MCID: OVR121
MIFTS: 31

Ovarian Sex-Cord Stromal Tumor

Categories: Cancer diseases, Rare 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 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, breast and colon.

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 220)
# Related Disease Score Top Affiliating Genes
1 leydig cell tumor 31.1 NR5A1 GNAS CALB2
2 gynandroblastoma 30.9 FOXL2 DICER1 CALB2
3 estrogen excess 30.9 FOXL2 EPPIN
4 cervical adenoma malignum 30.8 MME KRT7
5 luteoma 30.8 FOXL2 EPPIN CALB2
6 fibroma 30.6 KRT7 GNAS CALB2
7 gonadal dysgenesis 30.6 WT1 NR5A1 FOXL2
8 appendix adenocarcinoma 30.5 KRT7 GNAS
9 sertoli-leydig cell tumor 30.5 WT1 KRT7 FOXL2 DICER1 CALB2
10 gonadoblastoma 30.5 WT1 NR5A1 FOXL2
11 granulosa cell tumor of the ovary 30.5 KRT7 FOXL2
12 sertoli cell tumor 30.3 WT1 NR5A1 KRT7 CALB2
13 microcystic stromal tumor 30.2 WT1 NR5A1 FOXL2 DICER1
14 juvenile type testicular granulosa cell tumor 30.1 WT1 GNAS FOXL2 DICER1 CALB2
15 sex cord-gonadal stromal tumor 28.7 WT1 NR5A1 MLANA KRT7 GNAS FOXL2
16 malignant sex cord stromal tumor of ovary 12.0
17 adenoma 10.6
18 adrenal rest tumor 10.4 NR5A1 EPPIN
19 maligant granulosa cell tumor of the ovary 10.4 FOXL2 DICER1
20 46,xy partial gonadal dysgenesis 10.4 WT1 NR5A1
21 fallopian tube carcinosarcoma 10.4 WT1 CALB2
22 mucinous adenocarcinoma 10.4
23 peutz-jeghers syndrome 10.4
24 chromosomal triplication 10.4
25 precocious puberty 10.4
26 testicular fibroma 10.4 WT1 CALB2
27 ovarian gonadoblastoma 10.3 WT1 CALB2
28 ovarian melanoma 10.3 MLANA CALB2
29 kidney sarcoma 10.3 WT1 DICER1
30 46,xy sex reversal 9 10.3 WT1 NR5A1
31 mixed gonadal dysgenesis 10.3 NR5A1 FOXL2
32 wilms tumor predisposition 10.3 WT1 DICER1
33 46,xx sex reversal 10.3 NR5A1 FOXL2
34 premature menopause 10.3 FOXL2 EPPIN
35 ovarian small cell carcinoma 10.3 WT1 CALB2
36 fallopian tube adenocarcinoma 10.3 MME CALB2
37 pacinian tumor 10.3 MME CALB2
38 amenorrhea 10.3
39 infertility 10.3
40 blepharophimosis, ptosis, and epicanthus inversus 10.3 NR5A1 FOXL2
41 perivascular epithelioid cell tumor 10.3 MME MLANA
42 ovarian mixed germ cell neoplasm 10.2 WT1 MME
43 46 xx gonadal dysgenesis 10.2 NR5A1 FOXL2
44 fallopian tube endometrioid adenocarcinoma 10.2 MME CALB2
45 pseudohermaphroditism 10.2 WT1 NR5A1 EPPIN
46 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.2
47 endometrial cancer 10.2
48 endometrial hyperplasia 10.2
49 male infertility 10.2
50 endometrial adenocarcinoma 10.2

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
Cisplatin Approved Phase 3 15663-27-1 2767 441203 84093
3
Bleomycin Approved, Investigational Phase 3 11056-06-7 5360373
4
Etoposide Approved Phase 3 33419-42-0 36462
5
Carboplatin Approved Phase 3 41575-94-4 10339178 38904 498142
6 Albumin-Bound Paclitaxel Phase 3
7 Tubulin Modulators Phase 3
8 Antimitotic Agents Phase 3
9 Etoposide phosphate Phase 3
10 Anti-Bacterial Agents Phase 3
11 Antibiotics, Antitubercular 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 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, Breast, Colon, Liver, Cervix

Publications for Ovarian Sex-Cord Stromal Tumor

Articles related to Ovarian Sex-Cord Stromal Tumor:

(show top 50) (show all 91)
# Title Authors PMID Year
1
Right trisectionectomy for liver metastasis of granulosa cell tumor: a case report and literature review. 61
32494954 2020
2
Genomic profiling of primary and recurrent adult granulosa cell tumors of the ovary. 61
32203090 2020
3
Ovarian sex cord stromal tumor with annular tubules in a 7-year-old child: A case report. 61
31700964 2019
4
Endocrinological characterization of an ovarian sex cord-stromal tumor with a Sertoli cell pattern in a Japanese Black cow. 61
31448448 2019
5
[Ovarian sex cord tumor with annular tubules: report of a case]. 61
31422598 2019
6
RUNX3 Promotes the Tumorigenic Phenotype in KGN, a Human Granulosa Cell Tumor-Derived Cell Line. 61
31311113 2019
7
Impact of taxane plus bevacizumab for ovarian sex cord tumor with annular tubules. 61
31060113 2019
8
Mutational Landscape of Ovarian Adult Granulosa Cell Tumors from Whole Exome and Targeted TERT Promoter Sequencing. 61
30166312 2019
9
Ovarian Sex Cord-stromal Tumors With Melanin Pigment: Report of a Previously Undescribed Phenomenon. 61
29140884 2019
10
Extraovarian sex cord tumor with annular tubules discovered arising from a leiomyoma. 61
30148200 2018
11
Cytologic features of needle aspiration of ovarian sex cord tumor with annular tubules: Report of two cases and literature review. 61
29464881 2018
12
Prognostic significance of lymphadenectomyin malignant ovarian sex cord stromal tumor: A retrospective cohort study and meta-analysis. 61
29107349 2018
13
Pediatric imaging in DICER1 syndrome. 61
28474256 2017
14
[A very rare cause of peripheral precocious puberty in a girl: ovarian sex cord tumor with annular tubules]. 61
28504506 2017
15
Luteinizing hormone elevation in ovarian granulosa cell tumor: a case report and review of the literature. 61
28438188 2017
16
Histological changes of non-Peutz-Jegher syndrome associated ovarian sex cord tumor with annular tubules in childhood. 61
31966699 2017
17
Co-occurrence of a metastatic mammary liposarcoma and an ovarian sex-cord stromal tumor in a dog. 61
27892865 2016
18
A Rare Presentation of Paraovarian Sclerosing Stromal Tumor with High Mitotic Activity. 61
26327562 2016
19
Non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules: Report of a malignant case. 61
26807963 2016
20
Sclerosing Sromal Tumor of the Ovary in Postmenopausal Women: A Report of Two Cases. 61
26356607 2015
21
Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study. 61
25886261 2015
22
Clinicopathological significance of steroidogenic factor-1 expression in ovarian cancer versus ovarian sex cord stromal tumor. 61
25604140 2015
23
Ovarian sex cord tumor with annular tubules in children. 61
25879609 2014
24
Ovarian steroid cell tumor, not otherwise specified, producing testosterone. 61
25181629 2014
25
Recurrence of adult granulosa cell tumor of the ovary: experience at a tertiary care center. 61
24630126 2014
26
Metastatic ovarian sex-cord stromal tumor with annular tubules in a patient without Peutz-Jeghers syndrome. 61
24932834 2014
27
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature. 61
24439718 2014
28
Alfa-fetoprotein secreting ovarian sex cord-stromal tumor. 61
23924561 2013
29
Cancer problem in Peutz-Jeghers syndrome. 61
23977663 2013
30
A comprehensive analysis of PAX8 expression in human epithelial tumors. 61
21552115 2011
31
Laparoscopical management ovarian early sex cord-stromal tumors in postmenopausal women: a proposal method. 61
21116640 2011
32
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
33
Ovarian interstitial cell tumor in a South American sea lion (Otaria flavescens). 61
20688715 2010
34
Sex cord tumor with annular tubules in a young adolescent with Von Willebrand's disease. 61
19896399 2010
35
[Application of immunohistochemistry and its limitations in diagnosis of ovarian sex cord-stromal tumor]. 61
20388406 2010
36
Non-Peutz-Jeghers syndrome associated ovarian sex cord tumor with annular tubules: a case report. 61
19703688 2009
37
Sclerosing stromal tumor: an important differential diagnosis of ovarian neoplasms in childhood and adolescence. 61
19071970 2009
38
Mutation of FOXL2 in granulosa-cell tumors of the ovary. 61
19516027 2009
39
Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors. 61
19033865 2009
40
A rare cause of vaginal bleeding in a 7-month-old female infant. 61
18358265 2008
41
Ultrasound of ovarian sex-cord tumor with annular tubules. 61
17764009 2007
42
Bilateral sex cord tumor with annular tubules of ovary without Peutz-Jeghers syndrome: a case report. 61
17883091 2007
43
[Peutz-Jeghers syndrome complicated by cervical adenoma malignum and ovarian sex cord tumor with annular tubules: report of a case]. 61
17374267 2006
44
Peutz-Jeghers Syndrome with multiple genital tract tumors and breast cancer: a case report with a review of literatures. 61
16891826 2006
45
Chemotherapy-induced alterations in ovarian sex cord tumor with annular tubules (SCTAT): a diagnostic challenge. 61
16459027 2006
46
Ovarian sex cord-stromal tumor, unclassified: case report of endocrinologic findings and review of the literature. 61
16343200 2005
47
[Analysis of prognostic factors of malignant ovarian tumor after fertility-preserving treatment]. 61
16321304 2005
48
Histomorphologic assessment of formalin substitute fixatives for diagnostic surgical pathology. 61
15794674 2005
49
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
50
Colonic adenocarcinoma and bilateral malignant ovarian sex cord tumor with annular tubules in Peutz-Jeghers syndrome. 61
15524052 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

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 dendrite GO:0030425 8.92 MME GNAS DICER1 CALB2

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 9.77 WT1 SUZ12 JAZF1 FOXL2 DICER1
2 female gonad development GO:0008585 9.43 NR5A1 FOXL2
3 adrenal gland development GO:0030325 9.4 WT1 NR5A1
4 apoptotic DNA fragmentation GO:0006309 9.32 FOXL2 DICER1
5 production of miRNAs involved in gene silencing by miRNA GO:0035196 9.26 ZC3H7B DICER1
6 positive regulation of male gonad development GO:2000020 9.16 WT1 NR5A1
7 sex determination GO:0007530 8.96 WT1 NR5A1
8 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 9.28 ZC3H7B WT1 SUZ12 NR5A1 MME JAZF1

Sources for Ovarian Sex-Cord Stromal Tumor

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72 UMLS via Orphanet
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