PMID- 23947787
OWN - NLM
STAT- MEDLINE
DCOM- 20131028
LR  - 20211021
IS  - 1471-2377 (Electronic)
IS  - 1471-2377 (Linking)
VI  - 13
DP  - 2013 Aug 15
TI  - Primary cerebral low-grade B-cell lymphoma, monoclonal immunoglobulin deposition 
      disease, cerebral light chain deposition disease and "aggregoma": an update on 
      classification and diagnosis.
PG  - 107
LID - 10.1186/1471-2377-13-107 [doi]
AB  - 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. CASE PRESENTATION: A 61-year-old woman presented with a 
      3(1/2)-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. 
      CONCLUSION: 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.
FAU - Skardelly, Marco
AU  - Skardelly M
AD  - Department of Neurosurgery, University Hospital Tuebingen, Hoppe-Seyler-Str. 3, 
      72076, Tuebingen, Baden-Wuerttemberg, Germany. 
      marco.skardelly@med.uni-tuebingen.de
FAU - Pantazis, Georgios
AU  - Pantazis G
FAU - Bisdas, Sotirios
AU  - Bisdas S
FAU - Feigl, Guenther C
AU  - Feigl GC
FAU - Schuhmann, Martin U
AU  - Schuhmann MU
FAU - Tatagiba, Marcos S
AU  - Tatagiba MS
FAU - Ritz, Rainer
AU  - Ritz R
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20130815
PL  - England
TA  - BMC Neurol
JT  - BMC neurology
JID - 100968555
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Immunoglobulins)
SB  - IM
MH  - Amyloidosis
MH  - Brain/metabolism/pathology
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Immunoglobulin Light Chains/*cerebrospinal fluid
MH  - Immunoglobulins/metabolism
MH  - Lymphoma, B-Cell/*complications/*metabolism/surgery
MH  - Magnetic Resonance Imaging
MH  - Magnetic Resonance Spectroscopy
MH  - Middle Aged
MH  - Neoplasms, Plasma Cell/complications
PMC - PMC3751626
EDAT- 2013/08/21 06:00
MHDA- 2013/10/29 06:00
PMCR- 2013/08/15
CRDT- 2013/08/17 06:00
PHST- 2012/09/21 00:00 [received]
PHST- 2013/08/06 00:00 [accepted]
PHST- 2013/08/17 06:00 [entrez]
PHST- 2013/08/21 06:00 [pubmed]
PHST- 2013/10/29 06:00 [medline]
PHST- 2013/08/15 00:00 [pmc-release]
AID - 1471-2377-13-107 [pii]
AID - 10.1186/1471-2377-13-107 [doi]
PST - epublish
SO  - BMC Neurol. 2013 Aug 15;13:107. doi: 10.1186/1471-2377-13-107.