Large uterine leiomyoma presenting as pseudo-Meigs' syndrome with an elevated ca125: a case report and literature review.

A 35-year-old female presented to the emergency department with complaints of progressive abdominal pain, swelling and weight gain. She denied chest pain, shortness of breath, heat or cold intolerance, palpitations and any motor or sensory deficits. Surgical history was significant for a remote exploratory laparotomy for an abdominal stab wound. On examination, she was hemodynamically stable. Abdomen was distended and tender to palpation. A fluid shift was present. Computed tomography (CT) abdomen and pelvis demonstrated a 29 × 19-cm pelviabdominal mass with significant ascites. The mass contained both cystic and solid components as well as internal vascularity ( Fig. 1A and 1B ). (A) CT abdomen and pelvis with contrast identified a large pelviabdominal mass with internal vascularity measuring 29 × 19 cm and significant ascites; the mass contained both cystic and solid components. (B) The mass encasing the uterus. Pregnancy test was negative. She had an elevated CA-125 level of 272.9 ng/ml, suggesting ovarian cancer. The patient was taken to the operating room for an exploratory laparotomy, excision of intraabdominal mass, hysterectomy and bilateral salpingo-oophorectomy. Upon entry, 2 l of intrabdominal fluid were aspirated and sent to pathology. The mass was identified closely adherent to the anterior abdominal wall, sigmoid colon and two large areas of small bowel. It was dissected off the anterior abdominal wall, sigmoid colon and the two segments of small bowel. The two involved small intestinal segments were resected and anastomosed together. The tumor, which appeared to originate from the uterine fundus, was completely resected and sent to pathology. A total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. No intrabdominal metastasis were identified. The abdomen was closed and the patient tolerated the procedure well without complications. Pathology described an aggressive-behaving uterine leiomyoma. Ascitic cytology was negative for malignant cells.

Pseudo-Meigs’ syndrome presenting with an elevated CA-125 is a rare clinical entity. Although it often suggests malignancy, pseudo-Meigs’ syndrome should be considered in the differential diagnosis. Surgery is the mainstay of treatment with subsequent resolution of the ascites and hydrothorax.

Ascites, pelvic mass and an elevated CA-125 in women carries a grim prognosis, likely an ovarian carcinoma [ 9 , 10 ]. However, benign etiologies must also be considered [ 10 ]. Cases of Meigs’ and pseudo-Meigs’ syndrome with elevations in CA-125 have been reported [ 10–12 ]. An elevated CA-125 is not 100% specific for ovarian carcinoma [ 13 ]. It can be found in cases of pelvic inflammatory disease, pregnancy, endometriosis, benign ovarian tumors and uterine leiomyomas [ 14 ]. In cases of Meigs’, it has been suggested that mesothelial expression of CA-125, rather than expression by the fibroma, is the cause of the elevation [ 11 , 12 ]. In pseudo-Meigs’ peritoneal inflammation has been implicated as the cause of elevated CA-125 [ 10 ]. Clinically, Meigs’ and pseudo-Meigs’ syndrome are indistinguishable. Patients typically present with abdominal distention and a pelvic mass. Some report dyspnea due to pleural effusion [ 15 ]. These conditions are distinguished via imaging and pathology. Regardless of the underlying tumor in either Meigs’ or pseudo-Meigs, spontaneous resolution of the pleural effusion and ascites occurs after resection [ 15 ]. With this knowledge, surgeons can avoid potential morbidity of unnecessary tests and procedures in the workup and treatment. The pathophysiology of both Meigs’ and pseudo-Meigs’ syndromes are not entirely known but are thought to be similar [ 10 ]. In the formation of ascites, it is suggested that twisting of the tumor around its pedicle results in fluid production [ 16 ]. However, this fails to explain ascites in non-pedunculated tumors [ 10 ]. Others suggested that ascites could be caused by the mechanical pressure from the tumor on neighboring vessels and lymphatics [ 1 , 17 ]. This also seems unlikely, as tumors are often freely mobile within the abdomen and would not cause sufficient pressure for fluid transudation [ 1 , 17 ]. That said, fluid transudation resulting from insufficient venous and lymphatic drainage of a large tumor has also been suggested as a mechanism for ascites [ 18 ]. More likely, the formation of ascites and hydrothorax in Meigs’ and pseudo-Meigs’ is multifactorial in nature. It is reported that, intraoperatively, cutting a uterine leiomyoma causes fluid leakage from myometrial cysts within the tumor [ 10 ]. Thus, fluid production results from hydropic and subsequent cystic degeneration of the tumor [ 19 ]. Pressure within the leiomyoma itself might result in fluid transudation into the abdomen [ 10 ]. Also, peritoneal fluid production is also a factor in ascites. Mechanical irritation of the peritoneum from the tumor in addition to pressure from pre-existing ascites causes peritoneal inflammation, which results in further fluid production [ 20 ]. The formation of pleural effusion in these syndromes is thought to be a result of transdiaphragmatic transport of the ascitic fluid into the thorax [ 10 ]. The mechanism of fluid transport through the diaphragm is likely through intercellular gaps [ 21 , 22 ]. India ink studies have confirmed the transport of fluid into the pleural space [ 23 ]. A literature review using PubMed and Medline was performed, resulted in 21 cases describing pseudo-Meigs’ syndrome [ 3 , 6–8 , 10 , 13–15 , 24–36 ] ( Table 1 ). Mean age of presentation was 40. Fifteen of 21 cases presented with abdominal distention. Additionally, 8 of 21 cases reported dyspnea. Other reported symptoms were nausea, vomiting, dysuria and urinary frequency. Seventeen articles reported elevated CA-125 ( Table 1 ). Ascites was confirmed in all cases, and hydrothorax was reported in 16 cases. Effusion was demonstrated bilaterally in four patients. Uterine leiomyomas are the most common tumors found in association with pseudo-Meigs’ syndrome [ 3 ]. The average tumor size was 17.7 cm. All patients were treated surgically, with no reported cases of tumor recurrence. Articles reported pseudo-Meigs’ syndrome cases Y, years; cm, centimeter; NS, not specified; FU, follow-up; M, months; NR, no recurrence.

Meigs’ syndrome was first classified in 1954 as a triad of benign ovarian fibroma or thecoma with both ascites and hydrothorax [ 1 ]. A clinically similar yet distinct pathological entity is pseudo-Meigs’ syndrome. Like Meigs’, pseudo-Meigs’ syndrome involves ascites and hydrothorax. However, it is associated with non-thecoma, non-fibroma ovarian tumors and uterine leiomyomas [ 1 ]. Ovarian tumors implicated in pseudo-Meigs’s syndrome include struma-ovarii, germ cell tumors, ovarian metastases from gastric or colon cancer and serous or mucinous cystadenomas [ 2 ]. Uterine leiomyoma is commonly reported in cases of pseudo-Meigs’ syndrome [ 3 ]. Leiomyomas have an incidence of 20–50% in females over the age of 30, but rarely, they are found in association with ascites and hydrothorax [ 1 , 4 ]. Elevation in CA-125, in addition to the pseudo-Meigs’ syndrome is a rare finding [ 5–8 ]. We report a case of a pseudo-Meigs’ syndrome in a 35-year-old female who had markedly elevated CA-125. A literature review was performed, and cases of pseudo-Meigs’ syndrome were reported.