Thyroid disease associated with cowden syndrome: A meta-analysis.
Summary of "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. © 2012 Wiley Periodicals, Inc. Head Neck, 2012.
Affiliation
Department of Otolaryngology-Head and Neck Surgery, Vanderbilt University Medical Center, Nashville, Tennessee. joseph.e.hall@vanderbilt.edu.
Journal Details
This article was published in the following journal.
Name: Head & neck
ISSN: 1097-0347
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/22431287
- DOI: http://dx.doi.org/10.1002/hed.22971
Medical and Biotech [MESH] Definitions
Meta-analysis
Works consisting of studies using a quantitative method of combining the results of independent studies (usually drawn from the published literature) and synthesizing summaries and conclusions which may be used to evaluate therapeutic effectiveness, plan new studies, etc. It is often an overview of clinical trials. It is usually called a meta-analysis by the author or sponsoring body and should be differentiated from reviews of literature.
Thyroid Hormone Resistance Syndrome
An inherited autosomal recessive trait, characterized by peripheral resistance to THYROID HORMONES and the resulting elevation in serum levels of THYROXINE and TRIIODOTHYRONINE. This syndrome is caused by mutations of gene THRB encoding the THYROID HORMONE RECEPTORS BETA in target cells. HYPOTHYROIDISM in these patients is partly overcome by the increased thyroid hormone levels.
Pten Phosphohydrolase
A lipid phosphatase that acts on phosphatidylinositol-3,4,5-trisphosphate to regulate various SIGNAL TRANSDUCTION PATHWAYS. It modulates CELL GROWTH PROCESSES; CELL MIGRATION; and APOPTOSIS. Mutations in PTEN are associated with COWDEN DISEASE and PROTEUS SYNDROME as well as NEOPLASTIC CELL TRANSFORMATION.
Immunoglobulins, Thyroid-stimulating
Autoantibodies that bind to the thyroid-stimulating hormone (TSH) receptor (RECEPTORS, THYROTROPIN) on thyroid epithelial cells. The autoantibodies mimic TSH causing an unregulated production of thyroid hormones characteristic of GRAVES DISEASE.
Hamartoma Syndrome, Multiple
A hereditary disease characterized by multiple ectodermal, mesodermal, and endodermal nevoid and neoplastic anomalies. Facial trichilemmomas and papillomatous papules of the oral mucosa are the most characteristic lesions. Individuals with this syndrome have a high risk of BREAST CANCER; THYROID CANCER; and ENDOMETRIAL CANCER. This syndrome is associated with mutations in the gene for PTEN PHOSPHATASE.
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