Gonadoblastoma: Difference between revisions

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__NOTOC__
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{{SI}}
{{Gonadoblastoma}}
{{CMG}} {{AE}} {{Sahar}}
{{CMG}}; {{AE}} {{Sahar}}


{{SK}} Gonadoblastomas; Gonadoblastomata
{{SK}} [[Gonadoblastomas]]; [[Gonadoblastomata]]


==Overview==


==Historical Perspective==


Gonadoblastoma was first discovered by Dr. Robert E. Scully, an American pathologist, in 1953 following studying the pathology of a series of tumors suspected of being dysgerminomas. This tumor is called gonadoblastoma since it looks like embryonic tissue, but in an indifferent way regarding sexual development.<ref name="Scully1953">{{cite journal|last1=Scully|first1=Robert E.|title=Gonadoblastoma. A gonadal tumor related to the dysgerminoma (Seminoma) and capable of sex-hormone production|journal=Cancer|volume=6|issue=3|year=1953|pages=455–463|issn=0008-543X|doi=10.1002/1097-0142(195305)6:3<455::AID-CNCR2820060303>3.0.CO;2-U}}</ref><ref name="pmid4193741">{{cite journal| author=Scully RE| title=Gonadoblastoma. A review of 74 cases. | journal=Cancer | year= 1970 | volume= 25 | issue= 6 | pages= 1340-56 | pmid=4193741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193741  }} </ref>
==[[Gonadoblastoma overview|Overview]]==


==[[Gonadoblastoma historical perspective|Historical Perspective]]==


The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
==[[Gonadoblastoma classification|Classification]]==


In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
==[[Gonadoblastoma pathophysiology|Pathophysiology]]==


In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].
==[[Gonadoblastoma causes|Causes]]==


There have been several outbreaks of [disease name], including -----.
==[[Gonadoblastoma differential diagnosis|Differentiating Gonadoblastoma from other Diseases]]==


In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].
==[[Gonadoblastoma epidemiology and demographics|Epidemiology and Demographics]]==


==Classification==
==[[Gonadoblastoma risk factors|Risk Factors]]==
There is no established system for the classification of [disease name].


OR
==[[Gonadoblastoma screening|Screening]]==


[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
==[[Gonadoblastoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
 
OR
 
[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3].
[Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
 
OR
 
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
 
OR
 
If the staging system involves specific and characteristic findings and features:
According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
 
OR
 
The staging of [malignancy name] is based on the [staging system].
 
OR
 
There is no established system for the staging of [malignancy name].
 
==Pathophysiology and Associated Conditions==
The exact pathogenesis of gonadoblastoma is not fully understood. Gonadal development starts at 5 weeks of gestation and continues according to sex chromosomal contents. Any defects in this complicated process lead to defective gonadal development and gonadal dysgenesis and subsequently, it can be converted to gonadoblastoma in 20% to 30% of the cases. <ref>{{cite book | last = Carcangiu | first = M. L. | title = WHO Classification of Tumours of Female Reproductive Organs | publisher = International Agency for Research on Cancer | location = Lyon | year = 2014 | isbn = 978-9283224358 }}</ref><ref name="pmid16608895">{{cite journal |vauthors=Cools M, Stoop H, Kersemaekers AM, Drop SL, Wolffenbuttel KP, Bourguignon JP, Slowikowska-Hilczer J, Kula K, Faradz SM, Oosterhuis JW, Looijenga LH |title=Gonadoblastoma arising in undifferentiated gonadal tissue within dysgenetic gonads |journal=J. Clin. Endocrinol. Metab. |volume=91 |issue=6 |pages=2404–13 |date=June 2006 |pmid=16608895 |doi=10.1210/jc.2005-2554 |url=}}</ref>
 
Gonadoblastoma occurs bilaterally in 50% of the cases. It can be accompanied by dysgerminoma in 50% of cases as well as with other more malignant germinomatous neoplasms in 10%.<ref="name978-1-4557-4858-7">{{cite book | last = Saia | first = Philip | title = Clinical gynecologic oncology | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-40067-1 }}</ref>
 
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
 
OR
 
The progression to [disease name] usually involves the [molecular pathway].
 
OR
 
The pathophysiology of [disease/malignancy] depends on the histological subtype.
 
==Gross Pathology==
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].<br>Gross pathology of tumor greatly depends on the degree of germ cells overgrowth and calcification. The tumor is firm and cartilaginous with a yellow to a brown-grey color. It can be calcified partly or almost completely. It can be very large especially with when ccompanied by a dysgerminoma or be hardly detectable in gross examination.<ref name="pmid4193741">{{cite journal| author=Scully RE| title=Gonadoblastoma. A review of 74 cases. | journal=Cancer | year= 1970 | volume= 25 | issue= 6 | pages= 1340-56 | pmid=4193741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193741  }} </ref>
 
==Microscopic Pathology==
 
==Causes==
Disease name] may be caused by [cause1], [cause2], or [cause3].
 
OR
 
Common causes of [disease] include [cause1], [cause2], and [cause3].
 
OR
 
The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
 
OR
 
The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click [[Pericarditis causes#Overview|here]].
 
==Differentiating ((Page name)) from Other Diseases==
Gonadoblastoma must be differentiated from dysgerminoma and other sex-cord stromal tumors.<ref name="978-1-4557-4858-7">{{cite book | last = Sperling | first = M | title = Pediatric endocrinology | publisher = Elsevier/Saunders | location = Philadelphia, PA | year = 2014 | isbn = 978-1-4557-4858-7 }}</ref>
 
==Epidemiology and Demographics==
The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
 
OR
 
In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
 
OR
 
In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.
 
 
 
The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
 
OR
 
Gonadoblastoma affects individuals of any age but tends to present at a greater extent prior to 15 years of age. it commonly originated from dysgenetic gonad but has been seen in women with normal ovary and karyotypes, although rarely. Since this tumor affects gonads, the majority of affected individuals may appear phenotypically female, but in reality, they are intersex.<ref name="pmid4193741">{{cite journal| author=Scully RE| title=Gonadoblastoma. A review of 74 cases. | journal=Cancer | year= 1970 | volume= 25 | issue= 6 | pages= 1340-56 | pmid=4193741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193741  }} </ref>younger than/older than [number of years] years of age.
 
OR
 
[Chronic disease name] is usually first diagnosed among [age group].
 
 
 
 
There is no racial predilection to [disease name].
 
OR
 
[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
 
 
 
[Disease name] affects men and women equally.
 
OR
 
[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
 
 
 
The majority of [disease name] cases are reported in [geographical region].
 
OR
 
[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].
 
==Risk Factors==
The most potent risk factor in the development of gonadoblastoma is XY gonadal dysgenesis. The risk of gonadoblastoma development increases with age. In individuals with XY gonadal abnormalities and it reaches 30% by thirty years of age. Other risk factors include being affected with Turner syndrome, and having Y chromosome materials.<ref name="978-1-4557-4858-7">{{cite book | last = Sperling | first = M | title = Pediatric endocrinology | publisher = Elsevier/Saunders | location = Philadelphia, PA | year = 2014 | isbn = 978-1-4557-4858-7 }}</ref>
 
==Screening==
There is insufficient evidence to recommend routine screening for [disease/malignancy].
 
OR
 
According to the [guideline name], screening for [disease name] is not recommended.
 
OR
 
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].
 
==Natural History, Complications, and Prognosis==
Gonadoblastoma per se is a benign tumor, however, it has the capacity to convert to dysgerminoma or other more malignant germ cell tumors and produce steroids with resultant virilization.<ref name="978-1-4557-4858-7">{{cite book | last = Sperling | first = M | title = Pediatric endocrinology | publisher = Elsevier/Saunders | location = Philadelphia, PA | year = 2014 | isbn = 978-1-4557-4858-7 }}</ref> If complicated by an invasive germ cell tumor, then prognosis depends on the staging of the tumor.<ref name="978-1-4557-4858-7">{{cite book | last = Saia | first = Philip | title = Clinical gynecologic oncology | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-40067-1 }}</ref>


==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
[[Gonadoblastoma diagnostic study of choice|Diagnostic study of choice]] | [[Gonadoblastoma history and symptoms|History and Symptoms]] | [[Gonadoblastoma physical examination|Physical Examination]] | [[Gonadoblastoma laboratory findings|Laboratory Findings]] | [[Gonadoblastoma electrocardiogram|Electrocardiogram]] | [[Gonadoblastoma x ray|X-Ray Findings]] | [[Gonadoblastoma echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Gonadoblastoma CT scan|CT-Scan Findings]] | [[Gonadoblastoma MRI|MRI Findings]] | [[Gonadoblastoma other imaging findings|Other Imaging Findings]] | [[Gonadoblastoma other diagnostic studies|Other Diagnostic Studies]]
There are no established criteria for the diagnosis of Gonadoblastoma.
 
===History and Symptoms===
Patients with gonadoblastoma present either during infancy with ambiguous genitalia or later with sexual developmental complaints. The hallmark of gonadoblastoma is gonadal developmental disorders. The majority of affected individuals are phenotypically women and may be discovered during a workup for virilization and/or primary amenorrhea. Others are phenotypically men with sex organs developmental problems such as cryptorchidism and hypospadias.<ref name="978-1-4557-4858-7">{{cite book | last = Saia | first = Philip | title = Clinical gynecologic oncology | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-40067-1 }}</ref>
 
===Physical Examination===
Patients with gonadoblastoma can appear phenotypically female or male. It is of particular note that clinical presentation may be varied considering the nature of abnormal gonad and the amount of steroid hormone in it.<ref name="pmid4193741">{{cite journal| author=Scully RE| title=Gonadoblastoma. A review of 74 cases. | journal=Cancer | year= 1970 | volume= 25 | issue= 6 | pages= 1340-56 | pmid=4193741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193741  }} </ref>In women, physical examination of patients can be remarkable for virilization or the presence of a pelvic mass.
OR<ref name="978-0-323-40067-1">{{cite book | last = Saia | first = Philip | title = Clinical gynecologic oncology | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 978-0-323-40067-1 }}</ref>
 
 
 
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
 
OR
 
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
 
===Laboratory Findings===
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
 
OR
 
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
 
OR
 
[Test] is usually normal among patients with [disease name].
 
OR
 
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
 
OR
 
There are no diagnostic laboratory findings associated with [disease name].
 
===X-ray===
There are no x-ray findings associated with gonadoblastoma. However, an x-ray may be helpful in the diagnosis of calcification associated with gonadoblastoma.
 
===CT scan===
OR
 
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===MRI===
There are no MRI findings associated with [disease name].
 
OR
 
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===Other Imaging Findings===
There are no other imaging findings associated with [disease name].
 
OR
 
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
===Other Diagnostic Studies===
There are no other diagnostic studies associated with [disease name].
 
OR
 
[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].


==Treatment==
==Treatment==
===Medical Therapy===
[[Gonadoblastoma medical therapy|Medical Therapy]] | [[Gonadoblastoma surgery|Surgery]] | [[Gonadoblastoma primary prevention|Primary Prevention]] | [[Gonadoblastoma secondary prevention|Secondary Prevention]] | [[Gonadoblastoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Gonadoblastoma future or investigational therapies|Future or Investigational Therapies]]
The mainstay of treatment for gonadoblastoma is surgical removal of the tumor. Gonadectomy is applied only to those who have Y chromosome content and/or virilization.<ref name="978-1-4557-4858-7">{{cite book | last = Sperling | first = M | title = Pediatric endocrinology | publisher = Elsevier/Saunders | location = Philadelphia, PA | year = 2014 | isbn = 978-1-4557-4858-7 }}</ref>
 
 
OR
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
 
OR
 
[Therapy] is recommended among all patients who develop [disease name].
 
OR
 
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
 
 
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
 
OR
 
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
 
OR
 
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
 
===Surgery===
Surgical intervention is not recommended for the management of [disease name].
 
OR
 
 
OR
 
The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
 
OR
 
Surgery is the mainstay of treatment for [disease or malignancy].
 
===Primary Prevention===
There are no established measures for the primary prevention of [disease name].
 
 
 
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
 
OR
 
[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].
 
===Secondary Prevention===
There are no established measures for the secondary prevention of [disease name].


OR
==Case Studies==
[[Gonadoblastoma case study one|Case #1]]


Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].


==References==
[[Category:Medicine]]
{{reflist|2}}


{{WikiDoc Help Menu}}
[[Category:Oncology]]
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Latest revision as of 06:33, 28 July 2020

Gonadoblastoma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Gonadoblastoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Risk calculators and risk factors for Gonadoblastoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Synonyms and keywords: Gonadoblastomas; Gonadoblastomata


Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Gonadoblastoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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