MCID: SPN035
MIFTS: 50

Spindle Cell Sarcoma

Categories: Cancer diseases

Aliases & Classifications for Spindle Cell Sarcoma

MalaCards integrated aliases for Spindle Cell Sarcoma:

Name: Spindle Cell Sarcoma 12 6 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 epithelioid sarcoma and endometrial stromal sarcoma. An important gene associated with Spindle Cell Sarcoma is NF2 (Neurofibromin 2), and among its related pathways/superpathways are Epstein-Barr virus infection and AKT Signaling Pathway. The drugs Doxil and Fusilev have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and kidney, and related phenotypes are behavior/neurological 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 1262)
# Related Disease Score Top Affiliating Genes
1 epithelioid sarcoma 33.6 KRT7 MUC1 VIM
2 endometrial stromal sarcoma 33.6 ACTC1 DES VIM
3 small cell sarcoma 33.4 MDM2 NF2
4 biphasic synovial sarcoma 33.2 DES MUC1 VIM
5 reticulum cell sarcoma 33.1 ACTC1 DES MUC1 VIM
6 extraskeletal ewing sarcoma 33.1 DES MUC1 VIM
7 botryoid rhabdomyosarcoma 32.9 ACTC1 DES
8 embryonal sarcoma 32.8 DES MDM2 S100B VIM
9 monophasic synovial sarcoma 32.7 KRT7 MUC1 S100B VIM
10 gastrointestinal stromal tumor 32.1 ACTC1 DES NTRK3 S100B VIM
11 dendritic cell tumor 32.0 ACTC1 MUC1 S100B VIM
12 sarcoma, synovial 31.9 DES KRT7 MDM2 MUC1 VIM
13 malignant mesenchymoma 31.8 DES MDM2 NTRK3
14 desmoplastic small round cell tumor 31.8 DES MUC1 VIM
15 fibromatosis 31.7 ACTC1 DES VIM
16 pseudosarcomatous fibromatosis 31.7 ACTC1 DES VIM
17 malignant peripheral nerve sheath tumor 31.6 ACTC1 MDM2 MUC1 NF2 S100B VIM
18 mesothelioma, malignant 31.5 KRT7 MUC1 NF2 VIM
19 chordoma 31.3 DES KRT7 MUC1 S100B VIM
20 carcinosarcoma 31.2 ACTC1 DES KRT7 MUC1 VIM
21 leiomyosarcoma 31.2 ACTC1 DES MDM2 S100B VIM
22 hemangiopericytoma, malignant 31.0 ACTC1 DES MDM2 MUC1 S100B VIM
23 liposarcoma 30.5 DES MDM2 VIM
24 fibrosarcoma 30.3 DES NTRK3 VIM
25 leiomyoma 30.3 ACTC1 DES VIM
26 lymphangioma 30.1 ACTC1 VIM
27 lymphoma, non-hodgkin, familial 30.1 CDKN1B KMT2D MDM2
28 mesenchymoma 30.0 DES MDM2
29 myxofibrosarcoma 29.9 DES S100B
30 retroperitoneal fibrosis 29.8 MDM2 VIM
31 cholangiocarcinoma 29.8 CDKN1B KRT7 MUC1
32 intravascular papillary endothelial hyperplasia 29.8 ACTC1 VIM
33 epulis 29.8 DES VIM
34 mucinous cystadenocarcinoma 29.7 KRT7 MUC1 VIM
35 sertoli-leydig cell tumor 29.7 KRT7 MUC1
36 fibrous histiocytoma 29.7 ACTC1 DES S100B VIM
37 dedifferentiated liposarcoma 29.7 ACTC1 DES MDM2
38 osteochondroma 29.6 S100B VIM
39 rhabdomyosarcoma 29.6 ACTC1 DES MDM2 VIM
40 neurofibroma 29.6 MUC1 NF2 S100B
41 plasmablastic lymphoma 29.6 DES MUC1
42 cavernous hemangioma 29.6 KRT7 MUC1 VIM
43 cystic nephroma 29.6 DES VIM
44 smooth muscle tumor 29.6 ACTC1 DES VIM
45 fasciitis 29.5 ACTC1 DES MUC1 VIM
46 ewing sarcoma 29.5 DES MDM2 S100B VIM
47 giant cell tumor 29.5 ACTC1 DES MUC1 VIM
48 lipomatosis, multiple 29.5 DES MDM2
49 osteofibrous dysplasia 29.4 MUC1 VIM
50 proximal spinal muscular atrophy 29.4 DES MUC1

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



Diseases related to Spindle Cell Sarcoma

Symptoms & Phenotypes for Spindle Cell Sarcoma

MGI Mouse Phenotypes related to Spindle Cell Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.86 CDKN1B DES KMT2D MDM2 NF2 NTRK3
2 cardiovascular system MP:0005385 9.7 ACTC1 CDKN1B DES KMT2D MDM2 NTRK3
3 homeostasis/metabolism MP:0005376 9.61 ACTC1 CDKN1B DES KMT2D KRT7 MDM2
4 muscle MP:0005369 9.1 ACTC1 CDKN1B DES KMT2D MDM2 VIM

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 37)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 46835353 6436030 5284616
2
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177
3
Doxorubicin Approved, Investigational Phase 1, Phase 2 23214-92-8 31703
4
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
5
leucovorin Approved Phase 2 58-05-9 6006 143
6
Methotrexate Approved Phase 2 59-05-2, 1959-05-2 126941
7
Levoleucovorin Approved, Investigational Phase 2 68538-85-2
8
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
9
Doxil Approved June 1999 Phase 1, Phase 2 31703
10 Anti-Infective Agents Phase 1, Phase 2
11 Antibiotics, Antitubercular Phase 1, Phase 2
12 Topoisomerase Inhibitors Phase 1, Phase 2
13 Immunologic Factors Phase 1, Phase 2,Phase 2
14 Immunosuppressive Agents Phase 1, Phase 2,Phase 2
15 Antifungal Agents Phase 1, Phase 2
16 Anti-Bacterial Agents Phase 1, Phase 2
17 Vitamin B Complex Phase 2
18 Vitamin B9 Phase 2
19 Antimetabolites, Antineoplastic Phase 2
20 Antirheumatic Agents Phase 2
21 Antimetabolites Phase 2
22 Folic Acid Antagonists Phase 2
23 Folate Phase 2
24 Dermatologic Agents Phase 2
25 Nucleic Acid Synthesis Inhibitors Phase 2
26 Protective Agents Phase 2
27 Micronutrients Phase 2
28 Trace Elements Phase 2
29 Vitamins Phase 2
30 Hematinics Phase 2
31 Antidotes Phase 2
32
Cisplatin Approved 15663-27-1 2767 441203 84093
33
Mechlorethamine Approved, Investigational 51-75-2 4033
34
Ifosfamide Approved 3778-73-2 3690
35 Antineoplastic Agents, Alkylating
36
Isophosphamide mustard 0
37 Alkylating Agents

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
Bone, Lung, Kidney, Liver, Skin, Myeloid, Prostate

Publications for Spindle Cell Sarcoma

Articles related to Spindle Cell Sarcoma:

(show top 50) (show all 968)
# Title Authors Year
1
Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5). ( 30255545 )
2019
2
Early recognition and diagnosis of Ewing sarcoma of the cervical spine. ( 30416636 )
2019
3
Favorable response to nivolumab in a young adult patient with metastatic histiocytic sarcoma. ( 30270506 )
2019
4
KSHV viral load and Interleukin-6 in HIV-associated pediatric Kaposi sarcoma-Exploring the role of lytic activation in driving the unique clinical features seen in endemic regions. ( 30204240 )
2019
5
Kaposi's Sarcoma-Associated Herpesvirus (KSHV)-Associated Disease in the AIDS Patient: An Update. ( 30523621 )
2019
6
Cardiotoxicity with trabectedin in the treatment of advanced soft tissue sarcoma. ( 30540596 )
2019
7
A rare case of nasal biphenotypic sino-nasal sarcoma in a young female. ( 30555691 )
2019
8
Re‑irradiation plus hyperthermia for recurrent pediatric sarcoma; a simulation study to investigate feasibility. ( 30387837 )
2019
9
Localized synovial sarcoma of the foot or ankle: A series of 32 Cooperative Weichteilsarkom Study Group patients. ( 30421433 )
2019
10
Synovial sarcoma of bone: Sarcoma typically of soft tissues presenting as a primary bone tumor. ( 30425775 )
2019
11
miR‑494.3p expression in synovial sarcoma: Role of CXCR4 as a potential target gene. ( 30431073 )
2019
12
Outcomes of Elderly Patients Undergoing Curative Resection for Retroperitoneal Sarcomas: Analysis From the US Sarcoma Collaborative. ( 30502242 )
2019
13
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
14
Mutational analysis of high-grade spindle cell sarcoma of the femur in Mazabraud's syndrome. ( 29804209 )
2018
15
Spindle cell sarcoma: a SEER population-based analysis. ( 29568070 )
2018
16
Primary intracranial spindle cell sarcoma with rhabdomyosarcoma-like features share a highly distinct methylation profile and DICER1 mutations. ( 29881993 )
2018
17
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
18
Acquired Hemophilia Secondary to Soft-tissue Sarcoma: Case Report from a Latin American Hospital and Literature Review. ( 30027013 )
2018
19
Pleomorphic carcinoma of the lung mimicking synchronous pulmonary adenocarcinoma and small bowel sarcoma. ( 30416598 )
2018
20
Pseudoprogression of CNS metastatic disease of alveolar soft part sarcoma during anti-PDL1 treatment. ( 29991973 )
2018
21
Response to Immune Checkpoint Inhibition in Two Patients with Alveolar Soft-Part Sarcoma. ( 30018044 )
2018
22
Alveolar soft-part sarcoma: Primary metastatic disease and metastatic relapse occurring during long-term follow-up: Treatment results of four Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry. ( 30124238 )
2018
23
Alveolar Soft-Part Sarcoma of the Tongue. ( 30235167 )
2018
24
Positive Tumor Response to Combined Checkpoint Inhibitors in a Patient With Refractory Alveolar Soft Part Sarcoma: A Case Report. ( 30241159 )
2018
25
A Phase II Trial of Pazopanib in Patients with Metastatic Alveolar Soft Part Sarcoma. ( 30254189 )
2018
26
Diagnosis, Prognosis, and Treatment of Alveolar Soft-Part Sarcoma: A Review. ( 30347044 )
2018
27
Fine-needle aspiration of alveolar soft part sarcoma: Histologic correlation and aberrant CD68 expression. ( 30457206 )
2018
28
Radiation-associated sarcoma after breast cancer in a nationwide population: Increasing risk of angiosarcoma. ( 30044058 )
2018
29
Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment. ( 30262126 )
2018
30
Synovial sarcoma of the aortopulmonary window. ( 30467895 )
2018
31
Primary thyroid biphasic synovial sarcoma and synchronous papillary carcinoma: report of a remarkable case. ( 30546147 )
2018
32
Advanced Stage Breast Sarcoma Treatment in a Third World Country Public Hospital. ( 30174970 )
2018
33
Plastic Bronchitis in an AIDS Patient with Pulmonary Kaposi Sarcoma. ( 30363701 )
2018
34
Newly diagnosed AIDS with neurosyphilis, Kaposi sarcoma, pancytopenia, oropharyngeal candidiasis, and pseudomonal pneumonia: We shouldn't be seeing this anymore. ( 30101071 )
2018
35
Metastatic esophageal carcinosarcoma comprising neuroendocrine carcinoma, squamous cell carcinoma, and sarcoma: A case report. ( 30313107 )
2018
36
Could miRNA Signatures be Useful for Predicting Uterine Sarcoma and Carcinosarcoma Prognosis and Treatment? ( 30200635 )
2018
37
Characterizing sarcoma dominance pattern in uterine carcinosarcoma: Homologous versus heterologous element. ( 30217299 )
2018
38
Cardiac arrest due to coronary embolism from a metastatic sarcoma recovered through aspiration thrombectomy. ( 30069407 )
2018
39
Giant primary pericardial synovial sarcoma in an adolescent with cardiac tamponade: Contrast-enhanced CT and 18F-FDG PET/CT findings. ( 30088197 )
2018
40
Intraluminal EWSR1-CREB1 gene rearranged, low-grade myxoid sarcoma of the pulmonary artery resembling extraskeletal myxoid chondrosarcoma (EMC). ( 30326150 )
2018
41
Acroangiodermatitis of Mali (Pseudo-Kaposi Sarcoma) Associated with Chronic Venous Insufficiency and Obesity: A Case Report. ( 30457563 )
2018
42
Therapeutic potential of CPI-613 for targeting tumorous mitochondrial energy metabolism and inhibiting autophagy in clear cell sarcoma. ( 29879220 )
2018
43
Significance of MRI in the diagnosis and differentiation of clear cell sarcoma of tendon and aponeurosis (CCSTA): A case report. ( 30075493 )
2018
44
Preoperative diagnosis of clear cell sarcoma of the kidney by detection of BCOR internal tandem duplication in circulating tumor DNA. ( 30126017 )
2018
45
Review of the medical literature and assessment of current utilization patterns regarding the use of two common fluorescence in situ hybridization assays in the diagnosis of dermatofibrosarcoma protuberans and clear cell sarcoma. ( 30155908 )
2018
46
Establishment and Characterization of a Novel Human Clear-cell Sarcoma of Soft-tissue Cell Line, RSAR001, Derived from Pleural Effusion of a Patient with Pleural Dissemination. ( 30194147 )
2018
47
Clear cell sarcoma-A review. ( 30210202 )
2018
48
Detection of specific gene rearrangements by fluorescence in situ hybridization in 16 cases of clear cell sarcoma of soft tissue and 6 cases of clear cell sarcoma-like gastrointestinal tumor. ( 30219084 )
2018
49
Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report. ( 30262251 )
2018
50
Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for treatment of clear cell sarcoma of the kidney in children. ( 30318310 )
2018

Variations for Spindle Cell Sarcoma

ClinVar genetic disease variations for Spindle Cell Sarcoma:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 NF2 NM_000268.3(NF2): c.583_589delCACCGAG (p.His195Alafs) deletion Pathogenic GRCh37 Chromosome 22, 30051649: 30051655
2 NF2 NM_000268.3(NF2): c.583_589delCACCGAG (p.His195Alafs) deletion Pathogenic GRCh38 Chromosome 22, 29655660: 29655666
3 MGA; NUTM1 der(15)t(5: 15)(q23.2;q15.1) Translocation Pathogenic
4 subset of 62 genes:ACTC1; MEIS2; SPRED1 GRCh37/hg19 15q14-15.1 chr15: 34638237..42057083 complex variant complex Pathogenic GRCh37 Chromosome 15, 34638237: 42057083

Expression for Spindle Cell Sarcoma

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

Pathways for Spindle Cell Sarcoma

Pathways related to Spindle Cell Sarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.85 CDKN1B MDM2 VIM
2 11.65 CDKN1B MDM2 NTRK3
3 11.59 ACTC1 DES KRT7 MUC1 NF2 VIM
4 11.48 CDKN1B MDM2 NF2
5
Show member pathways
11.37 DES KRT7 VIM
6 10.99 ACTC1 DES VIM

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 cytoplasm GO:0005737 9.32 ACTC1 CDKN1B DES KRT7 MDM2 MUC1
2 intermediate filament GO:0005882 9.13 DES KRT7 VIM

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of apoptotic process GO:0043066 9.73 ACTC1 CDKN1B MDM2 NTRK3
2 positive regulation of gene expression GO:0010628 9.67 ACTC1 MDM2 NTRK3 VIM
3 positive regulation of cell proliferation GO:0008284 9.55 CDKN1B KMT2D MDM2 NTRK3 S100B
4 intermediate filament organization GO:0045109 9.4 DES VIM
5 lens fiber cell differentiation GO:0070306 9.32 NF2 NTRK3
6 cellular response to antibiotic GO:0071236 9.26 CDKN1B MDM2
7 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 9.13 CDKN1B MDM2 MUC1
8 muscle filament sliding GO:0030049 8.8 ACTC1 DES VIM

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

# Name GO ID Score Top Affiliating Genes
1 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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