Gynaecological endocrinology: McCune-Albright syndrome: practice question

A OBG 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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OBG · Gynaecological endocrinology: McCune-Albright syndrome

Sample item

A 5-year-old girl has vaginal bleeding and breast development. She has irregular café-au-lait macules with jagged borders and a bone scan shows fibrous dysplasia. Gonadotropin levels are suppressed. What is the diagnosis?

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

The answer and the reasoning

C. McCune-Albright syndrome

McCune-Albright syndrome results from a post-zygotic activating GNAS mutation and consists of peripheral (gonadotropin-independent) precocious puberty from autonomous ovarian cysts, café-au-lait spots and polyostotic fibrous dysplasia. LH and FSH are suppressed because oestrogen is produced autonomously. Treatment uses aromatase inhibitors or oestrogen receptor modulators.

Option by option

  • A. Turner syndrome

    Turner syndrome presents with short stature and delayed puberty.

  • B. Central precocious puberty

    Central precocity would have raised or pubertal LH levels and no fibrous dysplasia.

  • C. McCune-Albright syndrome (correct)

    Correct. The triad of peripheral precocity, café-au-lait spots and fibrous dysplasia is McCune-Albright syndrome.

  • D. Neurofibromatosis type 1

    NF1 has café-au-lait macules with smooth borders, neurofibromas and no peripheral precocity of this type.

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.

More OBG questions

Questions about this OBG question

What is the answer to this OBG question?

The best answer is C, McCune-Albright syndrome. McCune-Albright syndrome results from a post-zygotic activating GNAS mutation and consists of peripheral (gonadotropin-independent) precocious puberty from autonomous ovarian cysts, café-au-lait spots and polyostotic fibrous dysplasia.

Why is "Turner syndrome" not the answer?

Turner syndrome presents with short stature and delayed puberty.

Why is "Central precocious puberty" not the answer?

Central precocity would have raised or pubertal LH levels and no fibrous dysplasia.

Why is "Neurofibromatosis type 1" not the answer?

NF1 has café-au-lait macules with smooth borders, neurofibromas and no peripheral precocity of this type.

What concept does this question test?

It tests Gynaecological endocrinology: McCune-Albright syndrome in OBG. LH and FSH are suppressed because oestrogen is produced autonomously. Treatment uses aromatase inhibitors or oestrogen receptor modulators.

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 Gynaecological endocrinology: McCune-Albright syndrome?

Learn management algorithms as steps: PPH, eclampsia, ectopic pregnancy. Then use the flashcards and the other OBG 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.