Gastroenteropancreatic neuroendocrine tumour arising in Meckel’s diverticulum coexisting with colon adenocarcinoma

Abstract

Although colon cancer is the third most common cause of cancer-related death worldwide, the prevalence of gastroenteropancreatic neuroendocrine tumours (GEP-NETs) remains rare. To date, very few cases of GEP-NETs within Meckel’s diverticulum and synchronous colorectal cancer have been reported. Although the coexistence of these two tumour types is uncommon, it is important to be aware of their disease patterns. We present a rare case of a patient with an intestinal GEP-NET arising in Meckel’s diverticulum coexisting with metastatic colon adenocarcinoma, and we discuss the clinical manifestations and the diagnostic procedures and treatment modalities used. This case report underlines the importance of being aware of this particular coexistence, as well as the unlikely metastatic spread of GEP-NETs and the importance of a multidisciplinary approach to cancer treatment. Finally, individualizing the treatment according to the stages of the primaries will result in durable cancer control, particularly in synchronous double malignancy.

A rare case of metachronous neuroendocrine tumor after a colorectal adenocarcinoma: qualitative critical review of synchronous and metachronous gastrointestinal NET

“…To date, the literature in English reveals, 20 case reports (18 synchronous cases [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24], 2 metachronous cases [25,26]), 2 case series [25,27], 3 review articles [28][29][30], and about 10 retrospective studies [31][32][33][34] or population-based studies from a National Registry [35][36][37][38][39][40][41] of synchronous or metachronous NET with a second primary malignancy (SPM). A total of 31 patients in 24 articles were included in the study: 28 patients with a synchronous gastrointestinal NET and colorectal adenocarcinoma (Table 1) and 3 patients (including the present study) of 3 articles with metachronous gastrointestinal NET and colorectal adenocarcinoma ( Table 2).…”

“…Pearson and Fitzgerald were the first to report the high incidence (23%) of second primary malignancies (SPM) in patients with small intestinal NETs at autopsy [27]. A certain number of NETs were incidentally found during surgery for other [7] 1972 53 M Rectum Rectum -Lotlikar et al [25] 1981 53 M Rectum Small bowel -Lotlikar et al [25] 1981 67 F Right colon Small bowel -Lotlikar et al [25] 1981 60 F Sigmoid colon Small bowel -Sacchi et al [8] 1988 57 M Right colon Small bowel -Tse et al [9] 1997 72 M Hepatic flexure Small bowel -Habal et al [28] 2000 52 M Sigmoid colon Rectum Cioffi et al [10] 2005 64 F Ileum Small bowel -Klucinski et al [11] 2006 72 F Transverse colon Small bowel -Chemli et al [12] 2007 63 F Right colon Small bowel -Aslam et al [13] 2009 67 F Sigmoid colon Small bowel G1 Boland et al [14] 2009 77 F Left colon Meckel's diverticulum -McHugh et al [15] 2009 74 F Rectum Small bowel -Wohadlo et al [29] 2011 53 M Splenic-hepatic flexure Small bowel -Gurzu et al [16] 2012 78 F Sigmoid colon Small bowel G3 Pozzato et al [17] 2012 61 M Right colon Duodenum G1 Katalinic 2014 et al [18] 2014 63 M Right colon Meckel's diverticulum G1 Zhu et al [19] 2015 64 F Rectum Rectum G1 Almajano et al [20] 2016 66 M Right colon Small bowel G1 Mohapatra et al [21] 2016 83 M Sigmoid colon Left colon G3 Nejatollahi et al [22] 2016 83 M Sigmoid colon Small bowel G1 Vootla et al [23] 2016 46 F Hepatic flexure Rectum G1 Winn et al [30] 2017 40 M Sigmoid colon Rectum G1 Winn et al [30] 2017 70 M Sigmoid colon Rectum G1 Napolitano et al [24] 2017 72 M Right colon Small bowel G1-G2 cancers. The estimated rate of SPM associated with other malignancies was 2.3% in surgical cases and 8.1% in autopsy cases [42].…”

Serous Cystadenocarcinoma Arising in Presumed Vitelline Duct Remnant: A Case Report and Implications in the Management of Cancer of Unknown Primary

“…Nevertheless, the data support the existence of a subset of CUP that cannot be explained by occult or diminutive primary. In these cases, tumors arising in heterotopic tissues or embryonic remnants should be considered [1–5, 13–16]. …”

“…When attached to distal small bowel, it is called “Meckel's diverticulum”; heterotopic tissues such as pancreatic-type and gastric-type are often encountered. Various neoplasms can occur in Meckel's diverticulum, including gastrointestinal stromal tumor, neuroendocrine tumor, and gastric and intestinal adenocarcinoma [1–5]. Cates et al described an intraductal papillary mucinous adenoma that arose from pancreatic heterotopia within Meckel's diverticulum [5].…”

“…Neoplasms arising in Meckel's diverticulum, a vitelline duct remnant, are rare yet well documented. Most examples comprise carcinoid tumors, gastrointestinal stromal tumors, or gastric-type adenocarcinomas [1–5]. Herein we report the first case of serous cystadenocarcinoma arising from midline abdominal wall and discuss the implications of embryonic remnants, in this case vitelline duct remnant, as a potential origin of tumorigenesis on the management of CUP.…”