Desmoplastic Small Round Cell Tumor malady

NIH Rare Diseases: Desmoplastic small round cell tumors (DSRCT), a rare malignant cancer, is a soft tissue sarcoma that usually affects young boys and men and is found most often in the abdomen. Its name means that it is formed by small, round cancer cells surrounded by scarlike tissue. The most common symptoms include abdominal pain, abdominal mass and symptoms of gastrointestinal obstruction. DSRCTs are treated first with chemotherapy, then with surgery to remove the tumor, if possible. Radiation therapy is sometimes given, depending on the tumor. In addition, some people with DSRCT are candidates for a bone marrow transplant.³⁰

MalaCards: Desmoplastic Small Round Cell Tumor, also known as desmoplastic small round-cell tumor, is related to ewing's family of tumors and sertoli-leydig cell tumor. An important gene associated with Desmoplastic Small Round Cell Tumor is EWSR1 (Ewing sarcoma breakpoint region 1), and among its related pathways are Actin-Based Motility by Rho Family GTPases and CDO in myogenesis. The compounds progestin and orcein have been mentioned in the context of this disorder. Affiliated tissues include brain, liver and kidney, and related mouse phenotypes are respiratory system and reproductive system.

Wikipedia: Desmoplastic small-round-cell tumor is classified as a soft tissue sarcoma. It is an aggressive and rare...⁴⁴ more...

desmoplastic small round cell tumor 30 8 43 desmoplastic small round-cell tumor 30 32

dsrct 30

Diseases related to desmoplastic small round cell tumor by text searches and GeneDecks gene sharing:

(show top 50)    (show all 418)

id	Related Disease	Score	Top Affiliating Genes
1	ewing's family of tumors	33.5	VIM, EWSR1, SYP
2	sertoli-leydig cell tumor	29.5	VIM, MYOG, MUC1, ENO2, ALPP, CALB2
3	wilms tumor	28.1	VIM, MYOG, MYOD1, MUC1, FLI1, MB
4	acute lymphoblastic leukemia	27.9	KIT, CD99, FLI1, ENO2, IGF2, ALPP
5	lymphoblastic leukemia	27.9	KIT, CD99, FLI1, ENO2, IGF2, ALPP
6	leydig cell tumor	27.5	KIT, VIM, MYOG, MUC1, ENO2, IGF2
7	leukemia	24.2	KIT, VIM, MYOD1, BMP4, MUC1, CD99
8	sarcoma	23.7	KIT, VIM, MYOG, MYOD1, BMP4, PAX7
9	melanoma	21.5	KIT, VIM, MYOD1, BMP4, PAX7, MUC1
10	spindle cell thymoma	13.7	MUC1, DES
11	childhood mediastinal neurogenic tumor	13.7	WT1, DES
12	gallbladder sarcoma	13.6	MYOG, MYOD1
13	malignant mesenchymoma	13.6	MYOG, MYOD1, DES
14	bladder sarcoma	13.6	CD99, FLI1
15	pulmonary vein stenosis	13.6	CD99, ACTC1, DES
16	pigmented villonodular synovitis	13.6	MYOG, CALB2, DES
17	ovarian small cell carcinoma	13.6	CALB2, TTF1, DES
18	lymph node palisaded myofibroblastoma	13.6	VIM, DES
19	myxosarcoma	13.6	VIM, DES
20	infantile digital fibromatosis	13.5	VIM, DES
21	prostate leiomyoma	13.5	VIM, DES
22	clear cell ependymoma	13.5	VIM, MUC1
23	prostate stromal sarcoma	13.5	VIM, MUC1
24	central nervous system cancer	13.5	CD99, FLI1, EWSR1
25	adenoid squamous cell carcinoma	13.5	VIM, MUC1
26	endometrial mucinous adenocarcinoma	13.5	VIM, MUC1
27	clear cell meningioma	13.5	VIM, MUC1
28	mesenchymal chondrosarcoma	13.5	VIM, MUC1, DES
29	monophasic synovial sarcoma	13.5	MUC1, CALB2
30	ectomesenchymoma	13.5	MYOD1, PAX7, CD99
31	nodular hidradenoma	13.5	VIM, MUC1
32	endometrial stromal nodule	13.5	VIM, DES
33	osteoblastoma	13.5	VIM, MUC1
34	heart cancer	13.5	VIM, CALB2
35	myxoid liposarcoma of the ovary	13.4	EWSR1, PDGFA, DES
36	intravenous leiomyomatosis	13.4	ACTC1, DES
37	epithelioid leiomyosarcoma	13.4	VIM, ACTC1, DES
38	glomangiomyoma	13.4	VIM, ACTC1, DES
39	infantile myofibromatosis	13.4	VIM, ACTC1, DES
40	myofibroma	13.4	VIM, ACTC1, DES
41	epithelioid sarcoma	13.4	VIM, MUC1, CALB2
42	pneumothorax	13.4	MUC1, CALB2, ACTC1, DES
43	malignant peritoneal mesothelioma	13.4	VIM, MUC1, CALB2
44	metanephric adenoma	13.4	VIM, MUC1, WT1, DES
45	supratentorial neoplasm	13.4	CD99, ENO2, SYP
46	benign metastasizing leiomyoma	13.4	VIM, ACTC1, DES
47	sex cord-gonadal stromal tumor	13.4	VIM, CALB2, TTF1
48	mucinous cystadenocarcinoma	13.4	VIM, MUC1, ACTC1
49	bone cancer	13.3	ENO2, ALPP, EWSR1, DES
50	lymphoblastic lymphoma	13.3	CD99, FLI1, ENO2

Approved drugs:

Drug clinical trials:

MalaCards organs/tissues related to desmoplastic small round cell tumor:

²²

MGI Mouse Phenotypes related to desmoplastic small round cell tumor:

²⁵ (show all 19)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	respiratory system phenotype	MP:0005388	9.7	MYF5, MYOD1, PAX7, MB, MYOG
2	reproductive system phenotype	MP:0005389	9.2	PDGFA, MYOD1, ALPP, EWSR1, ERBB2, WT1
3	no phenotypic analysis	MP:0003012	8.9	BMP4, IGF2, MYOD1, MYF5, SYP, MUC1
4	craniofacial phenotype	MP:0005382	8.9	PDGFRB, PAX7, BMP4, KIT
5	renal/urinary system phenotype	MP:0005367	8.8	EWSR1, PDGFRB, BMP4, KIT, PDGFA, WT1
6	normal phenotype	MP:0002873	8.1	MYOG, MYF5, MYOD1, BMP4, PAX7, ALPP
7	skeleton phenotype	MP:0005390	8.0	PDGFA, IGF2, PAX7, BMP4, MYOD1, MYF5
8	embryogenesis phenotype	MP:0005380	7.8	PAX7, MYF5, ALPP, MB, KIT, ERBB2
9	mortality/aging	MP:0010768	7.5	EWSR1, PAX7, DES, TGFB3, THBD, BMP4
10	vision/eye phenotype	MP:0005391	7.5	BMP4, PAX7, MUC1, MYF5, PDGFRB, TGFB3
11	nervous system phenotype	MP:0003631	7.4	ETV1, PDGFA, THBD, TGFB3, SYP, KIT
12	digestive/alimentary phenotype	MP:0005381	7.2	KIT, BMP4, ERBB2, PAX7, MUC1, IGF2
13	behavior/neurological phenotype	MP:0005386	6.8	IGF2, TGFB3, DES, ETV1, CALB2, KIT
14	integument phenotype	MP:0010771	6.3	S100B, THBD, MUC1, PDGFRB, CSF3, PDGFA
15	cardiovascular system phenotype	MP:0005385	6.1	BMP4, KIT, FLI1, VIM, MYOD1, WT1
16	homeostasis/metabolism phenotype	MP:0005376	5.9	ERBB2, IGF2, MB, MUC1, BMP4, MYOD1
17	cellular phenotype	MP:0005384	5.7	BMP4, ENO2, ACTC1, TGFB3, THBD, IGF2
18	muscle phenotype	MP:0005369	5.7	IGF2, BMP4, PAX7, FLI1, MB, ETV1
19	growth/size phenotype	MP:0005378	5.4	EWSR1, ERBB2, MYOG, KIT, FLI1, PDGFA

Articles related to desmoplastic small round cell tumor:

(show all 40)

id	Title	Authors	Year	Affiliating Genes
1	Desmoplastic small round-cell tumor: an adult with pr evious exposure to agent orange. (20382635)	Baz W.... Dhar M.	2010	CSF3
2	Sequential intensified chemotherapy with stem cell re scue for children and adolescents with desmoplastic small round-cell tumor. (19802018)	Bisogno G.... Carli M.	2010	CSF3
3	Ovarian involvement by desmoplastic small round cell tumor with leydig cell hyperplasia showing an unusual immunophenotype (cytokera tin negative, calretinin and inhibin positive) mimicking poorly differentiated sertoli leydig cell tumor. (19851210)	Engohan-Aloghe C.... NoAl J.C.	2009	CALB2
4	A new molecular variant of desmoplastic small round cell tumor: significance of WT1 immunostaining in this entity. (18703217)	Murphy A.J.... Thorner P.S.	2008	WT1
5	Adenosine transporter ENT4 is a direct target of EWS/WT1 translocation product and is highly expressed in desmoplastic small round cell tumor. (18523561)	Li H.... Lee S.B.	2008	SLC29A4
6	Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients. (17964965)	Lee Y.S.... Hsiao C.H.	2007	VIM, DES, WT1
7	Desmoplastic small round cell tumor of the kidney in childhood. (17414105)	Wang L.L.... Kozakewich H.	2007	TGFB3
8	A novel EWS-WT1 gene fusion product in desmoplastic small round cell tumor is a potent transactivator of the insulin-like growth factor-I receptor (IGF-IR) gene. (16730884)	Werner H.... Roberts C.T.	2007	WT1, EWSR1
9	Epithelial marker-negative desmoplastic small round cell tumor with atypical morphology: definitive classification by fluorescence in situ hybridization. (17425400)	Zhang J.... Fuller C.	2007	ACTC1
10	Primary desmoplastic small round cell tumor of bone: report of a case with cytogenetic confirmation. (15642398)	Murphy A.... McDermott M.B.	2005	VIM, FLI1, CD99
11	PDGF-A, PDGF-Rbeta, TGFbeta3 and bone morphogenic protein-4 in desmoplastic small round cell tumors with EWS-WT1 gene fusion product and their role in stromal desmoplasia: an immunohistochemical study. (15389255)	Zhang P.J.... Barr F.G.	2005	PDGFRB, BMP4, TGFB3
12	A tetraspanin-family protein, T-cell acute lymphoblastic leukemia-associated antigen 1, is induced by the Ewing's sarcoma-Wilms' tumor 1 fusion protein of desmoplastic small round-cell tumor. (14633590)	Ito E.... Semba K.	2003	WT1, TSPAN7
13	Immunophenotype of desmoplastic small round cell tumors as detected in cases with EWS-WT1 gene fusion product. (12640103)	Zhang P.J.... Barr F.G.	2003	ERBB2, KIT, DES
14	Correspondence re: Kawano N, Inayama Y, Nagashima Y, Miyagi Y, Uemura H, Saitoh K, et al. Desmoplastic small round-cell tumor of the paratesticular region: report of an adult case with demonstration of EWS and WT1 gene fusion using paraffin-embedded tissue. Mod Pathol 1999;12:729-34. (10912946)	Yen T.S.	2000	WT1, EWSR1
15	Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor. (10842247)	Rosoff P.M.... West D.C.	2000	EWSR1
16	Immunohistochemical expression of WT1 by desmoplastic small round cell tumor: a comparative study with other small round cell tumors. (10843285)	Barnoud R.... Pasquier B.	2000	WT1
17	Molecular heterogeneity and function of EWS-WT1 fusion transcripts in desmoplastic small round cell tumors. (10999739)	Liu J.... Wright J.J.	2000	WT1, EWSR1
18	Chemotherapy dose-intensification for pediatric patie nts with Ewing's family of tumors and desmoplastic small round-cell tumors: a f easibility study at St. Jude Children's Research Hospital. (10458232)	Marina N.M.... Meyer W.H.	1999	CSF3
19	Cytokeratin-negative desmoplastic small round cell tumor: a report of two cases emphasizing the utility of reverse transcriptase-polymerase chain reaction. (10496592)	Trupiano J.K.... Goldblum J.R.	1999	ENO2, SYP, MYOG
20	Variant EWS-WT1 chimeric product in the desmoplastic small round cell tumor. (9949226)	Chan A.S.... Zielenska M.	1999	EWSR1
21	Intra-abdominal desmoplastic small round cell tumor: immunohistochemical evidence for up-regulation of autocrine and paracrine growth factors. (10074970)	Froberg K.... Manivel C.	1999	IGF2, WT1, EWSR1
22	Desmoplastic small round-cell tumor of the paratestic ular region: report of an adult case with demonstration of EWS and WT1 gene fus ion using paraffin-embedded tissue. (10430278)	Kawano N.... Nakatani Y.	1999	WT1, EWSR1
23	Detection of EWS-WT1 fusion mRNA in ascites of a patient with desmoplastic small round cell tumor by RT-PCR. (10029456)	Perez R.P.... Zhang P.J.	1999	EWSR1
24	A combined cytogenetic and molecular approach to diagnosis in a case of desmoplastic small round cell tumor with a complex translocation (11;22;21). (9973919)	Roberts P.... Lewis I.J.	1999	EWSR1
25	Fine needle aspiration cytology of desmoplastic small round cell tumor. A case report. (10432888)	Insabato L.... Pettinato G.	1999	EWSR1
26	Intra-abdominal desmoplastic small round cell tumor: immunohistochemical evidence for up-regulation of autocrine and paracrine growth factors. (9846206)	Froberg K.... Manivel C.	1998	IGF2, EWSR1
27	Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants. (9738572)	Gerald W.L.... Rosai J.	1998	EWSR1
28	Intraabdominal desmoplastic small round cell tumor with EWS/ERG fusion transcript. (9706984)	Ordi J.... Cardesa A.	1998	CD99
29	Desmoplastic small round cell tumor: II: an ultrastructural and immunohistochemical study with emphasis on new immunohistochemical markers. (9808124)	OrdA^A+ez N.G.	1998	S100B, VIM, DES
30	Novel breakpoints of the EWS gene and the WT1 gene in a desmoplastic small round cell tumor. (9797782)	Shimizu Y.... Hayasaka K.	1998	EWSR1
31	The desmoplastic small round cell tumor t(11;22) translocation produces EWS/WT1 isoforms with differing oncogenic properties. (9591781)	Kim J.... Pelletier J.	1998	WT1
32	Desmoplastic small round cell tumor. (8875710)	Leuschner I.... Harms D.	1996	EWSR1
33	Intra-abdominal desmoplastic small round-cell tumor: expansion of the pathologic profile. (8782211)	Dorsey B.V.... Weidner N.	1996	DES
34	Fusion of the EWS1 and WT1 genes as a result of the t(11;22)(p13;q12) translocation in desmoplastic small round cell tumors. (8827070)	Benjamin L.E.... Rauscher F.J.	1996	WT1
35	EWS and WT-1 gene fusion in desmoplastic small round cell tumor of the abdomen. (8522311)	Brodie S.G.... Williams T.M.	1995	WT1, EWSR1
36	Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor. (7862627)	Gerald W.L.... Ladanyi M.	1995	WT1
37	Detection of chimeric transcripts in desmoplastic small round cell tumor and related developmental tumors by reverse transcriptase polymerase chain reaction. A specific diagnostic assay. (7495283)	de Alava E.... Gerald W.L.	1995	EWSR1
38	Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumor. (8187063)	Ladanyi M.... Gerald W.	1994	WT1, EWSR1
39	The EWS gene, involved in Ewing family of tumors, malignant melanoma of soft parts and desmoplastic small round cell tumors, codes for an RNA binding protein with novel regulatory domains. (8084618)	Ohno T.... Reddy E.S.P.	1994	EWSR1
40	Novel oncogenic mutations in the WT1 Wilms' tumor suppressor gene: a t(11;22) fuses the Ewing's sarcoma gene, EWS1, to WT1 in desmoplastic small round cell tumor. (7587063)	Rauscher F.J. 3rd... Morris J.F.	1994	WT1

Genes related to desmoplastic small round cell tumor according to GeneCards:

(show all 33)

id	Symbol	Description	Score	GeneCards Section Context
1	EWSR1	Ewing sarcoma breakpoint region 1	11.1	Publications
2	WT1	Wilms tumor 1	11.1	Publications
3	DES	desmin	11.1	Publications
4	MUC1	mucin 1, cell surface associated	11.1
5	MYF5	myogenic factor 5	11.0	Publications
6	CD99	CD99 molecule	11.0	Publications
7	BAIAP3	BAI1-associated protein 3	11.0	Disorders
8	SLC29A4	solute carrier family 29 (nucleoside transporters), member 4	11.0	Publications
9	MYOG	myogenin (myogenic factor 4)	11.0	Publications
10	LRRC15	leucine rich repeat containing 15	11.0	Disorders
11	TGFB3	transforming growth factor, beta 3	11.0	Publications
12	MYOD1	myogenic differentiation 1	11.0	Publications
13	TTF1	transcription termination factor, RNA polymerase I	11.0
14	PDGFA	platelet-derived growth factor alpha polypeptide	11.0	Publications
15	CALB2	calbindin 2	11.0	Publications
16	TSPAN7	tetraspanin 7	11.0	Publications
17	BMP4	bone morphogenetic protein 4	11.0	Publications
18	SYP	synaptophysin	11.0	Publications
19	PAX7	paired box 7	11.0
20	VIM	vimentin	11.0	Publications
21	ENO2	enolase 2 (gamma, neuronal)	11.0	Publications
22	ACTC1	actin, alpha, cardiac muscle 1	11.0	Publications
23	FLI1	Friend leukemia virus integration 1	11.0	Publications
24	ETV1	ets variant 1	11.0
25	ALPP	alkaline phosphatase, placental	11.0	Publications
26	THBD	thrombomodulin	11.0	Publications
27	CSF3	colony stimulating factor 3 (granulocyte)	11.0	Publications
28	MB	myoglobin	11.0	Publications
29	IGF2	insulin-like growth factor 2 (somatomedin A)	11.0	Publications
30	KIT	v-kit Hardy-Zuckerman 4 feline sarcoma viral oncogene homolog	11.0	Publications
31	PDGFRB	platelet-derived growth factor receptor, beta polypeptide	11.0	Publications
32	S100B	S100 calcium binding protein B	11.0	Publications
33	ERBB2	v-erb-b2 erythroblastic leukemia viral oncogene homolog 2, neuro/glioblastoma derived oncogene homolog (avian)	11.0	Publications

Pathways related to desmoplastic small round cell tumor according to GeneDecks:

(show all 50)

id	Pathway	Score	Top Affiliating Genes
1	Actin-Based Motility by Rho Family GTPases36	10.4	BMP4, TGFB3
2	CDO in myogenesis38	10.3	MYOG, MYF5, MYOD1
3	Breast Cancer Regulation by Stathmin136	9.7	PDGFA, TGFB3, ERBB2, BMP4
4	Rac1 Pathway36	9.6	PDGFRB, PDGFA, BMP4, TGFB3
5	Molecular Mechanisms of Cancer36	9.6	BMP4, IGF2, PDGFA, TGFB3
6	JNK Pathway36	9.5	BMP4, PDGFRB, KIT
7	Pathways in cancer20	9.5	TGFB3, BMP4, ERBB2, PDGFA
8	14-3-3 Induced Intracellular Signaling36	9.4	BMP4, ERBB2, TGFB3, PDGFRB
9	PTEN Pathway36	9.1	PDGFA, KIT, BMP4, PDGFRB
10	Human Embryonic Stem Cell Pluripotency36	9.0	PDGFA, PDGFRB, TGFB3, BMP4
11	PPAR Pathway36	8.9	BMP4, KIT, TGFB3, PDGFRB, IGF2
12	Ras Pathway36	8.8	TGFB3, PDGFA, KIT, ERBB2, IGF2, BMP4
13	Apoptotic Pathways in Synovial Fibroblasts36	8.7	TGFB3, BMP4, IGF2, PDGFRB, PDGFA, KIT
14	p53 Mediated Apoptosis36	8.7	PDGFA, TGFB3, IGF2, BMP4, KIT, PDGFRB
15	Telomerase Components in Cell Signaling36	8.7	KIT, IGF2, PDGFA, PDGFRB, TGFB3, BMP4
16	Mitochondrial Apoptosis36	8.7	TGFB3, PDGFRB, PDGFA, BMP4, KIT, IGF2
17	DHA Signaling36	8.7	TGFB3, PDGFA, IGF2, BMP4, KIT, PDGFRB
18	ERK5 Signaling36	8.7	BMP4, IGF2, PDGFA, KIT, TGFB3, PDGFRB
19	Cellular Apoptosis Pathway36	8.7	PDGFRB, KIT, BMP4, PDGFA, TGFB3, IGF2
20	eIF2 Pathway36	8.7	BMP4, KIT, PDGFRB, IGF2, PDGFA, TGFB3
21	Nuclear Receptor Activation by Vitamin-A36	8.7	KIT, BMP4, IGF2, PDGFA, TGFB3, PDGFRB
22	p70S6K Signaling36	8.6	IGF2, BMP4, KIT, PDGFA, TGFB3, PDGFRB
23	Activation of PKC through GPCR36	8.6	BMP4, IGF2, KIT, PDGFA, PDGFRB, TGFB3
24	Cytokine-cytokine receptor interaction20	8.6	PDGFA, KIT, CSF3, TGFB3, PDGFRB
25	Renin-Angiotensin Pathway36	8.6	PDGFRB, PDGFA, IGF2, BMP4, KIT, TGFB3
26	Rap1 Pathway36	8.6	TGFB3, PDGFRB, PDGFA, BMP4, ERBB2, IGF2
27	CREB Pathway36	8.5	TGFB3, PDGFRB, PDGFA, IGF2, BMP4, KIT
28	Activation of cAMP-Dependent PKA36	8.5	BMP4, IGF2, ERBB2, PDGFA, PDGFRB, TGFB3
29	Endothelin-1 Signaling Pathway36	8.4	ERBB2, KIT, BMP4, CSF3, TGFB3, PDGFRB
30	PAK Pathway36	8.3	PDGFA, IGF2, BMP4, KIT, TGFB3, CSF3
31	Tec Kinases Signaling36	8.3	TGFB3, PDGFRB, PDGFA, ERBB2, IGF2, BMP4
32	Nanog in Mammalian ESC Pluripotency36	8.2	PDGFA, PDGFRB, KIT, TGFB3, BMP4, ERBB2
33	Paxillin Interactions36	8.2	PDGFA, ERBB2, IGF2, BMP4, KIT, PDGFRB
34	Pancreatic Adenocarcinoma36	8.2	TGFB3, KIT, BMP4, IGF2, ERBB2, PDGFA
35	eNOS Signaling36	8.2	PDGFA, IGF2, BMP4, PDGFRB, KIT, ERBB2
36	NFAT in Immune Response36	8.2	ERBB2, IGF2, BMP4, KIT, PDGFA, TGFB3
37	Phospholipase-C Pathway36	8.2	KIT, PDGFA, TGFB3, PDGFRB, BMP4, IGF2
38	Estrogen Pathway36	8.2	ERBB2, IGF2, BMP4, KIT, TGFB3, PDGFA
39	GPCR Pathway36	8.2	PDGFA, PDGFRB, ERBB2, IGF2, KIT, BMP4
40	GSK3 Signaling36	8.2	PDGFRB, KIT, BMP4, TGFB3, IGF2, PDGFA
41	Antioxidant Action of Vitamin-C36	8.2	BMP4, PDGFRB, TGFB3, CSF3, IGF2, KIT
42	JAK-STAT Pathway36	8.2	PDGFA, PDGFRB, BMP4, IGF2, ERBB2, TGFB3
43	Activation of PKA through GPCR36	8.1	ERBB2, TGFB3, PDGFRB, IGF2, BMP4, KIT
44	Intracellular Calcium Signaling36	8.1	BMP4, ERBB2, TGFB3, PDGFRB, PDGFA, KIT
45	MAPK Signaling36	8.0	TGFB3, PDGFRB, PDGFA, IGF2, BMP4, KIT
46	ILK Signaling36	7.9	TGFB3, KIT, VIM, BMP4, PDGFA, PDGFRB
47	Akt Signaling36	7.8	IGF2, CSF3, TGFB3, PDGFRB, PDGFA, ERBB2
48	ERK Signaling36	7.7	KIT, IGF2, ERBB2, CSF3, TGFB3, PDGFRB
49	p38 Signaling36	7.6	CSF3, ETV1, KIT, BMP4, IGF2, PDGFA
50	Rho Family GTPases36	7.5	VIM, IGF2, BMP4, CSF3, KIT, DES

Compounds related to desmoplastic small round cell tumor according to GeneDecks:

(show top 50)    (show all 125)

id	Compound	Score	Top Affiliating Genes
1	progestin32	9.7	IGF2, DES, ALPP, VIM
2	orcein32	9.3	ACTC1, VIM, S100B, DES
3	etoposide32 42 9 9	12.2	S100B, ERBB2, ENO2
4	azathioprine32 34 9 9	12.1	BMP4, VIM, MYOD1, IGF2, THBD, DES
5	reticulin32	9.1	DES, ACTC1, S100B, MUC1, VIM, SYP
6	toluidine32	9.0	DES, ENO2, VIM, KIT
7	hyaluronic acid32 18	10.0	DES, THBD, WT1, CALB2, ENO2, VIM
8	butyrate32	8.9	VIM, IGF2, ALPP, CALB2, MYOG, MUC1
9	mucicarmine32	8.9	CALB2, S100B, VIM, MUC1, DES, SYP
10	s 10032	8.8	DES, VIM, MUC1, S100B, ENO2, ACTC1
11	tgf beta132	8.7	MYOD1, VIM, BMP4, IGF2, PDGFA, TGFB3
12	glutamate32	8.6	ALPP, VIM, MYF5, ERBB2, CALB2, IGF2
13	heparin32 9 18 9	11.5	PDGFA, ENO2, IGF2, ALPP, ERBB2, DES
14	polyethylene glycol32	8.5	MUC1, MB, THBD, ERBB2, IGF2, ALPP
15	dbc-amp32	8.3	BMP4, SYP, THBD, IGF2, ENO2, S100B
16	bromodeoxyuridine32	8.1	ENO2, DES, PDGFRB, S100B, BMP4, MYF5
17	b 72332	8.1	MUC1, S100B, ALPP, CALB2, WT1, ACTC1
18	hematoxylin32	8.1	DES, SYP, ACTC1, CALB2, ENO2, S100B
19	forskolin32 42 9 9	10.9	WT1, VIM, MYOG, THBD, SYP, ENO2
20	ascorbic acid32 18	8.8	S100B, MYOG, BMP4, THBD, PDGFA, ALPP
21	vitamin d32	7.8	ALPP, ERBB2, PDGFA, PDGFRB, WT1, THBD
22	ribonucleic acid32	7.8	ENO2, MUC1, IGF2, PDGFA, KIT, PDGFRB
23	formaldehyde32 18	8.8	S100B, MUC1, VIM, KIT, SYP, ENO2
24	chloramphenicol32 9 9	9.7	ALPP, IGF2, S100B, ERBB2, MUC1, MYOD1
25	acetylcholine32 9 18 9	10.6	CALB2, MYF5, MYOG, SYP, VIM, S100B
26	norepinephrine32 9 18 9	10.6	MB, IGF2, ENO2, ALPP, SYP, DES
27	glutamine32	7.5	S100B, MB, ENO2, VIM, KIT, SYP
28	nitric oxide32 9 18 9	10.5	TGFB3, KIT, MYOG, MUC1, S100B, ENO2
29	glycogen32 18	8.5	MUC1, ACTC1, IGF2, ENO2, DES, KIT
30	cysteine32	7.4	BMP4, MB, S100B, MUC1, IGF2, THBD
31	cisplatin32 34 9 9	10.2	MB, CSF3, ENO2, PDGFRB, ERBB2, ALPP
32	thymidine32 18	8.2	CALB2, WT1, ALPP, IGF2, TGFB3, ENO2
33	progesterone32 42 9 18 9	11.1	S100B, BMP4, DES, KIT, ERBB2, PDGFA
34	valine32	7.1	THBD, ERBB2, IGF2, MB, S100B, KIT
35	arginine32	7.1	DES, MB, THBD, WT1, MYOD1, S100B
36	oligonucleotide32	7.0	PDGFRB, EWSR1, IGF2, PDGFA, WT1, THBD
37	imatinib32 34 9 9	10.0	ACTC1, S100B, KIT, IGF2, ERBB2, PDGFRB
38	calcium32 9 18 9	10.0	MYOD1, MYOG, KIT, BMP4, S100B, EWSR1
39	paclitaxel32 34 9 9	9.8	IGF2, S100B, CSF3, PDGFRB, VIM, KIT
40	lactate32	6.8	MB, S100B, VIM, KIT, ENO2, ALPP
41	creatinine32	6.7	MB, ENO2, IGF2, ALPP, WT1, ACTC1
42	steroid32	6.6	WT1, ACTC1, MYOG, MYOD1, DES, BMP4
43	dexamethasone32 42 34 9 9	10.4	BMP4, MUC1, ENO2, IGF2, ALPP, ERBB2
44	retinoic acid32 42 18	7.7	MYOD1, BMP4, MYF5, MYOG, S100B, WT1
45	testosterone32 9 18 9	7.9	IGF2, ENO2, MB, S100B, MUC1, MYOD1
46	estrogen32	4.8	THBD, ACTC1, WT1, PDGFRB, PDGFA, ERBB2
47	serine32	4.7	MB, MYOD1, ERBB2, ETV1, EWSR1, MYF5
48	tyrosine32	4.6	PDGFRB, MUC1, ERBB2, PDGFA, WT1, ACTC1
49	paraffin32	4.4	ALPP, CALB2, EWSR1, ERBB2, PDGFA, PDGFRB
50	vegf32	4.1	S100B, MUC1, BMP4, MYOD1, MYOG, VIM

Biological processes related to desmoplastic small round cell tumor according to GeneDecks:

(show all 12)

id	Name	GO ID	Score	Top Affiliating Genes
1	muscle cell fate commitment	GO:042693	10.3	MYOG, MYOD1, MYF5
2	muscle tissue morphogenesis	GO:060415	10.3	MYF5, PAX7
3	positive regulation of muscle cell differentiation	GO:051149	10.3	MYOG, MYOD1, MYF5
4	muscle cell differentiation	GO:042692	10.2	MYOG, MYF5, MYOD1
5	lung alveolus development	GO:048286	10.1	TGFB3, PDGFA, BMP4
6	skeletal muscle tissue development	GO:007519	10.0	MYOG, MYOD1, MYF5
7	muscle filament sliding	GO:030049	10.0	VIM, DES, ACTC1
8	cellular response to growth factor stimulus	GO:071363	9.9	MYOG, BMP4, TGFB3
9	positive regulation of metanephric mesenchymal cell migration by platelet-derived growth factor receptor-beta signaling pathway	GO:035793	9.9	PDGFRB, PDGFA
10	lymphoid progenitor cell differentiation	GO:002320	9.6	KIT, BMP4
11	positive regulation of MAP kinase activity	GO:043406	9.2	ERBB2, PDGFA, PDGFRB
12	positive regulation of phosphatidylinositol 3-kinase cascade	GO:014068	8.8	PDGFRB, PDGFA, KIT

Molecular functions related to desmoplastic small round cell tumor according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	E-box binding	GO:070888	10.0	MYOG, MYF5, MYOD1
2	growth factor activity	GO:008083	8.8	BMP4, CSF3, TGFB3, PDGFA, IGF2
3	protein heterodimerization activity	GO:046982	8.8	MYOD1, TGFB3, PDGFA, ERBB2, ENO2, MYOG