Introduction: Localized amyloidosis are isolated extracellular deposits of amyloid substance, called amyloidomas. It can rarely present with multiple myeloma, plasma cell dyscrasia, renal cell carcinoma or thyroid carcinoma1 2. We describe a case of cerebral amyloidoma in association to type B low grade non-Hodgkin lymphoma.
Methods: Case report and Review
Results: 70-year-old bilingual patient, with an unclassified brain lesion; presented itself with progressive speech impairment when using his non-native language. Physical examination revealed difficulty to naming things. A brain MRI showed a mass in the left posterior frontal lobe with high signal in T2-weighted sequences with a mild enhancing pattern. An elevation of Choline/NAA ratio was detected. (Figure 1). An awake craniotomy was performed. Afterward he exhibited non fluent aphasia, that recovered. Pathology showed findings of amyloidoma and lymphoma (Figure 2).
DISCUSSION
Primary amyloidomas of the brain parenchyma are rare.3 Its association with type B low grade non-Hodgkin Lymphoma is not yet understood given there are only two cases reported. According to literature, imaging characteristics of the lesion are neither for amyloidoma nor lymphoma but both of them. Given the spectroscopy surgery was performed, considering it to be a glioma.
It is possible that the amyloidoma might surge as a response to the tumor growth and it’s changes in the pH, enzymes misbalance and cytokines proliferation leading to polymerization and protein misfolding.
Considering that the clinical presentation or the imaging are not defining the histological study is of great importance. It was suggested that these lesions where not surgical given that the amyloidoma is a benign entity and that some lesions had shown growth after biopsied but more cases of amyloidomas accompanied by malignant neoplasms are being reported giving new significance to surgical treatment4 5.
Conclusions: More information about this pathology is needed, in order to stablish an entity´s profile to facilitate the diagnosis.
Patient Care: Make a contribution to the literature to increase available data about this rare condition.
Learning Objectives: 1. To study the data in the literature about characteristics in common of this pathology expressed in this patient.
2. To report a group of characteristics of this entity in this patient.
References: 1. Navarro San Francisco C, Luepke Estefan E, Camacho Siles J. Amiloidoma espinal como primera manifestación de mieloma múltiple. Med Clínica. :278-9.
2. Tabatabai G, Baehring J, Hochberg FH. Primary Amyloidoma of the Brain Parenchyma. Arch Neurol. 1 de marzo de 2005;62(3):477-80.
3. Landau D, Avgeropoulos N, Ma J. Cerebral amyloidoma mimicking intracranial tumor: a case report. J Med Case Reports. 2010;4:308.
4. Skardelly M, Pantazis G, Bisdas S, Feigl GC, Schuhmann MU, Tatagiba MS, et al. Primary cerebral low-grade B-cell lymphoma, monoclonal immunoglobulin deposition disease, cerebral light chain deposition disease and «aggregoma»: an update on classification and diagnosis. BMC Neurol. 15 de agosto de 2013;13:107.
5. Lehman NL, Horoupian DS, Warnke RA, Sundram UN, Peterson K, Harsh GR. Dural marginal zone lymphoma with massive amyloid deposition: rare low-grade primary central nervous system B-cell lymphoma. Case report. J Neurosurg. febrero de 2002;96(2):368-72.