Controversies in appendiceal pathology: mucinous and goblet cell neoplasms

Abstract

Appendiceal goblet cell adenocarcinomas and mucinous neoplasms are uncommon compared with other epithelial tumours of the gastrointestinal tract. Both tumour types have been subjected to terminology that belies their biological risk and leads to confusion. Goblet cell adenocarcinomas display patchy staining for endocrine markers and, thus, were previously classified as goblet cell carcinoids. Unlike well-differentiated endocrine neoplasms, however, they often contain high-grade glandular elements and pursue an aggressive course akin to that of conventional adenocarcinoma. Although several authors have recently proposed grading schemes to predict behaviour among goblet cell neoplasms, most that contain high-grade components have already spread beyond the appendix at the time of diagnosis, whereas those confined to the appendix almost always have low-grade features; the added value of grading these tumours is limited. Contradictions also surround the nomenclature of mucinous neoplasms. The World Health Organization and others promote non-malignant terminology to describe metastatic mucinous neoplasms of the peritoneum and eliminate a benign category entirely, even though virtually all neoplasms confined to the appendix pose no recurrence risk following appendectomy. 'Low-grade appendiceal mucinous neoplasm' now encompasses a spectrum of benign tumours and malignant neoplasms in the appendix and peritoneum. Although using an umbrella term in this fashion simplifies the roles of pathologists, it provides essentially no actionable information beyond that which is already clinically apparent. Broad strokes nomenclature also ensures that many patients with no risk of recurrence will receive unnecessary surveillance while others will undergo inappropriate surgical procedures due to lapses in communication. Moreover, a surprising number of non-neoplastic mucinous lesions are misclassified as low-grade appendiceal mucinous neoplasms, which can result in unwarranted patient concern or even mismanagement. The purpose of this review is to critically evaluate the literature and describe an approach to appendiceal neoplasms that more clearly denotes their biologic risk.

Keywords: Appendix; goblet cell carcinoid; low-grade appendiceal mucinous neoplasm; mucinous adenocarcinoma.