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Table 5

Cancer surveillance guidelines for Cowden syndrome

GeneralPhysical examination beginning at age 18 or 5 years before the diagnosis of breast or thyroid cancer in the family.
ThyroidBaseline ultrasound thyroid examination at age 18 and consider yearly thereafter.
SkinAnnual dermatologic exam.
ColonNone.
Breast [female]For those without a family history of breast cancer, self breast examination and clinical breast examination are recommended to commence at ages 18 and 25, respectively. Also, mammography and breast magnetic resonance imaging are recommended to begin at ages 30 to 35. Consider prophylactic mastectomy.
Endometrial [female]Educate on symptoms and consider enrollment in a clinical trial.

Adapted from the National Comprehensive Cancer Network (NCCN) guidelines for Cowden syndrome [9]. In addition to the NCCN recommendations, the authors recommend colon and rectum cancer screening with colonoscopy to begin at age 45 and every 5 years thereafter.