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Am J Surg Pathol. 2018 Jul;42(7):898-910. doi: 10.1097/PAS.0000000000001056.

Histologic and Outcome Study Supports Reclassifying Appendiceal Goblet Cell Carcinoids as Goblet Cell Adenocarcinomas, and Grading and Staging Similarly to Colonic Adenocarcinomas.

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Histopathology Department, Middlemore Hospital, Auckland, New Zealand.
Department of Pathology, Weill Cornell Medicine, New York, NY.
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School.
Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA.
Surgery, Massachusetts General Hospital and Harvard Medical School.
Department of Pathology, University of Chicago, Chicago IL.
Department of Pathology and Laboratory Medicine, University of Vermont Medical Center, Burlington, VT.
Department of Pathology, H. Lee Moffitt Cancer Center, Tampa, FL.
Departments of Pathology.


Goblet cell carcinoid tumors are amphicrine tumors whose biological behavior ranges from indolent to highly aggressive, depending on tumor grade. Current grading systems for these tumors are based on identifying an adenocarcinoma arising in the setting of a goblet cell carcinoid tumor, which distinguishes this tumor from other gastrointestinal tract adenocarcinomas. Because goblet cell tumors are predominantly tumors of mucin secreting cells, we propose that they be classified as goblet cell adenocarcinomas, and graded using a methodology that has parallels in colorectal adenocarcinoma grading. We graded a large series of goblet cell adenocarcinomas by assessing the proportion of the tumor that demonstrates tubular or clustered growth. Histologic grade correlated with overall survival independent of stage, with median overall survival of 204, 86, and 29 months for low-grade, intermediate-grade, and high-grade goblet cell adenocarcinomas, respectively. Tumor stage also correlated with overall survival. We also graded the tumors according to previously proposed grading systems, and found that these systems are valid, in that they segregate patients according to prognosis.

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