The Demons-Meigs syndrome (DMS) in its initial typical form is a pathology that combines ovarian fibroma or fibrothecoma, ascites, and hydrothorax [
We report the first documented case of a rare Demons-Meigs syndrome in our country associating bilateral fibrothecoma, ascites, and bilateral pleural effusion with significant elevation of the tumor marker CA-125.
The patient, Z.A.S, a 35-year-old black African woman, housewife, seventh gravida and fourth para, with 5 living children, was received at the gastrointestinal surgery emergency of the University Hospital of Cocody (Abidjan, Cote D’Ivoire) on 25/05/2012 for abdominopelvic pain and abdominal mass. The beginning dates back to about a year by the occurrence of progressive abdominal distension in a context of amenorrhea and asthenia. Abdominopelvic pain is of recent onset (approximately 10 days prior to the patient admission) associated with a dry cough and difficulty breathing.
On admission, his general condition was altered. She was emaciated, dyspneic, and very asthenic. Conjunctivae were slightly colored, without jaundice. She had no edema or collateral venous circulation. Taking constant gave blood pressure = 10/06 Cmmhg, pulse = 120 bpm, breathe frequency = 22 cycles/min.
Physical examination showed an abundant ascites and a large abdominopelvic mass, diffuse, solid, mobile, and slightly painful in its entirety with increased pain in the pelvis. This mass extended from the pelvis to the
The chest radiograph (Figure
Chest radiograph showing a bilateral hydrothorax.
Abdominal CT scan showing two abdominopelvic tissue masses multilobulated.
Given the importance of dyspnea, pleural paracentesis (500 mL) and ascites (4.5 liters) were performed in emergency. Microscopic and chemistry study of both effusions was in favor of a transudate without any suspected malignancy cells.
The biological study of the liver and kidney was normal. A complete blood count was noted anemia to 7.9 g/dL. Tumor markers were measured: CEA (carcinoembryonic antigen) = 4.77 ng/mL (normal < 5 ng/mL); serum CA-125 level = 1835 IU/mL, more than 60 times above the normal superior limit (normal < 30 IU/mL); AFP (alpha-fetoprotein) = 8.1 ng/mL (normal < 20 ng/mL); ECG (electrocardiogram) was normal.
Given the importance of the alteration of the general condition and particular high elevated serum CA-125 with normal levels of other markers, the probable preoperative diagnosis of ovarian cancer was retained despite the lack of evidence of ovarian carcinoma at CT scan. After multidisciplinary consultation, an exploratory laparotomy was decided and performed on 03/06/2012 one week after the patient’s admission.
Intraoperatively after a midline incision under and above the umbilicus, we find a 2.5 liter of ascites and two large ovarian masses (Figures
Macroscopic view of the masses.
View after formalin fixation: high tumors multilobulated. The smallest depended on the left ovary
Slice homogenous section
We performed a total hysterectomy with bilateral salpingoophorectomy. A blood transfusion of 1 liter of packed cells was required associated with analgesics. The postoperative course was uneventful without ascites reconstitution and the patient was discharged to J 12. Histological analysis of the two surgical specimens whose result is achieved 28 days later after the intervention concluded a double ovarian fibrothecoma which is a begnin tumor (Figures
Histology of both surgical specimens in favor of ovarian fibrothecoma.
HE×100: clusters of cells cytoplasm rich in fat backing the core periphery
HE×100: tumor proliferation of spindle cells without cytonuclear atypia entangled arranged in short beams
The patient was revisited in consultation each month until the 3rd postoperative month. Clinical examination noted a lack of pleural effusion confirmed by chest radiography and absence of ascites by ultrasound postoperative control. The level of serum CA-125 was normal at last control. It then concluded at a Demons-Meigs syndrome.
The Demons-Meigs syndrome typically though not exceptional is a rare clinical and pathological [
Ovarian fibroma is a benign tumor most frequently observed in this syndrome in the order of 80–85% and may be pure and nonsecreting or associated thecoma elements (fibrothecoma) sometimes responsible for estrogen secretion. Thecomas and fibrothecomas represent 10% of cases [
The prevalence of DMS begins to increase in the third decade and increases progressively to peak in the seventh decade. Although the syndrome may affect women of any age in both full genital activity (as our observation) and in prepubertal period [
Despite the many cases of the Demons-Meigs syndrome or pseudo-Meigs syndrome published in the literature, the mechanisms of formation of peritoneal and pleural effusions composing this syndrome remain unclear and speculative. Several theories attempt to explain the etiology of these two elements.
In DMS, ascites is present in 10–15% of cases and hydrothorax is found in only 1% of cases [
The hydrothorax is mostly unilateral and occurs most often on the right-sided (75%), rarely left-sided, but can be bilateral as in the observation that we present.
Indeed ascites once formed would spread through the diaphragm or via the lymphatic vessels of the foramen of Bochdalek in the chest cavity, more developed in the right than the left explaining the frequency of right-sided pleural effusion. Blockage of these lymphatics prevented the accumulation of pleural fluid and caused an increase in ascetic fluid. However, the minimum volume of ascites that can induce hydrothorax is not known [
Ascitic and pleural fluids in Demons-Meigs’ syndrome are similar in nature. It may be either transudative or exudative with a high frequency of exudate. The elevation of the tumor marker CA-125 is a confounding factor for that the CA-125 is increased in 80% of epithelial ovarian cancers but also in other malignancies and benign tumors [
The circumstances of discovery are variable and were the consequences of elements of the triad syndrome (ascites, abdominal mass, and hydrothorax). It can be a tension with abdominal bloating and weight gain or weight loss, respiratory distress associated with a cough or abdominal pain secondary to adnexal torsion as in the case we describe. A more or less pronounced alteration of the general condition can be observed. It may be a fortutious discovery during a routine gynecological examination, especially when the patient is asymptomatic.
Physical examination will typically find ascites, pleural effusion, and abdominal mass of variable importance. In the literature, most patients present an ovarian asymptomatic mass, solid, and unilateral, most often left-sided whose dimensions can be large [
Chest radiography confirmed hydrothorax while abdominopelvic ultrasound confirms the presence of a mass and ascites. The abdominopelvic CT scan specify the ovarian origin of mass and no signs of distant metastases. In our case, the CT scan did not diagnosis with certainty the organs which depend on the masses. Laparoscopic exploratory, which is still a luxury in our context, is reserved for masses of small dimensions. CA-125 can be increased but is not a diagnostic element because it is not specific to ovarian origin in the context of ascites; cytology of ascites and hydrothorax does not show any cells suggestive of malignancy [
In our case, such elevated rate of CA-125 (more of 60 times beyond the upper normal limit) with pronounced impairment of general condition and a tumor filling the whole abdominal cavity associated with serous effusions imitated advanced malignant ovarian tumor. In fact, any ovarian tumor with ascites and serous effusions should be considered malignant until proven otherwise histologically. Thus, the preoperative diagnosis of Demons-Meigs’ syndrome or pseudosyndrome is difficult because of the clinic [
Medical treatment is purely symptomatic and includes thoracocentesis and draining of ascites. Blood transfusion was necessary in our patient because of her preoperative anemia. Curative treatment is surgical by laparotomy in almost all cases [
The Demons-Meigs syndrome, although it strongly mimics the clinical picture of advanced ovarian cancer, remains a disease with benign prognosis after being properly managed. It should be considered systematically to all women presenting with a pelvic mass whatever the size or number associated with ascites and unilateral or bilateral hydrothorax with or without elevated CA-125 and in whom there is no concept of heart failure, nephrotic syndrome, or liver failure. The importance of general signs should not exclude the surgery. The histology of the tumor should confirm the syndrome but complete and definitive remission after surgery is also an important element of diagnosis. This is a rare condition that must be known and recognized by practitioners to avoid unnecessary practices.
A consent has been obtained from the patient for the publication of this paper and the accompanying images.
The authors declare that they have no conflict of interests.
The patient was admitted under the care of A. Kouassi during this episode. B. Akpa and S. Koui have made the review of the literature for the references. S. Koui has made the histological analysis. The patient was operated by V. A. S. Loue, E. Gbary, and B. Akpa. V. A. S. Loue and E. Gbary were major contributors in writing the paper. All the authors read and approved the final paper.