Early CNS relapse in a good-risk primary mediastinal large B-cell lymphoma after combined chemo- and radio-therapy

Excerpt

CNS recurrence of non-Hodgkin lymphoma is fetal in most cases and tends to occur 5–12 months after the initial diagnosis [1]. Risk factors for CNS involvement include advanced stage, increased IPI, raised LDH, young age, >1 extranodal site, B symptoms, low albumin and retroperitoneal disease. A 19-year-old Japanese man presented to our hospital because of persistent cough on October 2007. A CT scan showed an anterior mediastinal tumor of 8 cm in diameter. LDH value and bone marrow examination were normal. After the total resection, the tumor was diagnosed as primary mediastinal large B-cell lymphoma (PMLBL). Involvement of right infraclavicular lymph nodes detected by ⁶⁷Ga-SPECT and ¹⁸FDG-PET/CT determined the stage as IIA. One cycle of MACOP-B regime plus bi-weekly rituximab therapy started on November 2007. After confirming complete response (CR) by CT and ⁶⁷Ga-SPECT, 30 Gy of involved field irradiation to the mediastinum and bilateral infraclavicular lymph nodes followed. On June 2008, 3 months after the chemo- and radio-therapy, ¹⁸FDG-PET/CT revealed diminishment of abnormal accumulation. On July 2008, only 4 months after the completion of treatment, severe headache and nausea started. A cranial MRI scan showed an enhancing mass in the left frontal lobe with the right midline shift (Fig. 1). Although systemic evaluation revealed no recurrence in the other sites, brain biopsy confirmed infiltration of the lymphoma cells. Southern blot analysis of JH region of immunoglobulin heavy chain gene showed identical rearrangement bands in mediastinal tumor and CNS metastatic mass. The result confirmed that the CNS tumor is truly derived from primary mediastinal mass. The CNS lesion was resistant to two cycles of IVAM therapy containing high-dose methotrexate (MTX) and the following whole brain irradiation of 50 Gy. Then, after obtaining informed consent from the patient, we introduced via Ommaya reservoir three times of 10–25 mg of rituximab together with 3 ml of the autologous serum twice a week. The intracranial rituximab therapy failed to reduce the tumor size at least until day 15, when the patient’s condition rapidly deteriorated and subsequently he died of bacterial pneumonia.

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