Wesley R E, Cooper J, Litchford D W
Department of Ophthalmology, Vanderbilt University School of Medicine, Nashville, Tennessee.
Ann Ophthalmol. 1988 Apr;20(4):150-2.
A 52-year-old man developed proptosis from an orbital mass which was documented histopathologically to be an inflammatory orbital pseudotumor. When the lesion failed to resolve after six months of adrenocorticosteroid treatment, a second biopsy showed an inflammatory mass with nests of eosinophilic granuloma. The patient soon developed a lymphoma of the soft palate and a glioblastoma multiforme which led to the patient's rapid demise. Eosinophilic granuloma is known to represent the more benign end of the spectrum of histiocytic disorders in which a proliferation of Langerhans' cells and an abnormality of T-suppressor cells occur. Our case represents the first report of multifocal tumor immunoincompetence occurring with eosinophilic granuloma in an orbital inflammatory pseudotumor.
一名52岁男性因眼眶肿物出现眼球突出，组织病理学证实为炎性眼眶假瘤。肾上腺皮质类固醇治疗6个月后病变未消退，再次活检显示为伴有嗜酸性肉芽肿巢的炎性肿物。该患者很快出现软腭淋巴瘤和多形性胶质母细胞瘤，导致患者迅速死亡。嗜酸性肉芽肿是组织细胞疾病谱中较为良性的一端，其特征是朗格汉斯细胞增殖和T抑制细胞异常。我们的病例是眼眶炎性假瘤中嗜酸性肉芽肿伴发多灶性肿瘤免疫功能不全的首例报告。
