Author/Editor     Jerše, M; Mlakar, U; Popović, M; Vranič, A; Zidar, N
Title     Granulocytic sarcoma in a patient with essential thrombocythemia presented as acute spinal cord compression - case report and review of the literature
Type     članek
Source     Clin Neuropathol
Vol. and No.     Letnik 27, št. 4
Publication year     2008
Volume     str. 241-7
Language     eng
Abstract     Granulocytic sarcoma (GS) is a rare solid tumor of myeloid origin, which usually precedes or occurs concurrently with myeloid leukemia, or with other types of myeloproliferative and myelodysplastic disorders. Spinal affections of GS have been described but are uncommon, particularly in association with essential thrombocythemia. We present a case of a 7`-year-old woman with a long history of essential thrombocythemia who developed 2 tumors: l in the bodies of 1'3 - 6 vertebras extending epidurally, and the other in the right frontal lobe, adherent to dura, thus, mimicking meningioma. The patient died because of massive pulmonary thrombembolia. Microscopical and immunohistochemical features of spinal and intracranial tumor samples obtained at autopsy were consistent with the diagnosis of GS with focal megakaryocytic differentiation. Clinicians and pathologists should be aware of this rare tumor being so diverse in its clinical presentation, as well as in microscopical and immunohistochemical features. Careful evaluation of morphology, in conjunction with immunohistochemistry for evidence of myeloid differentiation are required Yo avoid frequent errors in diagnostics of GS. The suggested panel includes chloroacetate esterase, myeloperoxidase, lysozyme, CD117, CD43, CD79a and CD3. Only early correct diagnosis will enable proper treatment which may be successful despite the highly malignant potential of GS.
Descriptors     THROMBOCYTHEMIA, HEMORRHAGIC
SPINAL CORD COMPRESSION
SARCOMA
GRANULOCYTES
IMMUNOHISTOCHEMISTRY
AGED
AUTOPSY