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This article covers '''germ cell tumours''' which | [[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]]. | |||
== | ==Overview== | ||
===Germ cell tumour - types=== | |||
Precusor: | |||
*[[Intratubular germ cell neoplasia]]. | |||
Germ cell tumours (GCTs): | |||
*[[Germinoma]]/[[Seminoma]]/[[Dysgerminoma]]. | |||
*[[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]]. | |||
= | ===Grossing=== | ||
{{Main|Orchiectomy}} | |||
* | *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> | ||
=== | ===IHC for GCTs=== | ||
====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}} | |||
=== | ====ABCDs of GCTs==== | ||
*AFP - yolk sac tumour. | |||
* | *Beta-hCG - choriocarcinoma. | ||
* | *CD30 - embryonal carcinoma. | ||
* | *D2-40 - seminoma. | ||
* | |||
====GCT versus sex cord-stromal tumour==== | |||
*[ | 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). | |||
Alternates for SALL4 are:<ref name=pmid25025364/> | |||
*OCT4 and GPC3. | |||
* | |||
Image: | ===Tabular summary of GCTs=== | ||
{| 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)]] | |||
|} | |||
=== | ===Molecular pathology=== | ||
Most common cytogenetic abnormality in GCTs: | |||
*[[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> | |||
* | ** Isochromosome = one arm (p or q) is lost and replaced with a duplicate of the remaining one. | ||
* | ***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> | ||
* | |||
== | ==Germinoma== | ||
* | Comes in three flavours: | ||
*Germinoma. | |||
*Seminoma. | |||
*Dysgerminoma. | |||
== | ===Germinoma=== | ||
Is the generic version of this tumour. It is found in the midline ([[brain tumour|brain]], [[mediastinum]]). | |||
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> | |||
<gallery> | |||
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> | |||
=== | ===Seminoma=== | ||
{{Main|Seminoma}} | |||
A common GCT in males. | |||
=== | ===Dysgerminoma=== | ||
{{Main|Ovarian tumours#Dysgerminoma}} | |||
A common GCT in females. | |||
== | ==Yolk sac tumour== | ||
{{Main|Yolk sac tumour}} | |||
=== | ==Embryonal carcinoma== | ||
{{Main|Embryonal carcinoma}} | |||
==Choriocarcinoma== | |||
{{Main|Choriocarcinoma}} | |||
==Teratoma== | ==Teratoma== | ||
{{Main|Teratoma}} | |||
== | ==Gonadoblastoma== | ||
{{Main|Gonadoblastoma}} | |||
==Polyembryoma== | |||
{{Main|Polyembryoma}} | |||
=== | ==Mixed germ cell tumour== | ||
{{Main|Mixed germ cell tumour}} | |||
==See also== | ==See also== | ||
*[[Testis]]. | *[[Testis]]. | ||
*[[Ovarian tumours]]. | *[[Ovarian tumours]]. | ||
*[[Trophoblast]]. | |||
*[[Sex cord-stromal tumours]]. | |||
==References== | ==References== | ||
Line 173: | Line 184: | ||
[[Category:Gynecologic pathology]] | [[Category:Gynecologic pathology]] | ||
[[Category:Genitourinary pathology]] | [[Category:Genitourinary pathology]] | ||
[[Category:Germ cell tumours|Germ cell tumours]] |
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