Pediatric pseudo-Meigs' syndrome secondary to a Sertoli-Leydig cell tumor with elevated cancer antigen 125 and alpha-fetoprotein: A case report.

INTRODUCTION

Pseudo-Meigs' syndrome is described as the triad of a pleural effusion, ascites, and pelvic or abdominal tumor that is not an ovarian fibroma or thecoma. To our knowledge, this is the first reported pediatric case of pseudo-Meigs' syndrome secondary to a Sertoli-Leydig cell tumor (SLCT).

PRESENTATION OF CASE: A 12-year-old female with a past medical history of asthma and obesity (body mass index of 37) presented to the emergency department with complaints of abdominal pain, loss of appetite, irregular menses, and shortness of breath for one month. An abdominal ultrasound was obtained which showed a very large echoic heterogeneous structure involving the entire abdomen and moderate perihepatic and pelvic ascites. Computed Tomography (CT) imaging showed a large right pleural effusion with collapse of the right lower lobe, partial collapse of the right middle and upper lobes, and a 21.6 × 21.3 × 14.6 cm complex cystic mass in the abdomen. Tumor markers were significant for an elevated Cancer Antigen-125 (CA-125) of 1227 U/mL and alpha-fetoprotein (AFP) of 2385 ng/mL. She subsequently underwent right pleural thoracentesis, right tube thoracostomy, exploratory-laparotomy, and left salpingo-oophorectomy. Surgical pathology was consistent with a poorly differentiated SLCT.

DISCUSSION: SLCT are rare sex cord-stromal ovarian tumors that produce testosterone and account for less than 0.5 % of ovarian tumors. They are a rare cause of Psuedo-Meigs' syndrome.

CONCLUSION

Pseudo-Meigs' syndrome secondary to a SLCT in an adolescent is an exceedingly rare diagnosis. Multidisciplinary care for these patients consisting of pediatric surgery, general pediatrics, and pediatric hematology/oncology services is critical for management.