Service of Pathology. Hospital Universitario Dexeus, Barcelona, Spain.
Centro de Oftalmologia Barraquer, Barcelona, Spain.
Rev Esp Patol. 2022 Sep;55 Suppl 1:S7-S10. doi: 10.1016/j.patol.2020.06.007. Epub 2020 Jul 25.
We present a case of a 53-year-old female with an 11 year history of myasthenia gravis (MG) with palpebral ptosis in the left eye which had become more marked over the previous year. Examination revealed a painless left orbital mass causing ptosis. The tumor was surgically removed and histopathology revealed deposits of a hyaline substance which when stained with Congo Red had an apple-green birefringence with polarized light, typical of amyloid. In the absence of amyloid deposits elsewhere in the body, amyloid tumor (AT) was diagnosed. No myelo- or lymphoproliferative syndromes, systemic involvement by amyloidosis or any autoimmune disease were found. The evolution and aesthetic results where satisfactory. Only two cases of orbital AT associated with MG have been described previously; however, in one of the cases, the symptoms of the AT had led to a false diagnosis of MG.
我们报告了 1 例 53 岁女性患者,患有重症肌无力(MG)11 年,左眼上睑下垂,过去 1 年来症状逐渐加重。检查发现左侧眼眶无痛性肿块导致上睑下垂。肿瘤被手术切除,组织病理学显示透明物质沉积,刚果红染色后具有苹果绿双折射,偏振光下具有特征性,符合淀粉样变性。在身体其他部位无淀粉样沉积的情况下,诊断为淀粉样瘤(AT)。未发现骨髓或淋巴增生性综合征、淀粉样变性的全身受累或任何自身免疫性疾病。病情进展和美容效果均令人满意。先前仅描述过 2 例与 MG 相关的眶内 AT,但其中 1 例 AT 的症状导致了对 MG 的误诊。