Dysgerminoma

A dysgerminoma is a type of germ cell tumor;[1] it usually is malignant and usually occurs in the ovary.

Dysgerminoma
Micrograph of a dysgerminoma, H&E stain.
SpecialtyOncology, gynecology

A tumor of the identical histology but not occurring in the ovary may be described by an alternate name: seminoma in the testis[2] or germinoma in the central nervous system or other parts of the body.

Dysgerminoma accounts for less than 1% of ovarian tumors overall. Dysgerminoma usually occurs in adolescence and early adult life; about 5% occur in pre-pubertal children. Dysgerminoma is extremely rare after age 50. Dysgerminoma occurs in both ovaries in 10% of patients and, in a further 10%, there is microscopic tumor in the other ovary.

Abnormal gonads (due to gonadal dysgenesis and androgen insensitivity syndrome) have a high risk[3] of developing a dysgerminoma. Most dysgerminomas are associated with elevated serum lactic dehydrogenase (LDH), which is sometimes used as a tumor marker.

Signs and symptoms
Excision of a dysgerminoma

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.

Diagnosis

LDH tumour markers is elevated in 95% of the cases.

  • Dysgerminoma characterized by uniform cells separated by fibrous septa with lymphocytes, H&E stain.
  • Low-power view of a dysgerminoma infiltrating the colonic wall, H&E stain.

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.[4] Targeted treatments for dysgerminomas that do not respond to chemotherapy are being evaluated.[4]

References
  1. Behtash N, Karimi Zarchi M (June 2007). "Dysgerminoma in three patients with Swyer syndrome". World Journal of Surgical Oncology. 5 (1): 71. doi:10.1186/1477-7819-5-71. PMC 1934908. PMID 17587461.
  2. "dysgerminoma" at Dorland's Medical Dictionary
  3. Nelson Textbook of Pediatrics, 18th ed. Chapter 553. Question 11, Gynecologic Problems of Childhood
  4. 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.
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