Magnetic resonance imaging findings in pseudo-Meigs' syndrome associated with a large uterine leiomyoma: a case report

Danai Chourmouzi 0 Elissavet Papadopoulou 0 Antonios Drevelegas 0 0 Interbalkan Medical Center , Asklipiou, Pylaia 57001, Thessaloniki , Greece Introduction: Pseudo-Meigs' syndrome is a rare pathological entity characterized by the presence of a pelvic mass other than an ovarian fibroma. The mass is associated with ascites with or without hydrothorax. Case presentation: We describe the case of a 41-year-old Caucasian woman with a large uterine leiomyoma associated with massive ascites. A magnetic resonance imaging scan showed a large subserosal leiomyoma with multiple areas of cystic degeneration. Conclusion: To the best of our knowledge, this is the first reported case of pseudo-Meigs' syndrome caused by a uterine leiomyoma and diagnosed using magnetic resonance imaging. The pathophysiology of this syndrome and the role of magnetic resonance imaging are emphasized in this case report. - Introduction Pseudo-Meigs' syndrome is a rare pathological entity characterized by the presence of a pelvic mass other than an ovarian fibroma. The mass is associated with ascites with or without hydrothorax. We report the case of a patient with pseudo-Meigs' syndrome caused by a uterine leiomyoma. A magnetic resonance imaging (MRI) scan provided a detailed description of the tumor. Our patient's MRI results correlated well with histological findings and helped us make a diagnosis, which enabled the avoidance of a hysterectomy. To the best of our knowledge, currently fewer than 35 cases of pseudo-Meigs' syndrome have been reported in the literature and our case of pseudo-Meigs' syndrome is the first to be diagnosed using MRI. Case presentation A 41-year-old Caucasian woman presented at our hospital with a 12-month history of abdominal swelling, discomfort, urinary frequency and incontinence. She had regular menstrual cycles and had never been pregnant. Her clinical examination revealed a marked distension of her abdomen and a large palpable mass in her central pelvis. Gynecological vaginal ultrasound (US) showed abundant ascites in her pelvis, as well as a solid, smoothly outlined mass with heterogenous echogenicity. The mass seemed to extend from her pelvic cavity to her abdomen on the midline above the uterus. Her uterus and ovaries could not be identified separately from the pelvic mass. The mass was considered to be of adnexal origin. Laboratory test results of our patient showed the following values: serum carbohydrate antigen (CA)-125 level at 436.7 U/mL (normal value < 30 U/mL), fetoprotein (FP) at 2.8 ng/mL, (normal value<10 ng/ml) beta-human chorionic gonadotropin (-HCG) at 5.0 mIU/mL (normal value < 3), and carcinoembryonic antigen (CEA) 1.07 ng/mL (normal value < 5 ng/ml). An MRI scan was requested to further evaluate our patient and to determine the exact nature of her mass. The results of our patient's MRI scan revealed massive ascites and a heterogeneous ovoid pelvic mass measuring 13 16 cm. The mass had a broad connection to the uterus. It was located subserosally and extended superiorly from the posterior body and fundus of her uterus. The uterus was displaced inferiorly. She was noted to have a normal endometrial stripe, a normal junctional zone, and normal ovaries. The mass was heterogeneous and produced a predominantly low to intermediate signal intensity on T2-weighted images relative to that of the outer myometrium. Several small foci with very high signal intensities were also seen (Figure 1). The foci of high signal intensity on the T2-weighted images had low signal intensity on the T1-weighted images, and they showed no enhancement on the contrast-enhanced images, representing areas of cystic degeneration (Figure 2). Based on the above imaging findings, the diagnosis of a large subserosal leiomyoma with areas of cystic degeneration was made. We recommended excision of the mass without hysterectomy. Our patient underwent an exploratory laparotomy. A large firm mass, which originated from the uterine fundus, was seen. Multiple lobulated projections were also seen on the superior border of the mass (Figure 3). The mass was then excised and the ascitic fluid was drained. Histopathological examination of the mass revealed the presence of a uterine leiomyoma. Our patient had an uneventful post-operative course. Discussion Pseudo-Meigs' syndrome is characterized by the presence of ascites. It is also often characterized by pleural effusion caused by a pelvic tumor other than an ovarian fibroma. Tumors associated with pseudo-Meigs' syndrome are usually found in women's genitalia. The most commonly described tumor type is a leiomyoma, which is usually found in the uterus or the broad ligament [1]. Other reported ovarian tumors responsible for pseudo-Meigs' syndrome are struma ovarii tumors, mucinous or serous cystadenomas, germ cell tumors and ovarian metastasis from colon and stomach cancers [2]. Uterine leiomyomas are the most commonly reported cause of pseudo-Meigs' syndrome. They usually manifest as increased abdominal distension caused by a progressively enlarging pelvic mass and ascites. Respiratory Figure 1 (A) Axial and (B) coronal T2-weighted fast spin echo magnetic resonance images which show a large ovoid subserosal leiomyoma in the pelvis that extends superiorly from the body and fundus of the uterus. The mass is heterogeneous with predominantly low to intermediate signal intensity relative to that of the outer myometrium. Several small foci of very high signal intensity are also seen (arrows). A normal endometrial stripe and junctional zone are seen. Note the presence of massive ascites. Figure 2 (A) Axial T1-weighted spin echo and (B) contrast-enhanced axial T1-weighted spin echo magnetic resonance image which show similar enhancement of the normal myometrium and mass. The foci of high signal intensity on the T2-weighted image show no enhancement on the contrast-enhanced images representing areas of cystic degeneration (arrows). insufficiency caused by pleural effusion is also often encountered [3]. It is speculated that the presence of ascites results from mechanical irritation of the peritoneum and the leakage of intratumoral fluid from the degenerated leiomyoma. As leiomyomas enlarge, they may outgrow their blood supply, thus resulting in various types of degeneration. Cystic degeneration is considered to be a sequela of edema and is observed in about 4% of reported cases of leiomyomas. Cystic spaces appear as round, well-demarcated areas with signal intensities that have the characteristics of fluid, namely low on T1-weighted images and high on T2-weighted images with no enhancement [4]. Multiple lobulated, fluid-filled, grape-like cystic areas were seen in our patient. These cystic areas projected from the superior border into the peritoneal cavity which was presumed to be the cause of her massive ascites. Pleural effusions, which are commonly right-sided, result from transdiaphragmatic transport of ascitic fluid [5]. Although our patient (...truncated)