Guzman, Alexander Joseph Hipolito, April Lorraine Liver Mass Domingo, Elizabeth Guzman, Alexander Joseph Hipolito, April Lorraine

Case 1b General Data: 35 year old, male Nonsmoker, non-alcoholic elevated AFP (400 ng/ml) on routine tests Asymptomatic No history of Hepa B infection No history of blood transfusion PE is essentially unremarkable

Differential Diagnosis For elevated AFP: 400ng/ml (N= < 10ng/ml) Also consider laboratory error and ask for repeat AFP level determination 1. Hepatoma - primary malignant tumor of the liver - may present w/ hepatosplenomegaly, pain, hypoglycemia, weight loss or anorexia, ascites, portal hypertension, jaundice 2. Hepatocellular Carcinoma - has elevated AFP in 60-75% of the cases, commonly presents w/ enlarged liver on palpation, with sufficient irregularity or nodularity to permit differentiation from cirrhosis

Differential Diagnosis 3. Alcoholic Cirrhosis - may be clinically silent and in many cases(10-40%) are discovered incidentally at laparotomy or autopsy 4. Testicular tumor - malignant teratoma, embryonal cancer, yolk sac tumor. - unlikely in our case, px is already 35 y/o 5. Chronic hapatitis - unlikely in our case, no hx of hepatitis infection nor blood transfusions

Probable Diagnosis Fibrolamellar Hepatocellular Carcinoma - a distinctive clinical and pathologic subtype of hepatocellular carcinoma (HCC) - occurs in young adults (20-40 y/o) without underlying parenchymal liver disease - alpha-fetoprotein level are usually not elavated - non-encapsulated but well circumscribed and contains fibrous lamellae - slowly growing tumor

 AFP levels Hepatitis and cirrhosis 1° Liver CA CA of pancreas/stomach/biliary tract Malignant teratomas

Diagnostic Evaluation Abdominal CT scan is the preferred test for the detection, diagnosis, staging, and postoperative follow-up of HCC MRI is useful in detecting & characterizing primary tumors. It is more sensitive in detecting multiple intrahepatic recurrent lesions but less sensitive in detecting extrahepatic disease.

Ultrasound can be used intra-operatively as well as monitoring the progression of known intrahepatic lesions. Radionucleotide sulfur-colloid scans are ocassionally useful in the differentiation of fibrolamellar carcinoma from other tumors. Percutaneous biopsy with ultrasound guidance may be necessary for a definitive pre-operative diagnosis of fibrolamellar HCC.

Lab Studies AFP levels may be elevated since this protein is commonly produced by HCC. However, this is not a sensitive parameter since AFP levels may be normal in more than one-third of the patients. Normal adult levels of AFP are below 10 ng/mL

Follow-up Fibrolamellar carcinoma is an aggressive tumor that progresses to recurrent liver masses and metastatic lymph node metastases in most patients. Recurrent lesions often develop 6-18 months after attempted curative resection and may progress rapidly; therefore, follow-up imaging is recommended at 2- to 4-month intervals for at least 12-18 months after resection of the primary tumor. The early detection of metastatic disease is important because surgical resection of metastases improves patient survival rates.

Management Medical Care Surgical Care

Medical Care Patt et al reported a phase II clinical trial of systemic, continuous 5-fluorouracil and thrice-weekly subcutaneous recombinant interferon alfa-2b for liver cancers. demonstrated that this regimen was an effective treatment for patients with FLC, suggesting that neoadjuvant chemoimmunotherapy may play a previously unappreciated role in treatment of FLC

Surgical Care Hepatic Resection Liver Transplantation

Hepatic Resection problems: presents of advanced-stage disease direct invasion of adjacent organs lymphadenopathy limited metastais presence of extensive liver involvement contraindication: involvement of the main portal vein or hepatic artery

Liver Transplantation problems: limited donor availability with attendant waiting lists high cost sustained morbidity contraindications: presence of extrahepatic disease comorbid factors presence of vascular invasion poorly differentiated histologic grade

Prognosis 48% resectability rate 32 months average survival time 63% 5-year survival time prognosis better than usual type of hepatocellular carcinoma