Goblet cell adenocarcinoma (GCA) is a rare and distinctive amphicrine tumor comprised of goblet-like mucinous cells and neuroendocrine cells. It is believed to originate from pluripotent stem cells located at the base of crypts. GCA predominantly arises from the appendix, with a few reported cases in extra-appendiceal locations such as the colorectum, small intestine, and stomach. In this case report, we present a unique instance of a 64-year-old male who initially received a diagnosis of neuroendocrine carcinoma in the distal esophagus based on biopsy but, following resection, was subsequently re-diagnosed with GCA arising from Barrett’s esophagus.
Carcinoid tumors of the appendix are common incidental findings, but appendiceal tumors with histologic features of both carcinoids and adenocarcinomas are rare, and their biologic behavior and histogenesis are still unclear. We report a case of goblet cell carcinoid of the appendix in a 54-year-old male, who exhibited pain in the right lower abdomen. Microscopically, the tumor contained smooth-bordered, widely separated nests composed of tumor cells with abundant mucin. The principal tumor cell type had a close resemblance to the normal goblet cell.
Histochemically, the tumor cells revealed positive reaction for PAS and alcian blue stain. Immunohistochemically, the tumor showed strong reactivity for carcinoembryonic antigen, chromogranin and, neuron specific enolase but none for cytokeratin and epithelial membrane antigen.