Journal List > Korean J Obstet Gynecol > v.55(12) > 1088392

Ko, Kim, Park, Jeon, Kim, Rhim, Kang, and Park: A case of ovarian cancer developed in Mayer-Rokitansky-Küster-Hauser syndrome: Innate carcinogenesis of ovarian cancer

Abstract

Vaginal aplasia is an unusual congenital anomaly of the genital tract with an incidence of 1 in 4,000 female births. The vast majority of the cases are part of Mayer-Rokitansky-Küster-Hauser syndrome (MRKHs). In this syndrome, while the vagina is absent and the uterus is missing or extremely rudimentary, the ovarian function is normal and the secondary sexual characteristics are normally developed. The aim of the study is to present a case of ovarian cancer that developed on the ovary of a woman with MRKH syndrome.

Figures and Tables

Fig. 1
A computed tomography scan shows rim-enhancing ovarian mass with solid part in pelvic cavity in patient with Mayer-Rokitansky-Küster-Hauser syndrome.
kjog-55-1001-g001
Fig. 2
Right ovary contains solid nodule composed of gray tan friable tissue and multiple cysts which are filled with serous and hemorrhagic fluid. Two triangular small rudimentary uteruses are seen in the center.
kjog-55-1001-g002
Fig. 3
Serous carcinoma of the ovary illustrates poor differentiation (H&E, ×100).
kjog-55-1001-g003

References

1. Capraro VJ, Gallego MB. Vaginal agenesis. Am J Obstet Gynecol. 1976. 124:98–107.
2. Ghirardini G, Magnani A. Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian cancer. Report of a case. Clin Exp Obstet Gynecol. 1995. 22:247–248.
3. Griffin JE, Edwards C, Madden JD, Harrod MJ, Wilson JD. Congenital absence of the vagina. The Mayer-Rokitansky-Kuster-Hauser syndrome. Ann Intern Med. 1976. 85:224–236.
4. Morcel K, Camborieux L, Guerrier D. Programme de Recherches sur les Aplasies Müllériennes. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Orphanet J Rare Dis. 2007. 2:13.
5. Willemsen WN. Combination of the Mayer-Rokitansky-Kuster and Klippel-Feil syndrome: a case report and literature review. Eur J Obstet Gynecol Reprod Biol. 1982. 13:229–235.
6. Strübbe EH, Cremers CW, Dikkers FG, Willemsen WN. Hearing loss and the Mayer-Rokitansky-Kuster-Hauser syndrome. Am J Otol. 1994. 15:431–436.
7. Shokeir MH. Aplasia of the Mullerian system: evidence for probable sex-limited autosomal dominant inheritance. Birth Defects Orig Artic Ser. 1978. 14:147–165.
8. Wottgen M, Brucker S, Renner SP, Strissel PL, Strick R, Kellermann A, et al. Higher incidence of linked malformations in siblings of Mayer-Rokitansky-Kuster-Hauser-syndrome patients. Hum Reprod. 2008. 23:1226–1231.
9. Resendes BL, Sohn SH, Stelling JR, Tineo R, Davis AJ, Gray MR, et al. Role for anti-Mullerian hormone in congenital absence of the uterus and vagina. Am J Med Genet. 2001. 98:129–136.
10. Zenteno JC, Carranza-Lira S, Kofman-Alfaro S. Molecular analysis of the anti-Mullerian hormone, the anti-Mullerian hormone receptor, and galactose-1-phosphate uridyl transferase genes in patients with the Mayer-Rokitansky-Kuster-Hauser syndrome. Arch Gynecol Obstet. 2004. 269:270–273.
11. Cramer DW, Goldstein DP, Fraer C, Reichardt JK. Vaginal agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome) associated with the N314D mutation of galactose-1-phosphate uridyl transferase (GALT). Mol Hum Reprod. 1996. 2:145–148.
12. Klipstein S, Bhagavath B, Topipat C, Sasur L, Reindollar RH, Gray MR. The N314D polymorphism of the GALT gene is not associated with congenital absence of the uterus and vagina. Mol Hum Reprod. 2003. 9:171–174.
13. Pompili G, Munari A, Franceschelli G, Flor N, Meroni R, Frontino G, et al. Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med. 2009. 114:811–826.
14. Appelman Z, Hazan Y, Hagay Z. High prevalence of müllerian anomalies diagnosed by ultrasound in women with polycystic ovaries. J Reprod Med. 2003. 48:362–364.
TOOLS
Similar articles