Document Detail

A meningioma with islets of extramedullary myeloid metaplasia: case report.
MedLine Citation:
PMID:  17762725     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Idiopathic myelofibrosis is a clonal stem cell disorder that leads to ineffective erythropoiesis accompanied by reactive myelofibrosis (bone marrow fibrosis). As a consequence, extramedullary hematopoiesis characteristically develops. The central nervous system is rarely affected; the spinal canal and the cranial meninges are generally the preferred locations. Extramedullary hematopoiesis within central nervous system primary tumors have already been reported but, to our knowledge, never before in a patient with evidence of idiopathic myelofibrosis. CLINICAL PRESENTATION: A patient experiencing generalized idiopathic myelofibrosis developed a hemorrhagic intracranial meningioma containing islets of extramedullary myeloid metaplasia. INTERVENTION: The tumor was radically removed through a right frontal craniotomy. After surgery, the patient recovered completely and was discharged with a normal neurological status. After 6 years, the patient is in excellent condition with no sign of recurrence on magnetic resonance imaging scans. CONCLUSION: The reasons for this uncommon association are uncertain, but we hypothesize that myeloid islets may be involved in the origin of the tumor as well as in its acute hemorrhagic onset. Moreover, we suggest that in the presence of proven idiopathic myelofibrosis intracranial myeloid metaplasia should be ruled out by appropriate neuroimaging and considered as a potential diagnosis in the presence of brain lesions.
Gianluigi Zona; Giannantonio Spena; Pier F Sbaffi; Renato Spaziante
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neurosurgery     Volume:  61     ISSN:  1524-4040     ISO Abbreviation:  Neurosurgery     Publication Date:  2007 Aug 
Date Detail:
Created Date:  2007-08-31     Completed Date:  2007-09-25     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  7802914     Medline TA:  Neurosurgery     Country:  United States    
Other Details:
Languages:  eng     Pagination:  E418-9; discussion E419     Citation Subset:  IM    
Department of Neurosurgery, San Martino University Hospital, Genoa, Italy.
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MeSH Terms
Brain / pathology,  radiography
Brain Neoplasms / pathology*,  radiography,  surgery
Hematopoiesis, Extramedullary*
Magnetic Resonance Imaging
Meningeal Neoplasms / pathology*,  radiography,  surgery
Meningioma / pathology*,  radiography,  surgery
Middle Aged
Primary Myelofibrosis / pathology*
Tomography, X-Ray Computed

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