Rauba Darius, Lesinskas Eugenijus, Petrulionis Mindaugas, Sukytė Donata, Valevičienė Nomeda, Palionis Darius, Tamošiūnas Algirdas
Radiology and Nuclear Medicine Centre, Vilnius University Hospital Santariškių Klinikos, Santariškių 2, 08661 Vilnius, Lithuania.
Medicina (Kaunas). 2013;49(11):497-503.
Amyloidosis is a rare condition characterized by the deposition of abnormal protein filaments into the extracellular tissue. Only 16 cases of true primary idiopathic amyloidosis localized in the nose and the paranasal sinuses have previously been reported. We present a case of localized amyloidosis involving the sinonasal cavities and the nose in a 53-year-old woman. Our patient was operated on as a typical case of chronic rhinosinusitis with polyps, but after 2 years, the patient came back with severe symptoms of nasal obstruction and pain in the frontal and maxillary regions with proptosis of both eyes. Magnetic resonance imaging revealed sinonasal-intraorbital masses. Primary amyloidosis (AL type) was confirmed by tissue biopsy. A systemic workup for additional amyloid deposits revealed no evidence of other diseases. Extended surgery produced a symptomatic improvement.
淀粉样变性是一种罕见疾病，其特征是异常蛋白细丝沉积于细胞外组织。此前仅有16例真正的原发性特发性淀粉样变性局限于鼻腔和鼻窦的病例报道。我们报告一例53岁女性发生的累及鼻窦腔和鼻腔的局限性淀粉样变性病例。我们的患者最初作为典型的慢性鼻窦炎伴息肉病例接受了手术，但2年后，患者因严重的鼻塞、额部和上颌部疼痛以及双眼突出症状复诊。磁共振成像显示鼻窦-眶内肿块。组织活检确诊为原发性淀粉样变性（AL型）。针对其他淀粉样沉积物的全身检查未发现其他疾病证据。扩大手术使症状得到改善。
