Abstract
BackgroundThis work aims to add evidence and provide an update on the classification and diagnosis of monoclonal immunoglobulin deposition disease (MIDD) and primary central nervous system low-grade lymphomas. MIDD is characterized by the deposition of light and heavy chain proteins. Depending on the spatial arrangement of the secreted proteins, light chain-derived amyloidosis (AL) can be distinguished from non-amyloid light chain deposition disease (LCDD). We present a case of an extremely rare tumoral presentation of LCDD (aggregoma) and review the 3 previously published LCDD cases and discuss their presentation with respect to AL.Case presentationA 61-year-old woman presented with a 3½-year history of neurologic symptoms due to a progressive white matter lesion of the left subcortical parieto-insular lobe and basal ganglia. 2 former stereotactic biopsies conducted at different hospitals revealed no evidence of malignancy or inflammation; thus, no therapy had been initiated. After performing physiological and functional magnetic resonance imaging (MRI), the tumor was removed under intraoperative monitoring at our department. Histological analysis revealed large amorphous deposits and small islands of lymphoid cells.ConclusionLCCD is a very rare and obscure manifestation of primary central nervous system low-grade lymphomas that can be easily misdiagnosed by stereotactic biopsy sampling. If stereotactic biopsy does not reveal a definite result, a “wait-and-see” strategy can delay possible therapy for this disease. The impact of surgical removal, radiotherapy and chemotherapy in LCDD obviously remains controversial because of the low number of relevant cases.
Highlights
This work aims to add evidence and provide an update on the classification and diagnosis of monoclonal immunoglobulin deposition disease (MIDD) and primary central nervous system low-grade lymphomas
All forms of MIDD can be ascribed to monoclonal expansion of an immunoglobulin (Ig) light and/or heavy chain producing B-cells [7]. 2 subgroups of MIDD can be differentiated histologically based on the different spatial arrangement of the secreted proteins
Subsequent reports confirmed the existence of systemic heavy chain deposition disease (HCDD) as well as both light and heavy chain deposition disease (LHCDD) [10]
Summary
This work aims to add evidence and provide an update on the classification and diagnosis of monoclonal immunoglobulin deposition disease (MIDD) and primary central nervous system low-grade lymphomas. MIDD is characterized by the deposition of light and heavy chain proteins. Depending on the spatial arrangement of the secreted proteins, light chain-derived amyloidosis (AL) can be distinguished from non-amyloid light chain deposition disease (LCDD). A few low-grade PCNSL are associated with the deposition of monoclonal light and heavy chain immunoglobulins (Ig). Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the deposition of monotypic light and/or heavy chain proteins in various tissues and organs. All forms of MIDD can be ascribed to monoclonal expansion of an immunoglobulin (Ig) light and/or heavy chain producing B-cells [7]. Subsequent reports confirmed the existence of systemic heavy chain deposition disease (HCDD) as well as both light and heavy chain deposition disease (LHCDD) [10]
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