Abstract
Small bowel adenocarcinoma (SBA) complicating Crohn’s disease (CD) is rare and generally found incidentally on surgical specimens. We report our experience in CD-associated SBA observed this last decade in a tertiary referral centre in order to update its incidence, clinical presentation and pathological features. All SBAs diagnosed in patients who underwent surgery for CD between 2006 and 2016 were retrospectively included. Clinico-pathological characteristics were reviewed, and follow-up was updated. SBA was diagnosed in 9 (1.7%) of 522 patients who underwent SB resection(s) after a median CD duration of 15 years [0–32]. The median age at diagnosis was 46 years. Seven (78%) patients had obstructive symptoms refractory to medical treatment. Pre-operative biopsy revealed neoplasia in five (56%) patients (dysplasia in three and SBA in two) justifying the surgery. Two (29%) of the seven patients with imaging had features suggestive of cancer. In all specimens, SBA developed in active ileitis with adjacent dysplasia. Stage I low-grade tubulo-glandular adenocarcinoma was observed in 33% of patients. Stage IV high-grade adenocarcinoma was observed in 56% of patients, and mucinous/signet ring cell differentiation predominated in 44% of patients. Molecular analysis showed no BRAF mutation, a KRAS mutation in one case and a microsatellite instability phenotype suggestive of Lynch syndrome in one case. After a median follow-up of 24 months [7–82], four (44%) patients died with advanced stage IV SBA. This surgical series confirms that CD-associated SBA is rare with an incidence of 1.7%. Adjacent dysplasia was present in all specimens and was identified before surgery in all patients who benefit from ileal biopsies. This strengthens the importance of screening all longstanding CD by endoscopy if surgery is not considered.
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Abbreviations
- CD:
-
Crohn’s disease
- SBA:
-
Small bowel adenocarcinoma
- CT:
-
Computerized tomography
- MR:
-
Magnetic resonance
- MSI:
-
Microsatellite instability
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Chloé Grolleau: data collection, data analysis, writing and literature search; Nicolas Pote: data collection, figures and English editing; Nathalie S. Guedj: data collection; M. Zappa: data collection and data interpretation; Nathalie Theou-Anton: data interpretation; Yoram Bouhnik: data interpretation; Yves Panis: data interpretation; Dominique L. Cazals-Hatem: study design, data collection, data analysis, data interpretation and writing.
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Grolleau, C., Pote, N.M., Guedj, N.S. et al. Small bowel adenocarcinoma complicating Crohn’s disease: a single-centre experience emphasizing the importance of screening for dysplasia. Virchows Arch 471, 611–617 (2017). https://doi.org/10.1007/s00428-017-2125-z
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DOI: https://doi.org/10.1007/s00428-017-2125-z