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/start}}
{{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 }}
! colspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF| Treatment }}
! 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
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* 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]].
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*[[Orchiectomy|Radical inguinal orchiectomy]] is the first treatment for any stage of testicular seminoma. Usually done as diagnostic and therapeutic.
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*[[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]].
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*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]])
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*[[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]]
*
*
*
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* 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 />
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* The 5-year [[survival rate]] of the patient even with [[Disseminated disease|disseminated]][[dysgerminoma]] at the time of [[diagnosis]] is above 90%.
|-
| 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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*
|-
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Teratoma
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*[[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
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* 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]].
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* 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.
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The prognosis of teratoma depends on the following:
:* Whether or not the tumor can be removed by surgery.
:* The size and location of the tumor
:* The patient’s general health
:* Teratomas are not dangerous for the fetus unless there is either a [[Mass effect (medicine)|mass effect]] or a large amount of blood flow through the tumor (known as ''vascular steal''). The mass effect frequently consists of obstruction of normal passage of fluids from surrounding organs. The vascular steal can place a strain on the growing heart of the fetus, even resulting in heart failure, and thus must be monitored by fetal [[echocardiography]].  After surgery, there is a risk of regrowth in place, or in nearby organs [[Teratoma natural history#cite%20note-1|[1]]]
|-
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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
<br />
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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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==References==
{{reflist}}

Latest revision as of 14:23, 23 September 2019