Bone tumours: fibrous dysplasia: practice question

A Orthopaedics 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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Orthopaedics · Bone tumours: fibrous dysplasia

Sample item

A girl has café-au-lait spots, precocious puberty and polyostotic bone lesions with a ground-glass appearance and shepherd's crook deformity of the femur. Which syndrome is this?

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

The answer and the reasoning

A. McCune-Albright syndrome

McCune-Albright syndrome is polyostotic fibrous dysplasia with endocrine hyperfunction such as precocious puberty and café-au-lait spots with irregular borders, due to an activating GNAS mutation. Mazabraud syndrome is fibrous dysplasia with intramuscular myxomas.

Option by option

  • A. McCune-Albright syndrome (correct)

    Correct. The triad defines McCune-Albright syndrome.

  • B. Neurofibromatosis type 1

    NF1 has neurofibromas and smooth-edged cafe-au-lait spots.

  • C. Mazabraud syndrome

    Mazabraud syndrome has intramuscular myxomas.

  • D. Jaffe-Campanacci syndrome

    Jaffe-Campanacci syndrome has multiple non-ossifying fibromas.

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 Orthopaedics question

What is the answer to this Orthopaedics question?

The best answer is A, McCune-Albright syndrome. McCune-Albright syndrome is polyostotic fibrous dysplasia with endocrine hyperfunction such as precocious puberty and café-au-lait spots with irregular borders, due to an activating GNAS mutation.

Why is "Neurofibromatosis type 1" not the answer?

NF1 has neurofibromas and smooth-edged cafe-au-lait spots.

Why is "Mazabraud syndrome" not the answer?

Mazabraud syndrome has intramuscular myxomas.

Why is "Jaffe-Campanacci syndrome" not the answer?

Jaffe-Campanacci syndrome has multiple non-ossifying fibromas.

What concept does this question test?

It tests Bone tumours: fibrous dysplasia in Orthopaedics. Mazabraud syndrome is fibrous dysplasia with intramuscular myxomas.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. 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 Bone tumours: fibrous dysplasia?

Pair each fracture with its classic complication and nerve injured. Then use the flashcards and the other Orthopaedics 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.