Appendiceal Goblet Cell Carcinoma: Role of Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC)

Abstract

Goblet cell carcinoma (GCC) encompasses a separate entity in appendiceal neoplasms with mixed glandular and neuro-endocrine pathological features. GCC mostly presents as an acute appendicitis duo to luminal obstruction or as an incidental finding on the surgical appendectomy specimen. In case of tumour perforation or presence of other risk factors, guidelines suggest additional treatment with a completing right hemicolectomy or cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). We report the case of a 77-year-old male with symptoms of appendicitis for which an appendectomy was performed. The appendix was ruptured during the procedure. There was an incidental finding of GCC on the pathological specimen. Because of possible tumour soiling, the patient received a prophylactic CRS-HIPEC. A literature review was performed to investigate the potential role for CRS-HIPEC as a curative treatment in patients with GCC. GCC of the appendix is an aggressive type of tumour with a high risk of peritoneal and systemic dissemination. CRS and HIPEC is a treatment option: both in a prophylactic setting and in patients with established peritoneal metastases.

Keywords: Appendiceal carcinoma; Cytoreductive surgery; Goblet cell carcinoma; HIPEC; Peritoneal metastases.

Fig. 1

a Computed tomography (CT) with oral and intravenous iodine contrast in the axial plane demonstrates irregular thickening of the tip of the appendix (large arrow).The latter is adhesive with the medial wall of the caecum (small arrow). b CT scan with oral and intravenous iodine contrast in the axial plane: The proximal part of the appendix (small arrows) is normal. c CT with oral and intravenous iodine contrast in the axial plane: Streaky infiltration of the mesentery is seen in the vicinity of the thickened appendix (arrows)

Fig. 2

a A haematoxylin–eosin-stained section shows the presence of clusters and nest of cells composed of goblet-like mucinous cells and Paneth-like cells (haematoxylin–eosin, original magnification × 100). b An immunohistochemical staining for synaptophysin demonstrates endocrine cells in the cell clusters (original magnification × 400). c Immunohistochemistry with a pan-keratin antibody shows in addition to cell clusters, single infiltrating tumour cells (high-grade pattern) (original magnification × 100)