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Localized tongue Amyloidosis in a Patient with Neurofibromatosis type II
Dimitrios Andreadis1, Athanasios Poulopoulos, Petros Papadopoulos
1Department of Oral Medicine/Pathology, School of Dentistry, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece. dandrea@dent.auth.gr
Background:
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.
Case:
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.
Conclusion:
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.
