Mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with Zollinger-Ellison syndrome: an autopsy case with a 24-year survival period

Abstract

We report an autopsy case of mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with Zollinger-Ellison syndrome. A 38-year-old Japanese male was found to have Zollinger-Ellison syndrome and pancreatic gastrinoma, and gastrectomy and resection of the pancreatic tumor were performed. However, hypergastrinemia persisted, and the patient died of disseminated carcinomatosis at 62 years of age, 24 years after the onset of Zollinger-Ellison syndrome. At autopsy, the main tumor was present in the residual pancreas, and metastases were noted in many organs. In the pancreas and other organs, ductal and endocrine carcinoma areas were mixed and there was a gradual transition between the two. No acinar differentiation was noted. The ductal elements were positive for mucins and carcinoembryonic antigen but negative for neuroendocrine markers, while endocrine elements were positive for chromogranin A and synaptophysin and to a lesser extent for gastrin, but negative for mucins and carcinoembryonic antigen. The ductal elements comprised about 30% of the tumor cells, and endocrine elements 70%. According to the revised World Health Organization classification, our case was diagnosed as mixed ductal-endocrine carcinoma. Our case is rare because the tumor manifested as gastrinoma with Zollinger-Ellison syndrome and the patient survived for 24 years. To the best of our knowledge, no such case has been reported. Our case suggests that pancreatic endocrine tumors may evolve into mixed ductal-endocrine carcinomas.

Mixed Ductal-Endocrine Carcinoma of the Pancreas Occurring as a Double Cancer: Report of a Case

“…A resected specimen was used for the diagnosis of mixed ductal-endocrine carcinoma of the pancreas. Seventeen case reports of pancreatic mixed ductalendocrine tumor have been published in English language journals, [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] as shown in Table 1. A literature review with a case report by Iwamuro M et al showed 34 mixed-tumor cases in Japanese reports.…”

“…During the follow-up period, the case was complicated by gallbladder mucosal adenocarcinoma. 14 Chatelain et al reported mixed ductal-endocrine carcinoma complicated by squamous cell carcinoma of the gingiva. 16 Carter et al reported mixed ductalendocrine carcinoma complicated by papillary carcinoma in situ of the common bile duct.…”

A Pediatric Case of Mixed Acinar-Neuroendocrine Carcinoma

“…MANEC is more common in women, although the number of reported cases is small [4][5][6]. Most patients present with abdominal pain and weight loss [3,7,8]. A standardized management protocol has not been established owing to the small number of cases, but complete or debulking surgical resection is generally considered the first line treatment, after which additional chemotherapeutic agents can be applied [3].…”

Goblet cell carcinoids and other mixed neuroendocrine/nonneuroendocrine neoplasms

“…As for conventional pancreatic carcinomas, the majority of mixed ductal-endocrine carcinomas occur in the head of the pancreas [37,49], while acinarendocrine carcinomas are equally divided between the head and tail [36,50]. Endocrine-related symptoms may be present, and a case with hypergastrinemia has been reported [69].…”