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MCID: SRT003
MIFTS: 38

Sertoli-Leydig Cell Tumor

Categories: Cancer diseases, Rare diseases
Genes Tissues Related diseases Publications 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 43 15 71
Sertoli-Leydig Cell Tumor of Intermediate Differentiation 71
Sertoli-Leydig Cell Tumor of the Ovary 52
Sertoli-Leydig Cell Tumor of Ovary 71
Sertoli-Leydig Cell Tumors 52
Arrhenoblastoma of Ovary 52
Androblastoma of Ovary 52
Arrhenoblastoma 52

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
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External Ids:

Disease Ontology 12 DOID:2997
MeSH 43 D018310
UMLS 71 C0003810 C0206723 C1318541
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Summaries for Sertoli-Leydig Cell Tumor

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NIH Rare Diseases : 52 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 botryoid rhabdomyosarcoma and appendix adenocarcinoma, and has symptoms including hoarseness An important gene associated with Sertoli-Leydig Cell Tumor is CALB2 (Calbindin 2). The drugs Bevacizumab and Paclitaxel have been mentioned in the context of this disorder. Affiliated tissues include ovaries, ovary and testis, and related phenotypes are normal and 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 463)
# Related Disease Score Top Affiliating Genes
1 botryoid rhabdomyosarcoma 31.3 DICER1 AFP
2 appendix adenocarcinoma 31.1 SYP KRT7
3 cystadenocarcinoma 31.1 PGR MUC1 KRT7
4 endodermal sinus tumor 31.1 MIR302C KRT7 AFP
5 leydig cell tumor 31.1 SYP INSL3 INHA CYP17A1 CALB2 AFP
6 mucinous adenocarcinoma 31.0 PGR MUC1 KRT7
7 rete testis adenocarcinoma 30.8 KRT7 CALB2 AFP
8 carcinoid syndrome 30.8 SYP ENO2
9 gynandroblastoma 30.8 FOXL2 DICER1 CALB2
10 rhabdomyosarcoma 30.8 WT1 SYP ENO2 DICER1
11 desmoplastic small round cell tumor 30.7 WT1 MUC1
12 ovarian serous cystadenocarcinoma 30.7 SYP PGR MUC1 CALB2
13 serous cystadenocarcinoma 30.5 WT1 PGR KRT7
14 luteoma 30.4 FOXL2 CALB2
15 teratoma 30.4 SYP KRT7 ENO2 CALB2 AFP
16 germ cell cancer 30.4 MIR302C ENO2 AFP
17 ovarian brenner tumor 30.2 WT1 THBD KRT7
18 cystic teratoma 30.2 SYP KRT7 ENO2 CALB2 AFP
19 adrenal carcinoma 30.0 SYP ENO2 CYP17A1 CALB2
20 adenofibroma 29.9 WT1 MUC1 KRT7 INHA CALB2
21 cystadenoma 29.8 SYP PGR MUC1 KRT7 INHA CALB2
22 ovarian sex-cord stromal tumor 29.8 WT1 KRT7 FOXL2 DICER1 CALB2
23 granulosa cell tumor of the ovary 29.5 SYP KRT7 INHA FOXL2 ENO2
24 cystadenofibroma 29.4 WT1 MUC1 KRT7 INHA CALB2 AFP
25 nephroma 29.4 WT1 U2AF1 PGR MUC1 DICER1
26 endometrial cancer 29.1 WT1 PGR MUC1 KRT7 INHA CYP17A1
27 sertoli cell tumor 28.7 WT1 SYP PGR MUC1 KRT7 INHA
28 sex cord-gonadal stromal tumor 28.3 WT1 PGR MUC1 KRT7 INHA FOXL2
29 ovarian cancer 28.1 WT1 PGR MUC1 MIR302C KRT7 INHA
30 dysgerminoma 28.0 WT1 MUC1 MIR302C KRT7 INHA FOXL2
31 goiter, multinodular 1, with or without sertoli-leydig cell tumors 13.2
32 malignant sertoli-leydig cell tumor of the ovary 12.8
33 dicer1 tumor predisposition 12.2
34 amenorrhea 10.8
35 rare tumor 10.7
36 multinodular goiter 10.6
37 hyperandrogenism 10.6
38 goiter 10.6
39 dermoid cyst of ovary 10.5 CALB2 AFP
40 peritoneal benign neoplasm 10.5 PGR CALB2
41 androgen insensitivity syndrome 10.5
42 pleuropulmonary blastoma 10.5
43 embryoma 10.5
44 cervical clear cell adenocarcinoma 10.5 PGR AFP
45 bartholin's gland adenoid cystic carcinoma 10.5 PGR KRT7
46 intrahepatic bile duct adenoma 10.5 KRT7 AFP
47 nephrogenic adenoma 10.5 MUC1 KRT7
48 urethral villous adenoma 10.5 PGR KRT7
49 chronic tympanitis 10.5 PGR MUC1
50 intrahepatic biliary papillomatosis 10.5 MUC1 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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 normal MP:0002873 9.56 CALB2 DICER1 FOXL2 INHA PGR SYP
2 reproductive system MP:0005389 9.28 AFP CYP17A1 DICER1 FOXL2 INHA INSL3
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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
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
3
Cisplatin Approved Phase 2 15663-27-1 2767 441203 84093
4
Bleomycin Approved, Investigational Phase 2 11056-06-7 5360373
5
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
6
Etoposide Approved Phase 2 33419-42-0 36462
7 Immunoglobulins Phase 2
8 Mitogens Phase 2
9 Antibodies Phase 2
10 Immunologic Factors Phase 2
11 Endothelial Growth Factors Phase 2
12 Antibodies, Monoclonal Phase 2
13 Immunoglobulins, Intravenous Phase 2
14 Angiogenesis Inhibitors Phase 2
15 Immunoglobulin G Phase 2
16 Antineoplastic Agents, Immunological Phase 2
17 Albumin-Bound Paclitaxel Phase 2
18 Tubulin Modulators Phase 2
19 Antimitotic Agents Phase 2
20 Etoposide phosphate Phase 2
21 Anti-Bacterial Agents Phase 2
22 Antibiotics, Antitubercular Phase 2
23
Vincristine Approved, Investigational 57-22-7, 2068-78-2 5978
24
Doxorubicin Approved, Investigational 23214-92-8 31703
25
Dactinomycin Approved, Investigational 50-76-0 457193 2019
26
Ifosfamide Approved 3778-73-2 3690
27
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
28
Isophosphamide mustard 0
29
Liposomal doxorubicin 31703

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 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, Lung, Thyroid, Testes, Lymph Node
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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 408)
# 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. 54 61
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. 54 61
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. 61 54
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. 61 54
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]. 61 54
1698212 1990
14
Ovarian Sertoli-Leydig cell tumors: an analysis of 13 cases. 61
32430758 2020
15
Ovarian Sertoli-Leydig and granulosa cell tumor: comparison of epidemiology and survival outcomes. 61
32519016 2020
16
Clinical Characteristics and Mutation Analysis of Ovarian Sertoli-Leydig Cell Tumors. 61
32557933 2020
17
[Ovarian Sertoli-Leydig cell tumors: DICER1 hotspot mutations and associated clinicopathological features]. 61
32392927 2020
18
Pleuropulmonary blastoma-like peritoneal sarcoma: a newly described malignancy associated with biallelic DICER1 pathogenic variation. 61
32415267 2020
19
[The pathology of hereditary ovarian tumors]. 61
32178889 2020
20
An update on the central nervous system manifestations of DICER1 syndrome. 61
30953130 2020
21
Postmenopausal mild hirsutism and hyperandrogenemia due to ovarian Sertoli-Leydig cell tumor: A case report. 61
32280807 2020
22
Gynecologic and reproductive health in patients with pathogenic germline variants in DICER1. 61
31952842 2020
23
Ovarian tumors: a survey of selected advances of note during the life of this journal. 61
31654691 2020
24
NKX3.1 and Prostein Expression in Testicular Tissue and Sex Cord-stromal Tumors. 61
31498176 2020
25
Targeted Genomic Profiling of Female Adnexal Tumors of Probable Wolffian Origin (FATWO). 61
30134342 2019
26
Imaging of DICER1 syndrome. 61
31620849 2019
27
Ovarian Sertoli-Leydig Cell Tumor with Estrogenic Manifestations in a Postmenopausal Lady: a Case Report. 61
31496599 2019
28
The clinical features and reproductive prognosis of ovarian neoplasms with hyperandrogenemia: a retrospective analysis of 33 cases. 61
30990090 2019
29
Ovarian Sertoli-Leydig Cell Tumors: Epidemiological, Clinical and Prognostic Factors. 61
31344718 2019
30
Sertoli-Leydig cell tumor of the ovary: Analysis of a single institution database and review of the literature. 61
31106943 2019
31
Intraoperative Scrape Cytology from Ovarian Masss Lesions: A Study of 81 Cases. 61
31359919 2019
32
Pleuropulmonary Blastoma: More Than a Lung Neoplasm of Childhood. 61
31527943 2019
33
Ovarian Sertoli-Leydig Cell Tumor With Heterologous Hepatocytes and a Hepatocellular Carcinomatous Element. 61
29620585 2019
34
DICER1 and FOXL2 Mutation Status Correlates With Clinicopathologic Features in Ovarian Sertoli-Leydig Cell Tumors. 61
30986800 2019
35
Results from a Monocentric Long-Term Analysis of 23 Patients with Ovarian Sertoli-Leydig Cell Tumors. 61
30201740 2019
36
Mesenteric extraovarian Sertoli-Leydig cell tumor without DICER1 hotspot mutation: a case report. 61
30935425 2019
37
Role of adjuvant chemotherapy in the management of non-granulosa cell ovarian sex cord-stromal tumors. 61
30740951 2019
38
[Sex cord-stromal tumors of the ovary : Current aspects with a focus on granulosa cell tumors, Sertoli-Leydig cell tumors, and gynandroblastomas]. 61
30659330 2019
39
Malignant ascites occurs most often in patients with high-grade serous papillary ovarian cancer at initial diagnosis: a retrospective analysis of 191 women treated at Bayreuth Hospital, 2006-2015. 61
30415435 2019
40
Ovarian Sertoli-Leydig cell tumour with α-fetoprotein-producing intestinal glandular cells. Clinical case and short review of basic literature. 61
31820869 2019
41
Androgen-Secreting Ovarian Tumors. 61
31499493 2019
42
DICER1 Syndrome. 61
31409088 2019
43
Identification of two 14q32 deletions involving DICER1 associated with the development of DICER1-related tumors. 61
29698806 2019
44
Genetic Causes of Rare Pediatric Ovarian Tumors. 61
31409083 2019
45
Incidental Gynecologic Tract Neoplasms in Women Undergoing Anterior Pelvic Exenteration for Urothelial Carcinoma. 61
29257039 2019
46
Sertoli-Leydig Cell Tumor of Ovary: A Rare Case Report with Heterologous Elements and Focal Marked Anaplasia. 61
30820423 2019
47
Virilising ovarian tumors: a single-center experience. 61
30400027 2018
48
Ovarian Sex Cord-Stromal Tumors: Reflections on a 40-Year Experience With a Fascinating Group of Tumors, Including Comments on the Seminal Observations of Robert E. Scully, MD. 61
30500284 2018
49
Sertoli-Leydig cell tumors of ovary: A case series. 61
30334998 2018
50
Ovarian Gynandroblastoma with a Juvenile Granulosa Cell Tumor Component in a Postmenopausal Woman: A Case Report and Literature Review. 61
30011982 2018
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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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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 apoptotic DNA fragmentation GO:0006309 9.26 FOXL2 DICER1
2 progesterone metabolic process GO:0042448 9.16 CYP17A1 AFP
3 ovulation from ovarian follicle GO:0001542 8.96 PGR AFP
4 positive regulation of follicle-stimulating hormone secretion GO:0046881 8.62 INHA FOXL2
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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
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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