A dysgerminoma is a type of germ cell tumor;
A tumor of the identical histology but not occurring in the ovary may be described by an alternate name: seminoma in the testis
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 (due to gonadal dysgenesis and androgen insensitivity syndrome) have a high risk[Nelson Textbook of Pediatrics, 18th ed. Chapter 553. Question 11, Gynecologic Problems of Childhood] 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
They are exceptionally associated with hypercalcemia. On gross examination, dysgerminomas present with a smooth, (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
and about 20% of patients have sarcoid-like
.
Metastases are most often present in the
.
Diagnosis
LDH tumour markers is elevated in 95% of the cases.
Image:Dysgerminoma,_intermed._mag.1.jpg|Dysgerminoma characterized by uniform cells separated by fibrous septa with lymphocytes, H&E stain.
Image:Dysgerminoma,_low_mag.2.jpg|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.
Targeted treatments for dysgerminomas that do not respond to chemotherapy are being evaluated.
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