Jakobiec Frederick A, Wolkow Natalie, Zakka Fouad R, Rubin Peter A D
David G. Cogan Laboratory of Ophthalmic Pathology, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA.
Department of Pathology and Laboratory Medicine, Alpert Medical School, Brown University, Providence, Rhode Island, USA.
Ocul Oncol Pathol. 2019 Jan;5(1):28-35. doi: 10.1159/000488057. Epub 2018 May 22.
An 87-year-old woman not known to have either a lymphoma or leukemia developed a left multinodular, fish-flesh superior epibulbar and forniceal mass. A biopsy disclosed a blastic tumor with scattered multinucleated immature megakaryoblasts. Immunophenotyping of bone marrow cells revealed strong positivity for CD7, CD31, CD43, CD45, CD61, and CD117; CD71, myeloperoxidase, and lysozyme were also positive in scattered cells. Forty percent of the neoplastic cells were Ki-67 positive. Cytogenetic studies indicated a trisomy 8 (associated with worse prognosis) and a t(12; 17) translocation. Desmin, smooth muscle actin, pancytokeratin, CAM 5.2, adipophilin, tryptase, S100, SOX10, MART1, and E-cadherin were negative, ruling out a nonhematopoietic tumor. The conjunctival lesion was diagnosed as a myeloid sarcoma with megakaryoblastic differentiation, a rare variant. It probably arose from a myelodysplastic syndrome. This is the first case of its type to develop in the conjunctiva.
一名87岁女性,既往无淋巴瘤或白血病病史,出现左眼球上睑结膜和穹窿部多结节鱼肉样肿物。活检显示为一母细胞性肿瘤,散在有多核未成熟巨核母细胞。骨髓细胞免疫表型分析显示CD7、CD31、CD43、CD45、CD61和CD117呈强阳性;CD71、髓过氧化物酶和溶菌酶在散在细胞中也呈阳性。40%的肿瘤细胞Ki-67呈阳性。细胞遗传学研究显示8号染色体三体(与预后较差相关)和t(12; 17)易位。结蛋白、平滑肌肌动蛋白、全细胞角蛋白、CAM 5.2、脂联素、类胰蛋白酶、S100、SOX10、MART1和E-钙黏蛋白均为阴性,排除了非造血性肿瘤。结膜病变被诊断为具有巨核母细胞分化的髓系肉瘤,这是一种罕见的变异型。它可能起源于骨髓增生异常综合征。这是首例发生于结膜的此类病例。