Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case report

Abstract Background Primary cancer of the appendix, especially signet-ring cell carcinoma, is an uncommon disease, and it is rarely suspected before surgery. Diffuse intestinal ganglioneuromatosis that is not associated with neurofibromatosis-1 or multiple endocrine neoplasia 2b is also rare. The mo...

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Main Authors: Ayami Sato, Yu Sato, Nobuyuki Hiruta, Takashi Oshiro, Yutaka Yoshida, Tasuku Urita, Tomoaki Kitahara, Kengo Kadoya, Taiki Nabekura, Yuki Moriyama, Shinichi Okazumi
Format: Article
Language:English
Published: SpringerOpen 2022-08-01
Series:Surgical Case Reports
Subjects:
Online Access:https://doi.org/10.1186/s40792-022-01509-3
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author Ayami Sato
Yu Sato
Nobuyuki Hiruta
Takashi Oshiro
Yutaka Yoshida
Tasuku Urita
Tomoaki Kitahara
Kengo Kadoya
Taiki Nabekura
Yuki Moriyama
Shinichi Okazumi
author_facet Ayami Sato
Yu Sato
Nobuyuki Hiruta
Takashi Oshiro
Yutaka Yoshida
Tasuku Urita
Tomoaki Kitahara
Kengo Kadoya
Taiki Nabekura
Yuki Moriyama
Shinichi Okazumi
author_sort Ayami Sato
collection DOAJ
description Abstract Background Primary cancer of the appendix, especially signet-ring cell carcinoma, is an uncommon disease, and it is rarely suspected before surgery. Diffuse intestinal ganglioneuromatosis that is not associated with neurofibromatosis-1 or multiple endocrine neoplasia 2b is also rare. The most frequent symptoms caused by it are changes in bowel habits, abdominal pain, and occlusive episodes. Case presentation The patient was a 48-year-old woman who had a month-long history of chronic abdominal pain, fullness, constipation, and diarrhoea. Enhanced computed tomography showed a 100-mm irregular swelling in the appendix and thickening of the appendiceal wall with cystic dilatation. Based on a preoperative diagnosis of appendiceal cancer, the patient underwent laparoscopic ileocecal resection with D3 lymph node dissection. Pathological diagnosis revealed a signet-ring cell carcinoma of the appendix with ganglioneuromatosis. The patient completed four courses of capecitabine plus oxaliplatin (CAPEOX) as postoperative adjuvant chemotherapy, and 23-month postoperative outcome was noneventful without recurrence. Conclusion We report a signet-ring cell carcinoma of the appendix that was detected early because of its presence with ganglioneuromatosis.
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spelling doaj.art-6c8577e6165244768ac36cbd4ac40f3d2022-12-22T02:33:00ZengSpringerOpenSurgical Case Reports2198-77932022-08-01811510.1186/s40792-022-01509-3Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case reportAyami Sato0Yu Sato1Nobuyuki Hiruta2Takashi Oshiro3Yutaka Yoshida4Tasuku Urita5Tomoaki Kitahara6Kengo Kadoya7Taiki Nabekura8Yuki Moriyama9Shinichi Okazumi10Department of Surgery, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterDepartment of Surgical Pathology, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterDepartment of Surgery, Toho University Sakura Medical CenterAbstract Background Primary cancer of the appendix, especially signet-ring cell carcinoma, is an uncommon disease, and it is rarely suspected before surgery. Diffuse intestinal ganglioneuromatosis that is not associated with neurofibromatosis-1 or multiple endocrine neoplasia 2b is also rare. The most frequent symptoms caused by it are changes in bowel habits, abdominal pain, and occlusive episodes. Case presentation The patient was a 48-year-old woman who had a month-long history of chronic abdominal pain, fullness, constipation, and diarrhoea. Enhanced computed tomography showed a 100-mm irregular swelling in the appendix and thickening of the appendiceal wall with cystic dilatation. Based on a preoperative diagnosis of appendiceal cancer, the patient underwent laparoscopic ileocecal resection with D3 lymph node dissection. Pathological diagnosis revealed a signet-ring cell carcinoma of the appendix with ganglioneuromatosis. The patient completed four courses of capecitabine plus oxaliplatin (CAPEOX) as postoperative adjuvant chemotherapy, and 23-month postoperative outcome was noneventful without recurrence. Conclusion We report a signet-ring cell carcinoma of the appendix that was detected early because of its presence with ganglioneuromatosis.https://doi.org/10.1186/s40792-022-01509-3Signet-ring cell carcinomaAppendiceal cancerIntestinal ganglioneuromatosis
spellingShingle Ayami Sato
Yu Sato
Nobuyuki Hiruta
Takashi Oshiro
Yutaka Yoshida
Tasuku Urita
Tomoaki Kitahara
Kengo Kadoya
Taiki Nabekura
Yuki Moriyama
Shinichi Okazumi
Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case report
Surgical Case Reports
Signet-ring cell carcinoma
Appendiceal cancer
Intestinal ganglioneuromatosis
title Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case report
title_full Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case report
title_fullStr Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case report
title_full_unstemmed Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case report
title_short Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case report
title_sort signet ring cell carcinoma of the appendix with ganglioneuromatosis a case report
topic Signet-ring cell carcinoma
Appendiceal cancer
Intestinal ganglioneuromatosis
url https://doi.org/10.1186/s40792-022-01509-3
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