Purpose Primary CNS lymphoma (PCNSL) is an aggressive lymphoma but clinically validated biologic markers that can predict natural history to tailor treatment according to risk are lacking. Several genetic changes including BCL6 rearrangements and deletion of 6q22, containing the putative tumor suppressor gene PTPRK, are potential risk predictors. Herein we determined the prevalence and survival impact of del(6)(q22) and BCL6, immunoglobulin heavy chain (IGH), and MYC gene rearrangements in a large PCNSL cohort treated in a single center. Patients and Methods Interphase fluorescence in situ hybridization was performed using two-color probes for BCL6, MYC, IGH-BCL6, and del(6)(q22) on thin sections of 75 paraffin-embedded samples from 75 HIV-negative, immunocompetent patients newly diagnosed with PCNSL. Survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and proportional hazards regression adjusting for age, deep structure involvement, and high-dose methotrexate (HDMTX) treatment. Results The prevalence of del(6)(q22) and BCL6, IGH, and MYC translocations was 45%,17%, 13%, and 3%, respectively. The presence of del(6)(q22) and/or a BCL6 translocation was associated with inferior overall survival (OS; P = .0097). The presence of either del(6)(q22) alone or a BCL6 translocation alone was also associated with inferior OS (P = .0087). Univariable results held after adjusting for age, deep structure involvement, and HDMTX. Conclusion Del (6)(q22) and BCL6 rearrangements are common in PCNSL and predict for decreased OS independent of deep structure involvement and HDMTX. Unlike systemic diffuse large B-cell lymphoma, del(6)(q22) is common and IGH translocations are infrequent and usually involve BCL6 rather than BCL2, suggesting a distinct pathogenesis.
PISCL mimics other causes of myelopathy. Spinal MRI demonstrating multifocal lesions, persistent gadolinium enhancement, and conus medullaris or cauda equina involvement is characteristic. Pathologic confirmation often requires CNS biopsy. Despite chemotherapy, morbidity and mortality is high.
The objective is to determine whether corticosteroid administration before biopsy prevents histopathological diagnosis of primary central nervous system lymphoma (PCNSL). A retrospective review was performed of immunocompetent PCNSL patients from 1985 to 2005. A total of 109 patients was identified. Sixty-eight (63.6%) patients received corticosteroids before diagnosis. Thirteen patients (of 109; 12%) had undergone repeat brain biopsy to confirm PCNSL. These included 8 (of 68) patients who had received corticosteroids (12%), and 5 (of 39) who had not (13%) (p = 1.0). The majority of PCNSL patients who received corticosteroids before diagnosis did not experience significant radiographic change or require second biopsy for diagnosis.
The strong association between higher SES and greater glioblastoma risk is unlikely to represent an ascertainment effect because glioblastoma is rapidly progressive and ultimately fatal. A number of previously proposed glioma risk factors may be correlated with SES, including atopy and allergy rates, cellular telephone use, and body morphometric measures. Further research is needed to define the mechanism of this association.
This small retrospective series suggests that adjuvant therapy for patients with high grade, Stage C esthesioneuroblastoma is of benefit following complete resection. Radiation therapy alone provides an improvement in time to relapse, which may be increased further with the addition of cisplatin and etoposide based chemotherapy.
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