- Part 1 Principles of international endocrine practice
- Part 2 Pituitary and hypothalamic diseases
- Part 3 The thyroid
- Part 4 Parathyroid, calcium, and bone metabolism
- Part 5 The adrenal gland and endocrine hypertension
- Part 6 Neuroendocrine tumours and genetic disorders
- 6.1 Neuroendocrine tumours of the gastrointestinal tract: an appraisal of the past and perspectives for the future
- 6.2 Neuroendocrine tumour markers
- 6.3 Neuroendocrine (carcinoid) tumours and the carcinoid syndrome
- 6.4 Gastrinoma
- 6.5 Insulinoma and hypoglycaemia
- 6.6 Glucagonoma
- 6.7 VIPomas
- 6.8 Somatostatinoma
- 6.9 Imaging neuroendocrine tumours of the gastrointestinal tract
- 6.10 Systemic mastocytosis
- 6.11 Multiple endocrine neoplasia type 1
- 6.12 Multiple endocrine neoplasia type 2
- 6.13 von Hippel–Lindau disease and succinate dehydrogenase subunit (SDHB, SDHC, and SDHD) genes
- 6.14 Neurofibromatosis
- 6.15 Carney’s complex
- 6.16 Molecular and clinical characteristics of the McCune–Albright syndrome
- 6.17 Cowden’s syndrome
- Part 7 Growth and development during childhood
- Part 8 Female endocrinology and pregnancy
- Part 9 Male hypogonadism and infertility
- Part 10 Endocrinology of ageing and systemic disease
- Part 11 Endocrinology of cancer
- Part 12 Obesity, lipids, and metabolic disorders
- Part 13 Diabetes mellitus
(p. 980) Cowden’s syndrome
- Chapter:
- (p. 980) Cowden’s syndrome
- Author(s):
Charis Eng
- DOI:
- 10.1093/med/9780199235292.003.0713
Cowden’s syndrome (OMIM 158350), named after Rachel Cowden, is an autosomal dominant inherited cancer syndrome characterized by multiple hamartomas involving organ systems derived from all three germ cell layers and a risk of breast and thyroid cancers (1, 2). Endocrinologists may make the diagnosis of Cowden’s syndrome when they are presented with these patients’ endocrine lesions, chief of which are multinodular goitre, thyroid adenomas, and epithelial thyroid cancer. The Cowden’s syndrome susceptibility gene, PTEN, is located on chromosome sub-band 10q23.3 (3, 4).
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- Part 1 Principles of international endocrine practice
- Part 2 Pituitary and hypothalamic diseases
- Part 3 The thyroid
- Part 4 Parathyroid, calcium, and bone metabolism
- Part 5 The adrenal gland and endocrine hypertension
- Part 6 Neuroendocrine tumours and genetic disorders
- 6.1 Neuroendocrine tumours of the gastrointestinal tract: an appraisal of the past and perspectives for the future
- 6.2 Neuroendocrine tumour markers
- 6.3 Neuroendocrine (carcinoid) tumours and the carcinoid syndrome
- 6.4 Gastrinoma
- 6.5 Insulinoma and hypoglycaemia
- 6.6 Glucagonoma
- 6.7 VIPomas
- 6.8 Somatostatinoma
- 6.9 Imaging neuroendocrine tumours of the gastrointestinal tract
- 6.10 Systemic mastocytosis
- 6.11 Multiple endocrine neoplasia type 1
- 6.12 Multiple endocrine neoplasia type 2
- 6.13 von Hippel–Lindau disease and succinate dehydrogenase subunit (SDHB, SDHC, and SDHD) genes
- 6.14 Neurofibromatosis
- 6.15 Carney’s complex
- 6.16 Molecular and clinical characteristics of the McCune–Albright syndrome
- 6.17 Cowden’s syndrome
- Part 7 Growth and development during childhood
- Part 8 Female endocrinology and pregnancy
- Part 9 Male hypogonadism and infertility
- Part 10 Endocrinology of ageing and systemic disease
- Part 11 Endocrinology of cancer
- Part 12 Obesity, lipids, and metabolic disorders
- Part 13 Diabetes mellitus