Germ cell tumor classification: Difference between revisions

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Germ cell tumors can be classified based on their histologic features into:


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{{familytree| | | | | | | | | A01 | | | |A01=Germ cell tumors}}
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{{familytree| | | | B01 | | | | | | | | | B02 | | B01=Germinomatous/Undifferentiated/Immature|B02=Nongerminomatous/Differentiated/Embryonal}}
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{{familytree| | C01 | | | C02 | | | | | | |!| | | | | C01=[[Dysgerminoma(Ovary)]]|C02=[[Seminoma(Testis)]]}}
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{{familytree| | | | D01 | | | | | | D02 | | | | | D03 | | |D01=[[Embryonal carcinoma]]| D02=[[embryonic tissue]]| D03=[[Extraembryonic tissue]]}}
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{{familytree| | | | | | | | | | | | E01 | | | | | |!| | |E01=[[Teratoma]]}}
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{{familytree| | | | | | | | | | | | | | | | | F01 | | F02 | |F01=[[Yolk sac tumor]]|F02=[[Choriocarcinoma]]}}
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{{familytree| | | | | | | | | A01 | | | |A01=Germ cell tumors}}
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{{familytree| | | | B01 | | | | | | | | | B02 | | B01=Germinomatous/Undifferentiated/Immature|B02=Nongerminomatous/Differentiated/Embryonal}}
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{{familytree| | C01 | | | C02 | | | | | | |!| | | | | C01=[[Dysgerminoma(Ovary)]]|C02=[[Seminoma(Testis)]]}}
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{{familytree| | | | |!| | | | | | | |!| | | | | | |!| | |}}
{{familytree| | | | D01 | | | | | | D02 | | | | | D03 | | |D01=[[Embryonal carcinoma]]| D02=[[embryonic tissue]]| D03=[[Extraembryonic tissue]]}}
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{{familytree| | | | | | | | | | | | E01 | | | E02 | | E03| | |E01=[[Teratoma]]|E02=[[Yolk sac tumor]]|E03=[[Choriocarcinoma]]}}
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Germ cell tumors classification is based on the histologic features and whether they are differentiated or not into:
* Germinomatous tumors: (non-differentiated): Gonadal and Extra-gonadal
** Germinoma ( Gonadal: dysgerminoma and seminoma), (Extra-gonadal: mediastinum, or pineal region)
** Dysgerminoma (Ovary)
** Seminoma (testes)
* Nongerminomatous tumors: all other germ-cell tumors, pure and mixed
** Embryonal carcinoma
** Endodermal sinus tumor, also known as yolk sac tumor (EST, YST)
** Choriocarcinoma
** Teratoma including mature teratoma, dermoid cyst, immature teratoma, teratoma with malignant transformation
** Polyembryoma
** Gonadoblastoma
* Mixed germ cell tumors:
Germ cell tumors can also be classified based on their location into:
* Gonadal (ovary and testes)
* Extra-gonadal (MC:mediastinum, retroperitoneal. Less common: Pineal gland, sacrococcigeal) 
* Ovarian germ cell tumors (OGCTs ): The histologic types that arise from the ovary are similar to those arising from the testes of men
** Embryo-like neoplasms
*** Teratomas and their subtypes
*** Dysgerminomas: The female version of the male seminoma (comprised of immature germ cells)
** Extraembryonic fetal-derived (placenta-like) cell populations
*** Yolk sac/primitive placenta forms (epithelial neoplasms differentiate into yolk sac tumors)
** Rare OGCTs
*** Pure embryonal carcinomas
*** Nongestational choriocarcinomas
*** Pure polyembryoma.
** Mixed germ cell tumors (teratoma with yolk sac, dysgerminoma, and/or embryonal carcinoma)
* Extragonadal germ cell tumors (GCTs): no evidence of a primary tumor in the testes or ovaries
** Typically arise in midline locations,
** Specific sites vary with age
** The most common sites in order of frequency
** In adults:
*** Anterior mediastinum
**** Mature teratomas
**** Immature teratoma
**** Mediastinal seminoma
**** Mediastinal non-seminomatous GCT: 
***** Yolk sac tumor (most common, pure or mixed)
***** Choriocarcinoma (less common)
***** Embryonal carcinoma (infrequent)
***** Mixed GCTs (a mixture of teratoma, seminoma, and other cell types)
*** Retroperitoneum
**** Retroperitoneal seminoma
**** Retroperitoneal non-seminomatous GCTs (Embryonal carcinoma is common)
**** Retroperitoneal teratomas (rare)
*** Pineal and suprasellar regions
** In infants and young children:
*** Sacrococcygeal
*** Intracranial GCTs
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF| Types}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF|Subtypes}}
! colspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF|Signs and Symptoms}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF|Histopathology}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF| Lab finding }}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF| Prognosis}}
|-
| rowspan="2;" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" |Germinomatous
/Undifferentiated
<br />
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Seminoma (Testis)
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* Painless [[testicular mass]] with discomfort
*[[Back pain]]
*[[Abdominal discomfort]]
*[[Abdominal mass]].
| style="padding: 5px 5px; background: #F5F5F5;" |Gross: pale gray to yellow nodules that are uniform or slightly lobulated and often bulge from the cut surface
| style="padding: 5px 5px; background: #F5F5F5;" |
* Complete blood count and blood chemistry tests.
* Abnormal serum tumor marker levels ([[LDH]], [[HCG]]).<ref name="Diagnosisoftesticularcancer1" />
* CT: Metastases to the para-aortic, inguinal, or iliac lymph nodes. Visceral metastasis may also be seen.
* Pelvic MRI: may be diagnostic. multinodular tumors of uniform signal intensity
* Hypo- to isointense on T2-weighted images and inhomogenous enhancement on contrast enhanced T1-weighted images.
* Other diagnostic studies for seminoma include [[biopsy]], [[PET|FDG-PET scan]], and [[bone scan]].
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Prognosis]] of [[seminoma]] is good for all stages with greater than 90% cure rate.
* The International Germ Cell Cancer Consensus Group divides [[seminoma]] into two prognosis groups: good and intermediate.
* Common complications of [[seminoma]] include recurrence, [[lymph node]] [[metastasis]], distant [[metastasis]], and secondary [[malignancies]].
|-
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Dysgerminoma
(Ovary)
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* Depend on the type of the [[tumor]] and its potential to produce [[hormonal]]<nowiki/>materials
*[[Abdominal pain]] or 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]].
| style="padding: 5px 5px; background: #F5F5F5;" |
*The majority of [[ovarian]] [[germ cell]][[tumors]] have a [[solid]] and [[cystic]] appearance with areas of [[hemorrhage]]<nowiki/>and [[necrosis]]
* A uniform “fried egg” appearance ([[dysgerminoma]])
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Beta-hCG]] to rule out [[pregnancy]] in women with abdominopelvic [[symptoms]]
*Cultures for [[gonorrhea]] and [[chlamydia]] and a wet mount in [[reproductive]] and [[sexually active]] women to role out and treat before [[surgery]] if [[positive]].
*[[Lactate dehydrogenase]] ([[LDH]]), [[alpha-fetoprotein]] ([[AFP]]), [[beta-human chorionic gonadotropin]] ([[beta-hCG]]) levels. If any levels are elevated, they may assist in [[diagnosis]] and/ or follow-up of women [[Diagnosis|diagnosed]] with [[malignant]] [[Ovarian germ cell tumor|ovarian GCTs]].
*[[Inhibin A]] and B
*[[CA-125|Cancer antigen 125]] ([[CA-125]]) - For epithelial tumors
*[[Ultrasound]]: [[Dysgerminoma]] often appears as a [[Echogenicity|hypoechoic]] [[mass]]
*
*
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* Chemotherapy: except those with stage 1a, stage 1a, 1b [[dysgerminoma]]
* Radiotherapy:
<nowiki>**</nowiki>  [[Dysgerminoma]] is radiosensitive.
[[Radiotherapy|** Radiotherapy]] is not anymore the first option of treatment for [[dysgerminoma]] considering its association with [[ovarian failure]]<nowiki/>development.
* Surgery: for diagnostic grading and therapy depending on if the patient prefers to preserve the ovary or not.
<br />
|-
| rowspan="6;" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" | Germinomatous/
Differentiated
| rowspan="1;" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" | Embryonic
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| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*
|-
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Teratoma
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Chest pain]]
*[[Cough]]
*[[Shortness of breath]]
*[[Abdominal pain]]
*[[Lump]], Abdominal(ovarian teratoma)
* Abnormal [[bleeding]] from the vagina
*[[Fatigue]], [[weight loss]]
* Limited ability to tolerate exercise
| style="padding: 5px 5px; background: #F5F5F5;" |
* Teratomas belong to a class of tumors known as [[Nonseminoma|nonseminomatous]] [[germ cell tumor]] (NSGCT).
* All tumors of this class are the result of abnormal development of [[pluripotent]] cells: [[Germ cell|germ cells]] and [[Embryo|embryonal cells]].
* Teratomas of embryonal origin are [[Congenital disorder|congenital]]; teratomas of germ cell origin may or may not be congenital (this is not known).
* Embryonal teratomas most commonly occur in the sacrococcygeal region: [[sacrococcygeal teratoma]] is the single most common tumor found in [[Infant|newborn babies]].
| style="padding: 5px 5px; background: #F5F5F5;" |
* AFP
* MSAFP
* CT scans are often used to diagnose teratoma.
<br />
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* For malignant teratomas, usually, surgery is followed by chemotherapy.
* Teratomas that are in surgically inaccessible locations, or are very complex, or are likely to be malignant (due to late discovery and/or treatment) sometimes are treated first with chemotherapy.
|-
| rowspan="1;" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" |
Extraembryonic
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|-
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[[Choriocarcinoma]]([[Gestational Trophoblastic Neoplasia]])
| style="padding: 5px 5px; background: #F5F5F5;" |Early Symptoms:
*[[Vaginal bleeding]] <ref name="xxx">Signs and symptoms of gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/signs-and-symptoms/?region=ns Accessed on October 10, 2015</ref>
*[[Nausea]] and [[vomiting]]
*Passing of [[Tissue (biology)|tissue]] resembling a “bunch of grapes” from the [[vagina]]
*Absent [[fetal]] movement during [[pregnancy]]
*[[Abdomen|Abdominal]] distension 
Rare Symptoms:
*[[Headache]]
*[[Edema ]]of the [[Hand|hands]] and feet
*[[Abdomen|Abdominal]] or [[Pelvis|pelvic]] pain
*[[Vaginal discharge]]
*Overactive [[thyroid gland]] ([[hyperthyroidism]]) that causes:
*[[Tachycardia]]
*[[Sweating]]
*Shaking
*Heat intolerance
*[[Fever]]
Late Symptoms
*[[Hemoptysis]]
*Dry [[cough]]
*[[Chest pain]]
*Trouble [[breathing]]
*[[Headache]]
*[[Dizziness]]
*[[Jaundice]]
*[[Paralysis]]
*[[Seizure]]
*[[Dysarthria]] and [[dysphasia]]
*[[Visual system|Vision]] problems
*[[Lump]] in the [[vagina]]
| style="padding: 5px 5px; background: #F5F5F5;" |[[Gross pathology|Gross pathological]]:
* Bulky, destructive mass with [[Bleeding|hemorrhage]] and [[necrosis]]<ref name="OberEdgcomb1971">{{cite journal|last1=Ober|first1=William B.|last2=Edgcomb|first2=John H.|last3=Price|first3=Edward B.|title=THE PATHOLOGY OF CHORIOCARCINOMA|journal=Annals of the New York Academy of Sciences|volume=172|issue=10 Physiology a|year=1971|pages=299–426|issn=0077-8923|doi=10.1111/j.1749-6632.1971.tb34943.x}}</ref><ref name="SmithKohorn2005">{{cite journal|last1=Smith|first1=Harriet O.|last2=Kohorn|first2=Ernest|last3=Cole|first3=Laurence A.|title=Choriocarcinoma and Gestational Trophoblastic Disease|journal=Obstetrics and Gynecology Clinics of North America|volume=32|issue=4|year=2005|pages=661–684|issn=08898545|doi=10.1016/j.ogc.2005.08.001}}</ref>
* Can be associated with deep [[Myometrium|myometrial]] invasion
[[Microscopic]] [[Histopathology|histopathological:]]
*Columns and sheets of [[Trophoblast|trophoblastic]] [[Tissue (biology)|tissue]] invading [[Uterus|uterine]] [[muscle]] and [[Blood vessel|blood vessels]]
*[[Syncytiotrophoblast|Syncytiotrophoblasts]] (large [[eosinophilic]] smudgy [[Multinucleate|multinucleated]] [[Cell (biology)|cells]] with large [[Hyperchromicity|hyperchromatic]] [[Cell nucleus|nuclei]]) are intermixed with [[Cytotrophoblast|cytotrophoblasts]] (polygonal [[Cell (biology)|cells]] with distinct borders, and single irregular [[Cell nucleus|nuclei]])
<br />
| style="padding: 5px 5px; background: #F5F5F5;" |[[Human chorionic gonadotropin]] (HCG or b-HCG) is the most common [[tumor]] marker test used to diagnose GTD<ref name="abc">Diagnosing gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/diagnosis/?region=ns Accessed on October 13, 2015</ref>
*Human placental lactogen (hPL) is a tumor marker that may be used to follow women with placental site [[trophoblastic]] tumors
* Elevated hPL levels are found in women with some types of GTD
*[[Complete blood count]] can check for [[anemia]] from long-term (chronic) [[vaginal bleeding]]
<br />
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|-
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[[Yolk sac tumor]]
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* On microscopic [[pathology]]
* Presence of Schiller-Duval bodies ([[yolk sac tumor]])
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|-
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==References==
{{reflist}}

Latest revision as of 14:23, 23 September 2019