DSRCT
MCID: DSM007
MIFTS: 50

Desmoplastic Small Round Cell Tumor (DSRCT)

Categories: Cancer diseases, Gastrointestinal diseases, Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Desmoplastic Small Round Cell Tumor

MalaCards integrated aliases for Desmoplastic Small Round Cell Tumor:

Name: Desmoplastic Small Round Cell Tumor 53 59 37 6 73
Desmoplastic Small Round-Cell Tumor 53 55
Dsrct 53 59

Characteristics:

Orphanet epidemiological data:

59
desmoplastic small round cell tumor
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: Adolescent,Adult;

Classifications:



External Ids:

Orphanet 59 ORPHA83469
MESH via Orphanet 45 D058405
UMLS via Orphanet 74 C0281508
ICD10 via Orphanet 34 C48.2
KEGG 37 H01985
UMLS 73 C0281508

Summaries for Desmoplastic Small Round Cell Tumor

NIH Rare Diseases : 53 Desmoplastic small round celltumor (DSRCT) is a rare type of soft tissuecancer (sarcoma) that usually begins in the abdomen. It primarily affects children and young adults and is more common in males. It is formed by small, round cancer cells surrounded by scar-like tissue and is often found in the tissue (peritoneum) that lines the inside of the abdomen and pelvis. The tumor cells have a characteristic genetic change involving a translocation between chromosomes 11 and 22, which is important in differentiating from other similar tumors. The genetic change involved in DSRCT is acquired throughout a person's lifetime and is not inherited. Symptoms may include abdominal pain and a feeling of fullness, abdominal mass, and symptoms of gastrointestinal obstruction, such as constipation. Treatment options may include: chemotherapy, radiation therapy, surgery, and stem cell transplantation.

MalaCards based summary : Desmoplastic Small Round Cell Tumor, also known as desmoplastic small round-cell tumor, is related to sarcoma, synovial and sertoli-leydig cell tumor, and has symptoms including pain An important gene associated with Desmoplastic Small Round Cell Tumor is EWSR1 (EWS RNA Binding Protein 1), and among its related pathways/superpathways are Transcriptional misregulation in cancer and Cytoskeletal Signaling. The drugs Carboplatin and Dactinomycin have been mentioned in the context of this disorder. Affiliated tissues include lung, bone and liver, and related phenotypes are nausea and vomiting and hepatomegaly

Wikipedia : 76 Desmoplastic small-round-cell tumor is an aggressive and rare cancer that primarily occurs as masses in... more...

Related Diseases for Desmoplastic Small Round Cell Tumor

Diseases related to Desmoplastic Small Round Cell Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 260)
# Related Disease Score Top Affiliating Genes
1 sarcoma, synovial 30.4 DES MUC1 VIM
2 sertoli-leydig cell tumor 30.3 MUC1 WT1
3 rhabdomyosarcoma 2 30.2 DES ENO2 EWSR1 WT1
4 rhabdomyosarcoma 30.2 DES ENO2 EWSR1 VIM
5 heart sarcoma 30.1 ENO2 WT1
6 neurofibroma 30.1 MUC1 SYP
7 spindle cell sarcoma 30.0 DES MUC1 VIM
8 wilms tumor 1 29.9 ENO2 EWSR1 WT1
9 ewing sarcoma 29.6 DES ENO2 EWSR1 SYP VIM WT1
10 gastrointestinal stromal tumor 29.6 DES ENO2 SYP VIM
11 sarcoma 10.7
12 wilms tumor 6 10.4
13 ewing's family of tumors 10.3
14 hypoglycemia 10.2
15 uterine sarcoma 10.2 DES WT1
16 sarcomatosis 10.2 EWSR1 WT1
17 embryonal rhabdomyosarcoma 10.2 DES EWSR1
18 dysgerminoma of ovary 10.2 ENO2 WT1
19 myoepithelioma 10.2 EWSR1 VIM
20 connective tissue cancer 10.2 ENO2 EWSR1
21 extraosseous ewing's sarcoma 10.2 ENO2 EWSR1
22 dermatofibrosarcoma protuberans 10.2 DES VIM
23 inflammatory myofibroblastic tumor 10.1 DES VIM
24 infantile myofibromatosis 10.1 DES VIM
25 smooth muscle tumor 10.1 DES VIM
26 embryonal sarcoma 10.1 DES VIM
27 malignant ovarian surface epithelial-stromal neoplasm 10.1 MUC1 WT1
28 ovary epithelial cancer 10.1 MUC1 WT1
29 chondromyxoid fibroma 10.1 DES VIM
30 small cell osteogenic sarcoma 10.1 ENO2 EWSR1
31 malignant pleural mesothelioma 10.1 MUC1 WT1
32 epithelioid cell melanoma 10.1 ENO2 WT1
33 askin's tumor 10.1 ENO2 EWSR1
34 infantile digital fibromatosis 10.1 DES VIM
35 fibromatosis 10.1 DES VIM
36 liver angiosarcoma 10.1 ENO2 VIM
37 lymphangiomatosis 10.1 DES VIM
38 angiolipoma 10.1 DES VIM
39 cutaneous leiomyosarcoma 10.1 DES VIM
40 sarcomatoid mesothelioma 10.1 VIM WT1
41 malignant ectomesenchymoma 10.1 DES ENO2
42 ectomesenchymoma 10.1 DES ENO2
43 spindle cell lipoma 10.1 DES VIM
44 peritoneal mesothelioma 10.1 MUC1 WT1
45 endosalpingiosis 10.1 MUC1 WT1
46 benign metastasizing leiomyoma 10.1 DES VIM
47 metanephric adenoma 10.1 VIM WT1
48 adenomatoid tumor 10.1 VIM WT1
49 bednar tumor 10.1 ENO2 VIM
50 meningioma, radiation-induced 10.1 MUC1 VIM

Graphical network of the top 20 diseases related to Desmoplastic Small Round Cell Tumor:



Diseases related to Desmoplastic Small Round Cell Tumor

Symptoms & Phenotypes for Desmoplastic Small Round Cell Tumor

Human phenotypes related to Desmoplastic Small Round Cell Tumor:

59 32 (show all 18)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 nausea and vomiting 59 32 frequent (33%) Frequent (79-30%) HP:0002017
2 hepatomegaly 59 32 frequent (33%) Frequent (79-30%) HP:0002240
3 ascites 59 32 occasional (7.5%) Occasional (29-5%) HP:0001541
4 anemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001903
5 abdominal pain 59 32 hallmark (90%) Very frequent (99-80%) HP:0002027
6 cachexia 59 32 occasional (7.5%) Occasional (29-5%) HP:0004326
7 ileus 59 32 frequent (33%) Frequent (79-30%) HP:0002595
8 neoplasm of the central nervous system 59 32 occasional (7.5%) Occasional (29-5%) HP:0100006
9 sarcoma 59 32 hallmark (90%) Very frequent (99-80%) HP:0100242
10 ovarian neoplasm 59 32 occasional (7.5%) Occasional (29-5%) HP:0100615
11 mediastinal lymphadenopathy 59 32 frequent (33%) Frequent (79-30%) HP:0100721
12 neoplasm of the pancreas 59 32 occasional (7.5%) Occasional (29-5%) HP:0002894
13 neoplasm of the lung 59 32 occasional (7.5%) Occasional (29-5%) HP:0100526
14 abdominal distention 59 32 hallmark (90%) Very frequent (99-80%) HP:0003270
15 testicular neoplasm 59 32 occasional (7.5%) Occasional (29-5%) HP:0010788
16 abnormality of the peritoneum 59 32 hallmark (90%) Very frequent (99-80%) HP:0002585
17 weight loss 59 Frequent (79-30%)
18 lymphadenopathy 59 Very frequent (99-80%)

UMLS symptoms related to Desmoplastic Small Round Cell Tumor:


pain

Drugs & Therapeutics for Desmoplastic Small Round Cell Tumor

Drugs for Desmoplastic Small Round Cell Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 109)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Carboplatin Approved Phase 3,Phase 2 41575-94-4 10339178 38904 498142
2
Dactinomycin Approved, Investigational Phase 3,Phase 2 50-76-0 457193 2019
3
Etoposide Approved Phase 3,Phase 2,Early Phase 1 33419-42-0 36462
4
Cyclophosphamide Approved, Investigational Phase 3,Phase 1,Phase 2,Early Phase 1 6055-19-2, 50-18-0 2907
5
Vincristine Approved, Investigational Phase 3,Phase 2,Early Phase 1 57-22-7, 2068-78-2 5978
6
Ifosfamide Approved Phase 3,Phase 2,Early Phase 1 3778-73-2 3690
7
Epirubicin Approved Phase 3,Phase 2 56420-45-2 41867
8
Doxorubicin Approved, Investigational Phase 2, Phase 3,Phase 1,Early Phase 1 23214-92-8 31703
9
Trabectedin Approved, Investigational Phase 2, Phase 3 114899-77-3 108150
10
Doxil Approved June 1999 Phase 2, Phase 3,Phase 1,Early Phase 1 31703
11 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1,Early Phase 1
12
Isophosphamide mustard Phase 3,Phase 2,Early Phase 1 0
13 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Early Phase 1
14 Immunologic Factors Phase 3,Phase 2,Phase 1,Early Phase 1
15 Anti-Infective Agents Phase 3,Phase 2,Phase 1
16 Alkylating Agents Phase 3,Phase 2,Phase 1,Early Phase 1
17 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Phase 1,Early Phase 1
18 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1,Early Phase 1
19 Etoposide phosphate Phase 3,Phase 2,Early Phase 1
20 Topoisomerase Inhibitors Phase 3,Phase 2,Phase 1,Early Phase 1
21 Antimitotic Agents Phase 3,Phase 2,Early Phase 1
22 Antirheumatic Agents Phase 3,Phase 2,Early Phase 1
23 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2
24 Immunosuppressive Agents Phase 3,Phase 2,Phase 1,Early Phase 1
25
Cisplatin Approved Phase 2,Phase 1 15663-27-1 2767 441203 84093
26
Mecasermin Approved, Investigational Phase 2 68562-41-4
27
Fludarabine Approved Phase 1, Phase 2,Phase 2 75607-67-9, 21679-14-1 30751
28
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 6436030 5284616
29
Lenograstim Approved, Investigational Phase 2,Phase 1 135968-09-1
30
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492
31
Melphalan Approved Phase 2,Phase 1 148-82-3 4053 460612
32
Everolimus Approved Phase 2 159351-69-6 6442177
33
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
34
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
35
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
36
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
37
Arsenic trioxide Approved, Investigational Phase 2 1327-53-3 518740
38
Thiotepa Approved, Investigational Phase 2,Phase 1 52-24-4 5453
39
Topotecan Approved, Investigational Phase 2 123948-87-8, 119413-54-6 60700
40
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
41
Aldesleukin Approved Phase 1, Phase 2 85898-30-2, 110942-02-4
42
Gemcitabine Approved Phase 2 95058-81-4 60750
43
Iodine Approved, Investigational Phase 2,Phase 1 7553-56-2 807
44
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
45
Bevacizumab Approved, Investigational Phase 2,Early Phase 1 216974-75-3
46
Temozolomide Approved, Investigational Phase 2,Early Phase 1 85622-93-1 5394
47
Irinotecan Approved, Investigational Phase 2,Early Phase 1 100286-90-6, 97682-44-5 60838
48
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
49
Promethazine Approved, Investigational Phase 2 60-87-7 4927
50
Acetaminophen Approved Phase 2 103-90-2 1983

Interventional clinical trials:

(show all 42)
# Name Status NCT ID Phase Drugs
1 Surgery Followed by Chemotherapy in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00002898 Phase 3 carboplatin;cyclophosphamide;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
2 Doxorubicin Hydrochloride or Trabectedin in Treating Patients With Previously Untreated Advanced or Metastatic Soft Tissue Sarcoma Terminated NCT01189253 Phase 2, Phase 3 doxorubicin hydrochloride;trabectedin
3 Imatinib Mesylate in Patients With Refractory Desmoplastic Small Round Cell Tumors Completed NCT00417807 Phase 1, Phase 2 Imatinib mesilate
4 QUILT-3.025: A Phase 2 Study of AMG 479 in Relapsed or Refractory Ewing's Family Tumor and Desmoplastic Small Round Cell Tumors Completed NCT00563680 Phase 2 AMG 479
5 Imatinib Mesylate in Treating Patients With Recurrent Ewing's Family of Tumors or Desmoplastic Small Round-Cell Tumor Completed NCT00062205 Phase 2 imatinib mesylate
6 Exatecan Mesylate in Treating Patients With Ewing's Sarcoma, Primitive Neuroectodermal Tumor, or Desmoplastic Small Round Cell Tumor Completed NCT00055952 Phase 2 exatecan mesylate
7 Pilot Study of Hyperthermic Peritoneal Perfusion (HIPEC) for Adolescent and Young Adults With Desmoplastic Small Round Cell Tumor Completed NCT01277744 Phase 2 Cisplatin
8 A Study of R1507 in Participants With Recurrent or Refractory Sarcoma Completed NCT00642941 Phase 2 RG1507
9 Therapy to Treat Ewing's Sarcoma, Rhabdomyosarcoma or Neuroblastoma Completed NCT00923351 Phase 1, Phase 2 Tumor Purged/CD25 Depleted Lymphocytes;rhIL-7
10 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
11 Imatinib Mesylate in Treating Patients With Relapsed or Refractory Solid Tumors of Childhood Completed NCT00030667 Phase 2 imatinib mesylate
12 Stem Cell Transplantation in Patients With High-Risk and Recurrent Pediatric Sarcomas Completed NCT00043979 Phase 2 F-18 Fluorodeoxyglucose;cyclophosphamide;cyclosporine;doxorubicin hydrochloride;etoposide;fludarabine phosphate;melphalan;prednisone;sirolimus;tacrolimus;vincristine sulfate;Filgrastim
13 BI 2536 in Treating Patients With Recurrent or Metastatic Solid Tumors Completed NCT00526149 Phase 2 BI 2536
14 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
15 Combination Chemotherapy and Radiation Therapy in Treating Patients With Peripheral Neuroectodermal Tumors, Ewing's Sarcoma, Wilms' Tumor, or Bone Cancer Completed NCT00002466 Phase 2 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
16 Combination Chemotherapy in Treating Children With Metastatic Rhabdomyosarcoma or Other Malignant Mesenchymal Tumors Completed NCT00025441 Phase 2 carboplatin;cyclophosphamide;doxorubicin hydrochloride;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
17 Arsenic Trioxide in Treating Patients With Advanced Neuroblastoma or Other Childhood Solid Tumors Completed NCT00024258 Phase 2 arsenic trioxide
18 Combination Chemotherapy Followed by Bone Marrow Transplantation in Treating Patients With Rare Cancer Completed NCT00002515 Phase 2 carboplatin;thiotepa;topotecan hydrochloride
19 Trial of Nab-paclitaxel in Patients With Desmoid Tumors and Multiply Relapsed/Refractory Desmoplastic Small Round Cell Tumors and Ewing Sarcoma Recruiting NCT03275818 Phase 2 nab paclitaxel
20 Activated T Cells Armed With GD2 Bispecific Antibody in Children and Young Adults With Neuroblastoma and Osteosarcoma Recruiting NCT02173093 Phase 1, Phase 2
21 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
22 Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors Active, not recruiting NCT01946529 Phase 2 vincristine;doxorubicin;cyclophosphamide;ifosfamide;etoposide;temozolomide;temsirolimus;bevacizumab;sorafenib
23 Iodine I 131 Monoclonal Antibody 3F8 in Treating Patients With Central Nervous System Cancer or Leptomeningeal Cancer Active, not recruiting NCT00445965 Phase 2
24 A Phase II of Nivolumab Plus Ipilimumab in Non-resectable Sarcoma and Endometrial Carcinoma Not yet recruiting NCT02982486 Phase 2 Ipilimumab;Nivolumab
25 Study of High Dose Intravenous (IV) Ascorbic Acid in Measurable Solid Tumor Disease Suspended NCT01125449 Phase 2 Ascorbic acid (vitamin C)
26 Tanespimycin in Treating Young Patients With Recurrent or Refractory Leukemia or Solid Tumors Completed NCT00093821 Phase 1 tanespimycin
27 Continuous Hyperthermic Peritoneal Perfusion (CHPP) With Cisplatin for Children With Peritoneal Cancer Completed NCT00436657 Phase 1 CHPP of Cisplatin
28 Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma Completed NCT00720174 Phase 1 Doxorubicin Hydrochloride
29 AMG-479 in Treating Patients With Advanced Solid Tumors or Non-Hodgkin Lymphoma Completed NCT00562380 Phase 1
30 Intraperitoneal Radioimmunotherapy With 131I-8H9 for Patients With Desmoplastic Small Round Cell Tumors and Other Solid Tumors Involving the Peritoneum Recruiting NCT01099644 Phase 1
31 EGFR806 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults Recruiting NCT03618381 Phase 1
32 Radiolabeled Monoclonal Antibody Therapy in Treating Patients With Refractory, Recurrent, or Advanced CNS or Leptomeningeal Cancer Recruiting NCT00089245 Phase 1
33 Enoblituzumab (MGA271) in Children With B7-H3-expressing Solid Tumors Active, not recruiting NCT02982941 Phase 1 Enoblituzumab
34 Amifostine to Protect From Side Effects of PSCT in Treating Patients With Solid Tumors Terminated NCT00003926 Phase 1 amifostine trihydrate;busulfan;filgrastim;melphalan;thiotepa
35 Studying Genes in Samples From Younger Patients With Desmoplastic Small Round Cell Tumor Registered on COG-D9902 or COG-ABTR01B1 Completed NCT01430637
36 Quality of Life Study for Pediatric and Adult Patients Undergoing Hyperthermic Peritoneal Perfusion With Cisplatin Completed NCT00502177
37 Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas Completed NCT01567046
38 Combination Chemotherapy in Treating Patients With Sarcoma Completed NCT00662233 Early Phase 1 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
39 Irinotecan, Temozolomide and Bevacizumab in Combination With Existing High Dose Alkylator Based Chemotherapy for Treatment of Newly Diagnosed Patients With Desmoplastic Small Round Cell Tumor Active, not recruiting NCT01189643 Early Phase 1 irinotecan, temozolomide, and bevacizumab incorporated into an existing schedule of high dose alkylator
40 French National Registry of Rare Peritoneal Surface Malignancies Active, not recruiting NCT02834169
41 Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma Active, not recruiting NCT00919269
42 Glutamine in Preventing Oral Mucositis in Patients Receiving Chemotherapy for Sarcoma Withdrawn NCT00334984 Not Applicable glutamine

Search NIH Clinical Center for Desmoplastic Small Round Cell Tumor

Genetic Tests for Desmoplastic Small Round Cell Tumor

Anatomical Context for Desmoplastic Small Round Cell Tumor

MalaCards organs/tissues related to Desmoplastic Small Round Cell Tumor:

41
Lung, Bone, Liver, Brain, Ovary, Lymph Node, Spinal Cord

Publications for Desmoplastic Small Round Cell Tumor

Articles related to Desmoplastic Small Round Cell Tumor:

(show top 50) (show all 298)
# Title Authors Year
1
Desmoplastic small round cell tumor of the middle ear: A case report. ( 29703011 )
2018
2
Effective treatment of apatinib in desmoplastic small round cell tumor: a case report and literature review. ( 29587657 )
2018
3
Desmoplastic Small Round Cell Tumor Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Results of a Phase 2 Trial. ( 29383611 )
2018
4
A Case Report of Abdominal Desmoplastic Small Round Cell Tumor in a Young Tunisian Woman. ( 29313236 )
2018
5
Multimodality Treatment of Desmoplastic small round cell tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival. ( 29871905 )
2018
6
A novel image-based system for risk stratification in patients with desmoplastic small round cell tumor. ( 29605262 )
2018
7
High-Dose Chemotherapy with Stem Cell Rescue in Desmoplastic Small Round Cell Tumor: A Single-Institution Experience and Review of the Literature. ( 29853779 )
2018
8
Desmoplastic small round cell tumor: A nationwide study of a rare sarcoma. ( 29878371 )
2018
9
Primary desmoplastic small round cell tumor in the left orbit: a case report and literature review. ( 29383463 )
2018
10
Efficacy of ONC201 in Desmoplastic Small Round Cell Tumor. ( 29626752 )
2018
11
Immune profiles of desmoplastic small round cell tumor and synovial sarcoma suggest different immunotherapeutic susceptibility upfront compared to relapse specimens. ( 30015384 )
2018
12
Refractory hypoglycemia in a pediatric patient with desmoplastic small round cell tumor. ( 30044763 )
2018
13
The genomics of desmoplastic small round cell tumor reveals the deregulation of genes related to DNA damage response, epithelial-mesenchymal transition, and immune response. ( 30486883 )
2018
14
Imaging and histopathologic findings of desmoplastic small round cell tumor. ( 29560653 )
2017
15
Desmoplastic Small Round-Cell Tumor in a Young Indigenous Australian Man: A Case Report. ( 28717745 )
2017
16
Clinical activity of eribulin in advanced desmoplastic small round-cell tumor. ( 28863036 )
2017
17
Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: Is there a benefit for HIPEC after macroscopically complete cytoreductive surgery? ( 28234908 )
2017
18
Desmoplastic Small Round Cell Tumor Presenting as an Ocular Mass: Unusual Localization and Remarkable Surgical Approach. ( 29043531 )
2017
19
Mechanism of action of trabectedin in desmoplastic small round cell tumor cells. ( 28166781 )
2017
20
Clinical features and outcomes of 20 patients with abdominopelvic desmoplastic small round cell tumor. ( 27890349 )
2017
21
Desmoplastic small round cell tumor: evaluation of reverse transcription-polymerase chain reaction and fluorescence in situ hybridization as ancillary molecular diagnostic techniques. ( 28748349 )
2017
22
Recurrent desmoplastic small round cell tumor responding to an mTOR inhibitor containing regimen. ( 28941151 )
2017
23
Clinical Activity of Pazopanib in Patients with Advanced Desmoplastic Small Round Cell Tumor. ( 29212731 )
2017
24
Antiangiogenic effects in patients with progressive desmoplastic small round cell tumor: data from the French national registry dedicated to the use of off-labeled targeted therapy in sarcoma (OUTC's). ( 28491276 )
2017
25
Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy combined with two-stage hepatectomy for multiple and bilobar desmoplastic small round cell tumor liver metastases. ( 28890830 )
2017
26
Desmoplastic Small Round Cell Tumor: A Rare Case of Extraluminal Bowel Obstruction and Review of the Literature. ( 29282625 )
2017
27
Sister Mary Joseph nodule caused by metastatic desmoplastic small round cell tumor: A clinicopathological report. ( 27900084 )
2016
28
Central nervous system recurrence of desmoplastic small round cell tumor following aggressive multimodal therapy: A case report. ( 26870296 )
2016
29
Diagnosis of desmoplastic small-round-cell tumor by cytogenetic analysis: a case report. ( 27190620 )
2016
30
Desmoplastic Small Round Cell Tumor: Pathology, Genetics, and Potential Therapeutic Strategies. ( 27621277 )
2016
31
Management of desmoplastic small round cell tumor. ( 27955733 )
2016
32
Ovarian involvement of a desmoplastic small round cell tumor of unknown primary origin with lymph node and lung metastases: A case report. ( 26893704 )
2016
33
Desmoplastic Small Round Cell Tumor of the Pleura Successfully Treated with a Lower Dose of Pazopanib. ( 27580551 )
2016
34
Desmoplastic Small Round Cell Tumor Presenting as an Isolated Testicular Mass in an Adult With Multiple Previous Malignancies. ( 26786560 )
2016
35
Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy: A case report. ( 26893693 )
2016
36
Annulate Lamellae in a Desmoplastic Small Round Cell Tumor. ( 27098589 )
2016
37
Solid-Pattern Desmoplastic Small Round Cell Tumor. ( 27431755 )
2016
38
A genomic case study of desmoplastic small round cell tumor: comprehensive analysis reveals insights into potential therapeutic targets and development of a monitoring tool for a rare and aggressive disease. ( 27863505 )
2016
39
Clinical Characteristics and Outcomes of Pediatric Patients with Desmoplastic Small Round Cell Tumor. ( 27134714 )
2016
40
Management of a rapidly enlarging new adnexal mass: a rare case of desmoplastic small round cell tumor of the ovary arising in pregnancy. ( 27354996 )
2016
41
Desmoplastic small round cell tumor: postoperative retroperitoneal mass. ( 27594960 )
2016
42
Birth and evolution of the desmoplastic small round-cell tumor. ( 27312937 )
2016
43
Desmoplastic Small Round Cell Tumor of the Kidney: AIRP Best Cases in Radiologic-Pathologic Correlation. ( 27618327 )
2016
44
Intracranial desmoplastic small round cell tumor presenting as a suprasellar mass. ( 25479120 )
2015
45
Desmoplastic Small Round Cell Tumor, a &amp;quot;Floating Island&amp;quot; Pattern in Pleural Fluid Cytology: A Case Report and Review of the Literature. ( 26413364 )
2015
46
Desmoplastic small round cell tumor of the kidney with unusual imaging features. ( 26093512 )
2015
47
Desmoplastic Small Round Cell Tumor (DSRCT): an Unusual Intra-abdominal Tumor. ( 25829717 )
2015
48
Myeloablative chemotherapy with autologous stem cell transplant for desmoplastic small round cell tumor. ( 25945075 )
2015
49
A Case of Desmoplastic Small Round Cell Tumor. ( 26629298 )
2015
50
Desmoplastic small round cell tumor: Diagnostic dilemma and uncertain prognosis: Report of few cases. ( 26881606 )
2015

Variations for Desmoplastic Small Round Cell Tumor

ClinVar genetic disease variations for Desmoplastic Small Round Cell Tumor:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 TNFRSF17 NM_001192.2(TNFRSF17): c.524C> T (p.Thr175Met) single nucleotide variant other rs371163866 GRCh37 Chromosome 16, 12061673: 12061673
2 TNFRSF17 NM_001192.2(TNFRSF17): c.524C> T (p.Thr175Met) single nucleotide variant other rs371163866 GRCh38 Chromosome 16, 11967816: 11967816

Copy number variations for Desmoplastic Small Round Cell Tumor from CNVD:

7
# CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 163402 22 27994016 28026515 Translate EWSR1 Desmoplastic small round cell tumor

Expression for Desmoplastic Small Round Cell Tumor

Search GEO for disease gene expression data for Desmoplastic Small Round Cell Tumor.

Pathways for Desmoplastic Small Round Cell Tumor

Pathways related to Desmoplastic Small Round Cell Tumor according to KEGG:

37
# Name Kegg Source Accession
1 Transcriptional misregulation in cancer hsa05202

Pathways related to Desmoplastic Small Round Cell Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.29 DES MUC1 VIM
2
Show member pathways
10.61 DES VIM

GO Terms for Desmoplastic Small Round Cell Tumor

Biological processes related to Desmoplastic Small Round Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 muscle filament sliding GO:0030049 8.96 DES VIM
2 intermediate filament organization GO:0045109 8.62 DES VIM

Molecular functions related to Desmoplastic Small Round Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 8.92 DES EWSR1 SYP VIM

Sources for Desmoplastic Small Round Cell Tumor

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
Content
Loading form....