Head and neck tumours: odontogenic keratocyst: practice question

A ENT question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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ENT · Head and neck tumours: odontogenic keratocyst

Sample item

A teenager with multiple jaw cysts, basal cell carcinomas and bifid ribs has a recurrence of an odontogenic keratocyst after enucleation. Which syndrome is likely?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

A. Gorlin-Goltz (basal cell naevus) syndrome

Gorlin-Goltz syndrome results from a PTCH1 mutation and includes multiple odontogenic keratocysts, basal cell carcinomas, bifid ribs and palmar pits. Keratocysts have a high recurrence rate due to satellite cysts. Gardner syndrome has osteomas and colonic polyps.

Option by option

  • A. Gorlin-Goltz (basal cell naevus) syndrome (correct)

    Correct. Multiple keratocysts with basal cell carcinomas define the syndrome.

  • B. Gardner syndrome

    Gardner syndrome has osteomas, polyposis and desmoids.

  • C. Peutz-Jeghers syndrome

    Peutz-Jeghers gives mucocutaneous pigmentation and hamartomas.

  • D. Cowden syndrome

    Cowden syndrome gives hamartomas and thyroid disease.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

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Questions about this ENT question

What is the answer to this ENT question?

The best answer is A, Gorlin-Goltz (basal cell naevus) syndrome. Gorlin-Goltz syndrome results from a PTCH1 mutation and includes multiple odontogenic keratocysts, basal cell carcinomas, bifid ribs and palmar pits.

Why is "Gardner syndrome" not the answer?

Gardner syndrome has osteomas, polyposis and desmoids.

Why is "Peutz-Jeghers syndrome" not the answer?

Peutz-Jeghers gives mucocutaneous pigmentation and hamartomas.

Why is "Cowden syndrome" not the answer?

Cowden syndrome gives hamartomas and thyroid disease.

What concept does this question test?

It tests Head and neck tumours: odontogenic keratocyst in ENT. Keratocysts have a high recurrence rate due to satellite cysts. Gardner syndrome has osteomas and colonic polyps.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Head and neck tumours: odontogenic keratocyst?

Learn tuning fork results and audiogram patterns by sketching them. Then use the flashcards and the other ENT questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.