Advanced appendiceal goblet cell carcinoids with intestinal obstruction: two case reports
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CASE REPORT
Advanced appendiceal goblet cell carcinoids with intestinal obstruction: two case reports Junichi Kato1 · Akinori Maruta1 · Yohei Shirakami1 · Taku Mizutani1 · Noritaka Ozawa1 · Takayasu Ideta1 · Jun Takada1 · Masaya Kubota1 · Hiroyasu Sakai1 · Takashi Ibuka1 · Hiroshi Araki1 · Masahito Shimizu1 Received: 18 May 2020 / Accepted: 31 August 2020 © Japanese Society of Gastroenterology 2020
Abstract A goblet cell carcinoid is quite rare and has features, wherein, a carcinoid-like image and an adenocarcinoma-like image coexist. We encountered two cases of rare goblet cell carcinoid originating in the appendix. Case 1 is that of a 48-year-old man with a chief complaint of abdominal distension and case 2 is that of a 64-year-old woman with a chief complaint of constipation. At the time of diagnosis, both cases had already metastasized to the peritoneum and other organs, and no radical surgical treatment could be administered in either case. Chemotherapies were performed according to the regimen for colon cancer, and they were effective to a certain extent. During the course of treatment, however, both cases developed intestinal obstruction, presumably due to peritoneal dissemination, which led to worse condition and death several months afterwards. Chemotherapy for goblet cell carcinoids has not yet reached a consensus, and further studies and establishment of therapeutic strategy are desired in the future. Keywords Appendiceal tumour · Goblet cell carcinoid · Intestinal obstruction Abbreviations FOLFIRI 5-Fluorouracil/leucovorin/irinotecan FOLFOX 5-Fluorouracil/leucovorin/oxaliplatin GCC Goblet cell carcinoid MiNEN Mixed neuroendocrine-non-neuroendocrine neoplasms PD Progressive disease PR Partial response SD Stable disease
Introduction Primary appendiceal cancer is extremely rare with less than 1% of all gastrointestinal malignancies. Neoplasms of the appendix are capable of developing into cancers with morphological diversity, which are classified into several groups, such as epithelial neoplasms and neuroendocrine Junichi Kato and Akinori Maruta contributed equally. * Yohei Shirakami ys2443@gifu‑u.ac.jp 1
Department of Gastroenterology, Gifu University Graduate School of Medicine, 1‑1 Yanagido, Gifu 501‑1194, Japan
neoplasms. The former include invasive adenocarcinomas; low-grade appendiceal mucinous neoplasm and high-grade appendiceal mucinous neoplasm. The latter include welldifferentiated neuroendocrine tumors, poorly differentiated neuroendocrine carcinomas, and mixed neuroendocrinenon-neuroendocrine neoplasms (MiNEN) [1, 2]. Among them, goblet cell carcinoid (GCC) is classified as MiNEN and is uncommon in the primary cancers of the appendix [2, 3]. Although there are no clinical trials or guidelines of treatment for advanced stage of GCC because of the rarity, chemotherapies based on the regimen for colorectal cancer is recommended and used in clinical practice [4]. We report two cases of appendiceal GCC with intestinal obstruction, inoperable due to the accompanying peritoneal diss
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