Alex Holt, DO1, Ioannis Papadopoulos, MD2; 1Madigan Army Medical Center, Lakewood, WA; 2Madigan Army Medical Center, University Place, WA
Introduction: Hepatoid carcinoma (HC) of the pancreas is a rare malignancy with only 34 cases reported in the literature. HC displays morphological and serological features similar to hepatocellular carcinoma and may arise in organs derived from the foregut endoderm, such as the pancreas. We present a case of a gentleman with unresectable HC of the pancreatic head who presented with painless jaundice and weight loss.
Methods: A 58-year-old Caucasian male presented acutely to our facility with a history of painless jaundice, scleral icterus, and 20 pound unintentional weight loss. Laboratory tests revealed a total bilirubin of 31.6 mg/dL and an alkaline phosphatase of 424 mg/dL. A 6 cm mass in the pancreatic head was seen on computed tomography. The patient underwent endoscopic ultrasound with fine needle aspiration of the mass and endoscopic retrograde cholangiography with biliary stenting to relieve the obstruction. Immunohistochemical studies revealed the mass to be CK7, PASD, CEA, and HepPar-1 positive, consistent with HC of the pancreas. Imaging studies demonstrated involvement of the portal vein with mediastinal lymphadenopathy and staged his tumor as T3N2M0. He therefore was not a candidate for surgical resection. FOLFIRINOX chemotherapy was started, however follow-up imaging displayed progression of disease, including metastatic disease to the left lung and the surrounding lymph nodes. He subsequently developed recurrent biliary obstruction and gastric outlet obstruction due to tumor growth. This was treated with repeat biliary stenting and placement of a duodenal stent. Unfortunately, he had continued disease progression and died 10 months after his initial presentation. Discussion: Hepatoid carcinoma of the pancreas is a rare and aggressive cancer with no known etiology or standardized treatment regimen. Explanations in the literature include origin from ectopic liver tissue or transdifferentiation. Patient prognosis depends on the stage at diagnosis. To date, all documented cases of unresectable HC led to patient death within one year of diagnosis. Diagnosis is made with a combination of clinical, laboratory, imaging, and immunochemical features. Further study is required to more fully understand the etiology of hepatoid carcinoma to help develop guidelines for adjuvant therapy options if the cancer is unresectable.
Disclaimer: The views expressed are the authors and do not reflect the official policy of the Department of the Army, the Department of Defense or the U.S. Government.
Fluoroscopic image of esophagogastroduodenoscopy post biliary stent placement.
Endoscopic ultrasound image of pancreatic head mass.
Computed tomographic image with IV contrast at the time of initial presentation showing the 4.6 x 5.3 x 6 cm mass in the pancreatic head abutting the portal vein.
Disclosures: Alex Holt indicated no relevant financial relationships. Ioannis Papadopoulos indicated no relevant financial relationships.