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MEN 2B — MFDS Part 1 MCQ

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HardOral PathologyMEN 2BMFDS Part 1

A 12-year-old is referred after a dental examination identifies multiple painless nodules affecting the labial mucosa and anterior tongue. The lesions have been present since early childhood, and biopsy demonstrates enlarged, tortuous peripheral nerve bundles. The patient also has prominent everted lips, a tall slender build with long limbs, and an elevated serum calcitonin concentration. Which inherited syndrome best explains these findings?

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Correct answer: D — Multiple endocrine neoplasia type 2B

The diagnosis is multiple endocrine neoplasia type 2B. Its characteristic phenotype includes multiple mucosal neuromas of the lips and tongue, prominent lips and a marfanoid body habitus. Raised calcitonin indicates associated medullary thyroid carcinoma; affected patients are also at increased risk of phaeochromocytoma. Oral neuromas may appear in early childhood and can permit diagnosis before the thyroid malignancy becomes clinically apparent. MEN1 instead causes predominantly parathyroid, pituitary and pancreatic neuroendocrine tumours. Gardner syndrome produces intestinal polyposis, osteomas and dental abnormalities. Nevoid basal cell carcinoma syndrome is associated with multiple jaw keratocysts and basal cell carcinomas. PTEN hamartoma tumour syndrome can cause multiple oral papillomatous papules, but not the combination of neural lesions, marfanoid habitus and medullary thyroid carcinoma.

Reference: South West Genomic Medicine Service, Multiple endocrine neoplasia type 2A (MEN2A) and multiple endocrine neoplasia type 2B (MEN2B), 2025. https://www.southwestgenomics.nhs.uk/healthcare-professionals/request-a-test/a-z-conditions/multiple-endocrine-neoplasia-type-2a-men2a-multiple-endocrine-neoplasia-type-2b-men2b/