Hypothalamic Lymphoma
Central nervous system lymphoma is an uncommon disease that accounts for less than 3 % of all intracranial tumours. It can appear in various anatomical locations, but only a few cases have been reported in hypothalamic region. Hypothalamic lymphoma is not
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15
Ali Akhaddar
Contents
Abbreviations
15.1 Introduction .................................................
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15.2 Literature Review........................................
133
15.3 Epidemiology ...............................................
134
15.4 Clinical Presentation ...................................
134
15.5 Diagnostic Studies .......................................
139
15.6 Pathology......................................................
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15.7 Treatment and Outcome .............................
140
Conclusion ..............................................................
141
References ..............................................................
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CNS CT scan MRI
15.1
University of Mohammed V Souissi, Rabat 10100, Morocco e-mail: [email protected]
Introduction
Central nervous system (CNS) lymphoma is an uncommon disease that accounts for less than 3 % of all intracranial tumours. It can appear in various anatomical locations, but only a few cases have been reported in hypothalamic region. To our knowledge, only 15 cases of hypothalamic lymphomas have been published until now. A variable natural history and a proximity to other central nervous structures make the management of these tumours both challenging and controversial especially with some clinical “apoplectic” presentations, making them indistinguishable from pituitary apoplexy. Effective treatment requires a multidisciplinary team to guide careful observation and judicious therapeutic intervention.
15.2 A. Akhaddar, MD Department of Neurosurgery, Avicenne Military Hospital, Marrakech 40000, Morocco
Central nervous system Computed tomography scan Magnetic resonance imaging
Literature Review
The literature used in the review was identified using the Medline database (PubMed, http//:www. ncbi.nlm.nih.gov/pubmed). The following English keywords were used: “lymphoma” and “hypothalamic”. In addition, only three references
M. Turgut et al. (eds.), Pituitary Apoplexy, DOI 10.1007/978-3-642-38508-7_15, © Springer-Verlag Berlin Heidelberg 2014
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A. Akhaddar
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(Lee et al. 2004; Akhaddar et al. 2009; Biasiotta et al. 2010) were found in English with the term “hypothalamic lymphoma”, but bibliographies of all relevant articles were scanned to identify additional references from 1988 until April 2012. Only first-source information was analysed; second- or third-line references to original contributions were not taken into consideration. Abstracts were not included. For the sake of group homogeneity, patients with both intrasellar and suprasellar lymphomas were excluded (Wolfe et al. 2009; Li et al. 2012; Rizek et al. 2012). Finally, fifteen references were found which reported 15 cases during the last 25 years (Table 15.1).
15.3
Epidemiology
Lymphoma may involve the CNS either as a primary tumour or after spreading from an established systemic lymphoma (secondary tumour). This occurs in 5–29 % of patients with systemic lymphoma during the natural history of the disease and is usually associated with progressive
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