Primary dural-based parafalcine diffuse large b-cell lymphoma mimicking meningioma
Amr El Mohamad1*, Ahmed Shaaban1, Kazim Mohammed1, Rayan M. Sibira2, Einas A. Alkuwari2,3, Ali Raza1,3
1Department of Neurosurgery, Hamad Medical Corporation, Doha, Qatar
2Department of Laboratory Medicine and Pathology, Hamad Medical Corporation, Doha, Qatar
3Weill Cornell Medical College, Doha, Qatar
*Amr El Mohamad, Department of Neurosurgery, Hamad Medical corporation, PO Box: 3050, Al Rayanstreet, Doha, Qatar
Figure 1:CT head with no contrast
Figure 2: MRI head, T1 and T2 sequence
Figure 3: MRI head with contrast
Figure 4: MRI Head, Diffusion-weighted imaging (DWI) and Apparent diffusion coefficient (ADC)
Figure 5: MRI head with contrast Post op day 1
Figure 6: First brain biopsy showing meningothelial hyperplasia.
The histopathology results of the first brain biopsy samples (Figs.6–7) obtained from the falx cerebri showed meningothelial hyperplasia with calcification and focal perivascular lymphocytic infiltrate composed of small and large, atypical lymphocytes. Immunohistochemical staining was performed; however, the area of interest disappeared. The pathology team recommended another fresh biopsy to have the final diagnosis and flowmetry studies. So, the patient underwent redo craniotomy using the same incision, and multiple biopsy samples were taken. The second fresh brain biopsy (Figs. 8–9) showed multiple brain fragments with predominant perivascular atypical lymphoid infiltrates. Most cells were medium to large with moderate cytoplasm, atypical irregular nuclei having vesicular chromatin, variably prominent nucleoli, and several mitoses, including atypical one. Necrotic areas were also seen. Immunohistochemistry of the second biopsy (Fig.10 A-D) showed that atypical perivascular cells were positive for CD45, CD20, CD79a, BCl2, BCl6, MUM1, OCT2, and C-MYC, and negative for CD10, CD21, TDT, ALK1, EBV-LMP1, CD3, and CD5; however, few reactive/residual lymphocytes were positive for these enzymes. Moreover, 80% of lymphoid cellular nuclei were positive for Ki67. These findings were consistent with diffuse large B-cell lymphoma, not otherwise specified.
Figure 7: First brain biopsy showing focal large atypical lymphocytes.
Figure 8: Second brain biopsy: Tumor cells showing perivascular spread.
Figure 9: Second brain biopsy: Tumor cells showing perivascular spread.
Figure 10: Second brain biopsy, Immunohistochemistry; Tumor cells are positive for E. BCL2, F. BCL6, G. MUM1, and H. C-MYC
Whole-body positron emission tomography (PET) showed intense fluorodeoxyglucose (FDG) uptake higher than that in the healthy brain cortex, without evidence of coexisting systemic disease. In addition to PET scan, contrast-enhanced chest, abdomen, pelvis CT did not show any other lesions in the body; furthermore, workup for viral markers and autoimmune conditions were all unremarkable, thus confirming the diagnosis of “primary dural-based diffuse large B-cell lymphoma,” distinguishing it from secondary CNSL. The patient was transferred to the Oncology Department and started on three cycles of the methotrexate, cytarabine, thiotepa, and rituximab (MATRIX) protocol, which is the current standard treatment regimen for PCNSLs . Three months after the diagnosis and after receiving two cycles of the MATRIX protocol, brain MRI with contrast enhancement (Fig. 11A, B) showed regression of the lesion, and PET scan showed complete metabolic resolution in terms of decreased FDG activity of the previously seen PCNSL without signs of lymphoma activity elsewhere. Subsequently, the patient received the third cycle of the MATRIX protocol without specific complications. Two weeks later, autologous stem cell transplantation (50 × 106/kg) was performed as part of the consolidation phase of treatment. Six weeks later, conditioning chemotherapy with carmustine–thiotepa was administered, followed by stem cell infusion (CD34 = 12 million/kg). The post-transplant course was complicated with mucositis, folliculitis, diarrhea, febrile neutropenia, and prolonged thrombocytopenia. Two months after transplantation, PET scan was repeated and showed complete metabolic resolution of initially seen PCNSL involvement. Currently, the patient is being followed by the hematology team; the patient is in good health and remission. The last outpatient follow-up was 8 months after the first surgery. The patient was seen by the vascular surgery (for permcath removal) and oncology teams. At this time, the patient was stable with complete remission; then, the patient was lost to follow-up. Another head MRI was performed and showed almost total regression of the lesion (Fig. 12A, B).
Figure 11(A-B): MRI head with contrast after second cycle chemotherapy A) T1 with contrast axial B)T2 Axial
Figure 12(A-B): Last MRI head after completion of treatment and after about 6 months from diagnosis A)T1 with contrast axial B)T2 Axial
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