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Multiple hamartoma syndrome (Cowden's syndrome): case report and literature review

Douglas D Wright1, James Whitney

  • 1Veterans Administration Medical Center, Washington, DC, USA.

General Dentistry
|December 1, 2006
PubMed

Insights

Cowden's syndrome (CS) is a genetic disorder with early oral and facial symptoms often missed by dentists. Recognizing these signs is crucial for early diagnosis and managing associated cancer risks.

Area of Science:

  • Genetics
  • Dermatology
  • Oncology

Background:

  • Multiple hamartoma syndrome, or Cowden's syndrome (CS), is an inherited disorder.
  • CS presents with variable signs and symptoms, often affecting multiple body systems.

Observation:

  • While dermatologic and gastrointestinal features are commonly discussed, unique oral and facial manifestations are also characteristic of CS.
  • These oral symptoms can appear early in the condition's progression.

Findings:

  • The dental community may overlook or underdiagnose CS due to a lack of awareness regarding its oral and facial symptoms.
  • CS is strongly associated with an increased risk of specific cancers, including breast, thyroid, and certain brain cancers, as well as gastrointestinal polyps.

Implications:

  • Increased dental awareness of Cowden's syndrome's oral manifestations is essential for timely diagnosis.
  • Early identification by dental professionals can facilitate prompt management and monitoring for associated malignancies.

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