CS-iCCA, A New Clinically Based Staging System for Intrahepatic Cholangiocarcinoma: Establishment and External Validation.

INTRODUCTION: Intrahepatic cholangiocarcinoma (iCCA) is a primary liver malignancy with poor prognosis. Current prognostic methods are most accurate for patients with surgically resectable disease. However, a significant proportion of patients with iCCA are not surgical candidates. We aimed to develop a generalizable staging system based on clinical variables to determine prognosis of all patients with iCCA. METHODS: The derivation cohort included 436 patients with iCCA seen between 2000 and 2011. For external validation, 249 patients with iCCA seen from 2000 to 2014 were enrolled. Survival analysis was performed to identify prognostic predictors. All-cause mortality was the primary end point. RESULTS: Eastern Cooperative Oncology Group status, tumor number, tumor size, metastasis, albumin, and carbohydrate antigen 19-9 were incorporated into a 4-stage algorithm. Kaplan-Meier estimates for 1-year survival were 87.1% (95% confidence interval [CI] 76.1-99.7), 72.7% (95% CI 63.4-83.4), 48.0% (95% CI 41.2-56.0), and 16% (95% CI 11-23.5), respectively, for stages I, II, III, and IV. Univariate analysis yielded significant differences in risk of death for stages II (hazard ratio [HR] 1.71; 95% CI 1.0-2.8), III (HR 3.32; 95% CI 2.07-5.31), and IV (HR 7.44; 95% CI 4.61-12.01) compared with stage I (reference). Concordance indices showed the new staging system was superior to the TNM staging for predicting mortality in the derivation cohort, P < 0.0001. In the validation cohort, however, the difference between the 2 staging systems was not significant. DISCUSSION: The proposed independently validated staging system uses nonhistopathologic data to successfully stratify patients into 4 stages. This staging system has better prognostic accuracy compared with the TNM staging and can assist physicians and patients in treatment of iCCA.

Acute Fatty Liver of Pregnancy.

BACKGROUND Acute fatty liver of pregnancy (AFLP) is a rare obstetric emergency resulting from microvesicular infiltration of the liver by fat, leading to liver failure. It usually presents at 36 weeks of gestation, and risk factors include twin pregnancy and low BMI. The presentation of AFLP is nonspecific, requiring a high index of suspicion. The Swansea Criteria is used to aid diagnosis. CASE REPORT Case 1: A 23-year-old woman, G1P0 at 39 weeks of gestation, presented to the hospital with a 1-week history of fever, nausea, vomiting, and diarrhea. Examination revealed a gravid uterus with generalized abdominal tenderness. Laboratory investigations revealed elevated liver enzymes, with elevated total bilirubin and an INR of 1.26. CBC showed leukocytosis. Abdominal ultrasound was normal. Workup for other etiologies, including acetaminophen and salicylate overdose and infections, was negative. The Swansea score for AFLP was 8, confirming the AFLP diagnosis. An emergency Cesarean-section was performed, causing liver enzymes to improve over 3 days. Case 2: A 41-year-old woman, G1P1 with a twin gestation at 36 weeks, presented with a 3-day history of abdominal pain. She was jaundiced, with right upper quadrant tenderness. Laboratory investigations showed elevated liver enzymes and total bilirubin, with an INR of 1.26. Workup for viral hepatitis and autoimmune etiology was negative. Salicylate levels were within normal limits. She met criteria for AFLP and underwent emergency Cesarean-section. Liver enzymes improved over 4 days. CONCLUSIONS AFLP is a potentially life-threatening medical condition. From our experience, prompt diagnosis and early delivery leads to improved maternal and fetal outcomes.