Thyroid disease associated with Cowden syndrome: A meta-analysis.

BACKGROUND: We investigated the pathology and surgical management of thyroid disease in patients with Cowden syndrome (CS).

METHODS: A query of the PubMed database between 2001 and January 2010 was performed using the terms "cowden syndrome OR cowden's syndrome OR cowden disease OR cowden's disease." Original articles, reviews, case reports, and case series were included. Articles were excluded if they were not written in English, did not present case/cases of CS, or did not include patient information to qualify for a diagnosis of CS. Ninety-five articles met the inclusion and exclusion criteria. A total of 181 cases were analyzed.

RESULTS: Of the 181 patients, 99 female (54.7%) and 77 male patients (42.5%) had sex reported. The age at presentation ranged from 3 days to 78 years. Ninety-six patients (96/181, 53.0%) were reported to have thyroid disease. Surgical management of thyroid disease was performed in 80.2% (77/96) of patients with thyroid disease, with total thyroidectomy being the most common operation reported (23/77, 29.9%). Thyroid pathology reported with CS patients included goiter (39/96, 40.6%), adenoma (24/96, 25%), unknown/unspecified pathology (8/96, 8.3%), follicular carcinoma (7/96, 7.3%), thyroiditis (7/96, 7.3%), papillary carcinoma (6/96, 6.3%), cancer (unknown type) (3/96, 3.1%), medullary carcinoma (1/96, 1%), and hyperthyroidism (1/96, 1%).

CONCLUSION: Careful monitoring of thyroid disease in CS patients is imperative.