1762 A Rare Case of an Alpha-Fetoprotein-Producing Esophageal Adenocarcinoma

Abstract

INTRODUCTION: AFP-producing esophageal adenocarcinoma (EAC) is a very rare occurrence. Elevation of serum AFP is commonly associated with hepatocellular carcinoma and yolk sac tumors, but rarely with esophageal carcinoma. This is a case of a 75-yr-old female who presented with progressive weakness and decrease in appetite, she was diagnosed with EAC despite high suspicion for HCC due to the extremely elevated AFP level. Prognosis is poor given the advanced presenting stage and high metastatic potential when finally diagnosed. CASE DESCRIPTION/METHODS: A 75-yr-old female with a past medical history of gastroesophageal reflux disease and Barrett’s esophagus status-post multiple upper endoscopies and a colonoscopy presented with progressive weakness, decrease in appetite, and abdominal pain over a few weeks. She also complained of dysphagia for solid food, and recent weight loss. On physical exam, she had mild epigastric tenderness. Her labs revealed markedly elevated CEA of 742, alpha-fetoprotein 46,135, and CA 19-9 of 6842. In the hospital, she had tachycardia so a CTA of her lungs was done to rule out a pulmonary embolism but instead revealed gastric thickening, celiac adenopathy, and multiple large liver masses. These findings were confirmed by a CT of her abdomen and pelvis with and without IV and oral contrast. Upper endoscopy revealed a large non-obstructing mass in the distal esophagus that extended into the stomach. A needle aspiration biopsy of a liver lesion revealed esophageal adenocarcinoma with metastasis. DISCUSSION: There have been only fifteen reported cases of AFP-producing esophageal adenocarcinoma in the literature. Our patient presented not only with EAC but also extremely high levels of AFP >40,000, with 320 ng/mL as the highest reported AFP level previously. Most EACs were located over the lower third of the esophagus and were related to Barrett’s esophagus as seen in our patient. The serum AFP level can be serially measured for monitoring clinical status, evaluating cure, and assessing recurrence or metastases. Most cases were treated with adjuvant chemotherapy with cisplatin plus 5-fluorouracil (5-FU) as first-line and combination of paclitaxel and cisplatin as second-line. Our patient was treated with oxaplatin, folinic acid, and 5-FU. She had temporary reduction of metastasis, but unfortunately there was recurrence. Clinicians should consider AFP-producing EAC in their differential diagnosis when working up a liver mass in the setting of elevated AFP or liver function impairment.