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Cancer of the supernumerary ovary in Mayer-Rokitansty-Kuster-Hauser Syndrome: A case report

Authors
Bae, Hyo SookRyu, Min JiKim, In SunKim, Sun HaengSong, Jae Yun
Issue Date
2월-2013
Publisher
SPANDIDOS PUBL LTD
Keywords
Mayer-Rokitansty-Kuster-Hauser syndrome; supernumerary ovary; ovarian cancer; pelvic mass; amenorrhea
Citation
ONCOLOGY LETTERS, v.5, no.2, pp.598 - 600
Indexed
SCIE
SCOPUS
Journal Title
ONCOLOGY LETTERS
Volume
5
Number
2
Start Page
598
End Page
600
URI
https://scholar.korea.ac.kr/handle/2021.sw.korea/104024
DOI
10.3892/ol.2012.1073
ISSN
1792-1074
Abstract
Mayer-Rokitansty-Kuster-Hauser (MRKH) syndrome is a Mullerian anomaly that presents with varying degrees of uterovaginal aplasia and is secondarily associated with cervicothoracic, auditory and skeletal anomalies. However, MRKH syndrome patients have normal and functional ovaries. A supernumerary ovary is an extremely rare form of an ectopic ovary and there are no reported cases of MRKH syndrome with cancer of the supernumerary ovary in the current literature. A 31-year-old female with a history of MRKH syndrome that was diagnosed 4 years previously presented with abdominal pain and a suspected malignant pelvic mass was identified. During the staging surgery, both ovaries were separated from the main mass, observed and removed. A third ovary was discovered in the pelvic mass and the diagnosis of primary ovarian cancer from the third ovary was confirmed by immunohistochemistry. We report the first known case of cancer of the supernumerary ovary in a patient with MRKH syndrome. Although both ovaries were confirmed to be normal in the patient with MRKH syndrome, we propose that an ovarian neoplasm should be considered in the diagnosis of a pelvic mass.
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