MCID: OVR121
MIFTS: 31

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 20 17
Ovarian Sex Cord-Stromal Tumor 20

Classifications:



External Ids:

Disease Ontology 12 DOID:0080369
NCIt 50 C4862

Summaries for Ovarian Sex-Cord Stromal Tumor

GARD : 20 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 cervical adenoma malignum and estrogen excess. An important gene associated with Ovarian Sex-Cord Stromal Tumor is NR5A1 (Nuclear Receptor Subfamily 5 Group A Member 1). The drugs Paclitaxel and Etoposide have been mentioned in the context of this disorder. Affiliated tissues include ovary, breast and cervix.

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 224)
# Related Disease Score Top Affiliating Genes
1 cervical adenoma malignum 30.8 MME KRT7
2 estrogen excess 30.8 FOXL2 EPPIN
3 gonadoblastoma 30.7 WT1 NR5A1 FOXL2
4 gonadal dysgenesis 30.7 WT1 NR5A1 FOXL2
5 sertoli cell tumor 30.7 WT1 NR5A1 KRT7 CALB2
6 gynandroblastoma 30.5 FOXL2 DICER1 CALB2
7 granulosa cell tumor of the ovary 30.5 KRT7 FOXL2
8 premature menopause 30.4 WT1 NR5A1 FOXL2 EPPIN
9 sertoli-leydig cell tumor 29.9 WT1 KRT7 DICER1 CALB2
10 malignant sex cord stromal tumor of ovary 11.5
11 adenoma 10.6
12 peutz-jeghers syndrome 10.4
13 maligant granulosa cell tumor of the ovary 10.3 FOXL2 DICER1
14 adrenal rest tumor 10.3 NR5A1 EPPIN
15 fallopian tube carcinosarcoma 10.3 WT1 CALB2
16 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.3
17 endometrial cancer 10.3
18 endometrial hyperplasia 10.3
19 male infertility 10.3
20 endometrial adenocarcinoma 10.3
21 mucinous adenocarcinoma 10.3
22 turner syndrome 10.3
23 appendix adenocarcinoma 10.3
24 cervical adenocarcinoma 10.3
25 48,xyyy 10.3
26 posttransplant acute limbic encephalitis 10.3
27 intestinal polyposis syndrome 10.3
28 46,xy partial gonadal dysgenesis 10.3 WT1 NR5A1
29 ovarian melanoma 10.3 MLANA CALB2
30 amenorrhea 10.3
31 precocious puberty 10.3
32 malignant biphasic mesothelioma 10.3 WT1 CALB2
33 ovarian gonadoblastoma 10.3 WT1 CALB2
34 kidney sarcoma 10.3 WT1 DICER1
35 thymus clear cell carcinoma 10.2 MLANA CALB2
36 pacinian tumor 10.2 MME CALB2
37 46,xy sex reversal 9 10.2 WT1 NR5A1
38 wilms tumor predisposition 10.2 WT1 DICER1
39 mixed gonadal dysgenesis 10.2 NR5A1 FOXL2
40 perivascular epithelioid cell tumor 10.2 MME MLANA
41 46,xx sex reversal 10.2 NR5A1 FOXL2
42 ovarian small cell carcinoma 10.2 WT1 CALB2
43 adenosarcoma 10.2 MME JAZF1
44 luteoma 10.2 FOXL2 EPPIN CALB2
45 ovarian mixed germ cell neoplasm 10.2 WT1 MME
46 nivelon-nivelon-mabille syndrome 10.2 WT1 NR5A1 FOXL2
47 fallopian tube endometrioid adenocarcinoma 10.2 MME CALB2
48 46,xy sex reversal 2 10.2 WT1 NR5A1 FOXL2
49 46,xy sex reversal 10.2 WT1 NR5A1 FOXL2
50 46 xx gonadal dysgenesis 10.2 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

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
Etoposide Approved Phase 3 33419-42-0 36462
3
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
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 Tubulin Modulators Phase 3
8 Antimitotic Agents Phase 3
9 Antibiotics, Antitubercular Phase 3
10 Etoposide phosphate Phase 3
11 Anti-Bacterial Agents Phase 3
12
Bevacizumab Approved, Investigational Phase 2 216974-75-3
13 Angiogenesis Inhibitors Phase 2
14 Antineoplastic Agents, Immunological 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 Unknown status 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, Breast, Cervix

Publications for Ovarian Sex-Cord Stromal Tumor

Articles related to Ovarian Sex-Cord Stromal Tumor:

(show top 50) (show all 98)
# Title Authors PMID Year
1
Ovarian Sex Cord Stromal Tumor, Steroid Cell, NOS in an Adolescent: A Case Report. 61
32781238 2021
2
The role of FOXL2, SOX9, and β-catenin expression and DICER1 mutation in differentiating sex cord tumor with annular tubules from other sex cord tumors of the ovary. 61
33566167 2021
3
Uterine Tumor Resembling Ovarian Sex Cord Stromal Tumor (UTROSCT): A Series of 3 Cases With Extensive Rhabdoid Differentiation, Malignant Behavior, and ESR1-NCOA2 Fusions. 61
32675660 2020
4
Development and Validation of a Prognostic Prediction Model for Postoperative Ovarian Sex Cord-Stromal Tumor Patients. 61
32943602 2020
5
Genomic profiling of primary and recurrent adult granulosa cell tumors of the ovary. 61
32203090 2020
6
[Application of molecular analysis in differential diagnosis of ovarian adult granulosa cell tumors]. 61
32746545 2020
7
Sex-Cord Tumor with Annular Tubules with Unusual Morphology in an Infant with Peutz-Jeghers Syndrome. 61
32609034 2020
8
Right trisectionectomy for liver metastasis of granulosa cell tumor: a case report and literature review. 61
32494954 2020
9
Non-syndromic bilateral ovarian sex cord stromal tumor with annular tubules in a postmenopausal elderly woman as an incidental finding. 61
33395920 2020
10
Ovarian sex cord stromal tumor with annular tubules in a 7-year-old child: A case report. 61
31700964 2019
11
Endocrinological characterization of an ovarian sex cord-stromal tumor with a Sertoli cell pattern in a Japanese Black cow. 61
31448448 2019
12
[Ovarian sex cord tumor with annular tubules: report of a case]. 61
31422598 2019
13
RUNX3 Promotes the Tumorigenic Phenotype in KGN, a Human Granulosa Cell Tumor-Derived Cell Line. 61
31311113 2019
14
Impact of taxane plus bevacizumab for ovarian sex cord tumor with annular tubules. 61
31060113 2019
15
Mutational Landscape of Ovarian Adult Granulosa Cell Tumors from Whole Exome and Targeted TERT Promoter Sequencing. 61
30166312 2019
16
Ovarian Sex Cord-stromal Tumors With Melanin Pigment: Report of a Previously Undescribed Phenomenon. 61
29140884 2019
17
Extraovarian sex cord tumor with annular tubules discovered arising from a leiomyoma. 61
30148200 2018
18
Cytologic features of needle aspiration of ovarian sex cord tumor with annular tubules: Report of two cases and literature review. 61
29464881 2018
19
Prognostic significance of lymphadenectomyin malignant ovarian sex cord stromal tumor: A retrospective cohort study and meta-analysis. 61
29107349 2018
20
Pediatric imaging in DICER1 syndrome. 61
28474256 2017
21
[A very rare cause of peripheral precocious puberty in a girl: ovarian sex cord tumor with annular tubules]. 61
28504506 2017
22
Luteinizing hormone elevation in ovarian granulosa cell tumor: a case report and review of the literature. 61
28438188 2017
23
Histological changes of non-Peutz-Jegher syndrome associated ovarian sex cord tumor with annular tubules in childhood. 61
31966699 2017
24
Co-occurrence of a metastatic mammary liposarcoma and an ovarian sex-cord stromal tumor in a dog. 61
27892865 2016
25
A Rare Presentation of Paraovarian Sclerosing Stromal Tumor with High Mitotic Activity. 61
26327562 2016
26
Non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules: Report of a malignant case. 61
26807963 2016
27
Sclerosing Sromal Tumor of the Ovary in Postmenopausal Women: A Report of Two Cases. 61
26356607 2015
28
Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study. 61
25886261 2015
29
Clinicopathological significance of steroidogenic factor-1 expression in ovarian cancer versus ovarian sex cord stromal tumor. 61
25604140 2015
30
Ovarian sex cord tumor with annular tubules in children. 61
25879609 2014
31
Ovarian steroid cell tumor, not otherwise specified, producing testosterone. 61
25181629 2014
32
Recurrence of adult granulosa cell tumor of the ovary: experience at a tertiary care center. 61
24630126 2014
33
Metastatic ovarian sex-cord stromal tumor with annular tubules in a patient without Peutz-Jeghers syndrome. 61
24932834 2014
34
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature. 61
24439718 2014
35
Alfa-fetoprotein secreting ovarian sex cord-stromal tumor. 61
23924561 2013
36
Cancer problem in Peutz-Jeghers syndrome. 61
23977663 2013
37
A comprehensive analysis of PAX8 expression in human epithelial tumors. 61
21552115 2011
38
Laparoscopical management ovarian early sex cord-stromal tumors in postmenopausal women: a proposal method. 61
21116640 2011
39
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
40
Ovarian interstitial cell tumor in a South American sea lion (Otaria flavescens). 61
20688715 2010
41
Sex cord tumor with annular tubules in a young adolescent with Von Willebrand's disease. 61
19896399 2010
42
[Application of immunohistochemistry and its limitations in diagnosis of ovarian sex cord-stromal tumor]. 61
20388406 2010
43
Non-Peutz-Jeghers syndrome associated ovarian sex cord tumor with annular tubules: a case report. 61
19703688 2009
44
Sclerosing stromal tumor: an important differential diagnosis of ovarian neoplasms in childhood and adolescence. 61
19071970 2009
45
Mutation of FOXL2 in granulosa-cell tumors of the ovary. 61
19516027 2009
46
Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors. 61
19033865 2009
47
A rare cause of vaginal bleeding in a 7-month-old female infant. 61
18358265 2008
48
Ultrasound of ovarian sex-cord tumor with annular tubules. 61
17764009 2007
49
Bilateral sex cord tumor with annular tubules of ovary without Peutz-Jeghers syndrome: a case report. 61
17883091 2007
50
[Peutz-Jeghers syndrome complicated by cervical adenoma malignum and ovarian sex cord tumor with annular tubules: report of a case]. 61
17374267 2006

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

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

# Name GO ID Score Top Affiliating Genes
1 adrenal gland development GO:0030325 9.37 WT1 NR5A1
2 production of miRNAs involved in gene silencing by miRNA GO:0035196 9.32 ZC3H7B DICER1
3 apoptotic DNA fragmentation GO:0006309 9.26 FOXL2 DICER1
4 positive regulation of male gonad development GO:2000020 9.16 WT1 NR5A1
5 sex determination GO:0007530 8.96 WT1 NR5A1
6 negative regulation of female gonad development GO:2000195 8.62 WT1 NR5A1

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

# Name GO ID Score Top Affiliating Genes
1 metal ion binding GO:0046872 9.23 ZC3H7B WT1 SUZ12 NR5A1 MME JAZF1

Sources for Ovarian Sex-Cord Stromal Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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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41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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