spontaneous regression of an intracerebral lymphoma (ghost tumor) in a liver-engrafted patient

Abstract

Posttransplantation lymphoproliferative disorder involving the central nervous system is a rare and serious complication associated with solid organ transplantation. We report a liver transplant recipient who noticed unbalance, dizziness, and headache 30 months after transplantation. Magnetic resonance imaging (MRI) showed a space-occupying lesion in the corpus callosum and adjacent parenchyma of the left hemisphere. In the following month, the neurological symptoms and the MRI findings regressed without any treatment. Four months later the patient developed a left-sided hemiparesis. MRI now revealed a considerable increase of the known lesion and new lesions in other locations. Stereotactic biopsy showed a B-cell non-Hodgkin lymphoma of high malignancy. A spontaneous regression of cerebral lymphoma is possible, even in immunosuppressed patients. Hence, this diagnosis must not be dismissed if there is spontaneous regression of a lesion in the MRI or an amelioration of the clinical symptoms. Owing to the high mortality rate associated with this disease, prompt pathologic diagnosis is required to initiate appropriate therapy.

Case Details

Disease Location

Brain (cns)

Personal Characteristics

53 -year-old male liver transplantion 30 months prior to presentation due to hepatitis b cirrhosis and hepatocellular carcinoma

Clinical Characteristics

Presented with transient unbalance, dizziness, and headache MRI of the brain showed a space-occupying lesion with considerable contrast enhancement in the cc and adjacent parenchyma bilaterally accentuated to the left hemisphere on admission, symptoms were on the decline and neurological exam was unremarkable. No therapy was initiated. Follow-up MRI 16 laters was scheduled. Magnetic resonance spectroscopy was performed and found compatibility with lymphoma and acute demyelinating inflammation or a primary cns tumor repeated csf exams showed a lymphocytic pleocytosis with moderately elevated cell count, there was no confirmation of an infectious agent or lymphocytic b-cells molecular pathologic analysis for clonality of the b-cells didn't succeed due to low number of cells in the csf. Serum and csf were tested for borrelia, lues, ebv, and cytomegaly virus and came up negative. Biopsy of the lesion was declined because of the volume of regression, so there was no diagnosis confirmation or start to therapy. Fluorodeoxy-PET-CT revealed no other lesions no other therapies were initiated, but previous medication was maintained new involvement of deep white matter of the right hemisphere was discovered one months later, two months later the patient developed a left-sided hemiparesis over a few days. Cerebral MRI revealed considerable increase of the known lesion and new lesions in other locations histopathologic assessment of a stereotactic biopsy revealed tumor-like infiltration of the cerebral white matter by large blastic lymphoid cells with numerous mitotic and apoptotic cells immunohistochemical analysis confirmed large b-cell type with CD20 and bc12 positivity and a proliferation rate of >90%. Ebv was negative. Burkitt lymphoma was excluded the situation was assessed as palliative and no chemo was administered, there was cerebral radiation therapy and immunosuppressive therapy was reduced after tumor reduction, the patient continued to suffer from epileptic convulsions and hemiparesis on the left side, he died approx. 12 months after showing the first symptoms

Remission Characteristics

Initial follow-up MRI found the lesion regressed compared to initial findings one months later, patient was symptom free, MRI showed a regression of the contrast enhancement but also new involvement of the deep white matter of the right hemisphere with radiation therapy and immunosuppressant reduction, remarkable reduction of the tumor was achieved

Treatment & Mechanisms

Proposed Remission Mechanisms

Infarction and necrosis, although unlikely the resolution of an infection or non-neoplastic process induced a resolution of the lesion

Clinical Treatment

Tacrolimus was used for long-term immunosuppression and was eventually reduced cerebral radiation therapy

Non-Clinical Treatment

None reported