Sertoli-Leydig Cell Tumor

A sex cord-gonadal stromal tumor consists of LEYDIG CELLS; SERTOLI CELLS; and FIBROBLASTS in varying proportions and degree of differentiation. Most such tumors produce ANDROGENS in the Leydig cells, formerly known as androblastoma or arrhenoblastoma. Androblastomas occur in the TESTIS or the OVARY causing precocious masculinization in the males, and defeminization, or virilization (VIRILISM) in the females. In some cases, the Sertoli cells produce ESTROGENS.

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Networked: 172 relevant articles (2 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Sertoli-Leydig Cell Tumor
2.	Neoplasms (Cancer)
3.	Granulosa Cell Tumor
4.	Sex Cord-Gonadal Stromal Tumors (Sex Cord-Stromal Tumor)
5.	Thecoma (Thecomas)

Experts

1.	McCluggage, W Glenn: 5 articles (11/2019 - 04/2007)
2.	Gilks, Blake: 3 articles (07/2018 - 01/2012)
3.	Young, Robert H: 3 articles (01/2012 - 04/2007)
4.	Yu, Qi: 2 articles (09/2019 - 11/2012)
5.	Foulkes, William D: 2 articles (07/2016 - 01/2012)
6.	Devouassoux-Shisheboran, Mojgan: 2 articles (05/2016 - 08/2006)
7.	Mauduit, Claire: 2 articles (05/2016 - 08/2006)
8.	Anglesio, Michael S: 2 articles (08/2015 - 01/2012)
9.	Huntsman, David G: 2 articles (08/2015 - 01/2012)
10.	Morin, Gregg B: 2 articles (08/2015 - 01/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sertoli-Leydig Cell Tumor:

1.	Vincristine (Oncovin)FDA LinkGeneric 06/01/1976 - "Vincristine, actinomycin-D, and cyclophosphamide were effective in Sertoli-Leydig--cell tumors." 01/01/1987 - "Cytosol steroid receptor contents of 2 tubular androblastomas were determined and that was correlated with the response to hormonal (tamoxifen and medroxyprogesterone acetate) and cytotoxic agents (chlorambucil, vincristine, cisplatin and etoposide) using a new in vitro method based on ATP measurement of cultured cells. "
2.	MicroRNAs (MicroRNA)IBA 08/01/2015 - "Here, we further revealed that the global expression of 5p-derived miRNAs was dramatically reduced in ovarian Sertoli-Leydig cell tumors carrying DICER1 hotspot mutations compared with those without DICER1 hotspot mutation. "
3.	Dactinomycin (Actinomycin)FDA LinkGeneric 06/01/1976 - "Vincristine, actinomycin-D, and cyclophosphamide were effective in Sertoli-Leydig--cell tumors."
4.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 06/01/1976 - "Vincristine, actinomycin-D, and cyclophosphamide were effective in Sertoli-Leydig--cell tumors."
5.	Testosterone (Sustanon)FDA Link 10/01/1986 - "Characterization of gonadotrophin receptors in a testosterone-producing ovarian androblastoma. " 05/01/1987 - "These results of in vitro and in vivo studies suggest that this tumor was an hCG-dependent testosterone producing Sertoli-Leydig cell tumor." 09/01/1996 - "A 33-year-old woman with abrupt cessation of menses, progressive hirsutism, and weight gain because of a unilateral ovarian mass identified sonographically and an increased serum testosterone level underwent laparotomy, which disclosed a left ovarian Sertoli-Leydig cell tumor. " 11/22/2020 - "Here in, we report a case of aprimary ovarian Sertoli-Leydig cell tumor in a 21-year-old student, previously diagnosed to have polycystic ovarian syndrome and subsequently congenital adrenal hyperplasia, who presented with a large abdominal mass and features of virilization along with elevated serum testosterone levels. " 07/01/1996 - "We report a case of a 34-year-old woman affected with ovarian arrhenoblastoma characterized by very high testosterone (T) levels (34.0-60.0 ng/ml; n.v.0.2-0.9) and suppressed gonadotropin levels. "
6.	SteroidsIBA 01/01/1984 - "Steroid biosynthesis in the Sertoli-Leydig cell tumor tissue of the human ovary; enzymhistochemical and immunohistochemical studies." 01/01/1995 - "Immunohistochemical and biochemical analysis of a human Sertoli-Leydig cell tumor: autonomous steroid production characteristic of ovarian theca cells." 02/01/1994 - "Analysis for steroid production of a Sertoli-Leydig cell tumor." 01/01/1979 - "The ovarian-peripheral gradients of various delta4 and delta5 steroids were determined for a patient with virilizing arrhenoblastoma. " 09/01/1974 - "Steroid metabolism in arrhenoblastoma."
7.	alpha-Fetoproteins (alpha-Fetoprotein)IBA 01/01/1989 - "Frequency of alpha-fetoprotein production by Sertoli-Leydig cell tumors of the ovary: an immunohistochemical study of eight cases." 01/01/2022 - "15-Year-Old Patient with an Unusual Alpha-Fetoprotein-Producing Sertoli-Leydig Cell Tumor of Ovary." 05/01/2018 - "A Report of Ovarian Sertoli-Leydig Cell Tumors With Heterologous Intestinal-type Glands and Alpha Fetoprotein Elevation and Review of the Literature." 07/01/2016 - "Ovarian Sertoli-Leydig cell tumor with heterologous elements containing an unusual type of alpha-fetoprotein-producing cells." 11/01/2014 - "Elevated serum levels of alpha-fetoprotein are rarely associated with Sertoli-Leydig cell tumors, with only approximately 30 such cases previously reported in the literature. "
8.	Inhibins (Inhibin)IBA 07/01/1998 - "Among nine Sertoli-Leydig cell tumors with available clinical data, four that were more than minimally inhibin-positive were accompanied by androgenic manifestations; five inhibin-negative or only minimally positive tumors lacked such evidence. " 01/01/2004 - "Remarkably, all 10 investigated inhibin-expressing granulosa cell tumors, Leydig cell tumors, and Sertoli-Leydig cell tumors expressed NPY receptors. " 01/01/1998 - "Thirty-two (97%) of 33 granulosa cell tumors (GCTs), 10 (91%) of 11 Sertoli-Leydig cell tumors (SLCTs), and 4 (8%) of 51 carcinomas showed inhibin alpha immunopositivity. " 09/01/2008 - "Immunostaining for inhibin was positive in 66% of granulosa cell tumor, in 50% of Sertoli-Leydig cell tumor and in 54% of thecoma-fibroma group. " 02/01/2005 - "Inhibin was expressed in 60% of granulosa cell tumors, 71% of Sertoli-Leydig cell tumors, 43% of fibromas, and 33% of thecomas. "
9.	EnzymesIBA 05/01/1974 - "It has been shown in cases of arrhenoblastoma that some of the anomalies of steroidogenesis are secondary to the enzyme disorders, the impression being that, with this kind of tumour, the tumour parenchyma begins to secrete like a normal testicle." 03/01/1967 - "Enzyme histochemistry of a masculinizing arrhenoblastoma." 06/01/1982 - "These data suggest that 1) androgens from the androblastoma are responsible for virilization despite aromatizing enzyme activities within normal limits, and 2) both the delta 5 and delta 4 pathways are involved in the biosynthesis of androgens, with that of delta 5 being predominant." 01/01/1983 - "Androblastoma: ultrastructure, enzyme histochemistry and immunoenzymatic detection of major steroid hormones (light and electron microscopy)." 01/01/1985 - "Demonstration of hydroxysteroid dehydrogenases and testosterone in the Sertoli-Leydig cell tumor (androblastoma) tissue of the human ovary: an enzyme histochemical and immunohistochemical study."
10.	Androstenedione (4 Androstene 3,17 dione)IBA 07/01/1975 - "A metabolic study with tissue from a virilizing arrhenoblastoma, using as precursors [7-3H]pregnenolone, [7-3H]17alpha-hydroxypregnenolone,[4-14C]17alpha-hydroxyprogesterone and [4-14C]testosterone, revealed that in spite of a deficient activity of 3beta-hydroxysteroid dehydrogenase-5-isomerase the overall production of testosterone was compensated by an increased activity of a lyase converting [4-14C]17alpha-hydroxyprogesterone to testosterone (via androstenedione) and was comparable to the production obtained by normal ovarian tissue. " 07/01/1988 - "The results are consistent with a Sertoli-Leydig cell tumor producing mainly testosterone and, to a lesser degree, androstenedione, progesterone, estrone and 17 alpha-hydroxyprogesterone. " 12/01/1989 - "We conclude that (1) increased testosterone secretion by Sertoli-Leydig cell tumor resulted from increased availability of precursors from both delta 4 and delta 5 pathways; (2) the tumor was responsive to luteinizing hormone with an increase in the secretion of androstenedione and dehydroepiandrosterone; (3) insulin acts synergistically with luteinizing hormone to increase secretion of androstenedione; (4) the tumor has specific binding sites for insulin; and (5) the increased levels of insulin and luteinizing hormone in polycystic ovarian disease may play a role in the pathogenesis of Sertoli-Leydig cell tumor." 12/01/2022 - "We measured testosterone and its precursors (dehydroepiandrosterone-sulfate and androstenedione) and 11-oxygenated androgens (11β-hydroxyandrostenedione (11-OHA4) and 11-ketotestosterone (11-KT)) in the periphery, adrenal and ovarian veins in four different cases of hyperandrogenism in females (polycystic ovary syndrome (PCOS), primary bilateral macronodular adrenal hyperplasia, Sertoli-Leydig cell tumor and ovarian steroid cell tumor). " 02/01/1994 - "The authors present a case of a poorly differentiated Sertoli-Leydig cell tumor in which ascitic fluid was found to have a high concentration of androgenic hormones and precursors from the delta steroid pathway (17-OH progesterone, testosterone, and androstenedione). "

Therapies and Procedures

1.	Adjuvant Chemotherapy 01/01/2014 - "The present study was to evaluate clinicalopahologic features and prognosis of patients with Sertoli-Leydig cell tumor treated by surgery and adjuvant chemotherapy during short term follow-up. " 11/22/2020 - "Fertility sparing unilateral salpingo-oophorectomy was done and adjuvant chemotherapy was given after histopathology showed moderate to poorly differentiated Sertoli-Leydig cell tumor. " 10/31/2023 - "Four patients received adjuvant chemotherapy, three because of advanced tumor stage, and one because of a poorly differentiated Sertoli-Leydig cell tumor. " 05/01/2004 - "One patient whose tumor was a poorly differentiated Sertoli-Leydig cell tumor with mesenchymal heterologous elements received adjuvant chemotherapy postoperatively but died of disease 2.5 years after surgery. "
2.	Salpingo-oophorectomy 08/01/2023 - "•This is the first report of a germline DICER1-associated Sertoli-Leydig cell tumor (SLCT) diagnosed in pregnancy.•SLCT is linked to DICER1 pathogenic variants, but little is known about management of DICER1-associated SLCT.•There is an extended risk for metachronous SLCT in patients with germline DICER1 pathogenic variants who retain an ovary.•Prophylactic contralateral salpingo-oophorectomy may be offered with shared decision making to patients with inherited SLCT.•Genetic testing for DICER1 should be offered to all patients with moderately or poorly differentiated SLCT." 07/01/2023 - "Right salpingo-oophorectomy was performed 1 year later, at which time the specimen showed a different growth pattern involving epithelioid cells and tubules, resembling a Sertoli-Leydig cell tumor. " 05/13/2014 - "Bilateral salpingo-oophorectomy was performed and histopathology showed features of Sertoli Leydig cell tumor with intermediate to poor differentiation. " 10/01/2008 - "The pathological diagnosis after left salpingo-oophorectomy was Sertoli-Leydig cell tumor. " 11/22/2020 - "Fertility sparing unilateral salpingo-oophorectomy was done and adjuvant chemotherapy was given after histopathology showed moderate to poorly differentiated Sertoli-Leydig cell tumor. "
3.	Laparotomy 06/01/1996 - "A laparotomy with salpingo-oophorectomy was performed and the pathological report confirmed the presence of Sertoli-Leydig cell tumor of intermediate differentiation. " 01/01/1983 - "An exploratory laparotomy with left adrenalectomy, right adrenal biopsy and left ovarian wedge resection revealed an incompletely removed Sertoli-Leydig cell tumor, but normal adrenal histology. " 11/01/1989 - "At laparotomy, a monolateral ovarian tumor was found, which was proved histologically to be a Sertoli-Leydig cell tumor. " 01/01/1986 - "Whereas the findings of an ovarian mass and elevated serum AFP typically suggest an ominous diagnosis, ie, endodermal sinus tumor of the ovary, both of these patients were found at laparotomy to have Sertoli-Leydig cell tumors of the ovary. " 09/01/1996 - "A 33-year-old woman with abrupt cessation of menses, progressive hirsutism, and weight gain because of a unilateral ovarian mass identified sonographically and an increased serum testosterone level underwent laparotomy, which disclosed a left ovarian Sertoli-Leydig cell tumor. "
4.	Therapeutics 11/01/2012 - "To evaluate the clinicopathological features of ovarian Sertoli-Leydig cell tumors (SLCTs) and to explore the reasonable therapy. " 02/01/1983 - "The optimal therapy for recurrent Sertoli Leydig cell tumor is unknown. " 04/01/2023 - "We report the case of a 17 years old patient hospitalized in the oncology and gynecology ward of the university hospital Ibn Rochd in Casablanca, presenting a moderately differentiated Sertoli-Leydig cell tumor in the right ovary, our aim is to analyze the clinical, radiological and histological characteristics of this rare tumor that can be tricky to diagnose and review the different management therapies available and the challenges they present. " 12/01/1989 - "E.g., operative therapy with conservation of fertility is possible--in particular in young women--with granulosa cell tumors or androblastomas; dysgerminomas are strikingly sensitive to irradiation; chorion carcinomas respond well to chemotherapy. "
5.	Ovariectomy (Oophorectomy) 07/01/2018 - "The patient underwent laparoscopic unilateral oophorectomy resulting in the diagnosis of Sertoli-Leydig cell tumor (SLCT). " 03/01/2009 - "Bilateral oophorectomy was performed, and an ovarian Sertoli-Leydig cell tumor was diagnosed. " 10/01/1997 - "The Sertoli-Leydig cell tumor was a stage 1a, 145-g mass removed piecemeal by right oophorectomy. " 02/01/1983 - "An unusual case of a moderately well-differentiated, encapsulated, Sertoli Leydig cell tumor with heterologous elements recurring 3 years after a conservative unilateral oophorectomy in a 15-year-old female is reported. "