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The Enigma of Goblet Cell Tumors of the Appendix

van Velthuysen, Marie-Louise F., MD, PhD*; van Eeden, Susanne, MD, PhD; Carr, Norman J., FRCPath

doi: 10.1097/PCR.0000000000000307

The term goblet cell carcinoid was first proposed in 1974 by Subbuswamy et al for a rare tumor of the appendix. Since then, it has been debated whether these tumors should be regarded as adenocarcinomas or as neuroendocrine tumors. Research on these neoplasms has been hampered by their rarity and the low density of tumor cells. However, their particular morphology legitimates their classification as an entity specific for the appendix. In this review the morphology, immunohistochemical profile and differential diagnosis are discussed. Recent literature on grading and molecular profiling is reviewed. These data show that grading these tumors using their morphologic growth patterns is of prognostic value. At the molecular level, goblet cell tumors differ from other appendiceal neoplasms, colon carcinoma, and neuroendocrine tumors and share similarities with other neoplasms such as diffuse-type gastric carcinoma. Goblet cell carcinomas thus emerge as an interesting target in this era of genetic profiling.

From the *Department of Pathology, Erasmus Medical Centre, Rotterdam; and

Department of Pathology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands; and

Peritoneal Malignancy Institute, Basingstoke, United Kingdom.

Reprints: Marie-Louise F. van Velthuysen, MD, PhD, Department of Pathology, Erasmus MC, University Medical Centre, PO Box 2040, 3000 CA Rotterdam, the Netherlands. E-mail:

The authors have no funding or conflicts to declare.

© 2019 Lippincott Williams & Wilkins, Inc.