Gonadoblastoma

A complex neoplasm composed of a mixture of gonadal elements, such as large primordial GERM CELLS, immature SERTOLI CELLS or GRANULOSA CELLS of the sex cord, and gonadal stromal cells. Gonadoblastomas are most often associated with gonadal dysgenesis, 46, XY.

Also Known As:

Networked: 204 relevant articles (0 outcomes, 5 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Dysgerminoma
2.	Neoplasms (Cancer)
3.	46,XY Gonadal Dysgenesis (Swyer Syndrome)
4.	Gonadal Dysgenesis (Gonadal Agenesis)
5.	Turner Syndrome (Turner's Syndrome)

Experts

1.	Lau, Yun-Fai Chris: 8 articles (08/2022 - 08/2005)
2.	Kido, Tatsuo: 6 articles (01/2017 - 08/2005)
3.	Looijenga, Leendert H J: 5 articles (09/2008 - 02/2002)
4.	Cheng, Liang: 4 articles (01/2020 - 10/2004)
5.	Roth, Lawrence M: 4 articles (01/2020 - 10/2004)
6.	Li, Yunmin: 4 articles (01/2017 - 03/2007)
7.	Oosterhuis, J Wolter: 3 articles (09/2008 - 02/2002)
8.	Wolffenbuttel, Katja P: 3 articles (09/2008 - 05/2003)
9.	Stoop, Hans: 3 articles (10/2004 - 02/2002)
10.	Tian, Qinjie: 2 articles (08/2021 - 04/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Gonadoblastoma:

1.	Proteins (Proteins, Gene)FDA Link 07/01/2013 - "In this study, we performed immunohistochemical analysis for SALL4 and steroidogenic factor-1 (SF-1) on 4 cases of gonadoblastoma to examine the expression patterns of these proteins. " 01/01/2020 - "Those gonadoblastomas that occur in an individual who has a Y chromosome or part thereof express testis specific protein Y-encoded 1 (TSPY1). " 03/01/2018 - "Almost all gonadoblastomas occur in patients who have a Y chromosome or part thereof; testis-specific protein Y-encoded 1 (TSPY1) is the putative gene. " 03/28/2014 - "The testis-specific protein Y-encoded (TSPY) is a repetitive gene located on the gonadoblastoma region of the Y chromosome, and has been considered to be the putative gene for this oncogenic locus on the male-only chromosome. " 02/01/2011 - "The testis-specific protein Y-linked (TSPY) repeat gene has recently been demonstrated to be the putative gene for GBY, based on its location on the GBY critical region, expression patterns in early and late stages of gonadoblastoma and ability to induce gonadoblastoma-like structures in the ovaries of transgenic female mice. "
2.	DNA (Deoxyribonucleic Acid)IBA 08/01/1992 - "Minute Y chromosome derived marker in a child with gonadoblastoma: cytogenetic and DNA studies." 03/01/2001 - "DNA analysis showed that the proposed critical region of the gonadoblastoma locus on the Y chromosome was contained within the patient's idic (Y). " 11/01/1988 - "The presence of Y-specific fragments in the patient's DNA led to surgical exploration and the detection of a gonadoblastoma." 01/01/1987 - "Y-DNA hybridization analysis of individuals with gonadoblastoma and partial deletions of the Y chromosome should be of use in testing this proposal. " 10/01/2012 - "Exclusion of a Y cell line, the presence of which increases the risk of gonadoblastomas and subsequent gonadal germ cell tumors, is best accomplished by karyotype, fluorescence in situ hybridization, and DNA analysis if necessary. "
3.	Transcription Factors (Transcription Factor)IBA 09/01/2011 - "This study characterized the sex cord-stromal cells of gonadoblastoma by evaluating the expression of these counteracting transcription factors. " 09/10/2008 - "Also the octamer binding transcription factor OCT3/4 is consistently expressed in all type II GCTs with pluripotent potential, as well as in the precursor lesions carcinoma in situ (CIS) in case of a testis and gonadoblastoma (GB) in the DSD gonad. "
4.	GD3 ganglioside (GD(3))IBA 01/01/2015 - "The study included 26 patients with GD, 3 of whom had evidence of GCT (11.5 %, gonadoblastoma, dysgerminoma): 2 had Swyer syndrome, 1 had 46,XY partial GD. "
5.	SteroidsIBA 03/01/1976 - "Determinations of multiple steroids were made on ovarian and peripheral blood in a 46,XY patient with bilateral gonadoblastoma. " 12/01/2019 - "This report reminds us the possibility of diagnosis of Swyer syndrome in the presence of normal pubertal development and normal sex steroid levels considered to be produced by gonadoblastoma." 01/01/2002 - "Further evaluation (including laparoscopy) suggested that she presented a variant of gonadal dysgenesis, with the particularity of having well-developed müllerian structures and testicular remnants alongside a steroid-producing gonadoblastoma." 03/01/2000 - "The sex cord stromal group included 21 adult granulosa cell tumours (AGCT), two juvenile granulosa cell tumours (JGCT), eight tumours showing Sertoli cell or Sertoli-Leydig cell differentiation, two unclassified tumours, two gonadoblastomas, one sex cord tumour with annular tubules, two steroid cell tumours, five thecomas/fibrothecomas, and two sclerosing stromal tumours. " 03/01/1976 - "Ovarian and peripheral venous steroids in XY gonadal dysgenesis and gonadoblastoma."
6.	Testosterone (Sustanon)FDA Link 01/01/1994 - "Plasma testosterone level was elevated and returned to normal after the successful removal of the bilateral gonadoblastoma. " 01/01/2018 - "The patient was diagnosed with peripheral precocious puberty caused by over-secretion of testosterone by gonadoblastoma originating from dysgenetic gonads with Y-chromosome-related DSD. " 01/01/2002 - "The karyotype was 46,XY/45,X. These findings confirmed the diagnosis of mixed gonadal dysgenesis with testosterone-producing gonadoblastoma." 09/01/2003 - "Gonadoblastoma often produces estradiol or testosterone. " 11/01/1989 - "The high serum testosterone level, being unrelated to the administration of human chorionic gonadotropin is valuable for the diagnosis of gonadoblastoma in an individual with dysgenetic gonads. "
7.	H-Y AntigenIBA 04/01/1986 - "H-Y antigen level in the second sibling showed an increase in the male range, presumably due to the presence of gonadoblastomas. " 01/01/1986 - "All patients with gonadoblastomas should be HY antigen positive. " 01/01/1984 - "They show the place this syndrome has in the group of syndromes of bands and once again draw attention that there is a great risk of gonadoblastoma development when the Y chromosome is present and especially when the HY antigen is present. " 01/01/1980 - "Finally, the case confirms the high risk of gonadoblastoma in women with a Y chromosome, even in the absence of H-Y antigen." 01/01/1978 - "Cytogenetic and clinical notes on a girl with a 46,X,i(Yq) karyotype, H-Y antigen-negative, and a gonadoblastoma."
8.	LuteinIBA 07/01/2019 - "Gonadoblastoma is a neoplasm comprised of a mixture of germ cells and elements resembling immature granulosa or Sertoli cells with or without Leydig cells or lutein-type cells in an ovarian type stroma. " 06/01/2010 - "Gonadoblastoma is a rare gonadal tumor with tumor cells arranged in nests surrounded by ovarian stroma containing Leydig or lutein-type cells. " 05/18/2008 - "Gonadoblastoma (GB) is an in situ tumor consisting of a heterogeneous population of mature and immature germ cells, other cells resembling immature Sertoli/granulosa cells, and Leydig/lutein-like cells, may also be present. " 02/01/1999 - "Pathology reports confirmed hemorrhagic infarctions (n = 19), dysgenic gonads (n = 8), simple cysts (n = 7), teratomas (n = 6), theca lutein cysts (n = 4), fibroma (n = 1), stromal tumor (n = 1), mucinous cystadenoma (n = 1), granulosa cell tumor (n = 1), uterine neuroblastoma (n = 1), mixed germ cell neoplasm (n = 1), metastatic Wilms' tumor (n = 1), and gonadoblastoma (n = 1). " 01/01/1990 - "It is suggested that gonadoblastoma is capable of producing testosterone and estradiol, and Leydig or lutein-like cells may be the actual source of these steroid hormones."
9.	ParaffinIBA 09/01/2011 - "Archival paraffin-embedded material of 7 gonadoblastomas, 5 of which were overgrown by dysgerminoma, was examined by immunohistochemistry for expression and localization of FOXL2 and SOX9. " 04/01/2006 - "We performed dual color fluorescence in situ hybridization (FISH) analyses with a centromeric alpha-satellite probe for chromosome 12 and a subtelomeric probe for 12p on paraffin-embedded tissue sections from 21 dysgerminomas and two gonadoblastomas. " 08/01/1997 - "To evaluate this association, we used two-color fluorescence in situ hybridization with chromosome-specific probes to determine the distribution of the X and Y chromosomes in the tumor nests and surrounding stromal cells in paraffin tissue sections of three gonadoblastomas in two patients with gonadal dysgenesis and 45,X/46,XY mosaicism. " 11/01/1997 - "Archival, paraffin-embedded tissue from two patients with gonadoblastoma, one bilateral and both with concurrent germinomatous areas, were evaluated immunohistochemically with antibodies directed against I, MIS, WT1, and p53. " 10/01/2004 - "Formalin-fixed, paraffin-embedded cell blocks of 33 cases of dysgerminoma including 2 cases of gonadoblastoma associated with dysgerminoma and 3 cases of metastatic dysgerminoma, and 111 cases of nondysgerminomatous neoplasms involving the ovary were stained using the antibody against OCT4. "
10.	Inhibins (Inhibin)IBA 09/23/2019 - "Sex cord-stromal cells of gonadoblastoma were immunoreactive for α-inhibin. " 11/01/1997 - "Gonadoblastoma: immunohistochemical localization of Müllerian-inhibiting substance, inhibin, WT-1, and p53." 06/01/2002 - "Inhibin positivity in gonadal stroma and in gonadoblastoma may indicate hormonal activity causing advanced puberty in patients with XY gonadal dysgenesis." 06/01/2002 - "Immunohistochemical analysis showed that inhibin was strongly positive in gonadoblastoma but negative in dysgerminoma. " 07/01/1997 - "Inhibin immunostaining was identified in eight cases of stromal hyperthecosis, 24 adult-type granulosa cell tumours, two juvenile granulosa cell tumours, nine Sertoli cell or Sertoli-Leydig cell tumours, one gynandroblastoma, three gonadoblastomas, two cases of sex cord tumour with annular tubules, two steroid cell tumours and two sclerosing stromal tumours. "

Therapies and Procedures

1.	Castration 02/01/2023 - "Bilateral gonadoblastomas were incidentally diagnosed at 17 months of age at the time of prophylactic gonadectomies. " 01/24/2023 - "The patient initially presented with right gonadoblastoma but chose only right gonadectomy by laparoscopy to preserve the female sex characteristics, which resulted in rapid deterioration of the left gonad and poor treatment outcomes. " 11/01/2022 - "The biopsy revealed bilateral gonadoblastoma, emphasizing the need for early gonadectomy in 46XY FS patients." 06/01/2021 - "Gonadectomy is recommended due to the increased risk of gonadoblastoma, however, surgical intervention is often followed by loss of libido. " 10/01/2020 - "For instance, identifying a Y chromosome can prevent the occurrence of a gonadoblastoma, as gonadectomy should be discussed. "
2.	Laparotomy 01/01/2002 - "Explorative laparotomy revealed a gonadoblastoma arising from testicular structures on the left, a female streak gonad on the right side, and normal uterus and fallopian tubes. " 01/01/1998 - "A laparotomy was performed and the streak gonads with bilateral gonadoblastoma were removed. " 02/01/1996 - "On laparotomy, she was found to have bilateral gonadoblastomas. " 01/01/1995 - "At laparotomy, the elder sister was found to have bilateral gonadoblastoma. " 01/01/1993 - "Exploratory laparotomy after irradiation revealed gonadoblastoma originating from dysgenetic gonads."
3.	Salpingo-oophorectomy 01/01/2017 - "Elective laparoscopic bilateral salpingo-oophorectomy was performed to avoid increased risk for gonadoblastoma. " 07/01/2002 - "One child underwent bilateral salpingo-oophorectomy due to a gonadoblastoma. " 04/01/2001 - "After an extensive discussion with the patient and her family regarding her high risk for developing a gonadoblastoma, a laparoscopic bilateral salpingo-oophorectomy using the abdominal wall-life method was performed. " 12/01/1980 - "Ovarian biopsy contained gonadoblastoma that was treated by total abdominal hysterectomy and bilateral salpingo-oophorectomy. " 07/01/2008 - "The patient, who was admitted to our department with severe vaginal bleeding, was performed right salpingo-oophorectomy because of pure gonadoblastoma in 1996. "
4.	Therapeutics 07/01/2005 - "Gonadectomy without adjuvant therapies could be done in 46,XY gonadal dysgenetic women with gonadoblastoma in view of preservation of future fertility." 01/01/2017 - "The final diagnosis of Swyer syndrome with gonadoblastoma was made, and she was further subjected to operative resection and hormonal therapy. " 02/01/1979 - "Germ cell tumors which originate in gonadoblastomas appear to have the same clinical behavior and response to therapy as those that arise de novo in the ovary, testis or extragonadal sites. " 02/01/2006 - "Diagnostic of rare genetic pathologies, such as androgen insensitivity syndrome, with female phenotype and primary amenorrhea, is very important for avoiding developing of gonadoblastoma and the timely therapy." 08/01/2010 - "We recommend that the patient with gonadoblastoma start on hormone therapy. "
5.	Hysterectomy 04/01/1993 - "Examination of both gonads revealed gonadoblastoma and bilateral salpingo-gonadectomy and partial hysterectomy were performed. " 12/01/1980 - "Ovarian biopsy contained gonadoblastoma that was treated by total abdominal hysterectomy and bilateral salpingo-oophorectomy. " 01/01/1979 - "Pathological examination of tissues removed at total hysterectomy and bilateral salpinog-gonadectomy showed gonadoblastoma and dysgerminoma of left streak. " 04/15/1977 - "Pathologic examination of tissues removed at total hysterectomy and bilateral salpingo-oophorectomy revealed a gonadoblastoma of the right gonad, dysgerminoma of the left gonad, and an infantile hypoplastic uterus. " 01/01/1980 - "The gonadoblastoma was discovered at the age of 18 following a hysterectomy with castration which had become necessary because of pyometra and a tubal abscess. "