Diagnosis and Management of Ovarian Tumor in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome

Abstract

In the most recent publications on Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, the uterine remnants and ovaries in patients may develop uterine remnant leiomyoma, adenomyosis, or ovarian tumor, and this can lead to problems in differential diagnosis. Here we summarize the diagnosis methods and available interventions for ovarian tumor in MRKH syndrome, with emphasis on the relevant clinical findings and illustrative relevant case. According to the clinical findings and illustrative relevant case, with the help of imaging techniques, ovarian tumors can be detected in the pelvis in patients with MRKH syndrome and evaluated in terms of size. Laparoscopy could further differentiate ovarian tumors into different pathological types. In addition, laparoscopic surgery not only is helpful for the diagnosis of MRKH combined ovarian tumor, but also has a good treatment role for excising ovarian tumor at the same time. Moreover, laparoscopic removals of ovarian tumor can be considered as a safe and reliable treatment for conservative management.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update

“…Ovary anomalies are rare and only found in ~ 5–10% [ 3 , 13 , 101 ]. Different anomalies previously reported include unilateral agenesis, ectopic ovaries, polycystic ovaries, streak ovaries, and rarely tumors [ 102 ].…”

Herlyn-Werner-Wunderlich syndrome with borderline serous cystadenoma of the ovary: case report and literature review

“…Ovarian remnants neoplasms are rarely reported in these patients as they have normal ovarian development and function. A literature review by Miao et al (2018) [5] , collected few case reports of Mullerian ducts anomalies with various types of ovarian neoplasms, the majority of which were found to be benign after histopathological evaluation. Huepenbecker et al (2017) [6] , reported a rare occurrence of ovarian cystadenocarcinoma in two sisters affected by MRKH syndrome with normal 46XY karyotype and negative ovarian cancer genetic panel screening.…”

“…Patients with Mullerian dysgenesis syndrome have normal ovarian development. Few cases have been reported in the current literature about the development of uterine or ovarian tumors in patients with different classes of Mullerian dysgenesis but up to the authors’ best knowledge, there are no previous reports of ovarian tumor development in HWWS [5] , [6] , [7] . Thus, we report the first case of ovarian tumor development in HWWS.…”

Torsion of a rudimentary uterine horn with multiple leiomyomas in a case of Mayer-Rokitansky-Küster-Hauser syndrome

“…The three most common methods of diagnosing MRKH syndrome are MRI, ultrasound, and laparoscopy [1,6]. However, in MRKH syndrome with a pelvic mass, imaging tools have limitations with respect to diagnosing the origin of the mass because of the indistinct uterus and abnormal anatomy [11]. In a review of 6 cases of MRKH syndrome with ovarian tumors, laparoscopy was shown to offer the possibility of concurrent diagnosis and treatment [11].…”