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Dermatology · Skin tumours: plexiform neurofibroma
Sample itemA child with NF1 has a soft, ropey, bag-of-worms swelling of the eyelid and temple with overlying hyperpigmentation. Which lesion is this, and what is its main risk?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Plexiform neurofibroma, which can transform into malignant peripheral nerve sheath tumour
Plexiform neurofibroma is pathognomonic of NF1, is usually congenital, and involves multiple fascicles with a bag-of-worms feel. A minority undergo malignant change to malignant peripheral nerve sheath tumour, so rapid growth or new pain needs urgent evaluation.
Option by option
A. Dermal neurofibroma, which commonly turns into angiosarcoma
Dermal neurofibromas are benign and do not typically transform to angiosarcoma.
B. Plexiform neurofibroma, which can transform into malignant peripheral nerve sheath tumour (correct)
Correct. Plexiform neurofibroma is characteristic of NF1 and may become malignant peripheral nerve sheath tumour.
C. Solitary schwannoma, which often metastasises
A solitary schwannoma is encapsulated and benign and does not metastasise.
D. Granular cell tumour, which carries a high melanoma risk
Granular cell tumour is a benign Schwann-derived tumour and is not linked with melanoma.
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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