Because Cowden syndrome is associated with an increased risk for certain types of cancer, management is typically focused on high-risk cancer screening. According to the National Comprehensive Cancer Network 2014, the recommended screening protocol for Cowden syndrome includes:
Baseline colonoscopy at age 35 with follow-up every 5 years (more frequent if polyps identified)
Consider renal (kidney) ultrasound every 1-2 years beginning at age 40
**or individualized based on the earliest diagnosis of cancer in the family.
Pediatric (age <18 years)
Yearly thyroid ultrasound examination** (on identification of a PTEN pathogenic variant)
Yearly skin check with physical examination
If there are not any symptoms, observation alone is prudent. Cutaneous lesions should be removed only if malignancy is suspected or symptoms (e.g., pain, deformity, increased scarring) are serious. When symptomatic, topical agents (e.g., 5-fluorouracil), curettage, cryosurgery, or laser ablation may offer temporary relief. [Hildenbrand et al 2001].