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Dysgerminoma

MedGen UID:
41680
Concept ID:
C0013377
Neoplastic Process
Synonyms: Disgerminoma; Disgerminomas; Dysgerminomas
SNOMED CT: Dysgerminoma (60718004)
 
HPO: HP:0100621

Definition

A malignant ovarian neoplasm, thought to be derived from primordial germ cells of the sexually undifferentiated embryonic gonad. It is the counterpart of the classical seminoma of the testis, to which it is both grossly and histologically identical. Dysgerminomas comprise 16% of all germ cell tumors but are rare before the age of 10, although nearly 50% occur before the age of 20. They are generally considered of low-grade malignancy but may spread if the tumor extends through its capsule and involves lymph nodes or blood vessels. (Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1646) [from MeSH]

Conditions with this feature

Neoplasm of ovary
MedGen UID:
181539
Concept ID:
C0919267
Neoplastic Process
Ovarian cancer, the leading cause of death from gynecologic malignancy, is characterized by advanced presentation with loco-regional dissemination in the peritoneal cavity and the rare incidence of visceral metastases (Chi et al., 2001). These typical features relate to the biology of the disease, which is a principal determinant of outcome (Auersperg et al., 2001). Epithelial ovarian cancer is the most common form and encompasses 5 major histologic subtypes: papillary serous, endometrioid, mucinous, clear cell, and transitional cell. Epithelial ovarian cancer arises as a result of genetic alterations sustained by the ovarian surface epithelium (Stany et al., 2008; Soslow, 2008).
46,XY sex reversal, type 6
MedGen UID:
462414
Concept ID:
C3151064
Disease or Syndrome
Swyer syndrome is a condition that affects sexual development. Sexual development is usually determined by an individual's chromosomes; however, in Swyer syndrome, sexual development does not match the affected individual's chromosomal makeup.People usually have 46 chromosomes in each cell. Two of the 46 chromosomes, known as X and Y, are called sex chromosomes because they help determine whether a person will develop male or female sex characteristics. Girls and women typically have two X chromosomes (46,XX karyotype), while boys and men usually have one X chromosome and one Y chromosome (46,XY karyotype). In Swyer syndrome, individuals with one X chromosome and one Y chromosome in each cell, the pattern typically found in boys and men, have female reproductive structures.People with Swyer syndrome have typical female external genitalia. The uterus and fallopian tubes are normally-formed, but the gonads (ovaries or testes) are not functional; affected individuals have undeveloped clumps of tissue called streak gonads. Because of the lack of development of the gonads, Swyer syndrome is also called 46,XY complete gonadal dysgenesis. The residual gonadal tissue often becomes cancerous, so it is usually removed surgically early in life.People with Swyer syndrome are typically raised as girls and have a female gender identity. Because they do not have functional ovaries, affected individuals usually begin hormone replacement therapy during adolescence to induce menstruation and development of female secondary sex characteristics such as breast enlargement and uterine growth. Hormone replacement therapy also helps reduce the risk of reduced bone density (osteopenia and osteoporosis). Women with this disorder do not produce eggs (ova), but they may be able to become pregnant with a donated egg or embryo.Swyer syndrome usually affects only sexual development; such cases are called isolated Swyer syndrome. However, depending on the genetic cause, Swyer syndrome may also occur along with health conditions such as nerve problems (neuropathy) or as part of a syndrome such as campomelic dysplasia, which causes severe skeletal abnormalities.
46,XY SEX REVERSAL 10
MedGen UID:
897538
Concept ID:
C4225331
Disease or Syndrome

Recent clinical studies

Etiology

Yüce Ö, Döğer E, Çelik N, Emeksiz HC, Çamurdan MO, Bideci A, Cinaz P
J Clin Res Pediatr Endocrinol 2015 Dec;7(4):336-9. doi: 10.4274/jcrpe.2022. PMID: 26777047Free PMC Article
Kefelı M, Karagöz F, Malpica A
Turk Patoloji Derg 2014;30(3):210-4. doi: 10.5146/tjpath.2013.01199. PMID: 24272934
Capito C, Arnaud A, Hameury F, Fremond B, Lardy H, Leclair MD, Heloury Y
J Pediatr Urol 2011 Jun;7(3):367-72. Epub 2011 Mar 12 doi: 10.1016/j.jpurol.2011.02.021. PMID: 21402494
Guerriero S, Testa AC, Timmerman D, Van Holsbeke C, Ajossa S, Fischerova D, Franchi D, Leone FP, Domali E, Alcazar JL, Parodo G, Mascilini F, Virgilio B, Demidov VN, Lipatenkova J, Valentin L
Ultrasound Obstet Gynecol 2011 May;37(5):596-602. Epub 2011 Apr 5 doi: 10.1002/uog.8958. PMID: 21305635
Gimelli S, Beri S, Drabkin HA, Gambini C, Gregorio A, Fiorio P, Zuffardi O, Gemmill RM, Giorda R, Gimelli G
Mol Cancer 2009 Jul 30;8:52. doi: 10.1186/1476-4598-8-52. PMID: 19642973Free PMC Article

Diagnosis

Kulkarni MM, Sinai Khandeparkar SG, Joshi AR, Bhayekar PV
Indian J Pathol Microbiol 2016 Oct-Dec;59(4):527-529. doi: 10.4103/0377-4929.191815. PMID: 27721289
Yüce Ö, Döğer E, Çelik N, Emeksiz HC, Çamurdan MO, Bideci A, Cinaz P
J Clin Res Pediatr Endocrinol 2015 Dec;7(4):336-9. doi: 10.4274/jcrpe.2022. PMID: 26777047Free PMC Article
Yeh KE, Marcus PS, Fong TL
Obstet Gynecol 2015 Aug;126(2):431-4. doi: 10.1097/AOG.0000000000000699. PMID: 25923022
Guida M, Gentile A, De Fazio M, Cramarossa A, Sabatelli A, Colucci G
J Pediatr Adolesc Gynecol 2013 Feb;26(1):e13-6. doi: 10.1016/j.jpag.2012.10.001. PMID: 23332198
Changchien YC, Haltrich I, Micsik T, Kiss E, Fónyad L, Papp G, Sápi Z
Pathol Res Pract 2012 Oct 15;208(10):628-32. Epub 2012 Aug 18 doi: 10.1016/j.prp.2012.07.006. PMID: 22906432

Therapy

Hayari L, Shir On E, Fedorenko A, Sergio S, Zidan J, Solomonov E
BMJ Case Rep 2017 Jan 30;2017 doi: 10.1136/bcr-2016-217787. PMID: 28137901
Ajao M, Vachon T, Snyder P
Mil Med 2013 Aug;178(8):e954-5. doi: 10.7205/MILMED-D-13-00091. PMID: 23929062
Guida M, Gentile A, De Fazio M, Cramarossa A, Sabatelli A, Colucci G
J Pediatr Adolesc Gynecol 2013 Feb;26(1):e13-6. doi: 10.1016/j.jpag.2012.10.001. PMID: 23332198
Alvarado-Cabrero I, Valencia-Cedillo R, Mohs-Alfaro M, De Anda-González J
Int J Gynecol Pathol 2011 Sep;30(5):466-9. doi: 10.1097/PGP.0b013e318217139e. PMID: 21804387
Yoo SH, Kim KR, Hong SJ, Cho KJ
Pathol Int 2011 Apr;61(4):248-51. Epub 2011 Feb 9 doi: 10.1111/j.1440-1827.2010.02640.x. PMID: 21418398

Prognosis

Hayari L, Shir On E, Fedorenko A, Sergio S, Zidan J, Solomonov E
BMJ Case Rep 2017 Jan 30;2017 doi: 10.1136/bcr-2016-217787. PMID: 28137901
Guida M, Gentile A, De Fazio M, Cramarossa A, Sabatelli A, Colucci G
J Pediatr Adolesc Gynecol 2013 Feb;26(1):e13-6. doi: 10.1016/j.jpag.2012.10.001. PMID: 23332198
Changchien YC, Haltrich I, Micsik T, Kiss E, Fónyad L, Papp G, Sápi Z
Pathol Res Pract 2012 Oct 15;208(10):628-32. Epub 2012 Aug 18 doi: 10.1016/j.prp.2012.07.006. PMID: 22906432
A L Husaini H, Soudy H, El Din Darwish A, Ahmed M, Eltigani A, A L Mubarak M, Sabaa AA, Edesa W, A L-Tweigeri T, Al-Badawi IA
Med Oncol 2012 Dec;29(4):2944-8. Epub 2012 Mar 10 doi: 10.1007/s12032-012-0194-z. PMID: 22407668
Guerriero S, Testa AC, Timmerman D, Van Holsbeke C, Ajossa S, Fischerova D, Franchi D, Leone FP, Domali E, Alcazar JL, Parodo G, Mascilini F, Virgilio B, Demidov VN, Lipatenkova J, Valentin L
Ultrasound Obstet Gynecol 2011 May;37(5):596-602. Epub 2011 Apr 5 doi: 10.1002/uog.8958. PMID: 21305635

Clinical prediction guides

Changchien YC, Haltrich I, Micsik T, Kiss E, Fónyad L, Papp G, Sápi Z
Pathol Res Pract 2012 Oct 15;208(10):628-32. Epub 2012 Aug 18 doi: 10.1016/j.prp.2012.07.006. PMID: 22906432
A L Husaini H, Soudy H, El Din Darwish A, Ahmed M, Eltigani A, A L Mubarak M, Sabaa AA, Edesa W, A L-Tweigeri T, Al-Badawi IA
Med Oncol 2012 Dec;29(4):2944-8. Epub 2012 Mar 10 doi: 10.1007/s12032-012-0194-z. PMID: 22407668
Rouzier C, Soler C, Hofman P, Brennetot C, Bieth E, Pedeutour F
Pediatr Blood Cancer 2008 Mar;50(3):696-8. doi: 10.1002/pbc.21156. PMID: 17243131
Cossu-Rocca P, Jones TD, Roth LM, Eble JN, Zheng W, Karim FW, Cheng L
Hum Pathol 2006 Aug;37(8):1015-21. Epub 2006 May 26 doi: 10.1016/j.humpath.2006.02.018. PMID: 16867864
Pauls K, Wardelmann E, Merkelbach-Bruse S, Büttner R, Zhou H
Virchows Arch 2004 Dec;445(6):651-4. Epub 2004 Sep 29 doi: 10.1007/s00428-004-1112-3. PMID: 15455230

Recent systematic reviews

Kodama M, Grubbs BH, Blake EA, Cahoon SS, Murakami R, Kimura T, Matsuo K
Eur J Obstet Gynecol Reprod Biol 2014 Oct;181:145-56. Epub 2014 Aug 7 doi: 10.1016/j.ejogrb.2014.07.047. PMID: 25150953
Ondrus D, Hornak M
Tumori 1994 Oct 31;80(5):362-4. PMID: 7839467
Marks LB, Anscher MS, Shipley WU
Oncology (Williston Park) 1992 Jun;6(6):43-8; discussion 51-2. PMID: 1535210
Thomas G, Jones W, VanOosterom A, Kawai T
Prog Clin Biol Res 1990;357:285-94. PMID: 2217472
Weissbach L, Boedefeld EA, Oberdörster W, Vahlensieck W
Eur Urol 1984;10(1):1-9. PMID: 6365569

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