Primary cerebral low-grade B-cell lymphoma, monoclonal immunoglobulin deposition disease, cerebral light chain deposition disease and "aggregoma": an update on classification and diagnosis.
BACKGROUND
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). 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.


METHODS
A 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.


CONCLUSIONS
LCCD 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.
Department of Neurosurgery, University Hospital Tuebingen, Hoppe-Seyler-Str. 3, 72076, Tuebingen, Baden-Wuerttemberg, Germany. marco.skardelly@med.uni-tuebingen.de
Marco
M
Skardelly
Georgios
G
Pantazis
Sotirios
S
Bisdas
Guenther C
GC
Feigl
Martin U
MU
Schuhmann
Marcos S
MS
Tatagiba
Rainer
R
Ritz
D000686:Amyloidosis; D001921:Brain / Q000378:metabolism / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000134:cerebrospinal fluid*; D007136:Immunoglobulins / Q000378:metabolism; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000378:metabolism* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008875:Middle Aged; D054219:Neoplasms, Plasma Cell / Q000150:complications
D007147:Immunoglobulin Light Chains; D007136:Immunoglobulins