MCID: SPN035
MIFTS: 53

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 endometrial stromal sarcoma and small cell sarcoma. An important gene associated with Spindle Cell Sarcoma is NF2 (Neurofibromin 2), and among its related pathways/superpathways are Development EGFR signaling pathway and MicroRNAs in cancer. The drugs Sirolimus and Doxorubicin 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 1389)
# Related Disease Score Top Affiliating Genes
1 endometrial stromal sarcoma 33.8 ACTC1 DES VIM
2 small cell sarcoma 33.7 MDM2 NF2
3 epithelioid sarcoma 33.3 KRT7 MUC1 VIM
4 botryoid rhabdomyosarcoma 33.1 ACTC1 DES
5 biphasic synovial sarcoma 32.9 DES MUC1 VIM
6 embryonal sarcoma 32.9 DES MDM2 S100B VIM
7 reticulum cell sarcoma 32.9 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 32.1 DES MDM2 VIM
12 dendritic cell tumor 31.9 ACTC1 MUC1 S100B VIM
13 fibromatosis 31.9 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 31.3 ACTC1 DES MDM2 S100B VIM
20 mesothelioma, malignant 31.1 KRT7 MUC1 NF2 VIM
21 chordoma 30.8 DES KRT7 MUC1 S100B VIM
22 carcinosarcoma 30.7 ACTC1 DES KRT7 MUC1 VIM
23 hemangiopericytoma, malignant 30.7 ACTC1 DES MDM2 MUC1 S100B VIM
24 lymphangioma 30.7 ACTC1 VIM
25 leiomyoma 30.4 ACTC1 DES VIM
26 fibrosarcoma 30.2 DES NTRK3 VIM
27 mesenchymoma 30.2 DES MDM2
28 intravascular papillary endothelial hyperplasia 30.1 ACTC1 VIM
29 retroperitoneal fibrosis 30.1 MDM2 VIM
30 mammary paget's disease 30.0 KRT7 MUC1
31 myxofibrosarcoma 30.0 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 ovarian fibrothecoma 29.7 ACTC1 DES
38 cystic nephroma 29.7 DES VIM
39 rhabdomyosarcoma 29.7 ACTC1 DES MDM2 VIM
40 ewing sarcoma 29.6 DES MDM2 S100B VIM
41 secretory meningioma 29.5 KRT7 MUC1 VIM
42 congenital fibrosarcoma 29.5 ACTC1 DES NTRK3 VIM
43 neurofibroma 29.5 MUC1 NF2 S100B
44 neurilemmoma 29.5 DES NF2 S100B VIM
45 cellular schwannoma 29.5 MDM2 NF2 S100B
46 dermatofibrosarcoma protuberans 29.5 ACTC1 DES S100B VIM
47 cholangiocarcinoma 29.5 CDKN1B KRT7 MUC1
48 fasciitis 29.4 ACTC1 DES MUC1
49 sertoli-leydig cell tumor 29.4 KRT7 MUC1
50 horseshoe kidney 29.4 ACTC1 KRT7

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 10.01 CCNE1 CDKN1B DES MDM2 NF2 NTRK3
2 cardiovascular system MP:0005385 9.97 ACTC1 CCNE1 CDKN1B DES KMT2B MDM2
3 cellular MP:0005384 9.92 ACTC1 CCNE1 CDKN1B DES KMT2B KRT7
4 homeostasis/metabolism MP:0005376 9.81 ACTC1 CDKN1B DES KMT2B KRT7 MDM2
5 embryo MP:0005380 9.73 CCNE1 CDKN1B KMT2B MDM2 NF2 VIM
6 mortality/aging MP:0010768 9.61 ACTC1 CCNE1 CDKN1B DES KMT2B MDM2
7 reproductive system MP:0005389 9.17 CCNE1 CDKN1B 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
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 46835353 5284616 6436030
2
Doxorubicin Approved, Investigational Phase 1, Phase 2,Phase 2 23214-92-8 31703
3
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
4
Everolimus Approved Phase 1, Phase 2 159351-69-6 70789204 6442177
5
Paclitaxel Approved, Vet_approved Phase 1, Phase 2 33069-62-4 36314
6
leucovorin Approved Phase 2 58-05-9 143 6006
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 Immunologic Factors Phase 1, Phase 2,Phase 2
12 Antifungal Agents Phase 1, Phase 2
13 Topoisomerase Inhibitors Phase 1, Phase 2,Phase 2
14 Anti-Bacterial Agents Phase 1, Phase 2,Phase 2
15 Antibiotics, Antitubercular Phase 1, Phase 2,Phase 2
16 Anti-Infective Agents Phase 1, Phase 2
17 Immunosuppressive Agents Phase 1, Phase 2,Phase 2
18 Antimitotic Agents Phase 1, Phase 2
19 taxane Phase 1, Phase 2
20 Albumin-Bound Paclitaxel Phase 1, Phase 2
21 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
22 Dermatologic Agents Phase 2
23 Antimetabolites, Antineoplastic Phase 2
24 Nucleic Acid Synthesis Inhibitors Phase 2
25 Vitamin B9 Phase 2
26 Vitamin B Complex Phase 2
27 Antirheumatic Agents Phase 2
28 Antimetabolites Phase 2
29 Folate Phase 2
30 Folic Acid Antagonists Phase 2
31 Antibodies Phase 2
32 Antibodies, Monoclonal Phase 2
33 Immunoglobulins Phase 2
34 Protective Agents Phase 2
35 Antidotes Phase 2
36 Trace Elements Phase 2
37 Hematinics Phase 2
38 Micronutrients Phase 2
39 Vitamins Phase 2
40 Nutrients Phase 2
41
Mechlorethamine Approved, Investigational 51-75-2 4033
42
Ifosfamide Approved 3778-73-2 3690
43
Cisplatin Approved 15663-27-1 2767 441203 84093
44 Antineoplastic Agents, Alkylating
45 Alkylating Agents
46
Isophosphamide mustard 0
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 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, Liver, T Cells, Skin, Thyroid

Publications for Spindle Cell Sarcoma

Articles related to Spindle Cell Sarcoma:

(show top 50) (show all 3102)
# Title Authors Year
1
Alveolar Soft-Part Sarcoma of the Tongue. ( 30235167 )
2019
2
A Phase II Trial of Pazopanib in Patients with Metastatic Alveolar Soft Part Sarcoma. ( 30254189 )
2019
3
Fine-needle aspiration of alveolar soft part sarcoma: Histologic correlation and aberrant CD68 expression. ( 30457206 )
2019
4
Brain Metastases and Place of Antiangiogenic Therapies in Alveolar Soft Part Sarcoma: A Retrospective Analysis of the French Sarcoma Group. ( 30626710 )
2019
5
Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion. ( 30744536 )
2019
6
Alveolar soft part sarcoma metastatic to the breast: a case report. ( 30832622 )
2019
7
Second Reported Case of Pediatric Bladder Alveolar Soft Part Sarcoma as Secondary Malignancy After Prior Cytotoxic Chemotherapy. ( 30986487 )
2019
8
Axitinib plus pembrolizumab in patients with advanced sarcomas including alveolar soft-part sarcoma: a single-centre, single-arm, phase 2 trial. ( 31078463 )
2019
9
Personalised management of alveolar soft part sarcoma: a promising phase 2 study. ( 31078464 )
2019
10
Blockade of VEGFR and PD-L1 Inhibits Alveolar Soft-Part Sarcoma. ( 31101632 )
2019
11
Alveolar soft part sarcoma: the new primary intracranial malignancy : A case report and review of the literature. ( 28744773 )
2019
12
Radiation-associated angiosarcoma with histological features reminiscent of Kaposi's sarcoma successfully treated by radiotherapy with paclitaxel. ( 31087582 )
2019
13
Neurofibromatosis Type 1 - Association with Breast Cancer, Basal Cell Carcinoma of the Skin, and Low-Grade Peripheral Nerve Sheath Sarcoma: Case Report and Literature Review. ( 31011321 )
2019
14
Outcome of localized liver-bile duct rhabdomyosarcoma according to local therapy: A report from the European Paediatric Soft-Tissue Sarcoma Study Group (EpSSG)-RMS 2005 study. ( 30920113 )
2019
15
Spindle Cell Sarcoma Mimicking Supraclavicular Lymph Node Metastasis of Breast Cancer. ( 30760697 )
2019
16
Reconstruction options and outcomes for breast sarcoma patients. ( 31025484 )
2019
17
Huge tumoral calcinosis mimicking a sarcoma. ( 30971573 )
2019
18
Intraluminal EWSR1-CREB1 gene rearranged, low-grade myxoid sarcoma of the pulmonary artery resembling extraskeletal myxoid chondrosarcoma (EMC). ( 30326150 )
2019
19
Activity and safety of crizotinib in patients with advanced clear-cell sarcoma with MET alterations: European Organization for Research and Treatment of Cancer phase II trial 90101 'CREATE'. ( 29741569 )
2019
20
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
21
Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for treatment of clear cell sarcoma of the kidney in children. ( 30318310 )
2019
22
Primary clear cell sarcoma of the calcaneus : Report of a rare case and review of the literature. ( 30623235 )
2019
23
Clear Cell Sarcoma of the Kidney. ( 30628851 )
2019
24
CRTC1-TRIM11 Fusion in a Case of Metastatic Clear Cell Sarcoma: Are CRTC1-TRIM11 Fusion-bearing Tumors Melanocytomas or Clear Cell Sarcomas? ( 30676334 )
2019
25
Expression of cyclin D1 in clear cell sarcoma of kidney. Is it useful in differentiating it from its histological mimics? ( 30736802 )
2019
26
Clear cell sarcoma of the soft palate mimicking unclassified melanoma. ( 30837084 )
2019
27
BCOR Overexpression in Renal Malignant Solitary Fibrous Tumors: A Close Mimic of Clear Cell Sarcoma of Kidney. ( 30864973 )
2019
28
Intra-extracranial primary clear cell sarcoma: the first report and review of the literature. ( 30880192 )
2019
29
Clear Cell Sarcoma of the Kidney. ( 30917048 )
2019
30
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
31
Undifferentiated pleomorphic sarcoma in a patient with Cowden syndrome after radiotherapy for breast cancer. ( 30047560 )
2019
32
Current Update on the Molecular Biology of Cutaneous Sarcoma: Dermatofibrosarcoma Protuberans. ( 30874910 )
2019
33
Kaposi's sarcoma-associated herpesvirus seropositivity is associated with type 2 diabetes mellitus: A case-control study in Xinjiang, China. ( 30639407 )
2019
34
Undifferentiated Embryonal Sarcoma of the Liver (UESL) in Adolescents: An Unexpected Diagnosis. ( 29702542 )
2019
35
Undifferentiated embryonal sarcoma of the liver in elderly: Case report and review of the literature. ( 30706877 )
2019
36
Undifferentiated Embryonal Sarcoma of the Liver: a Great Masquerader. ( 30714072 )
2019
37
Undifferentiated embryonal sarcoma of the liver mimicking venolymphatic malformation. ( 31011382 )
2019
38
Extrauterine, Extragenital Endometrial Stromal Sarcoma in the Absence of Endometriosis: A Rare Entity. ( 29210799 )
2019
39
Adjuvant Hormonal Therapy for Low-Grade Endometrial Stromal Sarcoma. ( 29843577 )
2019
40
Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis. ( 30640694 )
2019
41
High-grade endometrial stromal sarcoma: a retrospective study of factors influencing prognosis. ( 30697075 )
2019
42
Metastatic low-grade endometrial stromal sarcoma with sex cord and smooth muscle differentiation: A case report. ( 30705899 )
2019
43
Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group. ( 30772825 )
2019
44
Tumour lysis syndrome in a patient with undifferentiated endometrial stromal sarcoma. ( 30834285 )
2019
45
Treatment of uterine high-grade endometrial stromal sarcoma with apatinib combined with chemotherapy: A case report. ( 30921232 )
2019
46
The routine use of progestins might be a good choice for patients with recurrent low-grade endometrial stromal sarcoma after definite surgery. ( 30932937 )
2019
47
Hormone therapy and low-grade endometrial stromal sarcoma. ( 30932941 )
2019
48
Endometrial Stromal Sarcoma: Case Series with Emphasis on Gross Features. ( 30948880 )
2019
49
Survival outcomes of different treatment modalities in patients with low-grade endometrial stromal sarcoma. ( 30985313 )
2019
50
Reply to "The routine use of progestins might be a good choice for patients with recurrent low-grade endometrial stromal sarcoma after definite surgery ". ( 31008813 )
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

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 nucleus GO:0005634 9.65 CCNE1 CDKN1B DES KMT2B KRT7 MDM2
2 cytoplasm GO:0005737 9.36 ACTC1 CCNE1 CDKN1B DES KRT7 MDM2
3 intermediate filament GO:0005882 9.33 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 gene expression GO:0010628 9.73 ACTC1 MDM2 NTRK3 VIM
2 positive regulation of cell proliferation GO:0008284 9.65 CCNE1 CDKN1B MDM2 NTRK3 S100B
3 intermediate filament organization GO:0045109 9.4 DES VIM
4 lens fiber cell differentiation GO:0070306 9.37 NF2 NTRK3
5 positive regulation of cell cycle GO:0045787 9.33 CCNE1 CDKN1B MDM2
6 cellular response to antibiotic GO:0071236 9.32 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 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
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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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