[1808] Central Nervous System Involvement by Myeloid Sarcoma

Adriana Olar, Thor D Stein, Christian J Davidson, Arie Perry, Gabriela Gheorghe. The Methodist Hospital, Houston, TX; Boston VA Medical Center, Boston University School of Medicine, Jamaica Plain, MA; Massachusetts General Hospital, Harvard Medical School, Boston, MA; Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI; University of California, San Francisco, San Francisco, CA

Background: Myeloid sarcoma (MS) is an extramedullary hematopoietic neoplasm of myeloid origin. Central nervous system (CNS) involvement by MS is unusual. We report ten cases of biopsy-proven CNS MS.
Design: The pathology records across eight institutions were searched for CNS MS. The clinical course, radiology and pathology were reviewed.
Results: Our results are summarized below.

#Age y/SexAssociated hematologic neoplasiaCytogeneticsCNS involvementOther sites of involvementCSFSurvival/mo
10.5/MAMLTrisomy 8L FP-+AWD/12
23.9/FAMLN/AR F-+N/A
316/MAMLInv16R FP--ANED/8
437/FAMLNormalR ON/AN/AD
548/FCMLt(9:22)R S1-S2 foraminaR sacrumN/AD/2
653/MN/AN/AMultiple cervical and thoracic spinal cord rootsCervical and thoracic paraspinal soft tissueN/AA
764/MAML5q delT6-T9 bilateral neural foramina and epidural spaceT6-T9 paraspinal soft tissueN/AD/4
869/FPVComplex; JAK2 mutationL F dura-+D/3
976/MMDSTrisomy 21Choroid plexus, pituitaryWidespreadN/AD/8
1084/FMDSNormalL FT-N/AD/<6
AML-acute myeloid leukemia, CML-chronic myelogenous leukemia, PV-polycythemia vera, MDS-myelodysplastic syndrome, L/R-left/right, F/P/T/O-frontal/parietal/temporal/occipital lobe, A/WD/NED-alive/with disease/no evidence of disease, D-deceased

Four patients had a prior diagnosis of a myeloid neoplasm. In 5 patients (#1, 3, 4, 7, 8) CNS MS was the initial presentation. Three (#1, 3, 4) presented with hemorrhagic masses, one of which was clinically misdiagnosed as subdural hematoma. Two (#7, 8) had enhancing lesions, one (#8) presenting with a dural-based mass. In one case (#9), MS was diagnosed at autopsy. In our series, MS occurred in isolation, sparing other organs in 6/10 cases.
Conclusions: The clinical and imaging characteristics of CNS MS overlap with those of hemorrhage and primary CNS tumors. Differential diagnosis with intracranial bleeding is particularly challenging. It is important to maintain a high index of suspicion and perform a biopsy whenever clinically appropriate. A meticulous workup is necessary to avoid misdiagnosis of other hematopoietic or nonhematopoietic neoplasms. Since CNS MS is curable, timely recognition is paramount.
Category: Neuropathology

Tuesday, March 20, 2012 1:00 PM

Poster Session IV # 265, Tuesday Afternoon

 

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