Ovarian germ cell tumor pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Overveiw

The pathophysiology of ovarian germ cell tumors depends on the histological subtype. However, their origin is the primordial germ cells that transformed pathologically in different stages of development.

Pathophysiology

Physiology

The normal physiology of [name of process] can be understood as follows:

Pathogenesis

  • The exact pathogenesis of or is not completely understood.

OR

  • It is understood that ovarian germ cell tumors are the result of the pathologic transformation of primordial germ cells during different stages of the development.[1]

Mature teratoma

  • Mature teratomas are benign tumors originating from pathologic development of primordial germ cells.[2]
  • These tumors contain the well-differentiated component of three germ layers.
  • Their usual location is the embryonic fusion line over head and neck, mediastinum,a and pre sacral area and tend to present at greater extent in midline.
  • [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
  • The progression to [disease name] usually involves the [molecular pathway].
  • The pathophysiology of [disease/malignancy] depends on the histological subtype.

Genetics

[Disease name] is transmitted in [mode of genetic transmission] pattern.

OR

Genes involved in the pathogenesis of [disease name] include:

  • [Gene1]
  • [Gene2]
  • [Gene3]

OR

The development of [disease name] is the result of multiple genetic mutations such as:

  • [Mutation 1]
  • [Mutation 2]
  • [Mutation 3]

Associated Conditions

Conditions associated with mature teratoma include:

  • Anti-NMDA receptor encephalitis (although very rarely)[3]
  • [Condition 2]
  • [Condition 3]

Gross Pathology

On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Gross Pathology

  • The majority of mature teratomas are 5 to 10 cm in diameter.[4]
Ovarian germ cell tumor subtype Features on Gross Pathology
Dysgerminoma
  • External surface is smooth and bosselated (knobby)[5]
  • Soft, fleshy and either cream-colored, gray, pink or tan in color on cut surface
Endodermal sinus tumor or yolk sac tumors
  • External surface is smooth and glistening[6]
  • Cut surface is cystic with hemorrhage and necrosis
Embryonal Carcinoma
Teratoma

Teratoma-mature

  • Solid or cystic[8]
  • Cystic content may contain greasy material composed of keratin, hair, and teeth
  • Teeth may be found in Rokitansky’s protuberance - a well-defined, nipple-like structure covered with hair

Teratoma-immature

  • Bulky, solid or cystic with necrosis, hemorrhage[9]

Microscopic Pathology

Ovarian germ cell tumor subtype Features on Histopathological Microscopic Analysis Image
Dysgerminomas
  • Uniform cells with "fried egg appearance" (large cytoplasm and small nucleus) that resemble primordial germ cells[5]
  • The stroma contains lymphocytes and about 20% of patients have sarcoid-like granulomas
Micrograph a seminoma, a tumor that is histologically indistinguishable from dysgerminoma.
Endodermal sinus tumor or yolk sac tumors
  • Schiller-Duval bodies (resemble renal glomeruli) - key feature[10]
Micrograph showing the yolk sac component of a mixed germ cell tumor.


Embryonal carcinoma

Main features:[7]

  • Nuclear atypia
  • Nucleoli prominent
  • Necrosis common
  • Nuclei overlap

Additional features that are often mixed:

  • Variable architecture:
  • Tubulopapillary
  • Glandular
  • Solid
  • Embryoid bodies - ball of cells surrounded by empty space on three sides
  • Mitoses common
Embryonal carcinoma
Teratoma

Mature teratoma

  • The sections show ovarian parenchyma with a lesion consisting of benign dermal, gastrointestinal, and neural elements [11]
  • The neural elements show focal degenerative changes with macrophages and giant cells
  • Siderophages are present

Immature teratoma

  • Primitive neuroepithelium[12]
  • Neuron-specific enolase (NSE) +ve
  • Neuron-specific B tubulin +ve
  • Synaptophysin +ve
Teratoma

Mature teratoma

  • It appears as a well-established organization of tissues mimicking the relationship observed in normal organs such as:[13]
    • respiratory epithelial layer surrounded by smooth muscle and cartilage
  • Usually, there is scant mitosis in the tumor cells usually limited to the normal proliferative zone of the body part that they produce.
  • No cytologic atypia is present.
  • Different type of tissues may be observed in the mature teratomas of the ovary such as:
    • Choroid plexus
    • Thyroid tissues
    • Pituitary tissues, although not commonly.[14]
      • Rarely, they produce prolactin and is associated with prolactinoma.

Microscopic Pathology

On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

References

  1. ↑ El-Maarri, Osman; Rijlaarsdam, Martin A.; Tax, David M. J.; Gillis, Ad J. M.; Dorssers, Lambert C. J.; Koestler, Devin C.; de Ridder, Jeroen; Looijenga, Leendert H. J. (2015). "Genome Wide DNA Methylation Profiles Provide Clues to the Origin and Pathogenesis of Germ Cell Tumors". PLOS ONE. 10 (4): e0122146. doi:10.1371/journal.pone.0122146. ISSN 1932-6203.
  2. ↑ Vural, F.; Vural, B.; Paksoy, N. (2015). "Vaginal teratoma: A case report and review of the literature". Journal of Obstetrics and Gynaecology. 35 (7): 757–758. doi:10.3109/01443615.2015.1004525. ISSN 0144-3615.
  3. ↑ Dalmau, Josep; Gleichman, Amy J; Hughes, Ethan G; Rossi, Jeffrey E; Peng, Xiaoyu; Lai, Meizan; Dessain, Scott K; Rosenfeld, Myrna R; Balice-Gordon, Rita; Lynch, David R (2008). "Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies". The Lancet Neurology. 7 (12): 1091–1098. doi:10.1016/S1474-4422(08)70224-2. ISSN 1474-4422.
  4. ↑ Yayla Abide, Çiğdem; Bostancı Ergen, Evrim (2018). "Retrospective analysis of mature cystic teratomas in a single center and review of the literature". Journal of Turkish Society of Obstetric and Gynecology. 15 (2): 95–98. doi:10.4274/tjod.86244. ISSN 1307-699X.
  5. ↑ 5.0 5.1 Dysgerminoma. https://en.wikipedia.org/wiki/Dysgerminoma. URL Accessed on November 12, 2015
  6. ↑ Ovary tumor Germ cell tumors Yolk sac tumor. http://www.pathologyoutlines.com/topic/ovarytumoryolksac.html. URL Accessed on November 12, 2015
  7. ↑ 7.0 7.1 Abbas, Fausto, Mitchell (2010). Basic Pathology. Elsevier. pp. 696–697. ISBN 978-81-312-1036-9.
  8. ↑ Ovary tumor Germ cell tumors Teratoma-maturehttp://www.pathologyoutlines.com/topic/ovarytumorteratomamature.html. URL Accessed on November 12, 2015
  9. ↑ Ovary tumor Germ cell tumors Teratoma-immature. http://www.pathologyoutlines.com/topic/ovarytumorteratomaimmature.html. URL Accessed on November 12, 2015
  10. ↑ Endodermal sinus tumor. https://en.wikipedia.org/wiki/Endodermal_sinus_tumor. URL Accessed on November 12, 2015
  11. ↑ Mature teratoma. http://librepathology.org/wiki/index.php/Teratoma#Mature_teratoma. URL Accessed on November 12, 2015
  12. ↑ Immature teratoma. http://librepathology.org/wiki/index.php/Teratoma#Mature_teratoma. URL Accessed on November 12, 2015
  13. ↑ 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.
  14. ↑ Kallenberg, GA; Pesce, CM; Norman, B; Ratner, RE; Silverberg, SG (1991). "Ectopic hyperprolactinemia resulting from an ovarian teratoma". International Journal of Gynecology & Obstetrics. 34 (2): 194–195. doi:10.1016/0020-7292(91)90266-8. ISSN 0020-7292.

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