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Pathology · single question

Which of the following conditions is characterized by calcification of the falx cerebri and multiple keratocystic odontogenic tumors?

  1. Gorlin-Goltz syndrome (NBCCS)
  2. Gardner syndrome
  3. Treacher Collins syndrome
  4. Crouzon syndrome
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Correct answer: A. Gorlin-Goltz syndrome (NBCCS)

Gorlin-Goltz syndrome (Nevoid Basal Cell Carcinoma Syndrome, NBCCS) is characterized by: multiple OKCs (keratocystic odontogenic tumors), multiple basal cell carcinomas, calcification of the falx cerebri, bifid ribs, and skeletal anomalies. The PTCH1 gene on chromosome 9 is mutated. Calcified falx cerebri at a young age on CT/skull X-ray is highly suggestive of Gorlin syndrome.
  • A. ✅ CORRECT – Gorlin syndrome: multiple OKCs + basal cell carcinomas + calcified falx cerebri. PTCH1 mutation.
  • B. ❌ Gardner syndrome features multiple odontomas, osteomas, colorectal polyposis — NOT OKCs or falx calcification.
  • C. ❌ Treacher Collins (mandibulofacial dysostosis) affects facial bones, ear, and lower eyelids — no OKCs or falx calcification.
  • D. ❌ Crouzon syndrome causes craniosynostosis and midface hypoplasia — no relationship to OKCs or falx calcification.

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