Perianal Goblet Cell Carcinoid With Pagetoid Spread

We present a case of perianal goblet cell carcinoid with pagetoid spread. Goblet cell carcinoid, also known as adenocarcinoid tumor, predominantly arises as a primary appendiceal tumor and contains nests of neuroendocrine and mucin-containing cells. When this tumor type is seen in other sites it usually represents a metastasis. We present the case of an 81-year-old woman with a perianal mass. Histologic and immunohistochemical examination following surgical excision showed a goblet cell carcinoid demonstrating pagetoid spread along the perianal squamous mucosa. There was no evidence of a primary appendiceal tumor by history or imaging studies. To our knowledge, this is the first report of a goblet cell carcinoid presenting in this manner. The patient died due to complications of metastatic disease 26 months after initial diagnosis.