关键词: Amylose orbitaire Chirurgie. Orbital amyloidosis Radiation therapy Radiothérapie Surgery.

来  源:   DOI:10.1016/j.canrad.2022.04.006

Abstract:
An 80 year-old woman was referred for a one-eyed palpebral edema. She had ptosis and oblique diplopia resistant to corticotherapy. An orbit magnetic resonance imaging showed a mass infiltrating the optic nerve, with an enhancement of the whole orbital musculature. Biopsies of the lacrimal gland were positive for immunoglobulin light chain amyloidosis. Debulking surgery is the main treatment modality for symptomatic patients with localized orbital amyloidosis. Diffuse orbital involvement may make excision difficult. We managed to treat her through radiation therapy to allow a surgical procedure, delivering 20Gy in 10 fractions. The main aim of radiation therapy was to eradicate the B cells that produced the amyloid light chain deposition. After radiation therapy, the orbital inflammatory caused by the lymphoid infiltration diminished and corticotherapy could be stopped. The surgery could be performed with excellent clinical results. At 2-year follow-up, neither tumor regrowth nor late complications were detected.
摘要:
一名80岁的妇女因单眼眼睑水肿而被转诊。她对皮质治疗有抵抗的上睑下垂和斜视复视。轨道磁共振成像显示有肿块浸润视神经,整个轨道肌肉组织的增强。泪腺活检为免疫球蛋白轻链淀粉样变性阳性。对于有症状的局限性眼眶淀粉样变性患者,膨化手术是主要的治疗方式。弥漫性眼眶受累可能使切除困难。我们设法通过放射治疗来治疗她,以便进行外科手术,在10个部分提供20Gy。放射治疗的主要目的是根除产生淀粉样蛋白轻链沉积的B细胞。放射治疗后,由淋巴浸润引起的眼眶炎症减少,皮质治疗可以停止。该手术可以进行,具有良好的临床效果。在2年的随访中,未检测到肿瘤再生长或晚期并发症。
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