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{{Ovarian germ cell tumor}}
{{Ovarian germ cell tumor}}


{{CMG}}{{AE}} {{MD}}
{{CMG}}{{AE}} {{Sahar}} {{MD}}
==Overveiw==
==Overveiw==
Ovarian germ cell tumor is a disease in which [[malignant]] cells form in the germ cells of the ovary. Germ cell tumors begin in the reproductive cells of the body. Ovarian germ cell tumor are rare tumors, accounting for 2% to 3% of all ovarian cancers. The median age for [[diagnosis]] is 16 to 20 (range 6 to 40 years).<ref name=dfg> Updates in the Management of Ovarian Germ Cell Tumors. http://meetinglibrary.asco.org/content/31-132. URL Accessed on November 11, 2015</ref> Ovarian germ cell tumor may be classified into 7 subtypes based on [[histology]].<ref name=abc> Cellular Classification of Ovarian Germ Cell Tumors. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_. URL Accessed on November 4, 2015</ref> The most common ovarian germ cell tumor is called [[dysgerminoma]]. Abnormal [[gonad]]s (due to [[gonadal dysgenesis]] and [[androgen insensitivity syndrome]]) have a high risk of developing a dysgerminoma. Ovarian germ cell tumor must be differentiated from other [[neoplastic]] [[ovarian]] mass, non neoplastic ovarian mass, and adnexal mass. Symptoms of ovarian germ cell tumor include [[abdominal distention]], acute/ sub acute [[abdominal pain]], [[menstrual irregularities]], and [[precocious puberty]].<ref name= lmn>{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref>  Physical examination of patients with ovarian germ cell tumor is usually remarkable for [[vaginal bleeding]], [[abdominal distention]], and adnexal mass.<ref name= lmn>{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref>  The laboratory findings associated with ovarian germ cell tumor are the following: serum [[lactate dehydrogenase]] (LDH),  [[human chorionic gonadotropin]] (HCG), [[CA-125]], and [[alpha-fetoprotein]] (AFP). Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. [[CT]], [[MRI]], and [[ultrasound]] are used in combination to distinguish between the subtypes of ovarian germ cell tumors.<ref name= opq> Ovarian dysgerminoma. http://radiopaedia.org/articles/ovarian-dysgerminoma. URL Accessed on November 11, 2015</ref><ref name= qst> Mature (cystic) ovarian teratoma. http://radiopaedia.org/articles/mature-cystic-ovarian-teratoma. URL Accessed on November 11, 2015</ref><ref name=ppp> Ovarian yolk sac tumour. http://radiopaedia.org/articles/ovarian-yolk-sac-tumour. URL Accessed on November 11, 2015</ref> However, the prognosis is generally regarded as good.<ref name= mmm> Ovarian Germ Cell Tumors Treatment.http://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq.  URL Accessed on November 11, 2015</ref>
[[Ovarian]] [[germ cell]] [[tumors]] is a [[disease]] in which [[malignant|malignancy]] originates from the [[germ cells]] of the [[ovary]]. [[Ovarian]] [[germ cell]] [[tumors]] are rare, accounting for 2% to 3% of all [[ovarian cancers]]. The median age for [[diagnosis]] differs for each sub-type of germ cell tumor. World health organization (WHO) classified germ cell [[tumors]] into 7 types based on [[histology]]. The most common [[ovarian]] [[germ cell]] [[tumor]] is called [[dysgerminoma]]. Abnormal [[gonad]]s (due to [[gonadal dysgenesis]] and [[androgen insensitivity syndrome]]) have a high risk of developing a [[dysgerminoma]]. [[Ovarian]] [[germ cell]] [[tumors]] must be differentiated from other [[neoplastic]] [[ovarian]] masses that can present with similar complaints  non-neoplastic [[ovarian]] mass, and [[adnexal mass]]. Symptoms of [[ovarian]] [[germ cell]] [[tumors]] include [[abdominal distention]], acute/ subacute [[abdominal pain]], [[menstrual irregularities]], and [[precocious puberty]]. The laboratory findings associated with [[ovarian]] [[germ cell]] [[tumors]] include rise in serum [[lactate dehydrogenase]] (LDH),  [[human chorionic gonadotropin]] (HCG), [[CA-125]], and [[alpha-fetoprotein]] (AFP). Depending on the extent of the tumor at the time of [[diagnosis]], the [[prognosis]] may vary. [[CT]], [[MRI]], and [[ultrasound]] are used in combination with biopsy not only to distinguish between the subtypes of [[ovarian]] [[germ cell]] [[tumors]] but also for diagnosis confirmation. [[Surgery]] along with [[chemotherapy]] are the mainstay of treatment depending on the staging of the tumor.
The mainstay of therapy for ovarian germ cell tumor is [[chemotherapy]] and [[surgery]].<ref name= cba> Stage I Ovarian Germ Cell Tumors
 
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_33. URL Accessed on Nov 5, 2015</ref><ref name= sss> Stage II Ovarian Germ Cell Tumors
== Historical Perspective ==
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_43. URL Accessed on Nov 5, 2015</ref><ref name= www> Stage III Ovarian Germ Cell Tumors
There is limited information about the historical perspective of [[ovarian]] [[germ cell]] [[tumors]].
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_54. URL Accessed on Nov 5, 2015</ref><ref name= rrr> Stage IV Ovarian Germ Cell Tumors
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_65. URL Accessed on Nov 5, 2015</ref>


==Classification==
==Classification==
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==Pathophysiology==
==Pathophysiology==
The [[pathophysiology]] of [[ovarian]] [[germ cell]] [[tumors]] depends on the [[histological]] subtype. However, their origin is the [[primordial germ cells]] that [[Transformation|transformed]] [[Pathology|pathologically]] in different stages of development. It is difficult to distinguish subtypes of ovarian germ cell tumor on gross [[pathology]] alone. The majority of [[ovarian]] [[germ cell]] [[tumors]] have a [[solid]] and [[cystic]] appearance with areas of [[hemorrhage]] and [[necrosis]]. On microscopic [[pathology]], [[ovarian]] [[germ cell]] [[tumors]] may be characterized by a uniform “fried egg” appearance ([[dysgerminoma]]), presence of Schiller-Duval bodies ([[yolk sac tumor]]),  presence of embryonic-like neural, [[gastrointestinal]], and/or cartilaginous tissue ([[teratoma]]), or mixed histopathological features ([[embryonal cell carcinoma]]).
The [[pathophysiology]] of [[ovarian]] [[germ cell]] [[tumors]] depends on the [[histological]] subtype. Their common origin is believed to be from the [[primordial germ cells]] that [[Transformation|transformed]] [[Pathology|pathologically]] in different stages of development. It is difficult to distinguish subtypes of ovarian germ cell tumor on gross [[pathology]] alone. The majority of [[ovarian]] [[germ cell]] [[tumors]] have a [[solid]] and [[cystic]] appearance with areas of [[hemorrhage]] and [[necrosis]]. On microscopic [[pathology]], [[ovarian]] [[germ cell]] [[tumors]] may be characterized by a uniform “fried egg” appearance ([[dysgerminoma]]), presence of Schiller-Duval bodies ([[yolk sac tumor]]),  presence of embryonic-like neural, [[gastrointestinal]], and/or cartilaginous tissue ([[teratoma]]), or mixed histopathological features ([[embryonal cell carcinoma]]).


==Epidemiology and Demographics==
==Epidemiology and Demographics==
Ovarian germ cell tumor are rare [[tumor]]s, accounting for 2% to 3% of all ovarian cancers. The median age for [[diagnosis]] is 16 to 20 (range 6 to 40 years).<ref name=dfg> Updates in the Management of Ovarian Germ Cell Tumors. http://meetinglibrary.asco.org/content/31-132. URL Accessed on November 11, 2015</ref>
In USA, the age-adjusted [[incidence]] of [[malignant]] [[ovarian]] [[germ cell]] [[tumor]] is 0.41 per 100,000 women. [[Incidence]] of these [[tumors]] increases from 5 years of age, although it may be present during [[infancy]], and this increase continues to peak between the age of 15 to 19 years which is approximately 1.2 per 100,000 women. Another peak [[incidence]] of these [[tumors]] has been reported among those aged 65 years old or older where [[teratoma]] is the most common type observed. The [[incidence]] is higher among non-white [[Ethnicity and health|ethnicity]] (other than black, especially Hispanic and Asians) followed by white and black individuals. Females are more commonly affected by [[germ cell]] [[tumors]] than males. These [[tumors]] also account for a greater proportion of [[ovarian]] [[tumors]] in the Asia and Africa.


==Risk Factors==
==Risk Factors==
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==Differentiating From Ovarian Germ Cell Tumor Other Diseases==
==Differentiating From Ovarian Germ Cell Tumor Other Diseases==
Ovarian germ cell tumor must be differentiated from other diseases that cause ovarian mass, such as stein-Leventhal syndrome, [[ovary]] teratoma, tubal [[pregnancy]], ovary adenocarcinoma, and tubo-ovarian [[abscess]].<ref name= sba>Shaaban AM, Rezvani M, Elsayes KM, et al. Ovarian malignant germ cell tumors: cellular classification and clinical and imaging features. Radiographics. 2014;34(3):777-801.http://pubs.rsna.org/doi/pdf/10.1148/rg.343130067</ref><ref name= ajk>Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST. CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. Radiographics. 2002;22(6):1305-25.http://www.ncbi.nlm.nih.gov/pubmed/12432104</ref>
[[Ovarian]] [[germ cell]] [[tumor]] must be differentiated from other [[diseases]] that cause [[ovarian mass]], such as [[Stein-Leventhal syndrome]], [[ovarian]] teratoma, tubal [[pregnancy]], [[ovarian epithelial tumors]], [[ovarian]] sex-cord stromal [[tumors]], and tubo-ovarian [[abscess]].


==Prognosis==
==Prognosis==
Depending on the extent of the tumor at the time of diagnosis, the [[prognosis]] may vary. However, the prognosis is generally regarded as
The [[prognosis]] of [[Germ cell|germ cells]] of the [[ovary]] depends on the type of the [[tumor]] and its [[malignant]] potentials. Possible [[complications]] of benign [[Teratoma|teratomas]] are a [[rupture]] and [[ovarian torsion]] also [[malignant transformation]]. [[Prognosis|Prognosi]]<nowiki/>s is generally excellent in the [[Mature cystic teratoma|mature teratoma]], but in case of simultaneous [[malignant transformation]], the 5-year [[survival rate]] of patients is approximately [15-30]%. The 5-year [[survival rate]] of the patient even with [[Disseminated disease|disseminated]] [[dysgerminoma]] at the time of [[diagnosis]] is above 90%. The overall 5-year [[survival rate]] for [[yolk sac tumor]], [[embryonal carcinoma]] and [[choriocarcinoma]] are approximately 80%.
good.<ref name= mmm> Ovarian Germ Cell Tumors Treatment.http://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdqURL Accessed on November 11, 2015</ref>
 
 
 
== Diagnosis ==
 
===Staging===  
According to the [[FIGO]] and [[TNM]] [[cancer staging]] system, there are 4 [[stages]] of [[ovarian]] [[germ cell]] [[tumor]]. [[Surgery]] is the mainstay of staging for [[ovarian]] [[germ cell]] [[tumors]].
 
===History and Symptoms===
The [[clinical]] manifestations of patients with [[ovarian]] [[germ cell]] [[tumors]] depend on the type of the [[tumor]] and its potential to produce [[hormonal]] materials. Usually, they present with [[abdominal pain]] or [[Abdominal distention|distention]], [[menstrual irregularities]], symptoms of [[virilization]], rapidly growing [[abdominal]]/[[pelvic]] [[mass]], [[acute abdominal pain]] from [[complications]] such as [[necrosis]], [[Capsule|capsular]] distention, [[rupture]] or [[torsion]] and or simply they can be [[asymptomatic]].


==Staging==  
===Ovarian Germ Cell Tumor Physical Examination===
According to the FIGO [[cancer staging]] system, there are 4 stages of ovarian germ cell tumor.<ref name= mmx> Stage Information for Ovarian Germ Cell Tumors. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_8. URL Accessed on November 5, 2015</ref>
Patients with [[germ cells]] of the [[ovary]] usually appear normal. [[Physical examination]] of these patients is usually unremarkable and the [[tumors]] tend to be discovered incidentally or during [[imaging]] workups for another reason. When [[symptomatic]], the [[physical examination]] may be remarkable for [[Abdominal]]/[[pelvic]] [[mass]] and/or [[signs]] of [[virilization]], [[precocious puberty]], and [[pregnancy]] depending on the capacity of the [[tumor]] for the production of [[hormones]].


==History and Symptoms==  
===Ovarian germ cell tumor Laboratory Findings===
Symptoms of ovarian germ cell tumor include [[abdominal distention]], acute/ sub acute [[abdominal pain]], [[menstrual irregularities]], and symptoms of [[precocious puberty]].<ref name= lmn>{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref>
The laboratory findings associated with [[ovarian]] [[germ cell]] [[tumor]] are the following: elevated serum level of [[lactate dehydrogenase]] (LDH), [[human chorionic gonadotropin]] (HCG), [[CA-125]], and [[alpha-fetoprotein]] (AFP).


==Ovarian Germ Cell Tumor Physical Examination==
===CT===
[[Physical examination]] of patients with ovarian germ cell tumor is usually remarkable for [[vaginal bleeding]], [[abdominal distention]], and adnexal mass.<ref name= lmn>{{cite book | last = Hoffman | first = Barbara | title = Williams gynecology | publisher = McGraw-Hill Medical | location = New York | year = 2012 | isbn = 9780071716727 }}</ref>
It is difficult to distinguish [[ovarian]] [[germ cell]] [[tumors]] on [[CT]] alone, however, [[pelvic]]/[[abdominal]] CT scan may be helpful in the diagnosis. Sensitive findings on [[CT scan]] for the [[diagnosis]] of [[Mature cystic teratoma|mature teratoma]] include [[Fat]] attenuation, presence or absence of [[calcification]] in the [[cyst]] wall, palm-tree like protrusion, and [[fat]]-[[fluid]] levels. Presence of cauliflower appearance with irregular borders may warrant the necessity to search for [[malignant transformation]] of these [[tumors]]. [[CT scan]] findings characteristics of immature [[teratoma]] include A large, irregular [[solid]] [[mass]], presence of coarse [[calcification]], small foci of [[fat]], and [[Hemorrhage]]. CT findings associated with other [[ovarian]] [[germ cell]] [[tumors]] are not specific, but may be helpful.


==Ovarian germ cell tumor Laboratory Findings==
===MRI===
The laboratory findings associated with ovarian germ cell tumor are the following: serum [[lactate dehydrogenase]] (LDH), [[human chorionic gonadotropin]] (HCG), [[CA-125]], and [[alpha-fetoprotein]] (AFP).<ref name= ors> Ovary: Germ cell tumors. http://atlasgeneticsoncology.org/Tumors/OvarianGermCellID5067.html. URL Accessed on November 10, 2015</ref>
[[Abdominal]]/[[pelvic]] [[MRI]] may be helpful in the [[diagnosis]] of [[ovarian]] [[germ cell]] [[tumors]]. Findings on [[MRI]] suggestive of [[ovarian]] [[germ cell]] [[tumors]] include [[Mass|masses]] with a [[cystic]] and [[solid]] component and may contain [[fat]], [[calcification]], [[fat]]-[[fluid]] level, Tuft\[[Hairs]], Palm tree-like protrusion, and [[Dermoid]] nipples-like elements (Rokitansky [[nodules]]). The majority of [[ovarian]] [[germ cell]] [[tumors]] have a [[solid]] and [[cystic]] appearance with areas of [[hemorrhage]] and [[necrosis]]. The predominance of [[cystic]] or [[solid]] component differs for each [[tumor]].


==CT==
===Ultrasound===
It is difficult to distinguish ovarian germ cell tumors on [[CT]] alone. [[Dysgerminoma]] often appears as multilobulated solid masses with prominent fibrovascular [[septa]] while, mature ovarian teratoma may demonstrate [[fat]] (areas with very low Hounsfield values), fat-fluid level, calcification (sometimes dentiform), Rokitansky protuberance, and tufts of [[hair]].<ref name= opq> Ovarian dysgerminoma. http://radiopaedia.org/articles/ovarian-dysgerminoma. URL Accessed on November 11, 2015</ref><ref name= qst> Mature (cystic) ovarian teratoma. http://radiopaedia.org/articles/mature-cystic-ovarian-teratoma. URL Accessed on November 11, 2015</ref><ref name=ppp > Immature ovarian teratoma. http://radiopaedia.org/articles/immature-ovarian-teratoma. URL Accessed on November 11, 2015</ref>
It is difficult to distinguish [[ovarian]] [[germ cell]] [[tumors]] on [[ultrasound]] alone. Both [[solid]] and [[cystic]] [[lesions]] with [[calcification]] may be present. Dysgerminoma often appears as a [[Echogenicity|hypoechoic]] [[mass]] while other [[ovarian]] [[germ cell]] [[tumors]] often have variable [[echogenicity]]. Ovarian teratoma may be further characterized by the presence of [[sebaceous]] and [[hair]] components arising from the Rokitansky protuberance.


==MRI==
== Treatment ==
It is difficult to distinguish subtypes of ovarian germ cell tumor on [[MRI]] alone. The majority of ovarian germ cell tumors have a solid and cystic appearance with areas of [[hemorrhage]] and [[necrosis]]. On MRI, ovarian germ cell tumors may be characterized by T2: the septae are often hypointense or isointense T1 C+ (Gd): the septae often show marked enhancement ([[dysgerminoma]]), the presence of a prominent solid component containing calcifications and small foci of [[fat]] (mature [[teratoma]]), areas of haemorrhage can also be seen (yolk sac tumor).<ref name= opq> Ovarian dysgerminoma. http://radiopaedia.org/articles/ovarian-dysgerminoma. URL Accessed on November 11, 2015</ref><ref name= qst> Mature (cystic) ovarian teratoma. http://radiopaedia.org/articles/mature-cystic-ovarian-teratoma. URL Accessed on November 11, 2015</ref><ref name=ppp> Ovarian yolk sac tumour. http://radiopaedia.org/articles/ovarian-yolk-sac-tumour. URL Accessed on November 11, 2015</ref>


==Ultrasound==
===Chemotherapy===
It is difficult to distinguish ovarian germ cell tumors on [[ultrasound]] alone. Both solid and [[cystic]] lesions with calcification may be present. Dysgerminoma often appears as a hypoechoic mass while other ovarian germ cell tumors often have variable echogenicity. Ovarian teratoma may be further characterized by the presence of [[sebaceous]] and [[hair]] components arising from the rokitansky protuberance.<ref name= opq> Ovarian dysgerminoma. http://radiopaedia.org/articles/ovarian-dysgerminoma. URL Accessed on November 11, 2015</ref><ref name= qst> Mature (cystic) ovarian teratoma. http://radiopaedia.org/articles/mature-cystic-ovarian-teratoma. URL Accessed on November 11, 2015</ref><ref name= ppp> Ovarian yolk sac tumour. http://radiopaedia.org/articles/ovarian-yolk-sac-tumour. URL Accessed on November 11, 2015</ref><ref name=xyz> Immature ovarian teratoma. http://radiopaedia.org/articles/immature-ovarian-teratoma. URL Accessed on November 11, 2015</ref>
[[Adjuvant]] [[Chemotherapy]] is recommended for all the patients with [[Diagnosis|diagnosed]] [[malignant]] [[ovarian]] [[germ cell]] [[tumor]], except those with stage 1a, stage 1a, 1b [[dysgerminoma]], and grade 1 immature [[Teratoma|teratomas]]. The [[platinum]]-based regimen is currently the most effective management.


==Medical Therapy==
===Radiotherapy===
The mainstay of therapy for ovarian germ cell tumor is [[chemotherapy]].<ref name= cba> Stage I Ovarian Germ Cell Tumors
Among [[ovarian]] [[germ cell]] [[tumors]], only [[dysgerminoma]] is radiosensitive. [[Radiotherapy]] is not anymore the first option of treatment for [[dysgerminoma]] considering its association with [[ovarian failure]] development.
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_33. URL Accessed on Nov 5, 2015</ref><ref name= sss> Stage II Ovarian Germ Cell Tumors
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_43. URL Accessed on Nov 5, 2015</ref><ref name= www> Stage III Ovarian Germ Cell Tumors
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_54. URL Accessed on Nov 5, 2015</ref><ref name= rrr> Stage IV Ovarian Germ Cell Tumors
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_65. URL Accessed on Nov 5, 2015</ref>


==Surgery==
===Surgery===
[[Surgery]] is the mainstay of treatment for ovarian germ cell tumors.<ref name= cba> Stage I Ovarian Germ Cell Tumors
[[Surgical]] [[intervention]] is the mainstay of management of [[ovarian]] [[germ cell]] [[tumors]]. [[Surgery]] must be done for the purpose of [[Cancer staging|staging]] and maybe treatment according to the stage of the tumor. [[Surgical]] management of the [[ovarian]] [[germ cell]] [[tumors]], for the purpose of treatment, classified to two categories according to the preference of the patient to preserve the [[ovary]] or not. [[Surgery]] is the mainstay of treatment for [[Mature cystic teratoma|mature teratoma]].
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_33. URL Accessed on Nov 5, 2015</ref><ref name= sss> Stage II Ovarian Germ Cell Tumors
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_43. URL Accessed on Nov 5, 2015</ref><ref name= www> Stage III Ovarian Germ Cell Tumors
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_54. URL Accessed on Nov 5, 2015</ref><ref name= rrr> Stage IV Ovarian Germ Cell Tumors
. http://www.cancer.gov/types/ovarian/hp/ovarian-germ-cell-treatment-pdq#section/_65. URL Accessed on Nov 5, 2015</ref>


==References==
{{reflist|2}}
[[Category:Disease]]
[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Types of cancer]]

Latest revision as of 00:50, 19 April 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2] Monalisa Dmello, M.B,B.S., M.D. [3]

Overveiw

Ovarian germ cell tumors is a disease in which malignancy originates from the germ cells of the ovary. Ovarian germ cell tumors are rare, accounting for 2% to 3% of all ovarian cancers. The median age for diagnosis differs for each sub-type of germ cell tumor. World health organization (WHO) classified germ cell tumors into 7 types based on histology. The most common ovarian germ cell tumor is called dysgerminoma. Abnormal gonads (due to gonadal dysgenesis and androgen insensitivity syndrome) have a high risk of developing a dysgerminoma. Ovarian germ cell tumors must be differentiated from other neoplastic ovarian masses that can present with similar complaints non-neoplastic ovarian mass, and adnexal mass. Symptoms of ovarian germ cell tumors include abdominal distention, acute/ subacute abdominal pain, menstrual irregularities, and precocious puberty. The laboratory findings associated with ovarian germ cell tumors include rise in serum lactate dehydrogenase (LDH), human chorionic gonadotropin (HCG), CA-125, and alpha-fetoprotein (AFP). Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. CT, MRI, and ultrasound are used in combination with biopsy not only to distinguish between the subtypes of ovarian germ cell tumors but also for diagnosis confirmation. Surgery along with chemotherapy are the mainstay of treatment depending on the staging of the tumor.

Historical Perspective

There is limited information about the historical perspective of ovarian germ cell tumors.

Classification

Ovarian germ cell tumor may be benign or malignant. Each category sub classified to different types based on histologic features.

Pathophysiology

The pathophysiology of ovarian germ cell tumors depends on the histological subtype. Their common origin is believed to be from the primordial germ cells that transformed pathologically in different stages of development. It is difficult to distinguish subtypes of ovarian germ cell tumor on gross pathology alone. The majority of ovarian germ cell tumors have a solid and cystic appearance with areas of hemorrhage and necrosis. On microscopic pathology, ovarian germ cell tumors may be characterized by a uniform “fried egg” appearance (dysgerminoma), presence of Schiller-Duval bodies (yolk sac tumor), presence of embryonic-like neural, gastrointestinal, and/or cartilaginous tissue (teratoma), or mixed histopathological features (embryonal cell carcinoma).

Epidemiology and Demographics

In USA, the age-adjusted incidence of malignant ovarian germ cell tumor is 0.41 per 100,000 women. Incidence of these tumors increases from 5 years of age, although it may be present during infancy, and this increase continues to peak between the age of 15 to 19 years which is approximately 1.2 per 100,000 women. Another peak incidence of these tumors has been reported among those aged 65 years old or older where teratoma is the most common type observed. The incidence is higher among non-white ethnicity (other than black, especially Hispanic and Asians) followed by white and black individuals. Females are more commonly affected by germ cell tumors than males. These tumors also account for a greater proportion of ovarian tumors in the Asia and Africa.

Risk Factors

Gonadal dysgenesis and androgen insensitivity syndrome are known risk factors for the development of ovarian germ cell tumors. There are also other maternal factors that have been observed to be associated with increased risk of the development of these tumors in daughters. Factors such as maternal use of exogenous hormones, maternal elevated body mass index, and reproductive factors.

Differentiating From Ovarian Germ Cell Tumor Other Diseases

Ovarian germ cell tumor must be differentiated from other diseases that cause ovarian mass, such as Stein-Leventhal syndrome, ovarian teratoma, tubal pregnancy, ovarian epithelial tumors, ovarian sex-cord stromal tumors, and tubo-ovarian abscess.

Prognosis

The prognosis of germ cells of the ovary depends on the type of the tumor and its malignant potentials. Possible complications of benign teratomas are a rupture and ovarian torsion also malignant transformation. Prognosis is generally excellent in the mature teratoma, but in case of simultaneous malignant transformation, the 5-year survival rate of patients is approximately [15-30]%. The 5-year survival rate of the patient even with disseminated dysgerminoma at the time of diagnosis is above 90%. The overall 5-year survival rate for yolk sac tumor, embryonal carcinoma and choriocarcinoma are approximately 80%.


Diagnosis

Staging

According to the FIGO and TNM cancer staging system, there are 4 stages of ovarian germ cell tumor. Surgery is the mainstay of staging for ovarian germ cell tumors.

History and Symptoms

The clinical manifestations of patients with ovarian germ cell tumors depend on the type of the tumor and its potential to produce hormonal materials. Usually, they present with abdominal pain or distention, menstrual irregularities, symptoms of virilization, rapidly growing abdominal/pelvic mass, acute abdominal pain from complications such as necrosis, capsular distention, rupture or torsion and or simply they can be asymptomatic.

Ovarian Germ Cell Tumor Physical Examination

Patients with germ cells of the ovary usually appear normal. Physical examination of these patients is usually unremarkable and the tumors tend to be discovered incidentally or during imaging workups for another reason. When symptomatic, the physical examination may be remarkable for Abdominal/pelvic mass and/or signs of virilization, precocious puberty, and pregnancy depending on the capacity of the tumor for the production of hormones.

Ovarian germ cell tumor Laboratory Findings

The laboratory findings associated with ovarian germ cell tumor are the following: elevated serum level of lactate dehydrogenase (LDH), human chorionic gonadotropin (HCG), CA-125, and alpha-fetoprotein (AFP).

CT

It is difficult to distinguish ovarian germ cell tumors on CT alone, however, pelvic/abdominal CT scan may be helpful in the diagnosis. Sensitive findings on CT scan for the diagnosis of mature teratoma include Fat attenuation, presence or absence of calcification in the cyst wall, palm-tree like protrusion, and fat-fluid levels. Presence of cauliflower appearance with irregular borders may warrant the necessity to search for malignant transformation of these tumors. CT scan findings characteristics of immature teratoma include A large, irregular solid mass, presence of coarse calcification, small foci of fat, and Hemorrhage. CT findings associated with other ovarian germ cell tumors are not specific, but may be helpful.

MRI

Abdominal/pelvic MRI may be helpful in the diagnosis of ovarian germ cell tumors. Findings on MRI suggestive of ovarian germ cell tumors include masses with a cystic and solid component and may contain fat, calcification, fat-fluid level, Tuft\Hairs, Palm tree-like protrusion, and Dermoid nipples-like elements (Rokitansky nodules). The majority of ovarian germ cell tumors have a solid and cystic appearance with areas of hemorrhage and necrosis. The predominance of cystic or solid component differs for each tumor.

Ultrasound

It is difficult to distinguish ovarian germ cell tumors on ultrasound alone. Both solid and cystic lesions with calcification may be present. Dysgerminoma often appears as a hypoechoic mass while other ovarian germ cell tumors often have variable echogenicity. Ovarian teratoma may be further characterized by the presence of sebaceous and hair components arising from the Rokitansky protuberance.

Treatment

Chemotherapy

Adjuvant Chemotherapy is recommended for all the patients with diagnosed malignant ovarian germ cell tumor, except those with stage 1a, stage 1a, 1b dysgerminoma, and grade 1 immature teratomas. The platinum-based regimen is currently the most effective management.

Radiotherapy

Among ovarian germ cell tumors, only dysgerminoma is radiosensitive. Radiotherapy is not anymore the first option of treatment for dysgerminoma considering its association with ovarian failure development.

Surgery

Surgical intervention is the mainstay of management of ovarian germ cell tumors. Surgery must be done for the purpose of staging and maybe treatment according to the stage of the tumor. Surgical management of the ovarian germ cell tumors, for the purpose of treatment, classified to two categories according to the preference of the patient to preserve the ovary or not. Surgery is the mainstay of treatment for mature teratoma.

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