This report presents a rare case of a large twisted ovarian fibroma associated with pain, Meigs' syndrome and severe hemolytic anemia. This case highlights the complexities associated with the diagnosis and treatment of patients with severe complications of benign ovarian disease. In rare cases, ovarian fibromas are associated with ascites and pleural effusions, which is known as Meigs' syndrome. This syndrome is usually associated with large fibromas and high CA-125 levels.[8,9] In the present case, making an accurate diagnosis was complicated by a high CA-125 level associated with anemia and high levels of inflammatory markers (CRP and fibrinogen). These associations may indicate advanced ovarian cancer and our patient could potentially have been misdiagnosed with a malignant ovarian tumor. However, a high CA-125 level does not necessarily indicate ovarian cancer and can also be associated with ovarian fibroma and the accompanying ascites.[12,13] In addition, torsion of an ovarian fibroma with subsequent necrosis and inflammation can result in increased serum levels of CA-125 and inflammatory markers.[14,15] The serum CA-125 level does not seem to have high specificity for ovarian malignancy unless it is associated with specific ultrasound findings suggesting malignancy. Our patient did not have ultrasound findings suggesting malignancy and her condition was complicated by severe anemia. Anemia is associated with ovarian malignancy, which is also commonly associated with high levels of fibrinogen and CRP. In our patient, the serum bilirubin levels suggested hemolytic anemia and the hypoechogenic and acellular echographic characteristics of the effusions excluded ongoing hemorrhage. The association between hemolytic anemia and benign ovarian tumors has been recognized for a long time and complete resolution of the hemolysis has been reported after removal of the tumor. Payne et al. reviewed the clinical courses and responses to ovarian cystectomy in 19 patients with hemolytic anemia and benign ovarian tumors reported in the literature up to 1981. Further 11 cases have been reported since then,[20–30] bringing the total to 30 cases. The cases reported in the literature reveal the complexity of managing hemolytic anemia associated with ovarian tumors. One of the previously reported patients showed a good response to glucocorticoid therapy and recovered after ovarian cystectomy within 3 weeks of diagnosis. Two patients died prior to surgery, one because of intestinal obstruction and the other because of a transfusion reaction. One patient underwent simultaneous ovarian cystectomy and splenectomy, resulting in complete recovery. Some patients who did not respond well to initial glucocorticoid therapy or splenectomy recovered well after ovarian cystectomy. These different responses to therapy are interesting, but in our opinion it is difficult to understand why the therapeutic options are considered to conflict with one another, especially when the size of the ovarian tumor and the related symptoms indicate that surgical excision is the treatment of choice, as in our patient. Considering the high impact of splenectomy, it may be useful to initially plan glucocorticoid therapy and surgical excision of the ovarian tumor and perform splenectomy only in patients who do not respond to the initial therapy. We therefore first concentrated on effective medical management of our patient and chose the most appropriate surgical treatment after laparoscopic examination. The main aim of our initial approach was preoperative management of the anemia. Blood transfusions and glucocorticoid therapy resulted in stabilization of the hemoglobin level and normalization of the bilirubin levels, which confirmed the appropriateness of this approach. Laparoscopic surgery was performed after stabilization of the anemia.
We consider that a long waiting time before surgery should be avoided, as cure depends on surgical excision of the tumor, and the pathogenic mechanisms that trigger autoimmune responses in patients with benign ovarian tumors are unknown. It seems appropriate to administer medical therapy, including transfusion, to ensure that patients can undergo early surgery. In our patient, this approach was necessary because of the size of the ovarian mass, the ovarian torsion and the resulting severe pain. Furthermore, it is known that large ovarian masses in Meigs' syndrome are often associated with intra-abdominal hypertension up to abdominal compartment syndrome. The chronic development of abdominal hypertension and onset of the abdominal compartment syndrome associated with Meigs' syndrome must be recognized in a timely manner and promptly treated by performing as complete a resection of the pelvic mass as possible.
Laparoscopic surgery 4 days after admission enabled definitive diagnosis of the tumor, confirmed torsion and removed the bulky ovarian fibroma, resulting in timely resolution of symptoms, short hospitalization, relatively low morbidity and a rapid return to her social and professional life. Meigs' syndrome always requires surgical treatment and the laparoscopic approach was successful in this case, with careful handling of the tumor because 1% of well-circumscribed ovarian tumors are malignant.[32,33] In cases of large potentially malignant ovarian masses, laparoscopic surgery may have several potential limitations (tumor rupture, spillage, incomplete resection of lesion and trocar insertion site metastasis). On the other hand, a recent review concluded that there was no good-quality evidence to help quantify the risks and benefits of laparoscopy for the management of early-stage ovarian cancer as routine clinical practice.
In conclusion, laparoscopic surgery for potentially malignant tumors may be feasible and safe but requires an experienced gynecological oncology team and is beyond the expertise of the general surgeon. Then, diagnostic laparoscopy is useful in patients with potentially malignant tumors and laparoscopic tumor resection can be performed also if there are signs of malignancy and removal can be achieved without peritoneal contamination. In the present case, laparoscopic resection was feasible, safe and effective for removal of the large ovarian fibroma.
BMC Surg. 2014;14(38) © 2014 BioMed Central, Ltd.
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