MCID: OVR121
MIFTS: 35

Ovarian Sex-Cord Stromal Tumor

Categories: Cancer diseases, Rare 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
Ovarian Sex Cord Tumor with Annular Tubules 52 17
Ovarian Sex Cord-Stromal Tumor 52

Classifications:



External Ids:

Disease Ontology 12 DOID:0080369
NCIt 49 C4862

Summaries for Ovarian Sex-Cord Stromal Tumor

NIH Rare Diseases : 52 An ovarian sex cord tumor with annular tubules (SCTAT) is a tumor that grows from cells in the ovaries known as sex cord cells. As these cells grow, they form tube-like shapes in the tumor. SCTATs can develop in one or both ovaries, and may cause symptoms such as puberty at an exceptionally young age (precocious puberty), irregular menstrual cycles, or post-menopausal bleeding. Most ovarian SCTATs are benign . However, because there is a chance that an SCTAT may be malignant , treatment may include surgery to remove the tumor.

MalaCards based summary : Ovarian Sex-Cord Stromal Tumor, also known as ovarian sex cord tumor with annular tubules, is related to leydig cell tumor and cervical adenoma malignum. 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 Corticotropin-releasing hormone signaling pathway and Ovarian steroidogenesis. The drugs Paclitaxel and Cisplatin have been mentioned in the context of this disorder. Affiliated tissues include ovary, kidney and appendix, and related phenotypes are no phenotypic analysis and normal

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.

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 216)
# Related Disease Score Top Affiliating Genes
1 leydig cell tumor 31.3 NR5A1 GNAS CALB2
2 cervical adenoma malignum 31.0 MME KRT7
3 gynandroblastoma 30.9 FOXL2 DICER1 CALB2
4 sertoli-leydig cell tumor 30.9 WT1 KRT7 DICER1 CALB2
5 estrogen excess 30.8 FOXL2 EPPIN
6 fibroma 30.8 KRT7 GNAS CALB2
7 appendix adenocarcinoma 30.7 KRT7 GNAS
8 luteoma 30.6 EPPIN CALB2 AMHR2
9 gonadoblastoma 30.6 WT1 NR5A1 FOXL2
10 granulosa cell tumor of the ovary 30.5 KRT7 FOXL2
11 sertoli cell tumor 30.3 WT1 NR5A1 KRT7 CALB2
12 microcystic stromal tumor 30.3 WT1 NR5A1 FOXL2 DICER1
13 gonadal dysgenesis 30.3 WT1 NR5A1 FOXL2 AMHR2
14 premature ovarian failure 1 30.2 WT1 NR5A1 FOXL2 AMHR2
15 juvenile type testicular granulosa cell tumor 29.0 WT1 MLANA GNAS FOXL2 DICER1 CALB2
16 sex cord-gonadal stromal tumor 28.9 WT1 NR5A1 MLANA KRT7 GNAS FOXL2
17 malignant sex cord stromal tumor of ovary 12.0
18 adenoma 10.6
19 maligant granulosa cell tumor of the ovary 10.5 FOXL2 DICER1
20 mixed germ cell-sex cord neoplasm 10.5 FOXL2 CALB2
21 fallopian tube carcinosarcoma 10.4 WT1 CALB2
22 46,xy partial gonadal dysgenesis 10.4 WT1 NR5A1
23 peritoneal serous adenocarcinoma 10.4 WT1 CALB2
24 malignant epithelial mesothelioma 10.4 WT1 CALB2
25 malignant leydig cell tumor 10.4 MLANA CALB2
26 ovarian melanoma 10.4 MLANA CALB2
27 testicular fibroma 10.4 WT1 CALB2
28 malignant biphasic mesothelioma 10.4 WT1 CALB2
29 pacinian tumor 10.4 MME CALB2
30 mucinous adenocarcinoma 10.4
31 fallopian tube adenocarcinoma 10.4 MME CALB2
32 kidney sarcoma 10.4 WT1 DICER1
33 peutz-jeghers syndrome 10.4
34 dicer1-related disorders 10.4
35 chromosomal triplication 10.4
36 precocious puberty 10.4
37 retroperitoneal sarcoma 10.4 MLANA CALB2
38 ovarian small cell carcinoma 10.3 WT1 CALB2
39 sarcomatoid squamous cell skin carcinoma 10.3 MME MLANA
40 wilms tumor predisposition 10.3 WT1 DICER1
41 spermatocele 10.3 CALB2 AMHR2
42 fallopian tube endometrioid adenocarcinoma 10.3 MME CALB2
43 vaginal endometrial stromal sarcoma 10.3 MME MLANA
44 vagina sarcoma 10.3 MME MLANA
45 pleuropulmonary blastoma 10.3
46 amenorrhea 10.3
47 infertility 10.3
48 chondrodysplasia-pseudohermaphroditism syndrome 10.3 WT1 NR5A1 FOXL2
49 testicular disease 10.2 EPPIN DICER1 CALB2
50 adult type testicular granulosa cell tumor 10.2 MLANA FOXL2 CALB2

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 no phenotypic analysis MP:0003012 9.43 AMHR2 DICER1 FOXL2 GNAS MLANA WT1
2 normal MP:0002873 9.17 BMPR1B CALB2 DICER1 FOXL2 GNAS NR5A1

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 14)
# 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 Albumin-Bound Paclitaxel Phase 3
7 Antimitotic Agents Phase 3
8 Anti-Bacterial Agents Phase 3
9 Antibiotics, Antitubercular Phase 3
10 Etoposide phosphate Phase 3
11 Topoisomerase Inhibitors Phase 3
12
Bevacizumab Approved, Investigational Phase 2 216974-75-3
13 Antineoplastic Agents, Immunological Phase 2
14 Angiogenesis Inhibitors Phase 2

Interventional clinical trials:


# 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:

40
Ovary, Kidney, Appendix, Breast, Heart, Testis, Colon

Publications for Ovarian Sex-Cord Stromal Tumor

Articles related to Ovarian Sex-Cord Stromal Tumor:

(show top 50) (show all 89)
# Title Authors PMID Year
1
Endocrinological characterization of an ovarian sex cord-stromal tumor with a Sertoli cell pattern in a Japanese Black cow. 61
31448448 2019
2
Ovarian sex cord stromal tumor with annular tubules in a 7-year-old child: A case report. 61
31700964 2019
3
[Ovarian sex cord tumor with annular tubules: report of a case]. 61
31422598 2019
4
RUNX3 Promotes the Tumorigenic Phenotype in KGN, a Human Granulosa Cell Tumor-Derived Cell Line. 61
31311113 2019
5
Impact of taxane plus bevacizumab for ovarian sex cord tumor with annular tubules. 61
31060113 2019
6
Mutational Landscape of Ovarian Adult Granulosa Cell Tumors from Whole Exome and Targeted TERT Promoter Sequencing. 61
30166312 2019
7
Ovarian Sex Cord-stromal Tumors With Melanin Pigment: Report of a Previously Undescribed Phenomenon. 61
29140884 2019
8
Extraovarian sex cord tumor with annular tubules discovered arising from a leiomyoma. 61
30148200 2018
9
Cytologic features of needle aspiration of ovarian sex cord tumor with annular tubules: Report of two cases and literature review. 61
29464881 2018
10
Prognostic significance of lymphadenectomyin malignant ovarian sex cord stromal tumor: A retrospective cohort study and meta-analysis. 61
29107349 2018
11
Pediatric imaging in DICER1 syndrome. 61
28474256 2017
12
[A very rare cause of peripheral precocious puberty in a girl: ovarian sex cord tumor with annular tubules]. 61
28504506 2017
13
Luteinizing hormone elevation in ovarian granulosa cell tumor: a case report and review of the literature. 61
28438188 2017
14
Histological changes of non-Peutz-Jegher syndrome associated ovarian sex cord tumor with annular tubules in childhood. 61
31966699 2017
15
Co-occurrence of a metastatic mammary liposarcoma and an ovarian sex-cord stromal tumor in a dog. 61
27892865 2016
16
A Rare Presentation of Paraovarian Sclerosing Stromal Tumor with High Mitotic Activity. 61
26327562 2016
17
Non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules: Report of a malignant case. 61
26807963 2016
18
Sclerosing Sromal Tumor of the Ovary in Postmenopausal Women: A Report of Two Cases. 61
26356607 2015
19
Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study. 61
25886261 2015
20
Clinicopathological significance of steroidogenic factor-1 expression in ovarian cancer versus ovarian sex cord stromal tumor. 61
25604140 2015
21
Ovarian sex cord tumor with annular tubules in children. 61
25879609 2014
22
Ovarian steroid cell tumor, not otherwise specified, producing testosterone. 61
25181629 2014
23
Recurrence of adult granulosa cell tumor of the ovary: experience at a tertiary care center. 61
24630126 2014
24
Metastatic ovarian sex-cord stromal tumor with annular tubules in a patient without Peutz-Jeghers syndrome. 61
24932834 2014
25
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature. 61
24439718 2014
26
Alfa-fetoprotein secreting ovarian sex cord-stromal tumor. 61
23924561 2013
27
Cancer problem in Peutz-Jeghers syndrome. 61
23977663 2013
28
A comprehensive analysis of PAX8 expression in human epithelial tumors. 61
21552115 2011
29
Laparoscopical management ovarian early sex cord-stromal tumors in postmenopausal women: a proposal method. 61
21116640 2011
30
Endometrial carcinoma and ovarian sex cord tumor with annular tubules in a patient with history of Peutz-Jeghers syndrome and multiple malignancies. 61
21941977 2011
31
Ovarian interstitial cell tumor in a South American sea lion (Otaria flavescens). 61
20688715 2010
32
Sex cord tumor with annular tubules in a young adolescent with Von Willebrand's disease. 61
19896399 2010
33
[Application of immunohistochemistry and its limitations in diagnosis of ovarian sex cord-stromal tumor]. 61
20388406 2010
34
Non-Peutz-Jeghers syndrome associated ovarian sex cord tumor with annular tubules: a case report. 61
19703688 2009
35
Sclerosing stromal tumor: an important differential diagnosis of ovarian neoplasms in childhood and adolescence. 61
19071970 2009
36
Mutation of FOXL2 in granulosa-cell tumors of the ovary. 61
19516027 2009
37
Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors. 61
19033865 2009
38
A rare cause of vaginal bleeding in a 7-month-old female infant. 61
18358265 2008
39
Ultrasound of ovarian sex-cord tumor with annular tubules. 61
17764009 2007
40
Bilateral sex cord tumor with annular tubules of ovary without Peutz-Jeghers syndrome: a case report. 61
17883091 2007
41
[Peutz-Jeghers syndrome complicated by cervical adenoma malignum and ovarian sex cord tumor with annular tubules: report of a case]. 61
17374267 2006
42
Peutz-Jeghers Syndrome with multiple genital tract tumors and breast cancer: a case report with a review of literatures. 61
16891826 2006
43
Chemotherapy-induced alterations in ovarian sex cord tumor with annular tubules (SCTAT): a diagnostic challenge. 61
16459027 2006
44
Ovarian sex cord-stromal tumor, unclassified: case report of endocrinologic findings and review of the literature. 61
16343200 2005
45
[Analysis of prognostic factors of malignant ovarian tumor after fertility-preserving treatment]. 61
16321304 2005
46
Histomorphologic assessment of formalin substitute fixatives for diagnostic surgical pathology. 61
15794674 2005
47
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. 61
15343512 2004
48
Colonic adenocarcinoma and bilateral malignant ovarian sex cord tumor with annular tubules in Peutz-Jeghers syndrome. 61
15524052 2004
49
Malignant mesonephric tumors of the female genital tract: a clinicopathologic study of 9 cases. 61
15105647 2004
50
Pathologic quiz case: incidental ovarian lesions in a 48-year-old woman with Peutz-Jeghers syndrome. Ovarian sex cord tumor with annular tubules. 61
14987145 2004

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 11.1 HSD3B2 HSD3B1 GNAS
2 10.49 HSD3B2 HSD3B1 GNAS

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 intercellular bridge GO:0045171 9.33 HSD3B2 HSD3B1 FOXL2
2 dendrite GO:0030425 9.02 MME GNAS DICER1 CALB2 BMPR1B
3 smooth endoplasmic reticulum membrane GO:0030868 8.96 HSD3B2 HSD3B1

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 male gonad development GO:0008584 9.63 WT1 NR5A1 AMHR2
2 adrenal gland development GO:0030325 9.52 WT1 NR5A1
3 transmembrane receptor protein serine/threonine kinase signaling pathway GO:0007178 9.51 BMPR1B AMHR2
4 response to corticosterone GO:0051412 9.49 HSD3B2 HSD3B1
5 apoptotic DNA fragmentation GO:0006309 9.48 FOXL2 DICER1
6 glucocorticoid biosynthetic process GO:0006704 9.43 HSD3B2 HSD3B1
7 androgen biosynthetic process GO:0006702 9.4 HSD3B2 HSD3B1
8 positive regulation of male gonad development GO:2000020 9.37 WT1 NR5A1
9 sex determination GO:0007530 9.32 WT1 NR5A1
10 C21-steroid hormone metabolic process GO:0008207 9.26 HSD3B2 HSD3B1
11 mineralocorticoid biosynthetic process GO:0006705 9.16 HSD3B2 HSD3B1
12 negative regulation of female gonad development GO:2000195 8.96 WT1 NR5A1
13 female gonad development GO:0008585 8.8 NR5A1 FOXL2 AMHR2

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

# Name GO ID Score Top Affiliating Genes
1 transmembrane receptor protein serine/threonine kinase activity GO:0004675 9.26 BMPR1B AMHR2
2 3-beta-hydroxy-delta5-steroid dehydrogenase activity GO:0003854 9.16 HSD3B2 HSD3B1
3 steroid delta-isomerase activity GO:0004769 8.96 HSD3B2 HSD3B1
4 cholesterol dehydrogenase activity GO:0102294 8.62 HSD3B2 HSD3B1

Sources for Ovarian Sex-Cord Stromal Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
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43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
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56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
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72 UMLS via Orphanet
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