Fatal Case of Bilateral Luteinized Thecoma with Sclerosing Peritonitis in a 33-Year-Old Woman

Thecomas are sex-cord stromal tumors, included in the thecoma- fibroma group by the World Health Organization. Sclerosing peritonitis has characteristic peritoneal alterations and microscopic findings that can lead to intestinal occlusion. The coexistence of thecoma and sclerosing peritonitis is very rare: 25 cases have been reported in the literature, with most patients treated surgically by bilateral oophorectomy. The operative morbidity and mortality is estimated to be as high as 50%, varying by etiology: from 6% in young patients with idiopathic disease, 28% in adults, and up to 80% in patients undergoing peritoneal dialysis. We present a 33-year-old Caucasian woman who presented to the general emergency department of our hospital with abdominal pain and the sensation of abdominal fullness and distention. She underwent exploratory laparotomy, which revealed copious ascitic fluid and a 15-cm mass, twisted and necrotic, dependent from the left ovary. A similar 10-cm tumor was found in the pouch of Douglas, dependent from the right ovary. The peritoneum was thickened. The omentum was remarkably retracted and had the hard consistency suggestive of tumor infiltration. Bilateral salpingo-oophorectomy with peritoneal and omental biopsies was performed. Despite the high suspicion for malignancy, histology revealed acellular ascitic fluid. Histology of the ovarian sections showed a lax, edematous cellular substrate, with a few dense areas of spindle cells with elongated nuclei and very few mitoses. Inmediatly after surgery, the patient developed recurrent ascites, intestinal obstruction, and renal failure. Computed tomography revealed intense peritoneal involvement, so we started treatment with high-dose dexamethasone. Unfortunately, the treatment was unsuccessful and the patient died of multiorgan failure. Sclerosing peritonitis associated with ovarian thecoma is a rare entity. Clinicians should suspect this condition when bilateral ovarian tumors are accompanied by large amounts of ascites and severe peritoneal involvement, particularly when peritoneal histology reveals no malignancy.