A Rare Case of a Double Primary of the Pancreas and the Esophagus Diagnosed on Endoscopic Ultrasound

Abstract

1297_A.tif Figure 1: Upper endoscopic ultrasound revealing an ill-defined, hypoechogenic, and lobular area between the head and body of the pancreas. EGD demonstrating a nodule at the gastroesophageal junction. Pathology showing adenocarcinoma of the pancreas, positive for CK7, CK20, DPP4, and CDX2 and MLH1, MSH2, MSH6, and PMS2.Pancreatic ductal adenocarcinoma (PDAC) is the third leading cause of death from a solid malignancy in the United States, with a 5-year overall survival rate as low as 8%. Pancreatic cancer with synchronous primary tumors are rare with a reported incidence of 0.75-20.0%. The most common locations of the associated primary tumors are the stomach, colon, rectum, lung and thyroid. To the best of our knowledge, only three cases of synchronous cancers of the pancreas and the esophagus have been reported in the literature. We report the fourth such case of a double primary of the pancreas and the esophagus, diagnosed with endoscopic ultrasound (EUS). A 54 year-old man presented with 9 months of epigastric abdominal pain, decreased appetite, new onset diabestes and a 30 lb weight loss. A CT scan done at an outside facility showed a 4 x 3.8 x 2.6 cm mass in the pancreas. Hepatic panel had elevated transaminases (ALT 160 IU/L) and an alkaline phosphatase (ALP) of 256 IU/L. Carbohydrate antigen 19-9 ( CA 19-9 ) was elevated at 177.1 U/mL. An esophagoduodenoscopy and upper endoscopic ultrasound revealed an ill-defined, hypoechogenic, and lobular area, measuring 19.6 x 24.7 mm, seen between the head and body of the pancreas. A fine-needle biopsy was obtained. He was also incidentally found to have a gastroesophageal junction nodule, which was biopsied. A staging CT scan with pancreas protocol confirmed the same mass with thrombosis of the main portal vein and portal confluence, moderate amount of perihepatic and perisplenic ascites, multiple prominent lymph nodes along the gastrohepatic ligament and the root of the mesentery and multiple omental nodules, concerning for carcinomatosis. Pathology showed adenocarcinoma of the pancreas, positive for CK7, CK20, DPP4, and CDX2 and MLH1, MSH2, MSH6, and PMS2, consistent with microsatellite stability. The biopsy of the distal esophageal nodule revealed adenocarcinoma with mucinous features. Hence, the patient was diagnosed with a double primary of the pancreas and the esophagus and started on FOLFIRINOX (5-fluorouracil, leucovorin, irinotecan, and oxaliplatin) in consultation with medical oncology. He received 10 cycles of FOLFIRONOX and is doing well at a six month follow-up. Only three cases of synchronous cancers of the pancreas and esophagus have been reported in the literature. Interestingly, patients with double primary tumors have been observed to have better overall survival than those with pancreatic cancers alone.