Localized Tongue Amyloidosis in a Patient with Neurofibromatosis Type II

Department of Oral Medicine/Pathology, School of Dentistry, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece.
Head and Neck Pathology 02/2011; 5(3):302-5. DOI: 10.1007/s12105-011-0251-5
Source: PubMed


Localized Amyloidosis (AL) may rarely involve oral mucosa. This is the first known reported case describing the development of tongue AL in a 30-year-old patient with Neurofibromatosis (NF) type-2.
A female patient presented with a painless, well-circumscribed nodule of the tongue. Her medical history included NF type-2 with chromosome-22 abnormal karyotype (mosaicism), multiple intracranial and spinal meningiomas/schwannomas and unilateral blindness/deafness. The biopsy of the excised lesion of the tongue revealed subepithelial accumulation of an amorphous, nodular, fibrillar material positive for Congo red. Blood examination showed increased Thyroxine-T4 due to thyroid multinodular colloid goiter, but excluded any other hematological/immunological disorder or organ dysfunction. No recurrence was observed after a six-month follow-up.
This case highlights the possibility of oral manifestations as the only sign of AL and reveals the unexpected co-existence of AL and NF 2, for the first time.

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Available from: Apostolos Epivatianos, Feb 28, 2014
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