Background: Myeloid sarcoma (MS) is a rare solid
tumour made of myeloblasts or immature myeloid cells in an extramedullary
site or in bone, associated with systemic hematologic neoplasms. When they
occur in the brain parenchyma, they can often be misdiagnosed.
Methods: The authors report a case of a 4-year
old boy 6 months out of remission from AML, presenting with a short history
of headaches and vomiting, and found to have a heterogenous
contrast-enhancing lesion in the right cerebellar hemisphere, with
differential diagnosis of myeloid sarcoma, astrocytoma, medulloblastoma and
ATRT. Preliminary diagnosis was made flow cytometry from an intraoperative
biopsy. The patient had a long course of chemotherapy and radiation, but
eventually died from the systemic burden of his AML.
Results: The authors present a literature review
on 178 published cases of CNS myeloid sarcomas, and their radiological
presentation and the basis of immunohistochemical and pathological diagnosis
is discussed. Conclusions: Diagnosis rests on a
combination of immunohistochemistry and histopathology of biopsied tissue.
Surgical resection is controversial, especially given the efficacy of
chemotherapy and radiation, and prognosis remains unclear. As with all
uncommon and rare clinical entities, further investigation is warranted to
determine prognosis and optimal management of CNS myeloid sarcomas.