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.
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).
- Common combinations:
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 | - | - |
![[1]](http://commons.wikimedia.org/wiki/File:Intratubular_germ_cell_neoplasia_high_mag.jpg){kind=link}
![[2]](http://commons.wikimedia.org/wiki/File:Intratubular_germ_cell_neoplasia_high_mag_cropped.jpg){kind=link}
![[3]](http://commons.wikimedia.org/wiki/File:Seminoma_high_mag.jpg){kind=link}
![[4]](http://commons.wikimedia.org/wiki/File:Seminoma_intermed_mag.jpg){kind=link}
![[5]](http://commons.wikimedia.org/wiki/File:Embryonal_carcinoma_intermed_mag.jpg){kind=link}
![[9]](http://commons.wikimedia.org/wiki/File:Primitive_neuroepithelium_intermed_mag.jpg){kind=link}
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.
- Vaguely resembles liver.
- 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]
- 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:
- Cytoplasmic staining variable (eosinophilic to basophilic).
Images:
- Set 1:
- Set 2:
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):
- Choriocarcinoma - low mag. (webpathology.com).
- Choriocarcinoma (webpathology.com).
- Choriocarcinoma (webpathology.com).
Notes:
- See: Chorionic villi.
IHC
- beta-hCG +ve.
Teratoma
General
- Consists of all three germ layers:[9]
- Endoderm:
- Skin, CNS.
- Mesoderm:
- Muscle, bone, connective tissue, blood.
- Ectoderm:
- Internal organs.
- Endoderm:
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:
- Primitive neuroepithelium - high mag. (WC).
- Primitive neuroepithelium - intermed mag. (WC)
- Teratoma - mature components (WC).
Other images:
- Immature teratoma - myxomatous stroma (webpathology.com).
- Immature teratoma - blastema (webpathology.com).
- Immature teratoma - primitive neuroepithelium (webpathology.com).
- Immature teratoma - primitive neuroepithelium (pathconsultddx.com).
- Immature teratoma - primitive neuroepithelium (ouhsc.edu).
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
- ↑ 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.
- ↑ 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.
- ↑ URL: http://webpathology.com/image.asp?case=34&n=1. Accessed on: March 8, 2010.
- ↑ URL: http://webpathology.com/image.asp?case=34&n=6. Accessed on: March 8, 2010.
- ↑ URL: http://webpathology.com/image.asp?case=34&n=8. Accessed on: March 8, 2010.
- ↑ 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.
- ↑ 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.
- ↑ URL: http://www.webpathology.com/image.asp?n=4&Case=36. Accessed on: 8 February 2011.
- ↑ Moore, Keith L.; Persaud, T.V.N. (2002). The Developing Human: Clinically Oriented Embryology (7th ed.). Saunders. pp. 83. ISBN 978-0721694122.
- ↑ RS. 2 May 2010.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.