Difference between revisions of "Germ cell tumours"

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==Teratoma==
==Teratoma==
===General===
===General===
*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>  
*May be benign of malignant.
*Are supposed to consists of all three [[germ layers]] - this is not always true.
 
====Classification====
#Mature.
#*Common in females, not seen in males.
#*Usually benign; however, mature component may give rise to a malignancy like elsewhere in the body.
#**Most common malignancy arising from a mature teratoma: [[squamous cell carcinoma]].
#Immature.
#*Uncommon.
#*Malignant.
#Monodermal.
#*Rare.
#*Highly specialized.
 
===Mature===
Features - three germ cell layers (usually):<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:
*#Endoderm:
*#*Skin, CNS.
*#*Skin, (mature) CNS.
*#Mesoderm:
*#Mesoderm:
*#*Muscle, bone, connective tissue, blood.
*#*Muscle, bone, connective tissue, blood.
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*#*Internal organs.
*#*Internal organs.


===Classification===
Note:
====Two groups (simplified)====
*May consist of skin only - in which case it is commonly called a ''dermoid''.
**Mature (benign).
**Immature (malignant).


====Three types====
Images:
#Mature - common, usually benign.
*[http://commons.wikimedia.org/wiki/File:Teratoma_2_low_mag.jpg Mature teratoma - low mag. (WC)].
#*Mature component may give rise to a malignancy - most common [[squamous cell carcinoma]].
*[http://commons.wikimedia.org/wiki/File:Teratoma_2_high_mag.jpg Mature teratoma - high mag. (WC)].
#Immature - malignant.
#Monodermal - highly specialized.


===Immature===
===Immature===
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**Synaptophysin +ve.
**Synaptophysin +ve.


===Specialized teratomas===
===Monodermal teratomas===
====General====
*Rare.
*Monodermal.
 
====Struma ovarii====
====Struma ovarii====
Features:
Features:

Revision as of 04:01, 12 August 2011

This article covers germ cell tumours which classicaly 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 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 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 may be preceded by a complete hydatidiform mole [8], [9]
Teratoma, immature primitive neuroepithelium pseudostratified epithelium in rosettes (gland-like arrangement) None teratoma are always malignant in males [10]
Mixed germ cell tumour NA common combinations: teratoma + embryonal carcinoma + endodermal sinus tumour (yolk sac tumour) (TEE); seminoma + embryonal (SE); embryonal + teratoma (TE) NA - -
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 [11]

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

  • May be benign of malignant.
  • Are supposed to consists of all three germ layers - this is not always true.

Classification

  1. Mature.
    • Common in females, not seen in males.
    • Usually benign; however, mature component may give rise to a malignancy like elsewhere in the body.
  2. Immature.
    • Uncommon.
    • Malignant.
  3. Monodermal.
    • Rare.
    • Highly specialized.

Mature

Features - three germ cell layers (usually):[9]

    1. Endoderm:
      • Skin, (mature) CNS.
    2. Mesoderm:
      • Muscle, bone, connective tissue, blood.
    3. Ectoderm:
      • Internal organs.

Note:

  • May consist of skin only - in which case it is commonly called a dermoid.

Images:

Immature

Features:

  • 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 (immature)

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.

Monodermal teratomas

Struma ovarii

Features:

Images:

Carcinoid

Main article: Neuroendocrine neoplasms

Features:

  • Like neuroendocrine tumours elsewhere:
    • Nuclei with stippled chromatin (salt-and-pepper chromatin).

Gonadoblastoma

General

  • Associated with abnormal sexual development.
  • Often coexist with a dysgerminoma.
  • A mixed tumour that consists of (1) primitive germ cells and (2) sex cord elements.

Microscopic

Features:[15][16]

  • Immature germ cells resembling Sertoli cells or granulosa cells.
    • Sertoli cells = moderate cytoplasm in a trabecular or tubular architecture.
    • Granulosa cells = form follicle-like structures.
  • Primitive germ cells resemble those of a dysgerminoma.
    • Polygonal cells with a central nucleus, squared-off nuclear membrane and clear cytoplasm.
  • +/-Calcification (very common).

Images:

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.
  15. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1104. ISBN 0-7216-0187-1.
  16. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970245-5. Accessed on: 8 April 2011.
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