MCID: SPN035
MIFTS: 52

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 45 74
Sarcoma, Spindle Cell 74
Sarcoma Spindle Cell 56
Sarcoma - Category 74

Classifications:



External Ids:

Disease Ontology 12 DOID:4235
MeSH 45 D012509
NCIt 51 C27005
SNOMED-CT 69 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 small cell sarcoma and endometrial stromal sarcoma. An important gene associated with Spindle Cell Sarcoma is NF2 (Neurofibromin 2), and among its related pathways/superpathways are Dilated cardiomyopathy (DCM) and Cellular senescence (KEGG). The drugs Miconazole and Sirolimus have been mentioned in the context of this disorder. Affiliated tissues include bone, myeloid and kidney, and related phenotypes are behavior/neurological and cardiovascular system

Wikipedia : 77 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 1341)
# Related Disease Score Top Affiliating Genes
1 small cell sarcoma 33.7 MDM2 NF2
2 endometrial stromal sarcoma 33.5 ACTC1 DES IL6 VIM
3 epithelioid sarcoma 33.2 KRT7 MUC1 VIM
4 botryoid rhabdomyosarcoma 33.1 ACTC1 DES
5 biphasic synovial sarcoma 32.9 DES MUC1 VIM
6 embryonal sarcoma 32.8 DES MDM2 S100B VIM
7 reticulum cell sarcoma 32.8 ACTC1 DES MUC1 VIM
8 extraskeletal ewing sarcoma 32.8 DES MUC1 VIM
9 monophasic synovial sarcoma 32.4 KRT7 MUC1 S100B VIM
10 gastrointestinal stromal tumor 32.1 ACTC1 DES NTRK3 S100B VIM
11 liposarcoma 32.0 DES MDM2 VIM
12 dendritic cell tumor 31.9 ACTC1 MUC1 S100B VIM
13 fibromatosis 31.8 ACTC1 DES VIM
14 pseudosarcomatous fibromatosis 31.8 ACTC1 DES VIM
15 malignant mesenchymoma 31.8 DES MDM2 NTRK3
16 desmoplastic small round cell tumor 31.5 DES MUC1 VIM
17 malignant peripheral nerve sheath tumor 31.4 ACTC1 MDM2 MUC1 NF2 S100B VIM
18 sarcoma, synovial 31.3 DES KRT7 MDM2 MUC1 VIM
19 leiomyosarcoma 31.2 ACTC1 DES MDM2 S100B VIM
20 mesothelioma, malignant 31.0 KRT7 MUC1 NF2 VIM
21 chordoma 30.7 DES KRT7 MUC1 S100B VIM
22 carcinosarcoma 30.7 ACTC1 DES KRT7 MUC1 VIM
23 lymphangioma 30.7 ACTC1 VIM
24 hemangiopericytoma, malignant 30.6 ACTC1 DES MDM2 MUC1 S100B VIM
25 leiomyoma 30.4 ACTC1 DES VIM
26 fibrosarcoma 30.2 DES NTRK3 VIM
27 mesenchymoma 30.1 DES MDM2
28 intravascular papillary endothelial hyperplasia 30.1 ACTC1 VIM
29 retroperitoneal fibrosis 30.1 MDM2 VIM
30 mammary paget's disease 29.9 KRT7 MUC1
31 myxofibrosarcoma 29.9 DES MDM2 S100B
32 epulis 29.9 DES VIM
33 fibrous histiocytoma 29.8 ACTC1 DES S100B VIM
34 dedifferentiated liposarcoma 29.8 ACTC1 DES MDM2
35 gliosarcoma 29.7 ACTC1 S100B VIM
36 smooth muscle tumor 29.7 ACTC1 DES VIM
37 cystic nephroma 29.7 DES VIM
38 rhabdomyosarcoma 29.7 ACTC1 DES MDM2 VIM
39 congenital fibrosarcoma 29.5 ACTC1 DES NTRK3 VIM
40 neurofibroma 29.5 MUC1 NF2 S100B
41 cellular schwannoma 29.5 MDM2 NF2 S100B
42 dermatofibrosarcoma protuberans 29.5 ACTC1 DES S100B VIM
43 ovarian fibrothecoma 29.4 ACTC1 DES
44 cholangiocarcinoma 29.4 IL6 KRT7 MUC1
45 sertoli-leydig cell tumor 29.4 KRT7 MUC1
46 horseshoe kidney 29.4 ACTC1 KRT7
47 malignant giant cell tumor 29.4 ACTC1 S100B
48 myopericytoma 29.4 ACTC1 S100B
49 amelanotic melanoma 29.4 S100B VIM
50 mucinous cystadenocarcinoma 29.4 KRT7 MUC1 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

MGI Mouse Phenotypes related to Spindle Cell Sarcoma:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.97 CCNE1 DES IL6 MDM2 NF2 NTRK3
2 cardiovascular system MP:0005385 9.92 ACTC1 CCNE1 DES IL6 KMT2B MDM2
3 cellular MP:0005384 9.86 ACTC1 CCNE1 DES IL6 KMT2B KRT7
4 homeostasis/metabolism MP:0005376 9.81 ACTC1 DES IL6 KMT2B KRT7 MDM2
5 mortality/aging MP:0010768 9.61 ACTC1 CCNE1 DES IL6 KMT2B MDM2
6 reproductive system MP:0005389 9.17 CCNE1 IL6 KMT2B MDM2 NF2 NTRK3

Drugs & Therapeutics for Spindle Cell Sarcoma

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

(show all 47)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
2
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 46835353 6436030 5284616
3
Doxorubicin Approved, Investigational Phase 1, Phase 2,Phase 2 23214-92-8 31703
4
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177 70789204
5
Paclitaxel Approved, Vet_approved Phase 1, Phase 2 33069-62-4 36314
6
leucovorin Approved Phase 2 58-05-9 6006 143
7
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
8
Levoleucovorin Approved, Investigational Phase 2 68538-85-2
9
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
10
Doxil Approved June 1999 Phase 1, Phase 2,Phase 2 31703
11 Anti-Bacterial Agents Phase 1, Phase 2,Phase 2
12 Anti-Infective Agents Phase 1, Phase 2
13 Immunosuppressive Agents Phase 1, Phase 2,Phase 2
14 Immunologic Factors Phase 1, Phase 2,Phase 2
15 Topoisomerase Inhibitors Phase 1, Phase 2,Phase 2
16 Antibiotics, Antitubercular Phase 1, Phase 2,Phase 2
17 Antifungal Agents Phase 1, Phase 2
18 taxane Phase 1, Phase 2
19 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
20 Antimitotic Agents Phase 1, Phase 2
21 Albumin-Bound Paclitaxel Phase 1, Phase 2
22 Folate Phase 2
23 Vitamin B9 Phase 2
24 Antimetabolites, Antineoplastic Phase 2
25 Nucleic Acid Synthesis Inhibitors Phase 2
26 Dermatologic Agents Phase 2
27 Antirheumatic Agents Phase 2
28 Folic Acid Antagonists Phase 2
29 Antimetabolites Phase 2
30 Vitamin B Complex Phase 2
31 Immunoglobulins Phase 2
32 Antibodies, Monoclonal Phase 2
33 Antibodies Phase 2
34 Micronutrients Phase 2
35 Protective Agents Phase 2
36 Nutrients Phase 2
37 Vitamins Phase 2
38 Trace Elements Phase 2
39 Antidotes Phase 2
40 Hematinics Phase 2
41
Ifosfamide Approved 3778-73-2 3690
42
Cisplatin Approved 15663-27-1 441203 84093 2767
43
Mechlorethamine Approved, Investigational 51-75-2 4033
44 Antineoplastic Agents, Alkylating
45
Isophosphamide mustard 0
46 Alkylating Agents
47 lysine

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 plus liposomal doxorubicin
2 Study to Find a Safe Dose and Show Early Clinical Activity of Weekly Nab-paclitaxel in Pediatric Patients With Recurrent/ Refractory Solid Tumors Completed NCT01962103 Phase 1, Phase 2 nab-paclitaxel;nab-paclitaxel
3 APX005M and Doxorubicin in Advanced Sarcoma Not yet recruiting NCT03719430 Phase 2 Doxorubicin;APX005M
4 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
5 European Study in Bone Sarcoma Patients Over 40 Years Completed NCT02986503 Doxorubicin+cisplatin+ifosfamide;Doxorubicin+cisplatin+ifosfamide+methotrexate
6 Tazemetostat Expanded Access Program for Adults With Solid Tumors Temporarily not available NCT03874455 Tazemetostat

Search NIH Clinical Center for Spindle Cell Sarcoma

Inferred drug relations via UMLS 74 / NDF-RT 52 :


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:

42
Bone, Myeloid, Kidney, Skin, Endothelial, Prostate, Thyroid

Publications for Spindle Cell Sarcoma

Articles related to Spindle Cell Sarcoma:

(show top 50) (show all 2204)
# Title Authors Year
1
A Phase II Trial of Pazopanib in Patients with Metastatic Alveolar Soft Part Sarcoma. ( 30254189 )
2019
2
Fine-needle aspiration of alveolar soft part sarcoma: Histologic correlation and aberrant CD68 expression. ( 30457206 )
2019
3
Brain Metastases and Place of Antiangiogenic Therapies in Alveolar Soft Part Sarcoma: A Retrospective Analysis of the French Sarcoma Group. ( 30626710 )
2019
4
Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion. ( 30744536 )
2019
5
Alveolar soft part sarcoma metastatic to the breast: a case report. ( 30832622 )
2019
6
Strategies to target energy metabolism in consensus molecular subtype 3 along with Kirsten rat sarcoma viral oncogene homolog mutations for colorectal cancer therapy. ( 30341899 )
2019
7
Spindle Cell Sarcoma Mimicking Supraclavicular Lymph Node Metastasis of Breast Cancer. ( 30760697 )
2019
8
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
9
Primary clear cell sarcoma of the calcaneus : Report of a rare case and review of the literature. ( 30623235 )
2019
10
Clear Cell Sarcoma of the Kidney. ( 30628851 )
2019
11
CRTC1-TRIM11 Fusion in a Case of Metastatic Clear Cell Sarcoma: Are CRTC1-TRIM11 Fusion-bearing Tumors Melanocytomas or Clear Cell Sarcomas? ( 30676334 )
2019
12
Expression of cyclin D1 in clear cell sarcoma of kidney. Is it useful in differentiating it from its histological mimics? ( 30736802 )
2019
13
Clear cell sarcoma of the soft palate mimicking unclassified melanoma. ( 30837084 )
2019
14
BCOR Overexpression in Renal Malignant Solitary Fibrous Tumors: A Close Mimic of Clear Cell Sarcoma of Kidney. ( 30864973 )
2019
15
Intra-extracranial primary clear cell sarcoma: the first report and review of the literature. ( 30880192 )
2019
16
Current Update on the Molecular Biology of Cutaneous Sarcoma: Dermatofibrosarcoma Protuberans. ( 30874910 )
2019
17
Kaposi's sarcoma-associated herpesvirus seropositivity is associated with type 2 diabetes mellitus: A case-control study in Xinjiang, China. ( 30639407 )
2019
18
Undifferentiated embryonal sarcoma of the liver in elderly: Case report and review of the literature. ( 30706877 )
2019
19
Undifferentiated Embryonal Sarcoma of the Liver: a Great Masquerader. ( 30714072 )
2019
20
Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group. ( 30772825 )
2019
21
Tumour lysis syndrome in a patient with undifferentiated endometrial stromal sarcoma. ( 30834285 )
2019
22
Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis. ( 30640694 )
2019
23
High-grade endometrial stromal sarcoma: a retrospective study of factors influencing prognosis. ( 30697075 )
2019
24
Metastatic low-grade endometrial stromal sarcoma with sex cord and smooth muscle differentiation: A case report. ( 30705899 )
2019
25
Extra-uterine low grade endometrioid stromal sarcoma arising from ovarian endometriosis: a case report and review of the literature. ( 30723546 )
2019
26
Epithelioid Inflammatory Myofibroblastic Sarcoma. ( 29623737 )
2019
27
ALK-G1269A mutation in epithelioid inflammatory myofibroblastic sarcoma after progression on crizotinib: A case report. ( 30675302 )
2019
28
Undifferentiated epithelioid sarcoma presenting as a fever of unknown origin: a case report. ( 30684969 )
2019
29
A case of primary cutaneous Ewing sarcoma in a neutropenic patient. ( 30582191 )
2019
30
Primary Ewing sarcoma of the squamous temporal bone with intracranial and extracranial extension: A rare cause of sudden sensorineural hearing loss. ( 30584673 )
2019
31
Risk stratification by somatic mutation burden in Ewing sarcoma. ( 30602061 )
2019
32
Safety and Cost-effectiveness of Outpatient Administration of High-dose Chemotherapy in Children With Ewing Sarcoma. ( 30608490 )
2019
33
Clotam enhances anti-proliferative effect of vincristine in Ewing sarcoma cells. ( 30610505 )
2019
34
Ewing sarcoma, analysis of survival at 6 years with multidisciplinary therapy. ( 30642764 )
2019
35
Ewing sarcoma of the mobile spine; predictive factors for survival, neurological function and local control. A Scandinavian sarcoma group study with a mean follow-up of 12 years. ( 30666289 )
2019
36
Central nervous system manifestations due to iatrogenic adrenal insufficiency in a Ewing sarcoma patient. ( 30687450 )
2019
37
Primary Ewing sarcoma of the kidney. ( 30696641 )
2019
38
Precision medicine approaches for the management of Ewing sarcoma: current perspectives. ( 30697061 )
2019
39
Pregnancy-Associated Plasma Protein-A (PAPP-A) in Ewing Sarcoma: Role in Tumor Growth and Immune Evasion. ( 30698726 )
2019
40
USP19 deubiquitinates EWS-FLI1 to regulate Ewing sarcoma growth. ( 30700749 )
2019
41
Treatment Outcomes of Pediatric Patients With Ewing Sarcoma in a War-Torn Nation: A Single-Institute Experience From Iraq. ( 30707662 )
2019
42
Bone Sarcoma With EWSR1-NFATC2 Fusion: Sarcoma With Varied Morphology and Amplification of Fusion Gene Distinct From Ewing Sarcoma. ( 30714449 )
2019
43
NKX2.2 immunohistochemistry in the distinction of Ewing sarcoma from cytomorphologic mimics: Diagnostic utility and pitfalls-Comment on Russell-Goldman et al. ( 30720925 )
2019
44
Bone marrow biopsy in the initial staging of Ewing sarcoma: Experience from a single institution. ( 30724024 )
2019
45
Adamantinoma-Like Ewing Sarcoma of the Thyroid: A Case Report and Review of the Literature. ( 30734898 )
2019
46
Targeting the CALCB/RAMP1 axis inhibits growth of Ewing sarcoma. ( 30741933 )
2019
47
Getting control during follow-up visits: the views and experiences of parents on tumor surveillance after their children have completed therapy for rhabdomyosarcoma or Ewing sarcoma. ( 30747278 )
2019
48
Primary cutaneous Ewing sarcoma in a young girl. ( 30761992 )
2019
49
SCMCIE94: an intensified pilot treatment protocol known to be associated with cure in CD 56-negative non-pelvic isolated Ewing sarcoma (EWS) is also associated with no early relapses in non-metastatic extremity EWS. ( 30770960 )
2019
50
Outcomes in different age groups with primary Ewing sarcoma of the spine: a systematic review of the literature. ( 30771777 )
2019

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
Show member pathways
11.83 ACTC1 DES IL6
2 11.75 CCNE1 IL6 MDM2
3 11.59 ACTC1 DES KRT7 MUC1 NF2 VIM
4 11.55 IL6 MUC1 VIM
5 11.48 CCNE1 MDM2 NF2
6
Show member pathways
11.37 DES KRT7 VIM
7 10.99 ACTC1 DES VIM
8 10.64 DES IL6 NTRK3 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 CCNE1 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 positive regulation of cell proliferation GO:0008284 9.55 CCNE1 IL6 MDM2 NTRK3 S100B
2 positive regulation of gene expression GO:0010628 9.35 ACTC1 IL6 MDM2 NTRK3 VIM
3 intermediate filament organization GO:0045109 9.32 DES VIM
4 lens fiber cell differentiation GO:0070306 9.26 NF2 NTRK3
5 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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