Primary central nervous system post-transplant lymphoproliferative disorder
Presentation
Headache, expressive aphasia and left facial droop. Rule out cerebrovascular accident. History of renal transplant, polycystic kidney disease.
Patient Data
There are 3 intra-axial lesions, 2 in the left frontal lobe and 1 in the right frontal lobe, associated with significant surrounding vasogenic edema. The lesions are peripherally isodense to gray matter with a hypodense center. There is significant mass effect on the adjacent brain parenchyma manifested by effacement of sulci and partial effacement of the left lateral ventricle and rightward midline shift of 3 mm.
Multiple (6) intra-axial focal lesions in the subcortical white matter of the frontal lobes bilaterally and at the inferior aspect of the left parietal lobe, predominantly hypointense on T1, heterogeneous signal on T2, with avid ring enhancement after IV gadolinium infusion. The lesion at the left middle frontal gyrus shows punctate T1 hyperintensity, likely reflecting microhemorrhage. In the DWI sequence, there are areas of peripheral diffusion restriction.
Marked perilesional edema, more prominent in the left parietal lobe, with mild effacement of the left lateral ventricle and diffuse effacement of the left convexity sulci.
Pathology
- Post-transplant lymphoproliferative disorder, EBER-positive
- Histologically consistent with diffuse large B-cell lymphoma
Case Discussion
Post-transplant lymphoproliferative disorders are serious and life-threatening complications related to chronic immunosuppression in the setting of solid organ or hematopoietic cell transplantation. Most cases are associated with B-cell proliferation secondary to a chronic latent Epstein-Barr virus infection. Among the PTLDs, CNS involvement is rare, manifesting with nonspecific neurologic signs and symptoms. MR imaging is crucial for the diagnostic of this entity, demonstrating multifocal ring-enhancing lesions with ill-defined margins, involving the basal ganglia or with lobar distribution. The patients’ previous history of transplantation is relevant for differentiating PTLD from other similar-appearing entities, such as primary CNS lymphoma, glioblastoma, metastatic disease, or infections.
The case was contributed by Dr Raquel Del Carpio-O'Donovan, Professor of Radiology, Neurology, and Neurosurgery at McGill University.
Unable to process the form. Check for errors and try again.