Placental Site Trophoblastic Tumor—Unusual Cause of Nodular Regenerative Hyperplasia and Portal Hypertension: Case Report: 2430

INTRODUCTION: There are various causes of non-cirrhotic portal hypertension which can be classified as pre hepatic, intra hepatic and post hepatic. One of the causes of idiopathic non cirrhotic portal hypertension is nodular regenerative hyperplasia (NRH). There are various disorders which are associated with NRH. We report a case of placental site trophoblastic tumor as a cause of NRH and portal hypertension. CASE DESCRIPTION/METHODS: A 36 years old Caucasian female with no known medical illness presented with progressive abdominal swelling of 5 months duration. She had a normal vaginal delivery to a healthy baby one year back. She gained almost 40 pounds since she started to notice abdominal distention. She has no history of alcohol intake or medication use. On physical examination she had markedly distended abdomen, with fluid wave and small umbilical hernia. She had Pitting edema of 3+ in the extremities. Investigations revealed that the platelet count was 114, Sodium 124, ALT 41, AST 46 and albumin 2.6. Otherwise white cell count, hemoglobin, bilirubin, PT and INR values were within normal limit. Viral hepatitis panel and work up for other causes of chronic liver disease were unrevealing. Abdominal ultrasound showed ascites and mild splenomegaly. It also showed a normal liver echotexture, no ductal dilatation and a patent portal vein. Ascitic fluid analysis was suggestive of portal hypertension with SAAG of 2. Transjugular liver biopsy was suggestive of nodular regenerative hyperplasia. Portal pressure was elevated at 11 mmHg. On abdominal CT scan an incidental enlarged heterogeneous uterus with prominent sub endometrial and parametrial vessels was noted. Serum HCG was high (762). Subsequent hysteroscopy and endometrial biopsy revealed trophoblastic maturation suggestive of trophoblastic tumor. PET scan showed that the disease was limited to the uterus. The patient underwent total hysterectomy with bilateral salpingectomy. She was also treated with three cycles of chemotherapy. The patient experienced total resolution of here symptoms. Four months after her surgery the patient continues to be symptom free with resolution of ascites and leg swelling without any medications. DISCUSSION: Idiopathic non cirrhotic portal hypertension can be associated with a variety of conditions including hematologic, neoplastic, immunogenic and medication related. To our knowledge, this is the first report of nodular regenerative hyperplasia associated with trophoblastic tumor.