Dysgerminoma of ovary

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Dysgerminoma of ovary
Other names: Dysgerminoma, dysgerminoma of the ovary, germ cell dysgerminoma of ovary, germ cell dysgerminoma of the ovary, ovarian dysgerminoma, ovarian germ cell dysgerminoma, ovary dysgerminoma (disease)[1]
Dysgerminoma surgery.jpg
Excision of a dysgerminoma
SpecialtyOncology, gynecology
SymptomsTummy ache, localised enlargement[2]
Usual onsetTeenage, young adult[2]
CausesAbnormalities in chromosome 12[2]
Risk factorsYoung females[2]
Diagnostic methodRaised LDL[2]
TreatmentSurgery,[3] chemotherapy, radiotherapy[1]
Prognosis10-year survival >90%, 10% recur within 2-years[2]
Frequency1% of ovarian tumors overall[2]

Dysgerminoma of ovary, is a cancerous ovarian tumor of the germ cell type.[1] It typically presents with pain or localised enlargement in the tummy.[2] The tumor tends to grow slowly and occurs in young females in their teenage years and early adulthood.[4]

80% have abnormalities in chromosome 12.[2] It may occur in association with gonadoblastoma in people with gonadal dysgenesis.[2] A blood test typically shows a raised LDL.[2] Treatment is by surgery to remove the tumor.[3] It responds well to chemotherapy and radiotherapy.[1] 10-year survival rate is more than 90%, with 10% recurring typically within 2-years.[2]

Dysgerminoma of ovary is the most common cancerous germ cell tumor and accounts for around 1% of ovarian tumors overall.[2] It occurs in both ovaries in 10% of cases.[2]

Signs and symptoms

They are exceptionally associated with hypercalcemia. On gross examination, dysgerminomas present with a smooth, bosselated (knobby) external surface, and is soft, fleshy and either cream-coloured, gray, pink or tan when cut. Microscopic examination typically reveals uniform cells that resemble primordial germ cells. Typically, the stroma contains lymphocytes and about 20% of patients have sarcoid-like granulomas. Metastases are most often present in the lymph nodes.

Dysgerminoma usually occurs in adolescence and early adult life; about 5% occur in pre-pubertal children. Dysgerminoma is extremely rare after age 50.

Diagnosis

LDH tumour markers is elevated in 95% of the cases. A tumor of the identical histology but not occurring in the ovary may be described by an alternate name: seminoma in the testis[5] or germinoma in the central nervous system or other parts of the body.

Treatment

Dysgerminomas, like other seminomatous germ cell tumors, are very sensitive to both chemotherapy and radiotherapy. For this reason, with treatment patients' chances of long-term survival, even cure, is excellent.[6]

References

  1. 1.0 1.1 1.2 1.3 "Dysgerminoma of ovary (Concept Id: C0346185) - MedGen - NCBI". www.ncbi.nlm.nih.gov. National Institutes of health. Archived from the original on 2 July 2022. Retrieved 1 July 2022.
  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 WHO Classification of Tumours Editorial Board, ed. (2020). "1. Tumours of the ovary: dysgerminoma". Female genital tumours: WHO Classification of Tumours. Vol. 4 (5th ed.). Lyon (France): International Agency for Research on Cancer. pp. 123–124. ISBN 978-92-832-4504-9. Archived from the original on 2022-06-17. Retrieved 2022-06-28.
  3. 3.0 3.1 "Ovarian Germ Cell Tumors Treatment (PDQ®)–Patient Version - NCI". www.cancer.gov. 23 May 2022. Archived from the original on 11 January 2022. Retrieved 1 July 2022.
  4. "Dysgerminoma". www.cancer.gov. National Cancer Institute. 2 February 2011. Archived from the original on 23 February 2022. Retrieved 30 June 2022.
  5. "dysgerminoma" at Dorland's Medical Dictionary
  6. Maoz A, Matsuo K, Ciccone MA, Matsuzaki S, Klar M, Roman LD, et al. (May 2020). "Molecular Pathways and Targeted Therapies for Malignant Ovarian Germ Cell Tumors and Sex Cord-Stromal Tumors: A Contemporary Review". Cancers. 12 (6): 1398. doi:10.3390/cancers12061398. PMC 7353025. PMID 32485873.

External links

Classification