Pathology · single question

A patient has multiple odontogenic keratocysts (OKCs) and supernumerary teeth. Which systemic condition are they at risk for?

  1. Hyperthyroidism
  2. Basal cell carcinoma (Gorlin-Goltz syndrome / nevoid BCC syndrome)
  3. Hyperparathyroidism
  4. Scleroderma
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Correct answer: B. Basal cell carcinoma (Gorlin-Goltz syndrome / nevoid BCC syndrome)

  • A. Hyperthyroidism has no association with multiple OKCs.
  • B. Multiple OKCs + supernumerary teeth + skeletal anomalies + calcified falx cerebri + skin lesions = Gorlin-Goltz syndrome (nevoid basal cell carcinoma syndrome). PTCH1 gene mutation. These patients develop multiple BCCs of the skin.
  • C. Hyperparathyroidism causes brown tumors (giant cell lesions) in bone, not OKCs.
  • D. Scleroderma affects connective tissue systemically; not linked to OKCs.

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