MCID: OVR121
MIFTS: 35

Ovarian Sex-Cord Stromal Tumor

Categories: Cancer diseases, Reproductive diseases

Aliases & Classifications for Ovarian Sex-Cord Stromal Tumor

MalaCards integrated aliases for Ovarian Sex-Cord Stromal Tumor:

Name: Ovarian Sex-Cord Stromal Tumor 12 15

Classifications:



External Ids:

Disease Ontology 12 DOID:0080369
NCIt 51 C4862

Summaries for Ovarian Sex-Cord Stromal Tumor

Disease Ontology : 12 A sex cord-gonadal stromal tumor that arises from the ovary and is composed of granulosa cells, Sertoli cells, Leydig cells, theca cells, and fibroblasts.

MalaCards based summary : Ovarian Sex-Cord Stromal Tumor is related to sex cord-gonadal stromal tumor and ovarian sex cord tumor with annular tubules. An important gene associated with Ovarian Sex-Cord Stromal Tumor is NR5A1 (Nuclear Receptor Subfamily 5 Group A Member 1), and among its related pathways/superpathways are Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers and FOXA2 and FOXA3 transcription factor networks. The drugs Paclitaxel and Carboplatin have been mentioned in the context of this disorder. Affiliated tissues include ovary and endothelial, and related phenotypes are endocrine/exocrine gland and homeostasis/metabolism

Related Diseases for Ovarian Sex-Cord Stromal Tumor

Diseases related to Ovarian Sex-Cord Stromal Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 91)
# Related Disease Score Top Affiliating Genes
1 sex cord-gonadal stromal tumor 29.6 AFP CALB2 DICER1 FOXL2 NR5A1 WT1
2 ovarian sex cord tumor with annular tubules 11.9
3 46,xy partial gonadal dysgenesis 10.3 NR5A1 WT1
4 maligant granulosa cell tumor of the ovary 10.3 DICER1 FOXL2
5 kidney clear cell sarcoma 10.3 TERT WT1
6 kidney sarcoma 10.3 DICER1 WT1
7 peutz-jeghers syndrome 10.3
8 cystic nephroma 10.3 DICER1 WT1
9 denys-drash syndrome 10.2 NR5A1 WT1
10 endosalpingiosis 10.2 PAX8 WT1
11 leydig cell tumor 10.2 AFP NR5A1
12 gonadoblastoma 10.2 FOXL2 WT1
13 peritoneum cancer 10.2 PAX8 WT1
14 urethral diverticulum 10.2 PAX8 WT1
15 epithelioid cell melanoma 10.2 MLANA WT1
16 persistent mullerian duct syndrome 10.2 NR5A1 WT1
17 gonadal dysgenesis 10.2 FOXL2 NR5A1 WT1
18 sarcomatoid mesothelioma 10.2 NKX2-1 WT1
19 dysgerminoma 10.2 AFP WT1
20 premature ovarian failure 1 10.2 FOXL2 NR5A1 WT1
21 corpus luteum cyst 10.2 AFP FOXL2
22 prostatic acinar adenocarcinoma 10.2 PAX8 WT1
23 vulvar melanoma 10.2 MLANA TERT
24 ovarian cyst 10.1 AFP FOXL2
25 athyreosis 10.1 NKX2-1 PAX8
26 pulmonary blastoma 10.1 DICER1 NKX2-1
27 hypothyroidism, congenital, nongoitrous, 2 10.1 NKX2-1 PAX8
28 nephrogenic adenofibroma 10.1 PAX8 WT1
29 nephrotic syndrome, type 1 10.1 AFP WT1
30 atypical choroid plexus papilloma 10.1 DICER1 NKX2-1
31 ovarian cancer 10.1
32 pleuropulmonary blastoma 10.1
33 liposarcoma 10.1
34 childhood endodermal sinus tumor 10.1 AFP PAX8
35 frasier syndrome 10.1 NR5A1 WT1
36 renal pelvis adenocarcinoma 10.1 AFP PAX8
37 cloacal exstrophy 10.1 AFP NR5A1
38 ovarian cancer 1 10.1 PAX8 WT1
39 pineoblastoma 10.1 AFP DICER1
40 malignant ovarian surface epithelial-stromal neoplasm 10.0 PAX8 WT1
41 fallopian tube carcinosarcoma 10.0 CALB2 WT1
42 ovarian germ cell cancer 10.0 AFP NKX2-1
43 kidney benign neoplasm 10.0 DICER1 PAX8 WT1
44 malignant struma ovarii 10.0 AFP NKX2-1
45 ovarian cystic teratoma 10.0 MLANA NKX2-1
46 ovary epithelial cancer 10.0 PAX8 WT1
47 peritoneal benign neoplasm 10.0 CALB2 WT1
48 gonadal disease 10.0 AFP FOXL2 NR5A1
49 diffuse peritoneal leiomyomatosis 10.0 CALB2 WT1
50 venous hemangioma 10.0 CALB2 WT1

Graphical network of the top 20 diseases related to Ovarian Sex-Cord Stromal Tumor:



Diseases related to Ovarian Sex-Cord Stromal Tumor

Symptoms & Phenotypes for Ovarian Sex-Cord Stromal Tumor

MGI Mouse Phenotypes related to Ovarian Sex-Cord Stromal Tumor:

47 (show all 11)
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 10.13 AFP DICER1 FOXL2 NKX2-1 NR5A1 PAX8
2 homeostasis/metabolism MP:0005376 10.09 AFP DICER1 FOXL2 NKX2-1 NR5A1 PAX8
3 growth/size/body region MP:0005378 10.06 DICER1 FOXL2 NKX2-1 NR5A1 PAX8 STK11
4 mortality/aging MP:0010768 10.06 AFP DICER1 FOXL2 NKX2-1 NR5A1 PAX8
5 behavior/neurological MP:0005386 10.04 AFP CALB2 DICER1 NKX2-1 NR5A1 PAX8
6 nervous system MP:0003631 9.87 CALB2 DICER1 NKX2-1 NR5A1 PAX8 STK11
7 neoplasm MP:0002006 9.85 AFP DICER1 NKX2-1 STK11 TERT WT1
8 normal MP:0002873 9.81 CALB2 DICER1 FOXL2 NKX2-1 NR5A1 PAX8
9 no phenotypic analysis MP:0003012 9.8 DICER1 FOXL2 MLANA NKX2-1 PAX8 STK11
10 reproductive system MP:0005389 9.61 AFP DICER1 FOXL2 NKX2-1 NR5A1 PAX8
11 respiratory system MP:0005388 9.02 DICER1 NKX2-1 STK11 TERT WT1

Drugs & Therapeutics for Ovarian Sex-Cord Stromal Tumor

Drugs for Ovarian Sex-Cord Stromal Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 25)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Paclitaxel Approved, Vet_approved Phase 3,Phase 2 33069-62-4 36314
2
Carboplatin Approved Phase 3,Phase 2 41575-94-4 38904 10339178 498142
3
Bleomycin Approved, Investigational Phase 3,Phase 2 11056-06-7 5360373
4
Cisplatin Approved Phase 3,Phase 2 15663-27-1 441203 84093 2767
5
Etoposide Approved Phase 3,Phase 2 33419-42-0 36462
6 Anti-Bacterial Agents Phase 3,Phase 2
7 Etoposide phosphate Phase 3,Phase 2
8 Topoisomerase Inhibitors Phase 3,Phase 2
9 Albumin-Bound Paclitaxel Phase 3,Phase 2
10 Antibiotics, Antitubercular Phase 3,Phase 2
11 Antimitotic Agents Phase 3,Phase 2
12 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
13
Bevacizumab Approved, Investigational Phase 2 216974-75-3
14 Antineoplastic Agents, Immunological Phase 2
15 Angiogenesis Modulating Agents Phase 2
16 Angiogenesis Inhibitors Phase 2
17 Immunoglobulins Phase 2
18 Immunoglobulin G Phase 2
19 Antibodies, Monoclonal Phase 2
20 Antibodies Phase 2
21 Immunologic Factors Phase 2
22 Endothelial Growth Factors Phase 2
23 Mitogens Phase 2
24 Imatinib Mesylate Phase 2 220127-57-1 123596
25 Protein Kinase Inhibitors Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 TC or BEP in Treating Patients With Ovarian Malignant Sex Cord-Stromal Tumors Recruiting NCT02429700 Phase 3 Paclitaxel;Carboplatin;Bleomycin;Etoposide;Cisplatin
2 Efficacy and Safety of Bevacizumab (Avastin®) Combined to Weekly Paclitaxel Followed by Bevacizumab (Avastin®) Alone in Patients With Relapsed Ovarian Sex-cord Stromal Tumours (ALIENOR) Active, not recruiting NCT01770301 Phase 2 Paclitaxel;Bevacizumab
3 Paclitaxel and Carboplatin or Bleomycin Sulfate, Etoposide Phosphate, and Cisplatin in Treating Patients With Advanced or Recurrent Sex Cord-Ovarian Stromal Tumors Recruiting NCT01042522 Phase 2 Carboplatin;Cisplatin;Etoposide Phosphate;Paclitaxel
4 Paclitaxel in Treating Patients With Ovarian Stromal Cancer Completed NCT00006227 Phase 2 Paclitaxel
5 Bevacizumab in Treating Patients With Recurrent Sex Cord-Stromal Tumors of the Ovary Completed NCT00748657 Phase 2
6 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
7 International Ovarian & Testicular Stromal Tumor Registry Recruiting NCT01970696

Search NIH Clinical Center for Ovarian Sex-Cord Stromal Tumor

Genetic Tests for Ovarian Sex-Cord Stromal Tumor

Anatomical Context for Ovarian Sex-Cord Stromal Tumor

MalaCards organs/tissues related to Ovarian Sex-Cord Stromal Tumor:

42
Ovary, Endothelial

Publications for Ovarian Sex-Cord Stromal Tumor

Articles related to Ovarian Sex-Cord Stromal Tumor:

# Title Authors Year
1
Prognostic significance of lymphadenectomyin malignant ovarian sex cord stromal tumor: A retrospective cohort study and meta-analysis. ( 29107349 )
2018
2
Co-occurrence of a metastatic mammary liposarcoma and an ovarian sex-cord stromal tumor in a dog. ( 27892865 )
2016
3
Clinicopathological significance of steroidogenic factor-1 expression in ovarian cancer versus ovarian sex cord stromal tumor. ( 25604140 )
2015
4
Metastatic ovarian sex-cord stromal tumor with annular tubules in a patient without Peutz-Jeghers syndrome. ( 24932834 )
2014
5
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature. ( 24439718 )
2014
6
Alfa-fetoprotein secreting ovarian sex cord-stromal tumor. ( 23924561 )
2013
7
Ovarian sex cord-stromal tumor, unclassified: case report of endocrinologic findings and review of the literature. ( 16343200 )
2005
8
Conservative management of a uterine tumor resembling an ovarian sex cord-stromal tumor. ( 14751183 )
2004
9
A distinctive ovarian sex cord-stromal tumor causing sexual precocity in the Peutz-Jeghers syndrome. ( 6837833 )
1983

Variations for Ovarian Sex-Cord Stromal Tumor

Expression for Ovarian Sex-Cord Stromal Tumor

Search GEO for disease gene expression data for Ovarian Sex-Cord Stromal Tumor.

Pathways for Ovarian Sex-Cord Stromal Tumor

Pathways related to Ovarian Sex-Cord Stromal Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.92 AFP TERT WT1
2 10.6 AFP NKX2-1

GO Terms for Ovarian Sex-Cord Stromal Tumor

Cellular components related to Ovarian Sex-Cord Stromal Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 9.61 CALB2 DICER1 FOXL2 NKX2-1 NR5A1 PAX8
2 nucleoplasm GO:0005654 9.17 FOXL2 NKX2-1 NR5A1 PAX8 STK11 TERT

Biological processes related to Ovarian Sex-Cord Stromal Tumor according to GeneCards Suite gene sharing:

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 9.93 FOXL2 NKX2-1 NR5A1 PAX8 WT1
2 positive regulation of transcription, DNA-templated GO:0045893 9.65 FOXL2 NKX2-1 NR5A1 PAX8 WT1
3 branching involved in ureteric bud morphogenesis GO:0001658 9.57 PAX8 WT1
4 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.55 TERT WT1
5 thyroid gland development GO:0030878 9.52 NKX2-1 PAX8
6 female gonad development GO:0008585 9.51 FOXL2 NR5A1
7 tissue development GO:0009888 9.49 NR5A1 WT1
8 adrenal gland development GO:0030325 9.48 NR5A1 WT1
9 apoptotic DNA fragmentation GO:0006309 9.46 DICER1 FOXL2
10 production of siRNA involved in RNA interference GO:0030422 9.43 DICER1 TERT
11 cellular response to gonadotropin stimulus GO:0071371 9.4 PAX8 WT1
12 positive regulation of male gonad development GO:2000020 9.37 NR5A1 WT1
13 sex determination GO:0007530 9.32 NR5A1 WT1
14 transcription by RNA polymerase II GO:0006366 9.26 NR5A1
15 metanephric epithelium development GO:0072207 9.16 PAX8 WT1
16 metanephric S-shaped body morphogenesis GO:0072284 8.96 PAX8 WT1
17 negative regulation of female gonad development GO:2000195 8.62 NR5A1 WT1

Molecular functions related to Ovarian Sex-Cord Stromal Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 DNA-binding transcription factor activity GO:0003700 9.55 FOXL2 NKX2-1 NR5A1 PAX8 WT1
2 DNA binding GO:0003677 9.5 DICER1 FOXL2 NKX2-1 NR5A1 PAX8 TERT
3 transcription regulatory region DNA binding GO:0044212 9.43 NKX2-1 PAX8 WT1
4 sequence-specific DNA binding GO:0043565 9.02 FOXL2 NKX2-1 NR5A1 PAX8 WT1

Sources for Ovarian Sex-Cord Stromal Tumor

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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