Cerebral Amyloidoma Resulting from Central Nervous System Lymphoplasmacytic Lymphoma: A Case Report and Literature Review

Table 1

(a) Presentation, treatment, and outcome of primary CNS mature B-cell lymphomas presented as an amyloidomas published in literature

Reference	Age/sex	Symptoms at presentation	Duration of symptoms	site	Clinical diagnosis	Treatment	Follow-up	Diagnosis rendered
(1) Lehman et al. [1]	63F	Focal sensory seizure- trigeminal neuralgia and mild right sided hearing loss	3 years	Dural, frontal lobe	Meningioma	Surgical resection of the largest mass, radiation therapy	8 months- alive with disease, imaging showed no change in the size of the amyloid mass	Primary CNS Marginal zone lymphoma
(2) Pace et al. [2]	46F	Seizures	Acute onset	Left frontal lobe	Oligodendroglioma or metastasis	Surgical resection only	24 months- no evidence of disease	Primary CNS lymphoplasmacytic lymphoma
(3) Tu et al. [3]	49 M	Seizures	NR	Dural, left frontal	NR	Methotrexate, recurrence treated with fludarabine	7.6 years- Recurrence at 4 months, No evidence of disease after treatment with fludarabine	Primary CNS Marginal zone lymphoma
(4) Tu et al. [3]	62F	Ataxia	NR	Dural, left occipital	NR	Radiation	25 months- No evidence of disease	Primary CNS Marginal zone lymphoma
(5) Our case	87F	Left sided weakness	6 months	Right frontal lobe	Lymphoma, vasculitis, or sarcoidosis	Rituximab, status post 1st cycle	2 months- patient developed hemorrhagic stroke	Low grade B-cell lymphoma with plasmacytic differentiation

NR: not reported; ND: not done.

(b) Diagnostic testing performed on the primary CNS mature B-cell lymphomas presented as an amyloidomas published in literature

Reference	Imaging	Histopathology	Light chain restriction	Bone marrow	Systemic amyloidosis	Lab tests	Molecular testing
(1) Lehman et al. [1]	MRI- isointense on T1 and hyperintense on T2	Intracerebral and vascular amyloid deposition, CD 20+ B cells and plasmacytoid cell infiltrates, CD138+ plasma cells	Kappa	Normal	NR	SPEP and UPEP- normal,; CBC- normal	ND
(2) Pace et al. [2]	MRI- isointense on T1, hypointense on T2	Intracerebral and vascular amyloid deposition, CD 20+ B cells and plasmacytoid cell infiltrates, CD138+ plasma cells	Kappa	Normal	Ruled out by serum amyloid P component scan	SPEP- normal, normal serum free kappa, free lambda and kappa: lambda ratio	ND
(3) Tu et al. [3]	NR	Amyloid deposition, CD20 + B cell infiltrates	Kappa	NR	NR	NR	Trisomy 3 positive, Negative for MALT1 and IgH translocation
(4) Tu et al. [3]	NR	Amyloid deposition, cyclin D1-, CD20 + B-cell infiltrates	Kappa	NR	NR	NR	Trisomy 3 positive, Negative for MALT1 and IgH translocation
(5) Our case	MRI-T2 hyperintense	Intraparenchymal and perivascular amyloid deposition (AL subtype). CD20+, CD3-, CD5-, BCL1-, CD23- plasmacytoid lymphocytes and CD138+ plasma cells	Lambda	Normal	Ruled out by abdominal fat biopsy	Normal serum free kappa, free lambda and kappa: lambda ratio	MYD88 L265P mutation analysis was negative

NR: not reported; ND: not done.