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Adrenal Lesions in Patients With (Attenuated) Familial Adenomatous Polyposis and MUTYH-Associated Polyposis

Kallenberg, Frank G.J. M.D.1; Bastiaansen, Barbara A.J. M.D.1; Nio, C. Yung M.D.2; Soeters, Maarten R. M.D., Ph.D.3; Boermeester, Marja A. M.D., Ph.D.4; Aalfs, Cora M. M.D., Ph.D.5; Bossuyt, Patrick M.M. M.D., Ph.D.6; Dekker, Evelien M.D., Ph.D.1

doi: 10.1097/DCR.0000000000000809
Original Contributions: Colorectal/Anal Neoplasia
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BACKGROUND: The reported proportion of patients with familial adenomatous polyposis who have adrenal lesions varies between 7% and 13% compared with 4% in the general population; the prevalence of adrenal lesions in patients with attenuated familial adenomatous polyposis and MUTYH-associated polyposis is unknown. Data on the clinical relevance and clinical course are limited.

OBJECTIVE: We aimed to report on the frequency, characteristics, and progression of adrenal lesions in polyposis patients.

DESIGN: This was a historical cohort study.

SETTINGS: The study was performed at the Academic Medical Center, Amsterdam.

PATIENTS: All of the patients with familial adenomatous polyposis, attenuated familial adenomatous polyposis, and MUTYH-associated polyposis were included. Medical charts and imaging reports were analyzed for data on adrenal lesions. A radiologist reassessed all of the images. Patients had not routinely been screened for adrenal lesions.

MAIN OUTCOME MEASURES: The frequency, characteristics, and progression of adrenal lesions in patients with polyposis who underwent abdominal imaging were assessed. Findings were compared with a reference.

RESULTS: A total of 39 adrenal lesions were identified in 23 (26%) of 90 patients with familial adenomatous polyposis, 2 (18%) of 11 with attenuated familial adenomatous polyposis, and 5 (24%) of 21 with MUTYH-associated polyposis. Mean age at time of detection was 50.7 years (range, 17.1–83.3 y). Median lesion size at baseline was 1.4 cm (range, 1.0–5.0 cm) versus 1.7 cm (range, 1.0–5.7 cm) after a median of 3.5 years (range, 1.0–11.4 y). Two patients were diagnosed with a hyperfunctioning lesion, and 4 underwent adrenalectomy: 3 lesions appeared benign, and 1 was oncocytic of uncertain malignant potential. The OR for detecting at least 1 lesion in a patient with polyposis versus reference was 6.2 (95% CI, 3.2–12.3), with no significant differences in ORs among the 3 syndromes.

LIMITATIONS: The study was limited by its retrospective design.

CONCLUSIONS: Adrenal lesions are frequent in patients with polyposis who undergo abdominal imaging. They appear to follow a benign and slowly progressive course and are mostly nonhyperfunctioning. See Abstract Video at http://links.lww.com/DCR/A323.

1 Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands

2 Department of Radiology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands

3 Department of Endocrinology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands

4 Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands

5 Department of Clinical Genetics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands

6 Department of Clinical Epidemiology, Biostatistics, and Bioinformatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands

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Funding/Support: None reported.

Financial Disclosure: None reported.

Poster presentation at the meeting of the International Society for Gastrointestinal Hereditary Tumours, Sao Paolo, Brazil, June 18 to 20, 2015.

Correspondence: Evelien Dekker, M.D., Ph.D., Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, the Netherlands. E-mail: e.dekker@amc.uva.nl

© 2017 The American Society of Colon and Rectal Surgeons