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Atypical Mole Syndrome — SCE Dermatology MCQ

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HardSkin CancerAtypical Mole SyndromeSCE Dermatology

A 60-year-old man is being assessed for melanoma risk. He has >100 melanocytic naevi and ≥5 clinically atypical naevi with dysplastic features on dermoscopy. He has a first-degree relative with melanoma. What is this phenotype called?

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Correct answer: E — Atypical mole syndrome (dysplastic naevus syndrome/FAMMM)

The best answer is “Atypical mole syndrome (dysplastic naevus syndrome/FAMMM)”. NICE bases melanoma diagnosis, excision margins and nodal assessment on complete histology, Breslow thickness, ulceration and clinical stage, with specialist review of suspicious or changing lesions. The alternatives “Gorlin syndrome with multiple basal-cell carcinomas”, “Cowden syndrome with mucocutaneous hamartomas”, “Peutz–Jeghers syndrome with lentiginosis and polyposis”, “Birt–Hogg–Dubé syndrome with fibrofolliculomas” are clinically adjacent possibilities, but they do not fit the defining morphology, distribution, histopathology, risk signal or management sequence in this stem.

Reference: NICE NG14 melanoma recommendations: https://www.nice.org.uk/guidance/ng14/chapter/Recommendations