{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T08:35:40Z","timestamp":1777710940817,"version":"3.51.4"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,8,5]],"date-time":"2022-08-05T00:00:00Z","timestamp":1659657600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,8,5]],"date-time":"2022-08-05T00:00:00Z","timestamp":1659657600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["surg case rep"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>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.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>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.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>We report a signet-ring cell carcinoma of the appendix that was detected early because of its presence with ganglioneuromatosis.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s40792-022-01509-3","type":"journal-article","created":{"date-parts":[[2022,8,5]],"date-time":"2022-08-05T02:02:39Z","timestamp":1659664959000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Signet-ring cell carcinoma of the appendix with ganglioneuromatosis: a case report"],"prefix":"10.1186","volume":"8","author":[{"given":"Ayami","family":"Sato","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4939-5704","authenticated-orcid":false,"given":"Yu","family":"Sato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuyuki","family":"Hiruta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takashi","family":"Oshiro","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yutaka","family":"Yoshida","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tasuku","family":"Urita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomoaki","family":"Kitahara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kengo","family":"Kadoya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Taiki","family":"Nabekura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuki","family":"Moriyama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shinichi","family":"Okazumi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,8,5]]},"reference":[{"key":"1509_CR1","doi-asserted-by":"publisher","first-page":"3307","DOI":"10.1002\/cncr.10589","volume":"94","author":"ME McCusker","year":"2002","unstructured":"McCusker ME, Cot\u00e9 TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973\u20131998. Cancer. 2002;94:3307\u201312.","journal-title":"Cancer"},{"key":"1509_CR2","doi-asserted-by":"publisher","first-page":"247","DOI":"10.1055\/s-0035-1564433","volume":"28","author":"KJ Kelly","year":"2015","unstructured":"Kelly KJ. Management of appendix cancer. Clin Colon Rect Surg. 2015;28:247\u201355.","journal-title":"Clin Colon Rect Surg"},{"key":"1509_CR3","doi-asserted-by":"publisher","first-page":"551","DOI":"10.1016\/j.soc.2018.02.007","volume":"27","author":"KI Votanopoulos","year":"2018","unstructured":"Votanopoulos KI, Shen P, Skardal A, Levine EA. Peritoneal metastases from appendiceal cancer. Surg Oncol Clin N Am. 2018;27:551\u201361.","journal-title":"Surg Oncol Clin N Am"},{"key":"1509_CR4","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1007\/BF02236899","volume":"41","author":"SJ Connor","year":"1998","unstructured":"Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7970 appendectomies. Dis Colon Rectum. 1998;41:75\u201380.","journal-title":"Dis Colon Rectum"},{"issue":"1 Suppl","key":"1509_CR5","doi-asserted-by":"publisher","first-page":"154","DOI":"10.1002\/1097-0142(19950101)75:1+<154::AID-CNCR2820751305>3.0.CO;2-Z","volume":"75","author":"RM Thomas","year":"1995","unstructured":"Thomas RM, Sobin LH. Gastrointestinal cancer. Cancer. 1995;75(1 Suppl):154\u201370.","journal-title":"Cancer"},{"key":"1509_CR6","doi-asserted-by":"publisher","first-page":"2264","DOI":"10.1007\/s10350-005-0196-4","volume":"48","author":"ML McGory","year":"2005","unstructured":"McGory ML, Maggard MA, Kang H, O\u2019Connell JB, Ko CY. Malignancies of the appendix: beyond case series reports. Dis Colon Rectum. 2005;48:2264\u201371.","journal-title":"Dis Colon Rectum"},{"key":"1509_CR7","doi-asserted-by":"publisher","first-page":"871","DOI":"10.1097\/00000478-198612000-00006","volume":"10","author":"RC Haggitt","year":"1986","unstructured":"Haggitt RC, Reid BJ. Hereditary gastrointestinal polyposis syndromes. Am J Surg Pathol. 1986;10:871\u201387.","journal-title":"Am J Surg Pathol"},{"key":"1509_CR8","doi-asserted-by":"publisher","first-page":"1166","DOI":"10.2214\/ajr.182.5.1821166","volume":"182","author":"SR Charagundla","year":"2004","unstructured":"Charagundla SR, Levine MS, Torigian DA, Campbell MS, Furth EE, Rombeau J. Diffuse intestinal ganglioneuromatosis mimicking Crohn\u2019s disease. AJR Am J Roentgenol. 2004;182:1166\u20138.","journal-title":"AJR Am J Roentgenol"},{"key":"1509_CR9","doi-asserted-by":"publisher","first-page":"e35","DOI":"10.1097\/MPG.0000000000002072","volume":"68","author":"EH Rosenfeld","year":"2019","unstructured":"Rosenfeld EH, Chumpitazi BP, Castro E, Naik-Mathuria B. Diffuse intestinal ganglioneuromatosis causing severe intestinal dysmotility in a child with a PTEN mutation. J Pediatr Gastroenterol Nutr. 2019;68:e35\u20137.","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"1509_CR10","doi-asserted-by":"publisher","first-page":"333","DOI":"10.1002\/ijc.28981","volume":"136","author":"N Hugen","year":"2015","unstructured":"Hugen N, Verhoeven RH, Lemmens VE, van Aart CJ, Elferink MA, Radema SA, et al. Colorectal signet-ring cell carcinoma: benefit from adjuvant chemotherapy but a poor prognostic factor. Int J Cancer. 2015;136:333\u20139.","journal-title":"Int J Cancer"},{"key":"1509_CR11","doi-asserted-by":"publisher","first-page":"3400","DOI":"10.1002\/cam4.3009","volume":"9","author":"BC Kolla","year":"2020","unstructured":"Kolla BC, Petersen A, Chengappa M, Gummadi T, Ganesan C, Gaertner WB, et al. Impact of adjuvant chemotherapy on outcomes in appendiceal cancer. Cancer Med. 2020;9:3400\u20136.","journal-title":"Cancer Med"},{"key":"1509_CR12","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1093\/ajcp\/75.2.225","volume":"75","author":"DC Snover","year":"1981","unstructured":"Snover DC, Weigent CE, Sumner HW. Diffuse mucosal ganglioneuromatosis of the colon associated with adenocarcinoma. Am J Clin Pathol. 1981;75:225\u20139.","journal-title":"Am J Clin Pathol"},{"key":"1509_CR13","doi-asserted-by":"publisher","first-page":"105","DOI":"10.5414\/NPP28105","volume":"28","author":"S Qiao","year":"2009","unstructured":"Qiao S, Iwashita T, Ichihara M, Murakumo Y, Yamaguchi A, Isogai M, et al. Increased expression of glial cell line-derived neurotrophic factor and neurturin in a case of colon adenocarcinoma associated with diffuse ganglioneuromatosis. Clin Neuropathol. 2009;28:105\u201312.","journal-title":"Clin Neuropathol"},{"key":"1509_CR14","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1038\/bjc.2012.535","volume":"108","author":"OO Seminog","year":"2013","unstructured":"Seminog OO, Goldacre MJ. Risk of benign tumours of nervous system, and of malignant neoplasms, in people with neurofibromatosis: population-based record-linkage study. Br J Cancer. 2013;108:193\u20138.","journal-title":"Br J Cancer"},{"key":"1509_CR15","doi-asserted-by":"publisher","first-page":"873","DOI":"10.1016\/j.annonc.2020.03.291","volume":"31","author":"V Dunnett-Kane","year":"2020","unstructured":"Dunnett-Kane V, Burkitt-Wright E, Blackhall FH, Malliri A, Evans DG, Lindsay CR. Germline and sporadic cancers driven by the RAS pathway: parallels and contrasts. Ann Oncol. 2020;31:873\u201383.","journal-title":"Ann Oncol"}],"container-title":["Surgical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40792-022-01509-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s40792-022-01509-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40792-022-01509-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,8,5]],"date-time":"2022-08-05T02:02:41Z","timestamp":1659664961000},"score":1,"resource":{"primary":{"URL":"https:\/\/surgicalcasereports.springeropen.com\/articles\/10.1186\/s40792-022-01509-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,5]]},"references-count":15,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["1509"],"URL":"https:\/\/doi.org\/10.1186\/s40792-022-01509-3","relation":{},"ISSN":["2198-7793"],"issn-type":[{"value":"2198-7793","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,5]]},"assertion":[{"value":"1 May 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 July 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"5 August 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Informed consent was obtained from the patient described in this article.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors have no financial disclosures.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"151"}}