[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/Sex	Associated hematologic neoplasia	Cytogenetics	CNS involvement	Other sites of involvement	CSF	Survival/mo
1	0.5/M	AML	Trisomy 8	L FP	-	+	AWD/12
2	3.9/F	AML	N/A	R F	-	+	N/A
3	16/M	AML	Inv16	R FP	-	-	ANED/8
4	37/F	AML	Normal	R O	N/A	N/A	D
5	48/F	CML	t(9:22)	R S1-S2 foramina	R sacrum	N/A	D/2
6	53/M	N/A	N/A	Multiple cervical and thoracic spinal cord roots	Cervical and thoracic paraspinal soft tissue	N/A	A
7	64/M	AML	5q del	T6-T9 bilateral neural foramina and epidural space	T6-T9 paraspinal soft tissue	N/A	D/4
8	69/F	PV	Complex; JAK2 mutation	L F dura	-	+	D/3
9	76/M	MDS	Trisomy 21	Choroid plexus, pituitary	Widespread	N/A	D/8
10	84/F	MDS	Normal	L FT	-	N/A	D/<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