Multicentric Castleman’s Disease of the Central Nervous System

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Castleman’s disease (CD) is defined as a non-neoplastic reactive proliferation of lymphoid tissue, also known as angiofollicular lymph node hyperplasia and giant lymph node hyperplasia. Initial identification of this eponymous disease dates back to 1956 [1]. Two distinct histological classifications are described extensively in the literature: hyaline-vascular and plasma cell types [2]. However, remarkable advances have been made in the characterization of CD, including the most recent elaboration of human herpes virus-8 (HHV-8)-related CD [3]. Hyaline-vascular CD is more common than the plasma cell type and usually presents as localized or unicentric disease involving one or several lymph node aggregates. Perhaps the most well recognized manifestation of localized CD presents with avidly enhancing mediastinal adenopathy. Despite what the histology implies, extranodal CD has been documented in multiple locations. In rare instances, extranodal disease may affect the central nervous system (CNS) and bears a remarkably similar appearance to meningioma, both clinically and using various imaging methods (e.g. magnetic resonance imaging and arteriography, [4–10]). In this article a follow-up discourse of a case of intracranial CD is presented which will be a unique addition to the literature [11]. This is the first case of intracranial CD which has been evaluated using an advanced imaging technique, dynamic susceptibility contrast (DSC) perfusion magnetic resonance imaging (MRI). The goals are two-fold: first to chronicle the initial surgical resection of localized CD of the CNS and to remark on what was strongly felt to represented recurrent perineural spread of the disease in a separate, noncontiguous location; second it is hoped that the role that perfusion MRI may play in characterizing intracranial CD will be illuminated, as the inherent limitations of conventional imaging prevent adequate differentiation between CD of the CNS and more commonly encountered extra-axial lesions (e.g. meningioma).