell sarcoma of the mitral valve: a rare indication for mitral valve replacement. ( 29029000 )
2017
11
Recurrent BRAF Gene Fusions in a Subset of Pediatric Spindle Cell Sarcomas: Expanding the Genetic Spectrum of Tumors With Overlapping Features With Infantile Fibrosarcoma. ( 28877062 )
2017
12
Unusual case of spindle cell sarcoma metastases to right ventricle: a case report and a literature review. ( 28936438 )
2017
13
Intimal spindle cell sarcoma masquerading as adult-onset symptomatic pulmonic stenosis: a case report and review of the literature. ( 29084562 )
2017
14
Spindle-cell sarcoma involving the major pulmonary arteries. ( 28670068 )
2017
15
TFG-MET fusion in an infantile spindle cell sarcoma with neural features. ( 28510278 )
2017
16
Post-irradiation vaginal spindle cell sarcoma. ( 29154376 )
2017
17
High Efficacy of Pazopanib on an Undifferentiated Spindle-Cell Sarcoma Resistant to First-Line Therapy Is Identified With a Patient-Derived Orthotopic Xenograft (PDOX) Nude Mouse Model. ( 28176365 )
2017
18
Gemcitabine and docetaxel in relapsed and unresectable high-grade osteosarcoma and spindle cell sarcoma of bone. ( 27098543 )
2016
19
Right atrial spindle cell sarcoma as a rare cause of tricuspid stenosis. ( 27436036 )
2016
20
Paediatric and adult soft tissue sarcomas with NTRK1 gene fusions: a subset of spindle cell sarcomas unified by a prominent myopericytic/haemangiopericytic pattern. ( 26863915 )
2016
21
Spindle Cell Sarcoma Presenting as Pancoast Syndrome. ( 27504558 )
2016
22
Direct Tumor Embolization of Sinonasal Unclassified Spindle Cell Sarcoma with Onyx. ( 27652195 )
2016
23
Spindle cell sarcoma of the left atrium: an extremely rare and challenging tumour often masquerading as left atrial myxoma. ( 25547565 )
2015
24
Primary spindle cell sarcoma of the prostate and (18)F-fluorodeoxyglucose-positron-emission tomography/computed tomography findings. ( 25657562 )
2015
25
Primary undifferentiated spindle cell sarcoma of the lung with metastasis to bone - A rare clinical entity with even rarer clinical course. ( 26853426 )
2015
26
Delayed diagnosis of spindle cell sarcoma presenting as a large mass on the back. ( 26194416 )
2015
27
High-grade spindle cell sarcoma of the heart: a case report and review of literature. ( 25888133 )
2015
28
CT Imaging Findings of Metastatic Spindle Cell Sarcoma of Prostate: A Case Report and Review of the Literature. ( 26180501 )
2015
29
Intimal (spindle cell) sarcoma of the left atrium presenting with abnormal neurological examination. ( 26483389 )
2015
30
A Unilateral Hemothorax as a Presenting Manifestation of Mediastinal Spindle Cell Sarcoma. ( 25416071 )
2015
31
A spindle cell sarcoma of liver supplied by internal mammary artery. ( 25533497 )
2015
32
Identification of an MLL4-GPS2 fusion as an oncogenic driver of undifferentiated spindle cell sarcoma in a child. ( 25139254 )
2014
33
Spindle cell sarcoma of pulmonary artery mimicking thromboembolism with lung metastasis detected in fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography. ( 25400365 )
2014
34
Several fusion genes identified by whole transcriptome sequencing in a spindle cell sarcoma with rearrangements of chromosome arm 12q and MDM2 amplification. ( 25176350 )
2014
35
Metastatic Undifferentiated Spindle Cell Sarcoma of the Thyroid Gland Evaluated by 18F-FDG PET/CT. ( 25140545 )
2014
36
Spindle cell sarcoma of thyroid: a case report. ( 25767348 )
2014
37
Spindle cell sarcoma of the mitral valve: an unusual cause of acute coronary syndrome in a child. ( 25282213 )
2014
38
Spindle Cell Sarcoma of the Vulva With Myofibroblastic Differentiation. ( 25062403 )
2014
39
Primary Intimal (Spindle Cell) Sarcoma of the Left Atrium. ( 25196496 )
2014
40
Primary spindle cell sarcoma of the breast masquerading as necrotizing fasciitis. ( 24876317 )
2014
41
Transthoracic echocardiographic assessment of spindle cell sarcoma of the pulmonary artery in a child. ( 24606227 )
2014
42
Recurrent cardiac intimal (spindle cell) sarcoma of the left atrium. ( 21924626 )
2013
43
Images in vascular medicine. Spindle cell sarcoma of the common femoral vein as a possible differential for deep venous thrombosis on ultrasound. ( 22951535 )
2013
44
Primary undifferentiated spindle-cell sarcoma of sella turcica: successful treatment with adjuvant temozolomide. ( 23715844 )
2013
45
Primary intimal (spindle cell) sarcoma of the heart: a case report and review of the literature. ( 23424592 )
2013
46
An unusually large spindle cell sarcoma of the tongue in a male child. ( 24669028 )
2013
47
Left atrial spindle cell sarcoma - case report. ( 22929830 )
2012
48
Primary pericardial spindle cell sarcoma mimicking left main coronary artery disease. ( 22219472 )
2012
49
Pleomorphic spindle cell sarcoma (PSCS) formerly known as malignant fibrous histiocytoma (MFH): a complex malignant soft-tissue tumor. ( 23129168 )
2012
50
Radiation-induced high-grade spindle cell sarcoma of the sternomastoid muscle: a case report. ( 23250072 )
2012

Variations for Spindle Cell Sarcoma

Expression for Spindle Cell Sarcoma

Search GEO for disease gene expression data for Spindle Cell Sarcoma.

Pathways for Spindle Cell Sarcoma

GO Terms for Spindle Cell Sarcoma

Cellular components related to Spindle Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 8.8 DES KRT7 VIM

Biological processes related to Spindle Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.67 IL6 MUC1 VEGFA VIM
2 negative regulation of apoptotic process GO:0043066 9.65 ACTC1 IL6 MDM2 NTRK3 VEGFA
3 cellular response to hypoxia GO:0071456 9.61 MDM2 S100B VEGFA
4 positive regulation of cell proliferation GO:0008284 9.55 IL6 MDM2 NTRK3 S100B VEGFA
5 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.5 IL6 NTRK3 VEGFA
6 intermediate filament organization GO:0045109 9.46 DES VIM
7 positive regulation of positive chemotaxis GO:0050927 9.4 NTRK3 VEGFA
8 muscle filament sliding GO:0030049 9.13 ACTC1 DES VIM
9 positive regulation of gene expression GO:0010628 9.1 ACTC1 IL6 MDM2 NTRK3 VEGFA VIM

Molecular functions related to Spindle Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.35 DES MDM2 S100B VEGFA VIM
2 p53 binding GO:0002039 8.8 MDM2 MUC1 NTRK3

Sources for Spindle Cell Sarcoma

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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