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MCID: SRT003
MIFTS: 38

Sertoli-Leydig Cell Tumor

Categories: Cancer diseases, Rare diseases, Reproductive diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Sertoli-Leydig Cell Tumor

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MalaCards integrated aliases for Sertoli-Leydig Cell Tumor:

Name: Sertoli-Leydig Cell Tumor 12 54 44 15 71
Sertoli-Leydig Cell Tumor of Intermediate Differentiation 71
Sertoli-Leydig Cell Tumor of the Ovary 20
Sertoli-Leydig Cell Tumor of Ovary 71
Sertoli-Leydig Cell Tumors 20
Arrhenoblastoma of Ovary 20
Androblastoma of Ovary 20
Arrhenoblastoma 20

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Reproductive diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:2997
MeSH 44 D018310
SNOMED-CT 67 34110004
UMLS 71 C0003810 C0206723 C1318541
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Summaries for Sertoli-Leydig Cell Tumor

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GARD : 20 Sertoli-Leydig cell tumors are a cancer that starts in the female ovaries. The cancer cells produce and release a male sex hormone which may cause the development of male physical characteristics (virilization), including facial hair and a deep voice. This type of tumor is sometimes called arrhenoblastoma of the ovary or a stromal tumor. Sertoli-Leydig cell tumors are rare tumors which account for less than 0.5% of all ovarian tumors. While they can be found in women of all age groups, they are most common in young women. Treatment involves surgery and chemotherapy or radiation if the cancer has spread. Because Sertoli-Leydig cell tumors have a low chance of spreading, if the tumor is found early, chances for survival are good.

MalaCards based summary : Sertoli-Leydig Cell Tumor, also known as sertoli-leydig cell tumor of intermediate differentiation, is related to gynandroblastoma and botryoid rhabdomyosarcoma, and has symptoms including hoarseness An important gene associated with Sertoli-Leydig Cell Tumor is CALB2 (Calbindin 2), and among its related pathways/superpathways is Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Bevacizumab and Etoposide have been mentioned in the context of this disorder. Affiliated tissues include ovaries, ovary and testis, and related phenotype is reproductive system.

Disease Ontology : 12 A sex cord-gonadal stromal tumor characterized by variable proportions of Sertoli cells, Leydig cells, primitive gonadal stroma and/or heterologous elements located in the ovaries and testicles.

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Related Diseases for Sertoli-Leydig Cell Tumor

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Diseases related to Sertoli-Leydig Cell Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 481)
# Related Disease Score Top Affiliating Genes
1 gynandroblastoma 31.0 DICER1 CALB2
2 botryoid rhabdomyosarcoma 31.0 DICER1 AFP
3 appendix adenocarcinoma 30.9 SYP KRT7
4 cystadenocarcinoma 30.9 PGR MUC1 KRT7
5 leydig cell tumor 30.8 SYP INSL3 INHA CYP17A1 CALB2 AFP
6 mucinous adenocarcinoma 30.8 PGR MUC1 KRT7
7 androgen insensitivity syndrome 30.7 MUC1 INSL3 CYP17A1
8 rete testis adenocarcinoma 30.6 KRT7 CALB2 AFP
9 carcinoid syndrome 30.6 SYP ENO2
10 rhabdomyosarcoma 30.4 WT1 SYP KRT7 ENO2 DICER1
11 teratoma 30.3 SYP KRT7 ENO2 CALB2 AFP
12 cystic teratoma 30.3 SYP KRT7 ENO2 CALB2
13 female reproductive endometrioid cancer 30.3 WT1 PGR KRT7
14 basal cell carcinoma 30.2 SYP MUC1 KRT7 ENO2
15 ovarian sex-cord stromal tumor 30.1 WT1 KRT7 DICER1 CALB2
16 serous cystadenocarcinoma 30.1 WT1 PGR ALPP
17 nephroma 30.1 WT1 PGR MUC1 DICER1
18 germ cell cancer 30.0 MIR302C ENO2 ALPP AFP
19 endodermal sinus tumor 29.9 MIR302C KRT7 INHA ALPP AFP
20 granulosa cell tumor of the ovary 29.8 SYP KRT7 INHA ENO2
21 ovarian brenner tumor 29.7 WT1 THBD KRT7
22 juvenile type testicular granulosa cell tumor 29.7 WT1 MIR302C DICER1 CALB2 AFP
23 adrenal carcinoma 29.6 SYP ENO2 CYP17A1 CALB2
24 adenofibroma 29.6 WT1 MUC1 KRT7 INHA CALB2
25 cystadenoma 29.6 SYP PGR MUC1 KRT7 INHA CALB2
26 cystadenofibroma 29.4 WT1 MUC1 KRT7 INHA CALB2
27 desmoplastic small round cell tumor 29.2 WT1 THBD SYP MUC1 ENO2 ALPP
28 endometrial cancer 28.9 WT1 PGR MUC1 KRT7 INHA CYP17A1
29 dysgerminoma 28.6 WT1 MIR302C KRT7 INHA ENO2 ALPP
30 sex cord-gonadal stromal tumor 28.4 WT1 PGR MUC1 KRT7 INHA DICER1
31 sertoli cell tumor 28.3 WT1 SYP PGR MUC1 KRT7 INHA
32 goiter, multinodular 1, with or without sertoli-leydig cell tumors 12.0
33 malignant sertoli-leydig cell tumor of the ovary 11.7
34 amenorrhea 10.7
35 rare tumor 10.6
36 hyperandrogenism 10.4
37 dicer1 tumor predisposition 10.4
38 dermoid cyst of ovary 10.4 CALB2 AFP
39 spermatocele 10.4 CALB2 AFP
40 renal pelvis adenocarcinoma 10.4 KRT7 AFP
41 infected hydrocele 10.4 CALB2 AFP
42 nephrogenic adenoma 10.4 MUC1 KRT7
43 cervical clear cell adenocarcinoma 10.4 PGR AFP
44 bartholin's gland adenoid cystic carcinoma 10.4 PGR KRT7
45 urethral villous adenoma 10.4 PGR KRT7
46 ovarian primitive germ cell tumor 10.4 KRT7 AFP
47 ovarian endodermal sinus tumor 10.4 KRT7 AFP
48 spiradenoma 10.4 MUC1 KRT7
49 intrahepatic biliary papillomatosis 10.4 MUC1 AFP
50 intratubular embryonal carcinoma 10.4 KRT7 AFP

Graphical network of the top 20 diseases related to Sertoli-Leydig Cell Tumor:



Diseases related to Sertoli-Leydig Cell Tumor
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Symptoms & Phenotypes for Sertoli-Leydig Cell Tumor

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UMLS symptoms related to Sertoli-Leydig Cell Tumor:


hoarseness

MGI Mouse Phenotypes related to Sertoli-Leydig Cell Tumor:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 reproductive system MP:0005389 9.23 AFP CYP17A1 DICER1 INHA INSL3 PGR
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Drugs & Therapeutics for Sertoli-Leydig Cell Tumor

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Drugs for Sertoli-Leydig Cell Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 29)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bevacizumab Approved, Investigational Phase 2 216974-75-3
2
Etoposide Approved Phase 2 33419-42-0 36462
3
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
4
Bleomycin Approved, Investigational Phase 2 11056-06-7 5360373
5
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
6
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
7 Antineoplastic Agents, Immunological Phase 2
8 Immunoglobulin G Phase 2
9 Angiogenesis Inhibitors Phase 2
10 Immunoglobulins Phase 2
11 Mitogens Phase 2
12 Immunologic Factors Phase 2
13 Immunoglobulins, Intravenous Phase 2
14 Antibodies Phase 2
15 Antibodies, Monoclonal Phase 2
16 Endothelial Growth Factors Phase 2
17 Antibiotics, Antitubercular Phase 2
18 Etoposide phosphate Phase 2
19 Albumin-Bound Paclitaxel Phase 2
20 Antimitotic Agents Phase 2
21 Tubulin Modulators Phase 2
22 Anti-Bacterial Agents Phase 2
23
Vincristine Approved, Investigational 2068-78-2, 57-22-7 5978
24
Doxorubicin Approved, Investigational 23214-92-8 31703
25
Dactinomycin Approved, Investigational 50-76-0 2019 457193
26
Ifosfamide Approved 3778-73-2 3690
27
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
28
Liposomal doxorubicin 31703
29
Isophosphamide mustard 100427

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Study of Paclitaxel for Ovarian Stromal Tumors as Second-Line Therapy Completed NCT00006227 Phase 2 Paclitaxel
2 A Phase II Trial of NCI-Supplied Agent: Bevacizumab (rhuMAB VEGF) (NSC# 704865) for Recurrent Sex Cord-Stromal Tumors of the Ovary Completed NCT00748657 Phase 2
3 A Randomized Phase II Trial of Paclitaxel and Carboplatin vs. Bleomycin, Etoposide, and Cisplatin for Newly Diagnosed Advanced Stage and Recurrent Chemonaive Sex Cord-Stromal Tumors of the Ovary Active, not recruiting NCT01042522 Phase 2 Carboplatin;Cisplatin;Etoposide Phosphate;Paclitaxel
4 DICER1-Related Pleuropulmonary Blastoma Cancer Predisposition Syndrome: A Natural History Study Recruiting NCT01247597
5 International Pleuropulmonary Blastoma Registry for PPB, DICER1 and Associated Conditions Recruiting NCT03382158
6 Elucidating the Genetic Basis of the Pleuropulmonary Blastoma (PPB) Familial Cancer Syndrome Active, not recruiting NCT00565903

Search NIH Clinical Center for Sertoli-Leydig Cell Tumor

Cochrane evidence based reviews: sertoli-leydig cell tumor

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Genetic Tests for Sertoli-Leydig Cell Tumor

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Anatomical Context for Sertoli-Leydig Cell Tumor

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The Foundational Model of Anatomy Ontology organs/tissues related to Sertoli-Leydig Cell Tumor:

19
Ovaries

MalaCards organs/tissues related to Sertoli-Leydig Cell Tumor:

40
Ovary, Testis, Endothelial, Thyroid, Skeletal Muscle, Uterus, Cervix
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Publications for Sertoli-Leydig Cell Tumor

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Articles related to Sertoli-Leydig Cell Tumor:

(show top 50) (show all 424)
# Title Authors PMID Year
1
7,12-dimethylbenz[a]anthracene induces sertoli-leydig-cell tumors in the follicle-depleted ovaries of mice treated with 4-vinylcyclohexene diepoxide. 54 61
20158943 2010
2
Ovarian involvement by desmoplastic small round cell tumor with leydig cell hyperplasia showing an unusual immunophenotype (cytokeratin negative, calretinin and inhibin positive) mimicking poorly differentiated sertoli leydig cell tumor. 61 54
19851210 2009
3
Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors. 61 54
19033865 2009
4
Estrogen receptor alpha and progesterone receptor expression in ovarian adult granulosa cell tumors and Sertoli-Leydig cell tumors. 61 54
17885486 2007
5
The utility of calretinin, inhibin, and WT1 immunohistochemical staining in the differential diagnosis of ovarian tumors. 61 54
15754297 2005
6
Inhibin immunohistochemical staining: a practical approach for the surgical pathologist in the diagnoses of ovarian sex cord-stromal tumors. 61 54
12502966 2003
7
Calretinin, a more sensitive but less specific marker than alpha-inhibin for ovarian sex cord-stromal neoplasms: an immunohistochemical study of 215 cases. 61 54
12409724 2002
8
Expression of calretinin in human ovary, testis, and ovarian sex cord-stromal tumors. 54 61
11603218 2001
9
Ovarian endometrioid carcinomas simulating sex cord-stromal tumors: a study using inhibin and cytokeratin 7. 54 61
9656124 1998
10
Inhibin and epithelial membrane antigen immunohistochemistry assist in the diagnosis of sex cord-stromal tumors and provide clues to the histogenesis of hypercalcemic small cell carcinomas. 54 61
9475192 1998
11
Analysis of WT1 in granulosa cell and other sex cord-stromal tumors. 61 54
8504409 1993
12
Sertoli-Leydig cell tumor of the ovary: a rare cause of amenorrhea. 54 61
1565381 1992
13
[A case of ovarian Sertoli-Leydig cell tumor associated with high serum alpha-fetoprotein (AFP) concentration]. 54 61
1698212 1990
14
Sex Cord Stromal Tumors in Children and Adolescents: A First Report by The South African Children's Cancer Study Group (1990-2015). 61
33560080 2021
15
Rare DICER1 and Absent FOXL2 Mutations Characterize Ovarian Juvenile Granulosa Cell Tumors. 61
32910017 2021
16
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
17
Inhabitual presentation of Sertoli-Leydig cell tumor of the ovary with xeroderma pigmentosum: Case report with review of literature. 61
33383283 2021
18
Sertoli-Leydig Cell Tumors of the Ovary With Follicular Differentiation Often Resembling Juvenile Granulosa Cell Tumor: A Report of 38 Cases Including Comments on Sex Cord-Stromal Tumors of Mixed Forms (So-called Gynandroblastoma). 61
32815828 2021
19
Mixed granulosa-Sertoli sex cord-stromal tumor of the testis: clinical, ultrasonic and histopathological features: a case report. 61
33457255 2020
20
Granulosa and theca cell tumor in rat's ovary after modified Biskind operation. 61
33355865 2020
21
Krukenberg Tumor of Ovary During Pregnancy: Learning From A Mistake. 61
33252404 2020
22
Sertoli-Leydig cell tumor of the ovary: Radiologic-operative-histologic finding. 61
33218429 2020
23
Cytoreduction with hyperthermic intra peritoneal and intra thoracic chemotherapy for metastatic Sertoli-Leydig cell tumor of the ovary. 61
32995452 2020
24
Pleuropulmonary blastoma-like peritoneal sarcoma: a newly described malignancy associated with biallelic DICER1 pathogenic variation. 61
32415267 2020
25
Pediatric ovarian Sertoli-Leydig cell tumors with heterologous rhabdomyosarcoma elements: Clinical case series and review of the literature. 61
32729194 2020
26
Clinical Characteristics and Mutation Analyses of Ovarian Sertoli-Leydig Cell Tumors. 61
32557933 2020
27
Poorly Differentiated Ovarian Sertoli-Leydig Cell Tumor in a 12-Month-Old Girl. 61
33343121 2020
28
Metachronous Sporadic Sextuple Primary Malignancies Including Bilateral Breast Cancers. 61
32908793 2020
29
[Application of molecular analysis in differential diagnosis of ovarian adult granulosa cell tumors]. 61
32746545 2020
30
Ovarian Sertoli-Leydig and granulosa cell tumor: comparison of epidemiology and survival outcomes. 61
32519016 2020
31
Ovarian Sertoli-Leydig cell tumors: an analysis of 13 cases. 61
32430758 2020
32
Sertoli-Leydig cell tumor in two siblings with DICER1 syndrome: A case report and literature review. 61
32629665 2020
33
Ovarian Gynandroblastoma with a Juvenile Granulosa Cell Tumor Component in a Postmenopausal Woman. 61
32751726 2020
34
[Ovarian Sertoli-Leydig cell tumors: DICER1 hotspot mutations and associated clinicopathological features]. 61
32392927 2020
35
Postmenopausal mild hirsutism and hyperandrogenemia due to ovarian Sertoli-Leydig cell tumor: A case report. 61
32280807 2020
36
An update on the central nervous system manifestations of DICER1 syndrome. 61
30953130 2020
37
[The pathology of hereditary ovarian tumors]. 61
32178889 2020
38
Gynecologic and reproductive health in patients with pathogenic germline variants in DICER1. 61
31952842 2020
39
DICER1 Syndrome and Cancer Predisposition: From a Rare Pediatric Tumor to Lifetime Risk. 61
33552988 2020
40
Ovarian tumors: a survey of selected advances of note during the life of this journal. 61
31654691 2020
41
NKX3.1 and Prostein Expression in Testicular Tissue and Sex Cord-stromal Tumors. 61
31498176 2020
42
Targeted Genomic Profiling of Female Adnexal Tumors of Probable Wolffian Origin (FATWO). 61
30134342 2019
43
Imaging of DICER1 syndrome. 61
31620849 2019
44
Ovarian Sertoli-Leydig Cell Tumor with Estrogenic Manifestations in a Postmenopausal Lady: a Case Report. 61
31496599 2019
45
The clinical features and reproductive prognosis of ovarian neoplasms with hyperandrogenemia: a retrospective analysis of 33 cases. 61
30990090 2019
46
Ovarian Sertoli-Leydig Cell Tumors: Epidemiological, Clinical and Prognostic Factors. 61
31344718 2019
47
Sertoli-Leydig cell tumor of the ovary: Analysis of a single institution database and review of the literature. 61
31106943 2019
48
Intraoperative Scrape Cytology from Ovarian Masss Lesions: A Study of 81 Cases. 61
31359919 2019
49
Pleuropulmonary Blastoma: More Than a Lung Neoplasm of Childhood. 61
31527943 2019
50
Ovarian Sertoli-Leydig Cell Tumor With Heterologous Hepatocytes and a Hepatocellular Carcinomatous Element. 61
29620585 2019
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Variations for Sertoli-Leydig Cell Tumor

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Expression for Sertoli-Leydig Cell Tumor

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Search GEO for disease gene expression data for Sertoli-Leydig Cell Tumor.
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Pathways for Sertoli-Leydig Cell Tumor

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Pathways related to Sertoli-Leydig Cell Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers 64
10.92 WT1 ALPP AFP
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GO Terms for Sertoli-Leydig Cell Tumor

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Biological processes related to Sertoli-Leydig Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 progesterone metabolic process GO:0042448 8.96 CYP17A1 AFP
2 ovulation from ovarian follicle GO:0001542 8.62 PGR AFP
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Sources for Sertoli-Leydig Cell Tumor

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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
39 LOVD
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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