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LCH in Children — MFDS Part 1 MCQ

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HardOral PathologyLCH in ChildrenMFDS Part 1

A 6-year-old child presents with painful gingival swelling and increasing mobility of several primary teeth. Radiographs show sharply demarcated areas of alveolar bone destruction, leaving some teeth with a “floating” appearance. Biopsy demonstrates sheets of mononuclear cells with pale cytoplasm and grooved, coffee-bean-shaped nuclei, accompanied by numerous eosinophils. The lesional cells express CD1a and CD207 (langerin). What is the most likely diagnosis?

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Correct answer: ALangerhans cell histiocytosis

The diagnosis is Langerhans cell histiocytosis. In children, jaw involvement may cause destructive alveolar radiolucencies, severe tooth mobility and the characteristic “floating teeth” appearance. The decisive findings are grooved, coffee-bean-shaped lesional cells admixed with eosinophils and expression of CD1a and CD207 (langerin). Ewing sarcoma instead comprises small round blue cells, typically with CD99 expression. Osteosarcoma requires malignant osteoid formation. A peripheral giant cell granuloma contains numerous osteoclast-like giant cells and usually causes only superficial cupping of underlying bone. Fibrous dysplasia produces a diffuse ground-glass appearance and irregular woven bone in fibrous stroma rather than destructive alveolar lesions with this immunophenotype.

Reference: Krooks J, Minkov M, Weatherall AG. Langerhans cell histiocytosis in children: Diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. Journal of the American Academy of Dermatology. 2018;78(6):1047-1056. https://pubmed.ncbi.nlm.nih.gov/34091963/