Gilad R, Milillo P, Som P M
Department of Neurosurgery, Mount Sinai School of Medicine of New York University, New York, USA.
AJNR Am J Neuroradiol. 2007 Sep;28(8):1557-8. doi: 10.3174/ajnr.A0604.
Amyloidosis is a term applied to a diverse group of disorders that share the deposition of amyloid protein in various extracellular tissues. Systemic amyloidosis may involve almost any organ system in the body including regions in the head and neck; however, pharyngeal involvement is rare, with only 12 cases having been previously reported. Ten of these cases were localized disease, and only 2 cases were systemic amyloidosis. We present the case of a patient with severe diffuse systemic amyloidosis with extensive involvement of the pharynx, larynx, trachea, lungs, eyelids, and breasts. We also review the imaging characteristics and pertinent literature.
淀粉样变性是一个适用于多种疾病的术语,这些疾病的共同特征是淀粉样蛋白在各种细胞外组织中沉积。系统性淀粉样变性可能累及身体几乎任何器官系统,包括头颈部区域;然而,咽部受累罕见,此前仅有12例报道。其中10例为局限性疾病,仅有2例为系统性淀粉样变性。我们报告一例严重弥漫性系统性淀粉样变性患者,其咽部、喉部、气管、肺、眼睑和乳房广泛受累。我们还回顾了影像学特征及相关文献。