Dysgerminoma
| Dysgerminoma | |
|---|---|
 | |
| Micrograph of a dysgerminoma, H&E stain. | |
| Specialty | Oncology, gynecology |
A dysgerminoma is a type of germ cell tumor;[1] it usually is malignant and usually occurs in the ovary.
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. Locations outside the ovaries such as the abdomen, fallopian tubes and uterus are rarely involved.[3][4][5]
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.[6]
Abnormal gonads (due to gonadal dysgenesis and androgen insensitivity syndrome) have a high risk[7] 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.[8] On gross examination, dysgerminomas present with a smooth, bosselated (knobby) external surface, and are 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.
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.[9] Targeted treatments for dysgerminomas that do not respond to chemotherapy are being evaluated.[9]
References
- ↑ 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.
- ↑ "dysgerminoma" at Dorland's Medical Dictionary
- ↑ Akbarzadeh-Jahromi, Mojgan; Aslani, Fatemeh Sari; Najib, Fatemesadat; Hosseini, Shahla (2018). "Primary intrauterine dysgerminoma in a pregnant woman: A rare case report". Indian Journal of Pathology & Microbiology. 61 (4): 590–592. doi:10.4103/IJPM.IJPM_598_17. ISSN 0974-5130. PMID 30303159.
{{cite journal}}: CS1 maint: unflagged free DOI (link) - ↑ Guadagno, Elia; Gencarelli, Annarita; Russo, Daniela; Campanino, Maria Raffaela; De Rosa, Filippo; D'Ambra, Michele; Corcione, Francesco; Insabato, Luigi (2021-04). "Extraovarian dysgerminoma in a pregnant woman: an extremely rare finding". Current Problems in Cancer. 45 (2): 100667. doi:10.1016/j.currproblcancer.2020.100667. ISSN 1535-6345. PMID 33129566.
{{cite journal}}: Check date values in:|date=(help)CS1 maint: article number as page number (link) - ↑ Anwar, Aisha; Akhtar, Muhammad; Busby, Gail (2021-12). "Swyer Syndrome: A Case of Dysgerminoma Solely within the Fallopian Tube". Journal of Pediatric and Adolescent Gynecology. 34 (6): 869–871. doi:10.1016/j.jpag.2021.04.008. ISSN 1873-4332. PMID 33989803.
{{cite journal}}: Check date values in:|date=(help) - ↑ Pectasides, D.; Pectasides, E.; Kassanos, D. (2008-08). "Germ cell tumors of the ovary". Cancer Treatment Reviews. 34 (5): 427–441. doi:10.1016/j.ctrv.2008.02.002. ISSN 0305-7372. PMID 18378402.
{{cite journal}}: Check date values in:|date=(help) - ↑ Nelson Textbook of Pediatrics, 18th ed. Chapter 553. Question 11, Gynecologic Problems of Childhood
- ↑ Iqbal, Iqra; Khan, Muhammad Atique Alam; Khan, Yasir; Thalambedu, Nishanth; Munir, Samavia (2019-11-08). "Hypercalcemia Due to Paraneoplastic Production of 1,25- Dihydroxyvitamin D in a Young Female with Dysgerminoma". Cureus. 11 (11): e6097. doi:10.7759/cureus.6097. ISSN 2168-8184. PMC 6901377. PMID 31886038.
{{cite journal}}: CS1 maint: article number as page number (link) CS1 maint: unflagged free DOI (link) - 1 2 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.