Introduction

Meigs’ syndrome is characterized by nonmalignant ascites and pleural effusions in women with ovarian fibromas, thecomas, granulosa cell tumors, or Brenner’s tumors [1]. Importantly, both the ascites and pleural effusions spontaneously resolve after removal of the ovarian tumor. Pseudo-Meigs’ syndrome refers to the same clinical presentation and course in the setting of other ovarian tumors. Pseudo-Meigs’ syndrome is rare and most often associated with struma ovarii or uterine tumors such as leiomyomas; however, there are reports associated with ovarian metastases, including gastrointestinal malignancies [2]. Here, we report the first case of pseudo-Meigs’ syndrome secondary to metastatic pancreatic adenocarcinoma. In addition, the reported patient presented during pregnancy. Pseudo-Meigs’ has been described during pregnancy, though this is the first case reported from ovarian metastases.

Case presentation

A 31-year-old Caucasian female with no significant past medical history developed significant weight loss and progressive, positional dyspnea during the third trimester of her second pregnancy. Abdominal ultrasound revealed a normal pregnancy but bilateral ovarian cystic masses were noted and felt to reflect luteal cysts. At 34 weeks, she had a rupture of membranes and underwent a cesarean section. She was found to have significant ascites and large bilateral ovarian masses. Cytologic analysis of the ascites did not reveal any malignant cells. Postoperatively, her dyspnea worsened. A non-contrast computed axial tomography scan revealed a large right pleural effusion, a large amount of ascites, and bilateral ovarian masses (17.7 × 14.4 cm on the right and 16 × 10 cm on the left). With a suspicion for a primary ovarian neoplasm, she then underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histopathologic analysis of the ovarian tissue revealed a mucinous adenocarcinoma consistent with a metastatic pancreaticobiliary cancer. A serum CA 19-9 level was elevated to 258.8 U/mL. After recovery from her surgery, a positron emission tomography (PET) scan confirmed a hypermetabolic (SUV 9.18) pancreatic tail mass encasing the celiac axis measuring 3.3 × 5.5 cm. She was also found to have a hypermetabolic (SUV 15.88) sclerotic lesion of the left iliac bone, and biopsy of this lesion confirmed metastatic pancreatic adenocarcinoma. This PET scan, which was performed prior to initiation of systemic therapy, also revealed resolution of her ascites and her pleural effusion. She then began systemic therapy with gemcitabine and oxaliplatin, achieving a modest radiographic response and a decrease in her CA 19-9 level from 258.8 U/ml at diagnosis to 61 U/ml after 6 months of treatment. Due to progressive neuropathy, therapy was then changed to gemcitabine and nab-paclitaxel, which she has tolerated well. At the time of this report, she is doing well on therapy 10 months after diagnosis with minimal symptoms. Neither the ascites nor the pleural effusions have reaccumulated to date.

Discussion

Pancreatic cancer during pregnancy is a rare phenomenon; in the English literature, only nine cases have been reported. Due to the nonspecific signs and symptoms, diagnosis has unfortunately been late. In addition, efforts to minimize radiation exposure to the fetus often limit diagnostic radiographic procedures during pregnancy. Of the nine previously published cases (Table 1), three were initially diagnosed as pancreatitis [3–5]. The other published cases presented with either jaundice or nonspecific symptoms such as weight loss and epigastric pain [6–11]. The case we report details a patient with pancreatic adenocarcinoma during pregnancy that presented with pseudo-Meigs’ syndrome.

Table 1 Summary of cases of pancreatic carcinoma described during pregnancy
Full size table

Pseudo-Meigs’ syndrome is a rare occurrence and has not been previously described in a patient with pancreatic cancer; this may be partially due to underdiagnosis. Ascites is commonly found in patients with metastatic pancreatic adenocarcinoma and is often assumed to be malignant. When cytologic analysis yields no malignant cells, this may be regarded as a false-negative result, and in many cases, diagnostic testing may be bypassed altogether. Pseudo-Meigs’ syndrome should be considered when ovarian metastases are present. While not common, ovarian metastases are well described in patients with pancreatic cancer. One series of 59 patients with ovarian metastases noted a pancreatic primary in over 5% of cases [12]. The pathophysiology underlying pseudo-Meigs’ syndrome is poorly understood, but inflammatory cytokines such as IL-1 beta, IL-6, and IL-8, as well as vasoactive factors such as fibroblast growth factor and vascular endothelial growth factor may play a role in the fluid accumulation [13, 14].

Conclusion

Pseudo-Meigs’ syndrome is an uncommon cause of ascites. When treating a patient with ascites, establishing the etiology of ascites can be critical, as the management is potentially quite different. Generally, surgery in the setting of metastatic pancreatic adenocarcinoma is not expected to provide long-term disease-free survival and will not be offered. In cases of pseudo-Meigs’ syndrome, however, surgery may be improve refractory ascites and pleural effusions, and while not curative, surgery in this instance may provide dramatic relief of symptoms and an improvement in a patient’s quality of life. We report this unusual case to highlight the challenging diagnosis of pancreatic cancer during pregnancy and to draw attention to the rare phenomenon and unique management of pseudo-Meigs’ syndrome, previously undescribed in pancreatic adenocarcinoma.