## Abstract Cowden syndrome is an autosomal dominant disorder characterized by benign and malignant hamartomatous lesions that can develop from all three germ cell derivatives. This disorder predisposes patients to develop malignant tumors of the breast, endometrium, and thyroid. We present a patie
Insular Thyroid Carcinoma in a Patient with Cowden Syndrome
โ Scribed by Henry R. Diggelmann; Douglas J. Van; Thomas M. O'Dorisio; Henry T. Hoffman
- Publisher
- John Wiley and Sons
- Year
- 2009
- Tongue
- English
- Weight
- 427 KB
- Volume
- 119
- Category
- Article
- ISSN
- 0023-852X
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โฆ Synopsis
Cowden syndrome is characterized hamartomatous lesions that can develop from all three germ cell derivatives. This disorder predisposes patients to develop malignant tumors of the breast, endometrium, and thyroid. We present a patient with clinically relevant manifestations of Cowden syndrome, with genetic verification, impacting by way of airway compromise due to hamartomas, urinary tract abnormalities, and insular thyroid cancer. This case illustrates the value of recognizing Cowden syndrome at an earlier stage to (1) permit early management to decrease the morbidity of untreated hamartomatous growths, and (2) perform an elective thyroidectomy to preempt the appearance of the thyroid malignancy. Through this case report, we provide further insight into management of this disorder.
๐ SIMILAR VOLUMES
## Abstract ## BACKGROUND. An insular growth pattern may be observed focally both in papillary and follicular thyroid carcinoma. The aim of the current study was to determine whether a greater extension of the insular component (IC) influences different clinical and histologic features at diagnosi
We report on an 18-month-old boy with an interstitial deletion at 10q23.2-q24.1. This region includes the PTEN gene, mutations of which have been reported to cause Cowden disease. Our patient presented with manifestations of Bannayan-Riley-Ruvalcaba (BRR) syndrome. The BRR syndrome is a rare disorde