Appendiceal Goblet Cell Adenocarcinoma: A Historically Informed Reading of 6 Cases

Abstract

Context.— Appendiceal goblet cell adenocarcinoma is an uncommon appendiceal neoplasm that is typically diagnosed incidentally to a presentation of acute appendicitis. Over the years, there have been many changes to the histologic evaluation and classification of this neoplasm, culminating in the most recent (2019) World Health Organization recommendations. Objective.— To understand the evolution of the concept of “goblet cell” neoplasia in the appendix, we explore a series of cases of goblet cell adenocarcinoma encountered at a county hospital over a 20-year period. We performed a historically informed review of these cases to highlight the significance and blind spots of our current definition and approach to this disease entity. Data Sources.— The cases were recovered from the archives of a county hospital (1999–2019). The literature was assembled through PubMed searches on the various names given to “goblet cell” neoplasms since the 1970s. We also refer to standard reference books of gastrointestinal pathology. Conclusions.— Frequent and numerous changes in the nomenclature of “goblet cell” lesions of the appendix resulted in considerable confusion regarding the approach to this rare entity, especially pertaining to its grade, as well as to its management in the clinical setting. The newest approach, recommended by the World Health Organization, provides clarity to its pathologic and clinical management. Adherence to these recommendations will improve communication between pathologists, surgeons, and oncologists about the natural history and prognosis of this malignant neoplasm.