Coincidence of Crohn’s Disease and a High-Risk Gastrointestinal Stromal Tumor of the Terminal Ileum

Abstract

A 51-year-old male patient presented with characteristic radiologic features of Crohn’s disease in the terminal ileum plus a large tumorous lesion in the right lower abdomen. Because of rapid crescent symptoms of bowel obstruction, the patient underwent surgery revealing a high-risk gastrointestinal stromal tumor (GIST) of the terminal ileum within an area of Crohn’s ileitis. Whereas the association of chronic inflammatory bowel disease (IBD) and gastrointestinal adenocarcinoma is well known, other primary intestinal tumors are rare in these patients, particularly at the time of onset of clinical symptoms. This is the 3rd patient reported in the literature with a sarcoma complicating IBD, and in fact, the first description of the coincidence of Crohn’s disease and GIST. Though the present case is likely to be a mere coincidence of two pathologically distinct entities (without any potential causal relationship), it should remind one of the possibility of small bowel ‘Crohn’s carcinoma’ in patients with a sudden change in symptomatology as well as in those in whom intestinal obstruction fails to resolve with adequate therapy.

Gastrointestinal stromal tumor causing small bowelintussusception in a patient with Crohn’s disease

“…Finally, a malignant GIST of the small intestine causing small bowel obstruction due to ileal invagination has also been described [6] . Coincidence of Crohn's disease and gastrointestinal stromal tumor is extremely rare and only two cases have been reported to date [14,15] . The first one described a polypoid tumor within Meckel's diverticulum in an 81-yearold male patient with Crohn's disease and the tumor was immunohistochemically proved to be a GIST [14] .…”

“…The first one described a polypoid tumor within Meckel's diverticulum in an 81-yearold male patient with Crohn's disease and the tumor was immunohistochemically proved to be a GIST [14] . The second report presented a 51-year-old patient with a high risk GIST of the terminal ileum within an area of Crohn's ileitis [15] . …”

Colonic Gastrointestinal Autonomic Nervous Tumor in a Patient with Crohn’s Disease

“…Other primary intestinal tumors are rare in these patients, and to the best of our knowledge, this is the first case of GANT in a patient with CD. So far only four cases of histologically confirmed gastrointestinal sarcomas have been reported in patients with CD (15)(16)(17)(18): two leiomyosarcomas (rectum and small bowel), one enteroblastoma (excluded segment of the small bowel), and one GIST (terminal ileum). Patients with CD have a chronically activated and abnormal immune system, receive treatment with numerous immunossupressive therapies increasing their risk of infectious complications, and are more exposed to ionizing radiation through diagnostic workups.…”

Contribution of CT to treatment planning in patients with GIST

“…In alcuni rari casi sono state descritte localizzazioni atipiche quali la colecisti o la vescica [3,4]. È stata descritta una rara associazione fra il morbo di Crohn a carico del tratto terminale dell'ileo ed un elevato rischio di insorgenza di GIST in tale distretto anatomico [5].…”

“…A few sporadic cases of atypical location in the gallbladder or urinary bladder have been documented [3,4]. An uncommon association between Crohn's disease of the terminal ileum and a high risk of GIST development in this anatomical district region has also been reported [5].…”