Bone tumours: Hand-Schuller-Christian disease: 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: Hand-Schuller-Christian disease

Sample item

A 3-year-old has multiple lytic skull lesions, passes large volumes of dilute urine and has bulging eyes. Biopsy of a skull lesion shows Langerhans cells. What is the clinical form of this histiocytosis?

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

The answer and the reasoning

D. Hand-Schuller-Christian disease

The triad of lytic calvarial lesions, diabetes insipidus from pituitary stalk involvement and exophthalmos from orbital deposits defines Hand-Schuller-Christian disease, the chronic multifocal form of Langerhans cell histiocytosis. Letterer-Siwe disease is the acute disseminated form of infancy and eosinophilic granuloma is the solitary bone form.

Option by option

  • A. Letterer-Siwe disease

    Letterer-Siwe disease is acute, disseminated and mainly affects infants with skin and organ involvement.

  • B. Gaucher disease

    Gaucher disease is a storage disorder with Gaucher cells, not Langerhans cells.

  • C. Eosinophilic granuloma

    Eosinophilic granuloma is a solitary or few bone lesions without systemic features.

  • D. Hand-Schuller-Christian disease (correct)

    Correct. Skull lesions, polyuria and proptosis form the classic chronic multifocal picture.

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 D, Hand-Schuller-Christian disease. The triad of lytic calvarial lesions, diabetes insipidus from pituitary stalk involvement and exophthalmos from orbital deposits defines Hand-Schuller-Christian disease, the chronic multifocal form of Langerhans cell histiocytosis.

Why is "Letterer-Siwe disease" not the answer?

Letterer-Siwe disease is acute, disseminated and mainly affects infants with skin and organ involvement.

Why is "Gaucher disease" not the answer?

Gaucher disease is a storage disorder with Gaucher cells, not Langerhans cells.

Why is "Eosinophilic granuloma" not the answer?

Eosinophilic granuloma is a solitary or few bone lesions without systemic features.

What concept does this question test?

It tests Bone tumours: Hand-Schuller-Christian disease in Orthopaedics. Letterer-Siwe disease is the acute disseminated form of infancy and eosinophilic granuloma is the solitary bone form.

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: Hand-Schuller-Christian disease?

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.