Hepatocellular Carcinoma in Pregnancy withUnusual Presentations
A 41-year-old pregnant woman referred to our hospital in the 22nd week of gestational age with right upper abdominal pain and jaundice. She had mild abdominal pain six weeks before presentation which was aggravated one week before admission. Abdominal pain was not related to food intake and referred to her right shoulder. Four days before admission, she had icter, tea colored urine and edema in her lower extremities. She had nausea and vomiting but no headache, blurred vision or bleeding was mentioned. She mentioned fatigue and hypertension from the eighth week of pregnancy. A past history was remarkable for two abortions and one live birth prior to this pregnancy. She took only ferrous sulfate and aspirin in this pregnancy and no other drugs, herbal medicines or oral contraceptives (OCPs).\nThere was no history of blood transfusion, hepatitis or exposure to aflatoxin in her past history however her father died from HCC due to hepatitis B virus (HBV). Laboratory investigations showed features of microangiopathic hemolytic anemia that included schistocytes, elevated prothrombin time and erythrocyte sedimentation rate (ESR), and negative viral markers(negative HBsAg and HBcAb). Differential diagnoses of HELLP syndrome, thrombotic thrombocytopenic purpura (TTP) or acute fatty liver of pregnancy (AFLP) were made for further evaluation and management.\nShe seemed ill and icteric at admission but not toxic. Her blood pressure was 110/70 mmHg with a pulse rate of 110 beats/min, respiratory rate about 18/min and oral temperature of 370C. Conjuctivae was pale and sclera was icteric. Jugular venous pressure was normal. Pulmonary sounds were diminished in the basal parts but cardiac examination showed no abnormalities. Her abdomen was tender, particularly in the right upper quadrant, but without rebound and guarding. Lower extremities had 3+ pitting edema and there were ecchymotic lesions at previous injection sites.\nLaboratory examination showed leukocytosis, anemia and lower limit platelet counts. Blood electrolytes were normal but there was direct hyperbilirubinemia and liver enzymes showed a cholestatic pattern. Lactate dehydrogenase (LDH) was elevated and haptoglobin was low. Peripheral blood smear showed features of microangiopathic hemolytic anemia that included target cells, tear drop and nucleated RBCs, and numerous schistocytes. Urine analysis was positive for microscopic hematuria and proteinuria.A 24-hour urine collection showed 226 milligrams of protein.\nViral markers of HBV and HCV, autoantibodies and Adisintegrin-like and metalloproteinase with thrombospondin type 1 motif (Adamts)-13 antigen and antibody (markers for Thrombotic Thrombocytopenic pupura=TTP) were all negative. AFP level was 90.7 and cancer antigen 125 (CA125) titer was >1000.\nTransabdominalsonography showed a normal fetus with a gestational age of about 23 weeks and normal amniotic fluid in the breech position. The liver showed a coarse echopattern with a diameter of 20 cm. The biliary ducts were normal and the gallbladder was contracted. No lesion was noted in the portal vein, which had a diameter of 10 mm. The inferior vena cava and suprahepatic vessels were normal. The pancreas, spleen and kidneys were all normal. There were no ascites, however bilateral pleural effusion was detected.\nA possible diagnosis of HELLP was made and supportive care for correction of coagulation disorder with fresh frozen plasma (FFP) and corticosteroids were administrated. Because of deterioration in her mental state in conjunction with an increased bilirubin level and international normalized ratio (INR), the pregnancy was terminated. Vaginal delivery was not possible because of the patient’s decreased mental state so a cesarean section was performed. The fetus was delivered and transferred to NICU but expired because of immaturity. At laparotomy, approximately one liter of ascitic fluid was noted in the abdomen and the liver had multiple malignant appearing mass lesions, which were biopsied with wedge resection. Pathologic evaluation showed sheaths of malignant hepatocytes typical of HCC ( ).\nAfter delivery the patient experienced progressive loss of conscious along with multi-organ failure and unfortunately died after a few days.