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Thyroid pathologic findings in patients with Cowden disease
H R Harach1, I Soubeyran, A Brown
1Pathology Service, 'Dr. A. Onativia&rsquo Endocrinology and Metabolism Hospital, Salta, Argentina.
Annals of Diagnostic Pathology
|December 14, 1999
Summary
Cowden disease often presents with multiple benign thyroid nodules and adenomas. In some cases, these can progress to follicular carcinoma, necessitating total thyroidectomy.
Area of Science:
- Endocrinology
- Pathology
- Genetics
Background:
- Cowden disease is a rare genetic disorder associated with an increased risk of various cancers.
- Thyroid abnormalities are common in Cowden disease, but detailed histologic findings are not always well-characterized.
Purpose of the Study:
- To describe the spectrum of histologic findings in the thyroid glands of patients with Cowden disease.
- To correlate histologic features with clinical presentation and potential for malignant transformation.
Main Methods:
- Histologic examination of thyroid glands from 11 patients diagnosed with Cowden disease.
- Analysis of cytoarchitectural patterns, presence of specific lesions, and immunohistochemical staining (thyroglobulin, calcitonin).
Main Results:
- Multicentric follicular adenomas and adenomatous nodules were the most common findings.
- Microadenomas, oxyphil/clear cell lesions, hyalinizing trabecular adenoma, and adenolipoma were also observed.
- Two cases exhibited follicular carcinoma, which were larger and occurred at an older age than benign nodules, suggesting progression.
Conclusions:
- Histologic findings of multiple adenomatous goiter or follicular adenomas in young individuals should raise suspicion for Cowden disease.
- While tumors are typically benign, multicentricity and risk of progression to carcinoma warrant total thyroidectomy.
- Early diagnosis and family screening are crucial due to the inherited nature of Cowden disease.