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Relapse of acute myelogenous leukemia as a cerebellar myeloblastoma showing megakaryoblastic differentiation
R B Lorsbach1, R D Folkerth, G S Pinkus
1Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts, USA. robert.lorsbach@stjude.org
Abstract:
Myeloblastomas (granulocytic sarcomas) occurring within the central nervous system (CNS) are extremely rare lesions that may develop in patients with acute or chronic myeloproliferative disorders. The majority of such lesions involve brain or spinal cord by contiguous spread from meningeal or bony sites, rather than originating within the CNS parenchyma. We describe a patient with acute myelogenous leukemia in remission, who developed a purely intraparenchymal cerebellar myeloblastoma with megakaryocytic differentiation. The neoplastic cells expressed the megakaryocytic markers factor VIII-related antigen and platelet glycoprotein-IIIa (CD61), and showed ultrastructural features that were indicative of megakaryocytic differentiation. Clinically, myeloblastomas of the CNS invoke a broad differential diagnosis that includes abscess, hemorrhage, and metastatic neoplasms because of their intraparenchymal location and radiologic features. Although they are rare, myeloblastomas should be included in the histopathologic differential diagnosis of a poorly differentiated neoplasm occurring within the CNS, particularly in a patient with a history of myeloproliferative or myelodysplastic disease.
Insights
Central nervous system (CNS) myeloblastomas are rare. This case highlights a unique intraparenchymal cerebellar myeloblastoma with megakaryocytic differentiation in a patient with acute myelogenous leukemia.
Area of Science:
- Neuropathology
- Hematology
- Oncology
Background:
- Central nervous system (CNS) myeloblastomas, also known as granulocytic sarcomas, are uncommon manifestations of myeloproliferative disorders.
- Typically, these lesions arise from contiguous spread from meningeal or bony sites, rarely originating within the CNS parenchyma.
Observation:
- A case study of a patient with acute myelogenous leukemia in remission presenting with a rare, purely intraparenchymal cerebellar myeloblastoma is described.
- The tumor exhibited megakaryocytic differentiation, confirmed by the expression of megakaryocytic markers (factor VIII-related antigen and platelet glycoprotein-IIIa [CD61]) and ultrastructural analysis.
Findings:
- The neoplastic cells demonstrated specific markers and ultrastructural characteristics of megakaryocytic lineage.
- This case represents an unusual intraparenchymal origin within the cerebellum.
Implications:
- CNS myeloblastomas present a diagnostic challenge due to their intraparenchymal location and radiologic similarity to abscesses, hemorrhages, or metastatic neoplasms.
- In patients with a history of myeloproliferative or myelodysplastic diseases, CNS myeloblastomas should be considered in the differential diagnosis of poorly differentiated CNS neoplasms.
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