Ileal carcinoid tumor within Crohn's disease
Tumor carcinoide ileal en la enfermedad de Crohn
Key words: Carcinoid. Small-bowel neoplasma. Crohn.
Dear Editor,
We present a rare case combining Crohn's disease with intestinal carcinoid recently treated at our Division of Colorectal Surgery.
]]>Case report
A 51-year-old female with a long history of Crohn disease (CD) and ileocolic resection in the past, receiving medical treatment with mesalazine and azathioprine, was admitted reporting daily colicky abdominal pain, diarrhea, and asthenia. Evaluation with abdomen multislice computed tomography (MSCT) demonstrated huge adenopathies located at the neoileal mesentery (Fig. 1A), which were ecoguide biopsied. Colonoscopy ruled out colorectal carcinoma and test revealed high values of CEA in blood, 5HIIA in urine, with a completely normal value of the beta-2 microglobulin. Subsequent OctreoScan confirmed abnormal mesenteric adenopathies tracer accumulation, with a discrete focus in distal ileum. Biopsy was consistent with lymph node metastasis of typical carcinoid tumor.
The patient underwent scheduled laparotomy with adenopathic conglomerate resection, located in neoileal meso (Fig. 1B). The primary tumor, a 0.5 cm lesion located in ileum, 15 cm proximal to ileocolic anastomosis, was also resected. Pathology confirmed moderately differentiated neuroendocrine tumor (Ki < 1 %).
Discussion
Malignant tumors of the small bowel are extremely rare, being the major histological subtypes: Adenocarcinoma, lymphoma and carcinoid tumor. Patients with long standing inflammatory bowel disease have an increased risk, which is related to two main factors: the longer duration of the inflammation and the site and severity of the disease.
The association of CD and adenocarcinoma of the small intestine has been well established by several studies. On the other hand, malignant carcinoid of the small intestine associated with CD is rare. Higashi et al reported that out of the 286 cases of CD examined, there were 13 concomitant cases of malignant disorders. Among them, six cases had colorectal cancer, while only one of the cases had rectal carcinoid (1).
]]> Last studies about carcinoid tumors found in patients with inflammatory bowel disease have come up to the question of whether there is an association between the pathogenesis of these tumors arising from a background of active CD and the disease itself (2).It is believed that chronic inflammation, caused by proinflammatory cytokines (TNFα, IFNγ, IL 2), lead enteroendocrine cells to hyperplasia and carcinoid transformation over a 10-year period (3). The finding of the tumor in an area of uninflamed intestine can be explained by the healing effect that immunomodulator therapy has over the mucosa or due to the effect of distant proinflammatory mediators, rather than a local inflammatory effect from adjacent CD (4).
Some authors do not believe that CD is a causative factor for carcinoid tumors since almost all cases were found incidentally after surgery for inflammatory bowel disease. Others argue that the coexistence may be under-reported (5). The diagnosis of carcinoid tumor poses a challenge, and it is rare before surgery because both conditions can mimic one another as far as clinical symptoms and diagnostic tests are concerned.
In general, the presence of ileal CD refractory to medical therapy should alert us to the possibility of a small-bowel neoplasm.
Mónica Mogollón, Raquel Conde, Inmaculada Segura, Franscisco Huertas,
Carlos San-Miguel, J. Antonio Ferrón and Pablo Palma
Division of Colon and Rectal Surgery. Hospital Universitario Virgen de las Nieves. Granada, Spain
References
]]>1. Higashi D, Futami K, Kawahara K, Kamitami T, Seki K, Naritomi K, et al. Study of colorectal cancer with Crohn's disease. Anticancer research 2007;27:3771-4. [ Links ]
2. West NE, Wise PE, Herline AJ, Muldoon RL, Chopp WV, Scwartz DA. Carcinoid tumors are 15 times more common in patients with Crohn's disease. Inflamm Bowel Dis 1997;13:1129-34. [ Links ]
3. Samlani-Sebbane Z, Ghraba S, Narjis Y, Rabbani K, Diffaa A, Krati K, et al. Coexistence de tumeur carcinoïde et maladie de Crohn iléale: une nouvelle observation. Acta Endosc 2012;42:194-6. [ Links ]
4. Hamish H, Dole J, Chai B. Incidental malignant carcinoid within Crohn ileitis. Can J Surgery 2009;52:58-9. [ Links ]
5. Le Marc'hadour F, Bost F, Peoc'h M, Roux JJ, Pasquier D, Pasquier B. Carcinoid tumor complicating inflammatory bowel disease. A study of two cases with review of the literature. Pathol Res Pract 1994;190:1185-92. [ Links ]
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