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This article covers '''germ cell tumours''' which classicaly arise in the gonads ([[ovary]], [[testis]]).  They also show-up in [[neuropathology]] and in the mediastinum.
[[Image:Mixed_Germ_Cell_Tumor_of_Testis_(3260625567).jpg|thumb|right|Testicular [[mixed germ cell tumour]]. (WC/euthman)]]
This article covers '''germ cell tumours''', often abbreviated [[GCT]], which classically arise in the gonads ([[ovary]], [[testis]]).  They are also found in the midline and make appearances in [[neuropathology]] (e.g. [[pineal gland]]) and in the [[mediastinum]].


==Seminoma==
==Overview==
{{Main|Testis#Seminoma}}
===Germ cell tumour - types===
A common GCT in males.
Precusor:
*[[Intratubular germ cell neoplasia]].


==Dysgerminoma==
Germ cell tumours (GCTs):
{{Main|Ovarian tumours#Dysgerminoma}}
*[[Germinoma]]/[[Seminoma]]/[[Dysgerminoma]].
A common GCT in females.
*[[Yolk sac tumour]] (endodermal sinus tumour).
*[[Embryonal carcinoma]].
*[[Choriocarcinoma]].
*[[Teratoma]].
*[[Mixed germ cell tumour]] (mixed GCT) - 60% of GCTs are mixed.
**Common combinations:
**# teratoma + embryonal carcinoma + endodermal sinus tumour (yolk sac tumour) (TEE).
**# seminoma + embryonal (SE).
**# embryonal + teratoma (TE).
*[[Gonadoblastoma]].
*[[Polyembryoma]].


==Yolk sac tumour==
===Grossing===
===General===
{{Main|Orchiectomy}}
*Tumour also known as ''endodermal sinus tumour''.
*1 cm<sup>2</sup> of tumour per cm of maximal tumour dimension - guideline for testicular cancer.<ref>URL: [http://www.uroweb.org/gls/pdf/10_Testicular_Cancer.pdf http://www.uroweb.org/gls/pdf/10_Testicular_Cancer.pdf]. Accessed on: 30 October 2012.</ref>


===Epidemiology===
===IHC for GCTs===
*Most common GCT in infants and young boys.
====ISUP====
An algorithmic approach based on the ISUP consensus paper by Ulbright ''et al.'':<ref name=pmid24832161>{{cite journal |author=Ulbright TM, Tickoo SK, Berney DM, Srigley JR |title=Best practices recommendations in the application of immunohistochemistry in testicular tumors: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=e50–9 |year=2014 |month=August |pmid=24832161 |doi=10.1097/PAS.0000000000000233 |url=}}</ref>
{{familytree/start}}
{{familytree | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | |A01=[[Germ cell tumours]]}}
{{familytree | | | | | |,|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|.| | | | | | | | | | | |}}
{{familytree | | | | | B01 | | | | | | | | | | | | | | B02 | | | | | | | | | | |B01=OCT4 +ve | B02=OCT4 -ve}}
{{familytree | |,|-|-|-|^|-|-|-|.| | | | | |,|-|-|-|-|-|+|-|-|-|-|-|.| | | | | |}}
{{familytree | C01 | | | | | | C02 | | | | C03 | | | | C04 | | | | C05 | | | | |C01=CD117 +ve<br>CD30 -ve |C02= CD117 -ve<br>CD30 +ve|C03=[[Glypican 3]] +ve<br>AFP +ve<br>beta-hCG -ve|C04=Glypican 3 ?<br>AFP -ve<br>beta-hCG +ve|C05=Glypican 3 -ve<br>AFP -ve<br>beta-hCG -ve}}
{{familytree | |!| | | | | | | |!| | | | | |!| | | | | |!| | | | | |!| | | | | |}}
{{familytree | D01 | | | | | | D02 | | | | D03 | | | | D04 | | | | D05 | | | | |D01=[[Seminoma]]|D02=[[Embryonal carcinoma]]|D03=[[Yolk sac tumour]]|D04=[[Choriocarcinoma]] |D05= [[Spermatocytic seminoma]] }}
{{familytree/end}}


===Microscopy===
====ABCDs of GCTs====
Classic feature:
*AFP - yolk sac tumour.
*Schiller-Duval bodies.
*Beta-hCG - choriocarcinoma.
**Look like glomerulus - central blood vessel surrounded by epithelial-like cells a space and more epithelial-like cells
*CD30 - embryonal carcinoma.
*Architecure - variable.
*D2-40 - seminoma.
**Most common ''microcystic pattern''.<ref>URL: [http://webpathology.com/image.asp?case=34&n=1 http://webpathology.com/image.asp?case=34&n=1]. Accessed on: March 8, 2010.</ref>


Image:  
====GCT versus sex cord-stromal tumour====
*[http://www.webpathology.com/image.asp?case=34&n=5 Schiller-Duval body (webpathology.com)].
The ISUP recommends:<ref name=pmid25025364>{{cite journal |author=Amin MB, Epstein JI, Ulbright TM, ''et al.'' |title=Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference |journal=Am. J. Surg. Pathol. |volume=38 |issue=8 |pages=1017–22 |year=2014 |month=August |pmid=25025364 |doi=10.1097/PAS.0000000000000254 |url=}}</ref>
*[[SALL4]] +ve (in GCTs).<ref name=pmid24525512>{{cite journal |author=Miettinen M, Wang Z, McCue PA, ''et al.'' |title=SALL4 expression in germ cell and non-germ cell tumors: a systematic immunohistochemical study of 3215 cases |journal=Am. J. Surg. Pathol. |volume=38 |issue=3 |pages=410–20 |year=2014 |month=March |pmid=24525512 |doi=10.1097/PAS.0000000000000116 |url=}}</ref>
*Alpha-inhibin -ve (+ve in [[SCST]]).
*Calretinin -ve (+ve in SCST).


Variants:
Alternates for SALL4 are:<ref name=pmid25025364/>
*Hepatoid pattern.<ref>URL: [http://webpathology.com/image.asp?case=34&n=6 http://webpathology.com/image.asp?case=34&n=6]. Accessed on: March 8, 2010.</ref>
*OCT4 and GPC3.
**Vaguely resembles liver.
***Hyaline globules (light red well-circumscribed globs).
***Bile canaculi.
*Solid pattern.<ref>URL: [http://webpathology.com/image.asp?case=34&n=8 http://webpathology.com/image.asp?case=34&n=8]. Accessed on: March 8, 2010.</ref>
**Vaguely resembles ''seminoma''.


Image:
===Tabular summary of GCTs===
*[http://webpathology.com/image.asp?case=34&n=6 Hepatoid Pattern].
{| class="wikitable sortable"
! Tumour
! Key feature
! Microscopic
! IHC
! Other
! Image
|-
| [[Germ cell neoplasia in situ]] (GCNIS)
| nests of small fried egg cells
| large central nucleus, clear <br>cytoplasm, squared-off nuclear membrane, nucleoli<ref name=Ref_GUP538>{{Ref GUP|538}}</ref>
| CD117 +ve, CD30 -ve
| appearance similar to [[seminoma]]
| [[Image:Intratubular_germ_cell_neoplasia_high_mag_cropped.jpg|thumb|center|150px|GCNIS (WC/Nephron)]]
|-
| [[Germinoma]] / [[Seminoma]] / [[Dysgerminoma]]
| fried egg cells
| fried egg-like cells (central nucleus, clear <br>cytoplasm) with squared-off nuclear <br>membrane, nucleoli, lymphocytic infiltrate, granulomata,<br>syncytiotrophoblastic giant cells<ref name=Ref_GUP542>{{Ref GUP|542}}</ref>
| D2-40
| seminoma = male version of this tumour; dysgerminoma = female version of this tumour
| [[Image:Seminoma_intermed_mag.jpg|thumb|center|150px|Seminoma (WC/Nephron)]]
|-
| [[Yolk sac tumour]] (endodermal sinus tumour)
| Schiller-Duval bodies
| Schiller-Duval b. = central blood vessel surrounded by epithelial-like cells a space and more epithelial-like cells, variable arch.
| [[AFP]] +ve, [[Glypican 3]] +ve
| patterns: microcystic, solid, hepatoid
| [[Image:Mixed_germ_cell_tumour_-_very_high_mag.jpg|thumb|center|150px|Yolk sac tumour (WC)]]
|-
| [[Embryonal carcinoma]]
| prominent nucleoli, vescicular nuclei
| var. arch.: tubulopapillary, glandular, solid, embryoid bodies (ball of cells in surrounded by empty space on three sides), +/-nuclear overlap, mitoses common
| CD30 +ve, CD117 -ve
| usu. part of a mixed GCT
| [[Image:Embryonal_carcinoma_high_mag.jpg|thumb|center|150px|Embryonal carcinoma (WC/Nephron)]]
|-
| [[Choriocarcinoma]]
| clear cytoplasm
| cells with abundant clear cytoplasm and eccentric atypical nuclei (cytotrophoblast), very large (multinucleated) cells with abundant eosinophilic cytoplasm and extreme nuclear atypia (syncytiotrophoblast)
| beta-hCG +ve
| may be preceded by a [[complete mole|complete hydatidiform mole]]
| [[Image:Choriocarcinoma_-2-_very_high_mag.jpg|thumb|center|150px|Choriocarcinoma. (WC/Nephron)]]
|-
| [[Teratoma]], immature
| primitive neuroepithelium
| pseudostratified epithelium in rosettes (gland-like arrangement)
| None
| testicular teratomas in post-pubertal males are all considered malignant<ref>{{Cite journal  | last1 = Carver | first1 = BS. | last2 = Al-Ahmadie | first2 = H. | last3 = Sheinfeld | first3 = J. | title = Adult and pediatric testicular teratoma. | journal = Urol Clin North Am | volume = 34 | issue = 2 | pages = 245-51; abstract x | month = May | year = 2007 | doi = 10.1016/j.ucl.2007.02.013 | PMID = 17484929 }}</ref>
| [[Image:Primitive_neuroepithelium_intermed_mag.jpg|thumb|center|150px|Primitive neuroepithelium (WC/Nephron)]]
|-
| [[Mixed germ cell tumour]]
| NA
| common combinations: teratoma + embryonal carcinoma + endodermal sinus tumour (yolk sac tumour) (TEE); seminoma + embryonal (SE); embryonal + teratoma (TE)
| NA
| -
| [[Image:Mixed_germ_cell_tumour_-_intermed_mag.jpg|thumb|center|150px|Mixed GCT (WC/Nephron)]]
|-
| [[Gonadoblastoma]]
| primitive germ cells (central nucleus, moderate (eosinophilic) cytoplasm); sex cord element
| sex cord element may be either granulosa cells (follicle-like arch.) or Sertoli cells (trabecular arch.)
| ?
| often abnormal karyotype; usu. Y chromosome present
| [[Image:Gonadoblastoma_-_intermed_mag.jpg|thumb|center|150px|Gonadoblastoma (WC/Nephron)]]
|}


===IHC===
===Molecular pathology===
*AFP +ve.
Most common cytogenetic abnormality in GCTs:
*Glypican 3 +ve.
*[[Isochromosome 12p]].<ref name=pmid10357096>{{Cite journal  | last1 = Looijenga | first1 = LH. | last2 = Oosterhuis | first2 = JW. | title = Pathogenesis of testicular germ cell tumours. | journal = Rev Reprod | volume = 4 | issue = 2 | pages = 90-100 | month = May | year = 1999 | doi = | PMID = 10357096 }}</ref>
**More sensitive than AFP.<ref name=pmid20438407>{{Cite journal  | last1 = Emerson | first1 = RE. | last2 = Ulbright | first2 = TM. | title = Intratubular germ cell neoplasia of the testis and its associated cancers: the use of novel biomarkers. | journal = Pathology | volume = 42 | issue = 4 | pages = 344-55 | month = Jun | year = 2010 | doi = 10.3109/00313021003767355 | PMID = 20438407 }}</ref>
** Isochromosome = one arm (p or q) is lost and replaced with a duplicate of the remaining one.
*Alpha-1 AT +ve.
***Example: isochromosome 12p = chromosome 12 where q is lost and two p arms are present.<ref>URL: [http://ghr.nlm.nih.gov/handbook/illustrations/isochromosomes http://ghr.nlm.nih.gov/handbook/illustrations/isochromosomes]. Accessed on: 15 February 2012.</ref>
*Cytokeratin +ve. ???


===DDx===
==Germinoma==
*Embryonal carcinoma.
Comes in three flavours:
*Germinoma.
*Seminoma.
*Dysgerminoma.


==Embryonal carcinoma==
===Germinoma===
===General===
Is the generic version of this tumour. It is found in the midline ([[brain tumour|brain]], [[mediastinum]]).
*Affects young adults.
**May be seen in women.


===Microscopic===
Image: [http://path.upmc.edu/cases/case525/images/fig02a.jpg Germinoma (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case525.html http://path.upmc.edu/cases/case525.html]. Accessed on: 25 January 2012.</ref>
Features:<ref name=Ref_GUP549>{{Ref GUP|549}}</ref>
#Nucleoli - '''key feature'''.
#Vesicular nuclei (clear, empty appearing nuclei) - '''key feature'''.
#Nuclei overlap.
#[[Necrosis]] - common.
#*Not commonly present in seminoma.
#Indistinct cell borders
#Mitoses - common.
#Variable architecture:
#*Tubulopapillary.
#*Glandular.
#*Solid.  
#*Embryoid bodies - ball of cells in surrounded by empty space on three sides.


Notes:
<gallery>
*Cytoplasmic staining variable (eosinophilic to basophilic).
Image:Neuropathology_case_VIII_01.jpg | Germinoma  of the brain, low mag. (WC/jensflorian)
Image:Neuropathology_case_VIII_02.jpg | Germinoma intermed. mag. (WC/jensflorian)
Image:Neuropathology_case_VIII_03.jpg | Germinoma very high. mag. (WC/jensflorian)
Image:Neuropathology_case_VIII_05.jpg | Germinoma Oct3/4 staining (WC/jensflorian)
</gallery>


Images:
*Set 1:
**[http://commons.wikimedia.org/wiki/File:Embryonal_carcinoma_-_very_high_mag_-_cropped.jpg Embryonal carcinoma - very high mag. - cropped (WC)].
**[http://commons.wikimedia.org/wiki/File:Embryonal_carcinoma_-_high_mag.jpg Embryonal carcinoma - high mag. (WC)].
*Set 2:
**[http://commons.wikimedia.org/wiki/File:Embryonal_carcinoma_high_mag.jpg Embryonal carcinoma - high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Embryonal_carcinoma_intermed_mag.jpg Embryonal carcinoma - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Embryonal_carcinoma_low_mag.jpg Embryonal carcinoma - low mag. (WC)].


===DDx===
===Seminoma===
*Yolk sac tumour.
{{Main|Seminoma}}
A common GCT in males.


===IHC===
===Dysgerminoma===
*AE1/AE3 +ve.
{{Main|Ovarian tumours#Dysgerminoma}}
*CD30 +ve.
A common GCT in females.


==Choriocarcinoma==
==Yolk sac tumour==
===General===
{{Main|Yolk sac tumour}}
*Aggressive clinical course.


===Microscopic===
==Embryonal carcinoma==
Features:
{{Main|Embryonal carcinoma}}
*Syncytiotrophoblasts:
**Large + many irreg. or lobular hyperchromatic nuclei.
**Eosinophilic vacuolated cytoplasm (contains hCG).
*'''C'''ytotrophoblasts:
**'''C'''lear cytoplasm.
**Polygonal shaped cells in cords/masses.
**Distinct cell borders.
**Single uniform nucleus.
*+/-Hemorrhage.
*+/-Necrosis.


Image(s):
==Choriocarcinoma==
*[http://www.webpathology.com/image.asp?case=36&n=1 Choriocarcinoma (webpathology.com)]
{{Main|Choriocarcinoma}}
 
Notes:
*See: ''[[Chorionic villi]]''.
 
===IHC===
*beta-hCG +ve.


==Teratoma==
==Teratoma==
===General===
{{Main|Teratoma}}
*Consists of all three [[germ layers]]:<ref>{{cite book |author=Moore, Keith L.; Persaud, T.V.N.|title=The Developing Human: Clinically Oriented Embryology |publisher=Saunders |location= |year=2002 |pages= 83 |edition=7th |isbn=978-0721694122 |oclc= |doi= |accessdate=}}</ref>
*#Endoderm:
*#*Skin, CNS.
*#Mesoderm:
*#*Muscle, bone, connective tissue, blood.
*#Ectoderm:
*#*Internal organs.
 
===Classification===
*Divided into:
**Mature.
**Immature.
 
===Immature===
*Immature if neural tissue is present:<ref>RS. 2 May 2010.</ref>
**Vaguely resembles pseudostratified respiratory epithelium.
*Islands of small hyperchromatic cells - "blastema".
*+/-Cartilage.
*+/-Adipocytes.
*+/-Colonic type mucosa.
*+/-Stratified squamous epithelium (skin).
 
Images:
*[http://commons.wikimedia.org/wiki/File:Immature_teratoma_high_mag.jpg Primitive neuroepithelium - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Primitive_neuroepithelium_intermed_mag.jpg Primitive neuroepithelium - intermed mag. (WC)]
*[http://commons.wikimedia.org/wiki/File:Teratoma_intermed_mag.jpg Teratoma - mature components (WC)].
 
Other images:
*[http://www.webpathology.com/image.asp?n=5&Case=35 Immature teratoma - myxomatous stroma (webpathology.com)].
*[http://www.webpathology.com/image.asp?n=6&Case=35 Immature teratoma - blastema (webpathology.com)].
*[http://www.webpathology.com/image.asp?n=7&Case=35 Immature teratoma - primitive neuroepithelium (webpathology.com)].
*[http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675%2806%2970278-9&figureId=fig2 Immature teratoma - primitive neuroepithelium (pathconsultddx.com)].
*[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/N0C001-PQ01-M.htm Immature teratoma - primitive neuroepithelium (ouhsc.edu)].


===Grading===
==Gonadoblastoma==
Based on quantity of immature neuroepithelium:<ref name=pmid17080330>{{cite journal |author=Harms D, Zahn S, Göbel U, Schneider DT |title=Pathology and molecular biology of teratomas in childhood and adolescence |journal=Klin Padiatr |volume=218 |issue=6 |pages=296–302 |year=2006 |pmid=17080330 |doi=10.1055/s-2006-942271 |url=}}</ref><ref name=pmid15761467>{{cite journal |author=Ulbright TM |title=Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues |journal=Mod. Pathol. |volume=18 Suppl 2 |issue= |pages=S61–79 |year=2005 |month=February |pmid=15761467 |doi=10.1038/modpathol.3800310 |url=http://www.nature.com/modpathol/journal/v18/n2s/full/3800310a.html}}</ref><ref name=pmid7814189>{{cite journal |author=O'Connor DM, Norris HJ |title=The influence of grade on the outcome of stage I ovarian immature (malignant) teratomas and the reproducibility of grading |journal=Int. J. Gynecol. Pathol. |volume=13 |issue=4 |pages=283–9 |year=1994 |month=October |pmid=7814189 |doi= |url=}}</ref>
{{Main|Gonadoblastoma}}
*G0 - mature teratoma; no immature neuroepithelium.
*G1 - less than one lower power field ([[LPF]]) of immature neuroepithelium; LPF defined field at 4X magnification.
*G2 - 1-3 LPFs.
*G3 - more than 3 LPFs.


Note:
==Polyembryoma==
*LPF not adequately defined - see [[LPFitis]]. Same BS as HPF.
{{Main|Polyembryoma}}


===IHC (immature)===
==Mixed germ cell tumour==
Features:
{{Main|Mixed germ cell tumour}}
*Primitive neuroepithelium:<ref name=pmid11598856>{{cite journal |author=Craver RD, Lipscomb JT, Suskind D, Velez MC |title=Malignant teratoma of the thyroid with primitive neuroepithelial and mesenchymal sarcomatous components |journal=Ann Diagn Pathol |volume=5 |issue=5 |pages=285–92 |year=2001 |month=October |pmid=11598856 |doi=10.1053/adpa.2001.27918 |url=}}</ref>
**Neuron-specific enolase (NSE) +ve.
**Neuron-specific B tubulin +ve.
**Synaptophysin +ve.


==See also==
==See also==
*[[Testis]].
*[[Testis]].
*[[Ovarian tumours]].
*[[Ovarian tumours]].
*[[Trophoblast]].
*[[Sex cord-stromal tumours]].


==References==
==References==
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[[Category:Gynecologic pathology]]
[[Category:Gynecologic pathology]]
[[Category:Genitourinary pathology]]
[[Category:Genitourinary pathology]]
[[Category:Germ cell tumours|Germ cell tumours]]
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