Adenocarcinoma Ex Goblet Cell Carcinoid of Appendix: Two Case Reports

Abstract

Adenocarcinoma ex goblet cell carcinoid is a rare tumor incidentally found in specimens of appendicitis. Most patients present with acute abdomen, similar to acute appendicitis. Here we present two cases, which were found incidentally after operation. We give a brief summary about clinical and biological behavior of this entity.

A rare case of signet ring cell carcinoma of the appendix

“…The 5-year survival rate is 20.5% and will drop to 6.7–14% if there is diffuse peritoneal metastasis [ 1 ]. Since this tumor is morphologically and immunohistochemically different from all other appendiceal carcinomas, it is important that the entire appendix be removed at time of surgery because the morphology is prognostically important and prefers to make peritoneal folds and spread on the surface [ 5 ]. Follow-up for recurrence is important since it is a neuroendocrine-based tumor.…”

“…Therefore, it is difficult to diagnose and needs histopathological evaluation after the procedure by the clinical and surgical pathologist [ 1 ]. It was first described in 1969 by having both neuroendocrine and glandular differentiation and this therefore offsets it from the other types of appendiceal carcinomas making it an extremely rare primary cancer [ 5 ]. It most likely originates from pluripotent intestinal crypt epithelial stem cells as well as loss of the tumor suppressor, Notch [ 6 ].…”

“…It most likely originates from pluripotent intestinal crypt epithelial stem cells as well as loss of the tumor suppressor, Notch [ 6 ]. It is also noted that mucin droplets and neuroendocrine secretory granules are present in these epithelial intestinal crypts that help form the tumor [ 5 ]. Appendectomy is the treatment of choice followed by superimposed right hemicolectomy and adjuvant chemotherapy if warranted from perforation or invasion of location structures [ 7 ].…”

Colonic Goblet Cell Carcinoid: Rarity of a Rarity! A Case Report and Review of Literature

“…Also the goblet cells secrete anti-microbial proteins, chemokines, and cytokines which play role in innate immunity beyond barrier maintenance function of the mucin (11). Goblet cell carcinoid (GCC) is a rare tumour, was first described by Gagne in 1969 (14,15), in addition World Health Organization accepted the term "mucinous carcinoid" as a name for this condition (16).…”

Inusual localización de un tumor inusual. Comunicación breve de un caso

“…Tang et al (6) categorizaron este tumor, basados en un esquema terciario: GCC, adenocarcinoma ex carcinoide de células caliciformes (ACexGCC) con morfología de células en anillo de sello y ACexGCC pobremente diferenciado. (5,6,8) Sin embargo, otros autores han propuesto otros sistemas mejor reproducibles, basados en las categorías de bajo y alto grado, así como Yozu et al (2), quienes proponen tres grados, según el porcentaje de formación glandular: > 75 %, de bajo grado (grado 1); 50-75 %, grado intermedio (grado 2); y < 50 % alto grado (grado 3). ( 2) Actualmente, la OMS recomienda la denominación de adenocarcinoma de células caliciformes, y su clasificación en bajo y alto grado, según sus características histopatológicas.…”