Difference between revisions of "Germ cell tumours"

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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.
This article covers '''germ cell tumours''' which classicaly arise in the gonads ([[ovary]], [[testis]]).  They also show-up in [[neuropathology]] and in the mediastinum.
==Overview==
*Germ cell tumours (GCTs).
**Intratubular germ cell neoplasia.
**Germinoma/Seminoma/Dysgerminoma.
**Yolk sac tumour (endodermal sinus tumour).
**Embryonal carcinoma.
**Choriocarcinoma.
**Teratoma.
**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).
===IHC for GCTs===
ABCDs of GCTs:
*AFP - yolk sac tumour.
*Beta-hCG - choriocarcinoma.
*CD30 - embryonal carcinoma.
*D2-40 - seminoma.
===Tabular summary of GCTs===
{| class="wikitable"
!| Tumour
!| Key feature
!| Microscopic
!| IHC
!| Other
!| Image
|-
| Intratubular germ cell neoplasia (ITGCN)
| 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
| appearance similar to seminoma
| [http://commons.wikimedia.org/wiki/File:Intratubular_germ_cell_neoplasia_high_mag.jpg], [http://commons.wikimedia.org/wiki/File:Intratubular_germ_cell_neoplasia_high_mag_cropped.jpg]
|-
| 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
| [http://commons.wikimedia.org/wiki/File:Seminoma_high_mag.jpg], [http://commons.wikimedia.org/wiki/File:Seminoma_intermed_mag.jpg]
|-
| [[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
| patterns: microcystic, solid, hepatoid
| [http://webpathology.com/image.asp?case=34&n=6 hepatoid YST]
|-
| [[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
| usu. part of a mixed GCT
| [http://commons.wikimedia.org/wiki/File:Embryonal_carcinoma_intermed_mag.jpg], [http://www.webpathology.com/image.asp?case=33&n=1], [http://www.webpathology.com/image.asp?n=4&Case=37]
|-
| [[Choriocarcinoma]]
| marked nuclear atypia
| cells with clear cytoplasm (cytotrophoblast), multinucleated cells (syncytiotrophoblast)
| beta-hCG
| other
| [http://www.webpathology.com/image.asp?case=36&n=1]
|-
| [[Teratoma]], immature
| primitive neuroepithelium
| pseudostratified epithelium in rosettes (gland-like arrangement)
| None
| teratoma are always malignant in males
| [http://commons.wikimedia.org/wiki/File:Primitive_neuroepithelium_intermed_mag.jpg]
|-
| Mixed germ cell tumour
| NA
| common combinations: teratoma + embryonal carcinoma + endodermal sinus tumour (yolk sac tumour) (TEE); seminoma + embryonal (SE); embryonal + teratoma (TE)
| NA
| -
| -
|}


==Germinoma==
==Germinoma==

Revision as of 22:50, 8 February 2011

This article covers germ cell tumours which classicaly arise in the gonads (ovary, testis). They also show-up in neuropathology and in the mediastinum.

Overview

  • Germ cell tumours (GCTs).
    • Intratubular germ cell neoplasia.
    • Germinoma/Seminoma/Dysgerminoma.
    • Yolk sac tumour (endodermal sinus tumour).
    • Embryonal carcinoma.
    • Choriocarcinoma.
    • Teratoma.
    • Mixed GCT - 60% of GCTs are mixed.
      • Common combinations:
        1. teratoma + embryonal carcinoma + endodermal sinus tumour (yolk sac tumour) (TEE).
        2. seminoma + embryonal (SE).
        3. embryonal + teratoma (TE).

IHC for GCTs

ABCDs of GCTs:

  • AFP - yolk sac tumour.
  • Beta-hCG - choriocarcinoma.
  • CD30 - embryonal carcinoma.
  • D2-40 - seminoma.

Tabular summary of GCTs

Tumour Key feature Microscopic IHC Other Image
Intratubular germ cell neoplasia (ITGCN) nests of small fried egg cells large central nucleus, clear
cytoplasm, squared-off nuclear membrane, nucleoli[1] 		CD117 appearance similar to seminoma [1], [2]
Germinoma / Seminoma / Dysgerminoma fried egg cells fried egg-like cells (central nucleus, clear
cytoplasm) with squared-off nuclear
membrane, nucleoli, lymphocytic infiltrate, granulomata,
syncytiotrophoblastic giant cells[2] 		D2-40 seminoma = male version of this tumour; dysgerminoma = female version of this tumour [3], [4]
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 patterns: microcystic, solid, hepatoid hepatoid YST
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 usu. part of a mixed GCT [5], [6], [7]
Choriocarcinoma marked nuclear atypia cells with clear cytoplasm (cytotrophoblast), multinucleated cells (syncytiotrophoblast) beta-hCG other [8]
Teratoma, immature primitive neuroepithelium pseudostratified epithelium in rosettes (gland-like arrangement) None teratoma are always malignant in males [9]
Mixed germ cell tumour NA common combinations: teratoma + embryonal carcinoma + endodermal sinus tumour (yolk sac tumour) (TEE); seminoma + embryonal (SE); embryonal + teratoma (TE) NA - -

Germinoma

Comes in three flavours:

  • Germinoma.
  • Seminoma.
  • Dysgerminoma.

Germinoma

Is the generic version of this tumour. It is found in the midline (brain, mediastinum).

Seminoma

Main article: Testis#Seminoma

A common GCT in males.

Dysgerminoma

Main article: Ovarian tumours#Dysgerminoma

A common GCT in females.

Yolk sac tumour

General

  • Tumour also known as endodermal sinus tumour.

Epidemiology

  • Most common GCT in infants and young boys.

Microscopy

Classic feature:

  • Schiller-Duval bodies.
    • Look like glomerulus - central blood vessel surrounded by epithelial-like cells a space and more epithelial-like cells
  • Architecure - variable.
    • Most common microcystic pattern.[3]

Image:

Variants:

  • Hepatoid pattern.[4]
    • Vaguely resembles liver.
      • Hyaline globules (light red well-circumscribed globs).
      • Bile canaculi.
  • Solid pattern.[5]
    • Vaguely resembles seminoma.

Image:

IHC

  • AFP +ve.
  • Glypican 3 +ve.
    • More sensitive than AFP.[6]
  • Alpha-1 AT +ve.
  • Cytokeratin +ve. ???

DDx

  • Embryonal carcinoma.

Embryonal carcinoma

General

  • Affects young adults.
    • May be seen in women.

Microscopic

Features:[7]

  1. Nucleoli - key feature.
  2. Vesicular nuclei (clear, empty appearing nuclei) - key feature.
  3. Nuclei overlap.
  4. Necrosis - common.
    • Not commonly present in seminoma.
  5. Indistinct cell borders
  6. Mitoses - common.
  7. Variable architecture:
    • Tubulopapillary.
    • Glandular.
    • Solid.
    • Embryoid bodies - ball of cells in surrounded by empty space on three sides.

Notes:

  • Cytoplasmic staining variable (eosinophilic to basophilic).

Images:

DDx

  • Yolk sac tumour.

IHC

  • AE1/AE3 +ve.
  • CD30 +ve.

Choriocarcinoma

General

  • Aggressive clinical course.

Microscopic

Features:

  • Cytotrophoblasts - key feature.
    • Clear cytoplasm.
    • Polygonal shaped cells in cords/masses.
    • Distinct cell borders.
    • Single uniform nucleus.
  • +/-Hemorrhage.
  • +/-Necrosis.
  • Syncytiotrophoblasts - may be absent.[8]
    • Large + many irreg. or lobular hyperchromatic nuclei.
    • Eosinophilic vacuolated cytoplasm (contains hCG).

Image(s):

Notes:

IHC

  • beta-hCG +ve.

Teratoma

General

  • Consists of all three germ layers:[9]
    1. Endoderm:
      • Skin, CNS.
    2. Mesoderm:
      • Muscle, bone, connective tissue, blood.
    3. Ectoderm:
      • Internal organs.

Classification

  • Divided into:
    • Mature.
    • Immature.

Immature

  • Immature if neural tissue is present:[10]
    • Vaguely resembles pseudostratified respiratory epithelium.
  • Islands of small hyperchromatic cells - "blastema".
  • +/-Cartilage.
  • +/-Adipocytes.
  • +/-Colonic type mucosa.
  • +/-Stratified squamous epithelium (skin).

Images:

Other images:

Grading

Based on quantity of immature neuroepithelium:[11][12][13]

  • 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:

  • LPF not adequately defined - see LPFitis. Same BS as HPF.

IHC (immature)

Features:

  • Primitive neuroepithelium:[14]
    • Neuron-specific enolase (NSE) +ve.
    • Neuron-specific B tubulin +ve.
    • Synaptophysin +ve.

See also

References

  1. ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 538. ISBN 978-0443066771.
  2. ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 542. ISBN 978-0443066771.
  3. ↑ URL: http://webpathology.com/image.asp?case=34&n=1. Accessed on: March 8, 2010.
  4. ↑ URL: http://webpathology.com/image.asp?case=34&n=6. Accessed on: March 8, 2010.
  5. ↑ URL: http://webpathology.com/image.asp?case=34&n=8. Accessed on: March 8, 2010.
  6. ↑ Emerson, RE.; Ulbright, TM. (Jun 2010). "Intratubular germ cell neoplasia of the testis and its associated cancers: the use of novel biomarkers.". Pathology 42 (4): 344-55. doi:10.3109/00313021003767355. PMID 20438407.
  7. ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 549. ISBN 978-0443066771.
  8. ↑ URL: http://www.webpathology.com/image.asp?n=4&Case=36. Accessed on: 8 February 2011.
  9. ↑ Moore, Keith L.; Persaud, T.V.N. (2002). The Developing Human: Clinically Oriented Embryology (7th ed.). Saunders. pp. 83. ISBN 978-0721694122.
  10. ↑ RS. 2 May 2010.
  11. ↑ Harms D, Zahn S, Göbel U, Schneider DT (2006). "Pathology and molecular biology of teratomas in childhood and adolescence". Klin Padiatr 218 (6): 296–302. doi:10.1055/s-2006-942271. PMID 17080330.
  12. ↑ Ulbright TM (February 2005). "Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues". Mod. Pathol. 18 Suppl 2: S61–79. doi:10.1038/modpathol.3800310. PMID 15761467. http://www.nature.com/modpathol/journal/v18/n2s/full/3800310a.html.
  13. ↑ O'Connor DM, Norris HJ (October 1994). "The influence of grade on the outcome of stage I ovarian immature (malignant) teratomas and the reproducibility of grading". Int. J. Gynecol. Pathol. 13 (4): 283–9. PMID 7814189.
  14. ↑ Craver RD, Lipscomb JT, Suskind D, Velez MC (October 2001). "Malignant teratoma of the thyroid with primitive neuroepithelial and mesenchymal sarcomatous components". Ann Diagn Pathol 5 (5): 285–92. doi:10.1053/adpa.2001.27918. PMID 11598856.
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