Myeloid Sarcoma with Megakaryoblastic Differentiation Arising in the Conjunctiva

Ocul Oncol Pathol. 2019 Jan;5(1):28-35. doi: 10.1159/000488057. Epub 2018 May 22.

Abstract

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.

Keywords: Acute megakaryoblastic leukemia; Blast; Chloroma; Conjunctiva; Granulocytic sarcoma; JAK2; Megakaryocyte; Myelofibrosis; Myeloid sarcoma; Trisomy 8; V617F mutation; t(12; 17) translocation.