A case of neurofibroma located in the retroperitoneum involving the uncinate process of the pancreas

Abstract

A case of solitary neurofibroma in the retroperitoneum involving the uncinate process of the pancreas, which has been reported in only one case in Japan and less than 20 cases in the world literature, is described. A 57-year-old male complained of back pain and microhematuria. Plain and enhanced abdominal computed tomography (CT) indicated an 8 x 6 cm hypovascular tumor located in the uncinate process. We initially assumed it to be a cancer of the pancreas. His physical examination and blood chemistry result were normal. Various diagnostic imaging methods however, disclosed a rare type of tumor. The tumor was resected by pancreaticoduodenectomy and histological examination revealed it to be neurofibroma.

Neurofibroma of the porta hepatis

“…Neurofibromas are exceedingly rare in the pancreas . In fact, a Medline search found only three reported cases of neurofibromas in the pancreas (Table 1), with only two cases reported in the English language . The previous cases seem to demonstrate that these tumors are more prevalent in middle‐aged men, sharing the common symptom of upper abdominal or back pain.…”

Solitary neurofibroma of the pancreas body not associated with type 1 neurofibromatosis

“…6,7 Solitary sporadic neurofibroma of the pancreas is uncommon. 8 PubMed and Medline searches up to 2008 detected a limited number of related studies, all of which were case reports (Table 1) Neurofibroma of the pancreas was more commonly observed in middle age and was associated with upper quadrant abdominal pain; one case reported symptoms similar to acute pancreatitis with irregular obstruction of the main pancreatic duct and a narrowed intrapancreatic common bile duct upon ERCP (endoscopic retrograde cholagiopancreatography) examination.…”

“…6 The cut surface is primarily gray-white, often with whorls and spirals. 10 In the event of high lipid content, the tumor appears more yellowish, with a xanthomatous capsule, as in our case.…”

“…Despite the large tumor size, no significant nutritional wasting or weight loss was detected. 6 In all cases, pancreatic carcinoma could not be excluded until final pathological examination was undertaken.…”

Neurofibroma of the Common Bile Duct

“…Somewhat more commonly, although still comparatively rare, are cases of solitary neurofibroma in the hepatobiliary and gastrointestinal systems. King and Williamson discuss five cases of solitary neurofibroma in the gallbladder [2], and other individual case reports of solitary neurofibroma in the retroperitoneum and stomach exist as well [3,4].…”