Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
August 2013
Next Case of the Month coming September 3 . . .
Leptomeningeal Granulocytic Sarcoma
- Solid tumor composed of immature myeloid cells of granylocytic lineage; can occur in virtually any location of the body, most frequently in bone and soft tissue. When seen intracranially, it is typically extra-axial in location.
- May develop before, during, or after the onset of a myeloproliferative disorder, most frequently acute myelogenous leukemia or blastic transformation of chronic myelogenous leukemia. Half of patients are younger than 15 years.
- Clinical Features: Increased intracranial pressure and/or focal neurological signs
- Key Diagnostic Features: Intracranial granulocytic sarcoma is most frequently extra-axial, rarely intra-axial. The lesion enhances homogeneously, but may appear heterogeneous in cases of intratumoral hemorrhage. Hyperdensity on noncontrast CT and restricted diffusion in a context of a myeloproliferative disorder must raise suspicion of a granulocytic sarcoma.
- DDx: Meningioma, metastasis , lymphoma, TB, inflammatory granulomatosis
- Rx: Radiation therapy, chemotherapy and high-dose steroids. Overall prognosis: poor.
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