Cerebral amyloidoma is characterized by B-cell clonality and a stable clinical course.
Amyloidomas are rare amyloid-containing lesions, which may also occur in the central nervous system. Etiology, pathogenesis and clinical course are poorly understood. To gain more insight into the biology of cerebral amyloidoma, they aimed to characterize its histopathological, molecular and clinical features in a retrospective series of seven patients. FFPE tissue specimens were examined using immunohistochemistry, chromogenic in situ hybridization (CISH) for light chains kappa and lambda as well as an IgH gene clonality analysis. Follow-up information was gathered by reviewing patient records and imaging results. Median age of the three males and four females was 50 years (range: 35-53 years). All cerebral amyloidomas were located supratentorially and were classified as lambda light chain amyloidosis (AL-λ; n = 6) and kappa light chain amyloidosis (AL-κ; n = 1) on immunohistochemistry and CISH. B-cell clonality was confirmed by IgH gene clonality assay in all cases examined. After a median follow-up of 21 months, all patients were alive and showed stable disease. No progression to systemic disease was observed. In conclusion, their data suggest that cerebral amyloidoma is a local disease characterized by B-cell clonality and associated with a stable clinical course.
Institute of Neuropathology, University Hospital Münster, Münster, Germany.
Katharina
K
Heß
Amyloidosis Center, Heidelberg University Hospital, Heidelberg, Germany.|Department of Neurology, Heidelberg University Hospital, Heidelberg, Germany.
Jan
J
Purrucker
Amyloidosis Center, Heidelberg University Hospital, Heidelberg, Germany.
Ute
U
Hegenbart
Department of Neurosurgery, University Hospital Münster, Münster, Germany.
Benjamin
B
Brokinkel
Clinic of Cardiovascular Surgery, Vascular Inflammatory Research, Christian-Albrechts University Kiel, University Hospital Schleswig-Holstein Campus Kiel, Kiel, Germany.
Rouven
R
Berndt
Institute of Neuropathology, Faculty of Medicine, University of Duisburg-Essen, Essen, Germany.
Kathy
K
Keyvani
Department of Neuropathology, Institute of Pathology, University of Würzburg, Würzburg, Germany.
Camelia M
CM
Monoranu
Department of Neurosurgery, University Hospital Würzburg, Würzburg, Germany.
Mario
M
Löhr
Institute of Neuropathology, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany.
Guido
G
Reifenberger
Department of Neurosurgery, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany.
Christopher
C
Munoz-Bendix
Institute of Pathology, Hospital Schwarzwald-Baar, Villingen-Schwenningen, Germany.
Jörg
J
Kalla
Clinic of Cardiovascular Surgery, Vascular Inflammatory Research, Christian-Albrechts University Kiel, University Hospital Schleswig-Holstein Campus Kiel, Kiel, Germany.
Justus
J
Groß
Department of Neurosurgery, Clemenshospital, Münster, Germany.
Uta
U
Schick
Amyloidosis Center, Heidelberg University Hospital, Heidelberg, Germany.|Department of Neuroradiology, Heidelberg University Hospital, Heidelberg, Germany.
Jennifer
J
Kollmer
Institute of Pathology, Hematopathology Section, Christian-Albrechts University Kiel, University Hospital Schleswig-Holstein Campus Kiel, Kiel, Germany.
Wolfram
W
Klapper
Department of Pathology, Christian-Albrechts University Kiel University Hospital Schleswig-Holstein Campus Kiel, Kiel, Germany.
Christoph
C
Röcken
Institute of Neuropathology, University Hospital Münster, Münster, Germany.
Martin
0000-0003-2707-8484
M
Hasselblatt
Institute of Neuropathology, University Hospital Münster, Münster, Germany.
Werner
W
Paulus
D000328:Adult; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D001402:B-Lymphocytes / Q000473:pathology*; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D054022:Cerebrum / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012333:RNA, Messenger / Q000378:metabolism
D000682:Amyloid; D012333:RNA, Messenger
B-cell; amyloidoma; central nervous system; light chain amyloidosis; neoplasm