Co-occurrence of Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian cancer: A case report and review of the literature.

ABSTRACT:

Background

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a congenital disorder of yet unknown etiology, characterized by agenesis/hypoplasia of the müllerian duct system. The occurrence of ovarian cancer (OC) in MRKHS is rare, with <20 cases reported to date.

Case

A woman affected with MRKHS, developed an abdominal mass at the age of 33 years. Surgical examination revealed a blind vagina, small rudimentary uterus, two fully developed tubes and large bilateral ovarian tumors. The histological diagnosis was a low-grade serous carcinoma (LGSOC) of both ovaries, staged IIB. The patient showed a normal female karyotype and resulted negative at the BRCA1/2 genetic testing.

Conclusion

This is the first report of a LGSOC in a patient with MRKHS. Although the identificati

SUBMITTER: Villa R 

PROVIDER: S-EPMC6431729 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature