MCID: OVR121
MIFTS: 29

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 50 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 sertoli-leydig cell tumor and gynandroblastoma. An important gene associated with Ovarian Sex-Cord Stromal Tumor is NR5A1 (Nuclear Receptor Subfamily 5 Group A Member 1). The drugs Paclitaxel and Cisplatin have been mentioned in the context of this disorder. Affiliated tissues include ovary, 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:

(showing 59, show less)
# Related Disease Score Top Affiliating Genes
1 sertoli-leydig cell tumor 31.2 WT1 DICER1
2 gynandroblastoma 30.7 FOXL2 DICER1
3 gonadoblastoma 30.2 WT1 FOXL2
4 juvenile type testicular granulosa cell tumor 30.1 WT1 FOXL2 DICER1
5 sex cord-gonadal stromal tumor 29.3 WT1 NR5A1 GNAS FOXL2 DICER1
6 malignant sex cord stromal tumor of ovary 12.0
7 ovarian sex cord tumor with annular tubules 11.7
8 leydig cell tumor 10.6
9 fibroma 10.6
10 sertoli cell tumor 10.4
11 peutz-jeghers syndrome 10.4
12 dicer1-related disorders 10.4
13 chromosomal triplication 10.4
14 pleuropulmonary blastoma 10.3
15 amenorrhea 10.3
16 infertility 10.3
17 luteoma 10.3
18 precocious puberty 10.3
19 maligant granulosa cell tumor of the ovary 10.1 FOXL2 DICER1
20 leiomyoma, uterine 10.1
21 enchondromatosis, multiple, ollier type 10.1
22 ovarian cancer 10.1
23 scott syndrome 10.1
24 multiple enchondromatosis, maffucci type 10.1
25 endometriosis of ovary 10.1
26 leiomyoma 10.1
27 neuroendocrine tumor 10.1
28 endometriosis 10.1
29 liposarcoma 10.1
30 embryoma 10.1
31 ovarian cyst 10.1
32 pleomorphic liposarcoma 10.1
33 peritonitis 10.1
34 dicer1-related pleuropulmonary blastoma cancer predisposition syndrome 10.1
35 germ cells tumors 10.1
36 posttransplant acute limbic encephalitis 10.1
37 rare tumor 10.1
38 denys-drash syndrome 10.0 WT1 NR5A1
39 multinodular goiter 10.0 GNAS DICER1
40 gonadal disease 10.0 NR5A1 FOXL2
41 goiter 10.0 GNAS DICER1
42 persistent mullerian duct syndrome 10.0 WT1 NR5A1
43 kidney sarcoma 10.0 WT1 DICER1
44 cystic nephroma 10.0 WT1 DICER1
45 46 xy gonadal dysgenesis 10.0 WT1 NR5A1
46 kidney benign neoplasm 10.0 WT1 DICER1
47 frasier syndrome 9.9 WT1 NR5A1
48 ovarian disease 9.9 NR5A1 FOXL2
49 dysgerminoma of ovary 9.9 WT1 DICER1
50 pseudohermaphroditism 9.9 WT1 NR5A1
51 cryptorchidism, unilateral or bilateral 9.8 WT1 NR5A1
52 hypospadias 9.7 WT1 NR5A1
53 premature ovarian failure 1 9.7 WT1 NR5A1 FOXL2
54 adrenocortical carcinoma, hereditary 9.6 NR5A1 GNAS
55 testicular granulosa cell tumor 9.6 WT1 FOXL2 DICER1
56 diaphragmatic hernia, congenital 9.5 ZFPM2 WT1
57 46,xy partial gonadal dysgenesis 9.3 ZFPM2 WT1 NR5A1
58 male reproductive organ benign neoplasm 9.2 WT1 NR5A1 FOXL2 DICER1
59 gonadal dysgenesis 8.9 ZFPM2 WT1 NR5A1 FOXL2

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:

46 (showing 11, show less)
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.93 DICER1 FOXL2 GNAS NR5A1 WT1 ZFPM2
2 homeostasis/metabolism MP:0005376 9.91 DICER1 FOXL2 GNAS NR5A1 WT1 ZFPM2
3 cardiovascular system MP:0005385 9.89 DICER1 GNAS NR5A1 WT1 ZFPM2
4 cellular MP:0005384 9.88 DICER1 GNAS NR5A1 WT1 ZFPM2
5 mortality/aging MP:0010768 9.85 DICER1 FOXL2 GNAS NR5A1 WT1 ZFPM2
6 liver/biliary system MP:0005370 9.71 DICER1 GNAS WT1 ZFPM2
7 no phenotypic analysis MP:0003012 9.65 DICER1 FOXL2 GNAS WT1 ZFPM2
8 normal MP:0002873 9.63 DICER1 FOXL2 GNAS NR5A1 WT1 ZFPM2
9 muscle MP:0005369 9.62 DICER1 GNAS WT1 ZFPM2
10 reproductive system MP:0005389 9.35 DICER1 FOXL2 NR5A1 WT1 ZFPM2
11 respiratory system MP:0005388 8.92 DICER1 GNAS WT1 ZFPM2

Drugs & Therapeutics for Ovarian Sex-Cord Stromal Tumor

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

(showing 17, show less)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Paclitaxel Approved, Vet_approved Phase 3 33069-62-4 36314
2
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
3
Etoposide Approved Phase 3 33419-42-0 36462
4
Bleomycin Approved, Investigational Phase 3 11056-06-7 5360373
5
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
6 Antineoplastic Agents, Phytogenic Phase 3
7 Albumin-Bound Paclitaxel Phase 3
8 Tubulin Modulators Phase 3
9 Antimitotic Agents Phase 3
10 Antibiotics, Antitubercular Phase 3
11 Anti-Bacterial Agents Phase 3
12 Topoisomerase Inhibitors Phase 3
13 Etoposide phosphate Phase 3
14
Bevacizumab Approved, Investigational Phase 2 216974-75-3
15 Angiogenesis Inhibitors Phase 2
16 Angiogenesis Modulating Agents Phase 2
17 Antineoplastic Agents, Immunological Phase 2

Interventional clinical trials:

(showing 2, show less)
# Name Status NCT ID Phase Drugs
1 A Multicenter, Prospective, Randomized Trial Comparing Paclitaxel and Carboplatin or Bleomycin, Etoposide and Cisplatin in the Treatment of Ovarian Malignant Sex Cord-Stromal Tumors Recruiting NCT02429700 Phase 3 Paclitaxel;Carboplatin;Bleomycin;Etoposide;Cisplatin
2 A Randomized, Open Label, Phase II Trial of Bevacizumab Plus Weekly Paclitaxel Followed by Bevacizumab Monotherapy Maintenance Versus Weekly Paclitaxel Followed by Observation in Patients With Relapsed Ovarian Sex-cord Stromal Tumours Active, not recruiting NCT01770301 Phase 2 Paclitaxel;Bevacizumab

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:

41
Ovary

Publications for Ovarian Sex-Cord Stromal Tumor

Articles related to Ovarian Sex-Cord Stromal Tumor:

(showing 39, show less)
# Title Authors PMID Year
1
RUNX3 Promotes the Tumorigenic Phenotype in KGN, a Human Granulosa Cell Tumor-Derived Cell Line. 38
31311113 2019
2
Mutational Landscape of Ovarian Adult Granulosa Cell Tumors from Whole Exome and Targeted TERT Promoter Sequencing. 38
30166312 2019
3
Ovarian Sex Cord-stromal Tumors With Melanin Pigment: Report of a Previously Undescribed Phenomenon. 38
29140884 2019
4
Extraovarian sex cord tumor with annular tubules discovered arising from a leiomyoma. 38
30148200 2018
5
Prognostic significance of lymphadenectomyin malignant ovarian sex cord stromal tumor: A retrospective cohort study and meta-analysis. 38
29107349 2018
6
Pediatric imaging in DICER1 syndrome. 38
28474256 2017
7
Luteinizing hormone elevation in ovarian granulosa cell tumor: a case report and review of the literature. 38
28438188 2017
8
Co-occurrence of a metastatic mammary liposarcoma and an ovarian sex-cord stromal tumor in a dog. 38
27892865 2016
9
A Rare Presentation of Paraovarian Sclerosing Stromal Tumor with High Mitotic Activity. 38
26327562 2016
10
Sclerosing Sromal Tumor of the Ovary in Postmenopausal Women: A Report of Two Cases. 38
26356607 2015
11
Clinicopathological significance of steroidogenic factor-1 expression in ovarian cancer versus ovarian sex cord stromal tumor. 38
25604140 2015
12
Ovarian steroid cell tumor, not otherwise specified, producing testosterone. 38
25181629 2014
13
Metastatic ovarian sex-cord stromal tumor with annular tubules in a patient without Peutz-Jeghers syndrome. 38
24932834 2014
14
Recurrence of adult granulosa cell tumor of the ovary: experience at a tertiary care center. 38
24630126 2014
15
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature. 38
24439718 2014
16
Alfa-fetoprotein secreting ovarian sex cord-stromal tumor. 38
23924561 2013
17
A comprehensive analysis of PAX8 expression in human epithelial tumors. 38
21552115 2011
18
Laparoscopical management ovarian early sex cord-stromal tumors in postmenopausal women: a proposal method. 38
21116640 2011
19
Ovarian interstitial cell tumor in a South American sea lion (Otaria flavescens). 38
20688715 2010
20
[Application of immunohistochemistry and its limitations in diagnosis of ovarian sex cord-stromal tumor]. 38
20388406 2010
21
Sclerosing stromal tumor: an important differential diagnosis of ovarian neoplasms in childhood and adolescence. 38
19071970 2009
22
Mutation of FOXL2 in granulosa-cell tumors of the ovary. 38
19516027 2009
23
Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors. 38
19033865 2009
24
A rare cause of vaginal bleeding in a 7-month-old female infant. 38
18358265 2008
25
Ovarian sex cord-stromal tumor, unclassified: case report of endocrinologic findings and review of the literature. 38
16343200 2005
26
[Analysis of prognostic factors of malignant ovarian tumor after fertility-preserving treatment]. 38
16321304 2005
27
Histomorphologic assessment of formalin substitute fixatives for diagnostic surgical pathology. 38
15794674 2005
28
The STK11/LKB1 Peutz-Jegher gene is not involved in the pathogenesis of sporadic sex cord-stromal tumors, although loss of heterozygosity at 19p13.3 indicates other gene alteration in these tumors. 38
15343512 2004
29
Malignant mesonephric tumors of the female genital tract: a clinicopathologic study of 9 cases. 38
15105647 2004
30
Conservative management of a uterine tumor resembling an ovarian sex cord-stromal tumor. 38
14751183 2004
31
Positron emission tomography for detecting clinically occult surgically resectable metastatic ovarian cancer. 38
11925145 2002
32
[Immunohistochemical study on telomerase activity in ovarian sex-cord stromal tumors]. 38
12500406 2002
33
Value of inhibin staining in gynecological pathology. 38
11192074 2001
34
Androgen-secreting steroid cell tumor of the ovary. 38
11142457 2000
35
Ossifying well-differentiated Sertoli-Leydig cell tumor of the ovary. 38
10684379 2000
36
Benign and malignant neoplasms in a family with Peutz-Jeghers syndrome: study of three generations. 38
1925720 1991
37
Sex cord tumor with annular tubules associated with endometriosis of the fallopian tube. 38
1862780 1991
38
[Ovarian sex cord stromal tumors with annular tubules: a report of 6 cases]. 38
2209222 1990
39
A distinctive ovarian sex cord-stromal tumor causing sexual precocity in the Peutz-Jeghers syndrome. 38
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

GO Terms for Ovarian Sex-Cord Stromal Tumor

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

(showing 1, show less)
# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 9.1 ZFPM2 WT1 NR5A1 GNAS FOXL2 DICER1

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

(showing 15, show less)
# Name GO ID Score Top Affiliating Genes
1 regulation of transcription by RNA polymerase II GO:0006357 9.81 ZFPM2 WT1 NR5A1 FOXL2
2 positive regulation of transcription by RNA polymerase II GO:0045944 9.78 ZFPM2 WT1 NR5A1 FOXL2
3 negative regulation of transcription by RNA polymerase II GO:0000122 9.76 ZFPM2 WT1 FOXL2 DICER1
4 negative regulation of transcription, DNA-templated GO:0045892 9.7 ZFPM2 WT1 FOXL2
5 positive regulation of transcription, DNA-templated GO:0045893 9.56 ZFPM2 WT1 NR5A1 FOXL2
6 male gonad development GO:0008584 9.54 WT1 NR5A1
7 vasculogenesis GO:0001570 9.49 ZFPM2 WT1
8 tissue development GO:0009888 9.43 WT1 NR5A1
9 female gonad development GO:0008585 9.4 NR5A1 FOXL2
10 adrenal gland development GO:0030325 9.37 WT1 NR5A1
11 apoptotic DNA fragmentation GO:0006309 9.32 FOXL2 DICER1
12 transcription by RNA polymerase II GO:0006366 9.25 NR5A1
13 sex determination GO:0007530 9.16 WT1 NR5A1
14 positive regulation of male gonad development GO:2000020 9.13 ZFPM2 WT1 NR5A1
15 negative regulation of female gonad development GO:2000195 8.8 ZFPM2 WT1 NR5A1

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

(showing 4, show less)
# Name GO ID Score Top Affiliating Genes
1 metal ion binding GO:0046872 9.65 ZFPM2 WT1 NR5A1 GNAS DICER1
2 sequence-specific DNA binding GO:0043565 9.33 WT1 NR5A1 FOXL2
3 DNA-binding transcription factor activity, RNA polymerase II-specific GO:0000981 9.26 ZFPM2 WT1 NR5A1 FOXL2
4 DNA binding GO:0003677 9.02 ZFPM2 WT1 NR5A1 FOXL2 DICER1

Sources for Ovarian Sex-Cord Stromal Tumor

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10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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32 HPO
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34 ICD10 via Orphanet
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73 UMLS via Orphanet
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