Skin tumours: Maffucci syndrome: practice question

A Dermatology 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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Dermatology · Skin tumours: Maffucci syndrome

Sample item

A young man has multiple soft blue-purple spindle-cell haemangiomas on the hands and multiple enchondromas causing deformity of the fingers. Which malignancy carries the greatest risk?

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

The answer and the reasoning

B. Chondrosarcoma

Maffucci syndrome is multiple enchondromas with spindle-cell haemangiomas, from mosaic IDH1 or IDH2 mutations. Enchondromas have a high lifetime risk of malignant change to chondrosarcoma.

Option by option

  • A. Osteosarcoma

    Osteosarcoma arises from osteoid-forming cells and is not the main risk here.

  • B. Chondrosarcoma (correct)

    Correct. Enchondromas transform to chondrosarcoma in a high proportion of patients.

  • C. Melanoma

    Melanoma is unrelated to enchondromatosis.

  • D. Rhabdomyosarcoma

    Rhabdomyosarcoma is a paediatric muscle tumour unrelated to this syndrome.

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 Dermatology question

What is the answer to this Dermatology question?

The best answer is B, Chondrosarcoma. Maffucci syndrome is multiple enchondromas with spindle-cell haemangiomas, from mosaic IDH1 or IDH2 mutations.

Why is "Osteosarcoma" not the answer?

Osteosarcoma arises from osteoid-forming cells and is not the main risk here.

Why is "Melanoma" not the answer?

Melanoma is unrelated to enchondromatosis.

Why is "Rhabdomyosarcoma" not the answer?

Rhabdomyosarcoma is a paediatric muscle tumour unrelated to this syndrome.

What concept does this question test?

It tests Skin tumours: Maffucci syndrome in Dermatology. Enchondromas have a high lifetime risk of malignant change to chondrosarcoma.

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 Skin tumours: Maffucci syndrome?

Learn each condition by its primary lesion, site and signature sign. Then use the flashcards and the other Dermatology 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.