Four cases of carcinoid tumour in Crohn’s disease: coincidence or correlation?

Abstract

Dear Editor:Inflammatoryboweldiseasesareassociatedwithanincreasedrisk of large and small bowel cancers.The association between Crohn’s disease and adenocarci-noma of the small intestine has been established, but therelation between Crohn’s and carcinoid tumours remains un-certain, although the diagnosis of carcinoid tumours has sig-nificantly increased in the last years.We describe four cases of carcinoid tumour diagnosed inpatients with Crohn’s disease followed at our IBD outpa-tients’ clinic.First case A 46-year-old male non-smoker presented with26 years history of ileocolonic Crohn ’s disease and psoriasis.The patient underwent several surgeries over these years, endingup with colectomy with ileal–rectal anastomosis. After that, thedisease has been poorly controlled by the therapy (mesalazine),andseveralsteroidscourseswereneeded.In2011,anabdominalCT scan showed a pre-anastomotic Crohn ’s disease relapse andan unexpected expansive mass in the left kidney. The patientunderwent resection of the anastomosis and left nephrectomy.Post-operative histology showed active ileal and rectal Crohn ’sdisease and papillary renal can cer type 1, with Fuhrman nucleargradeof2andnopyeluminfiltration.Noadjuvantchemotherapytreatment was set. Few months after, he presented a Crohn’srelapse,withtwentybowelmovementsperdaywithurgencyandsevere weight loss. He also repor ted unusual skin rushes aftersomekindoffoodconsumption, whichwereself-solving.Bloodtests were normal, except for a mild creatinine rise. The endos-copy revealed normal ileal mucosa with a small polyp, whichwas removed, regular anastomo sis and rectal erosions. Polyp ’shistology pointed out muscularis propria infiltrating cells withdiffuse positivity for neuroe ndocrine markers, includingchromograninAandCD56andlownuclearproliferationmarkerKi-67(<2%).Immunohistochemist rystainingforsomatostatin2receptor (SSTR2A) was positive. These findings resulted in thediagnosis of well-differentiated neuroendocrine tumour (NET)-G1 (G1 category according to the WHO 2010).An abdominal CT scan showed no signs of renal tumourlocal recurrence but thickened anastomotic wall andoedematous mesenteric fat. The SPECTscan with a somato-statin receptor marker as contrast agent pointed out a roundarea on the anterior abdominal wall, lymph node like.A second endoscopy detected severe endoscopic Crohn’srelapse associated with another polyp, which was positivefor a new carcinoid recurrence (NET-G1). The patientunderwent proctectomy with ileal stoma.Second case A 45-year-old male smoker presented with12 years history of ileocolonic Crohn’s disease and vitiligo.Crohn’s diagnosis was made after an emergency rightemicolectomy due to peritonitis. In 2011, he started to expe-rience obstructive symptoms with low response to steroids.The enteric MRI detected two stenotic segments of the pre-anastomotic ileum and a voluminous solid lesion (O70×40×70 mm) in the retroperitoneum, adjacent to thepancreatic head, duodenum and inferior caval vein. Histologyof CT-guided biopsy revealed a well-differentiated NET-G1.The patient was referred to the surgeon and underwent apancreatic duodenectomy (Whipple procedure) and resectionof the previous ileal colonic anastomosis. Post-operative his-tology pointed out an infiltrating neoplasia of the duodenumwall, next to the duodenal papilla. The pancreas was cancerfree as well as the surgical resection borders; two peri-duodenal lymph nodes were infiltrated. Neoplastic cells werediffusely positive for the neuroendocrine marker synap-tophysin and for the nuclear proliferation marker Ki-67(1 %). Immunohistochemistry staining for SSTR2A was