Findings at presentation in primary CNS diffuse large B-cell lymphoma of the brain: A comparison of immunocompetent and immunodeficient patients
TL;DR: MRI and CT are able to define imaging characteristics of PCNSL, promoting a quick diagnosis and there are no significant differences between immunocompetent and immunodeficient patients for MR and CT features.
Abstract: Background/Objectives: Primary central nervous system lymphoma (PCNSL) is a rare tumour with poor prognosis. Due to the increased number of patients with PCNSL over the past two decades our purpose are to describe magnetic resonance imaging (MRI) and Computed Tomography (CT) findings in (PCNSL) of the brain, and to study the differences between immunocompetent and immunodeficient patients with PCNSL. Methods : A retrospective, descriptive study was performed with 59 patients diagnosed of PCNSL in two hospitals from 1997 to 2010. Immunocompetent (n=38) and immunodeficient (n=21) patients were compared and differences between both groups were analyzed. Patients were evaluated according to sex, age, median time from clinical symptoms presentation to pathologic diagnosis, clinical symptoms, location, number of lesions, size, MRI and CT characteristics. Significance was defined as p < 0.05. Results : MRI findings: 50% of lesions in immunocompetent and 52.4% in immunodeficient group were heterogeneous, 89.5% of lesions in immunocompetent and 85,7% in immunodeficient were hypo-isointense on T1WI; 63.2% of lesions in immunocompetent and 76.2% in immunodeficient group were hyperintense on T2WI. CT images: 48.39% of lesions in immunocompetent and 20% in immunodeficient group were hyperdense. Statistically significant differences between immunocompetent and immunodeficient patients were found when evaluating the age ( p < 0.000) and median time from clinical symptoms presentation to pathologic diagnosis ( p < 0.008). Conclusions: MRI and CT are able to define imaging characteristics of PCNSL, promoting a quick diagnosis. There are no significant differences between immunocompetent and immunodeficient patients for MR and CT features.
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TL;DR: Primary central nervous system lymphoma (PCNSL) is a rare aggressive high-grade type of extranodal lymphoma that can mimic other brain disorders such as encephalitis, demyelination, and stroke.
Abstract: Primary central nervous system lymphoma (PCNSL) is a rare aggressive high-grade type of extranodal lymphoma. PCNSL can have a variable imaging appearance and can mimic other brain disorders such as encephalitis, demyelination, and stroke. In addition to PCNSL, the CNS can be secondarily involved by systemic lymphoma. Computed tomography and conventional MRI are the initial imaging modalities to evaluate these lesions. Recently, however, advanced MRI techniques are more often used in an effort to narrow the differential diagnosis and potentially inform diagnostic and therapeutic decisions.
36 citations
TL;DR: This case demonstrates the rapidity with which a PCNSL lesion can develop, and the evolution of the imaging characteristics prior to definitive diagnosis and treatment.
Abstract: Background: Primary central nervous system lymphoma (PCNSL) is a rare central nervous system tumor, especially in the pediatric population. There are fewer than 20 described cases of pediatric primary central nervous system anaplastic large cell lymphoma. The child described in our case report demonstrated a dramatic evolution of this tumor in the first 4 weeks on serial imaging. Methods: Serial MRI imaging was performed followed by biopsy and chemotherapy. Results: Initial imaging revealed a T2 hyperintense lesion in the frontal lobe with abnormally enhancing sulci and minimal surrounding edema and diffusion restriction. Serial imaging revealed progressive increase in the degree of gadolinium enhancement, and the hyperintense T2 edema progressed markedly to exert mass effect. The lesion itself grew marginally. Biopsy revealed an anaplastic large cell lymphoma, only described in 14 previous pediatric patient case reports. The patient was successfully treated with chemotherapy and autologous stem cell transplant. Conclusions: Our case demonstrates the rapidity with which a PCNSL lesion can develop, and the evolution of the imaging characteristics prior to definitive diagnosis and treatment. Serial imaging by MRI may help differentiate the behavior of a PCNSL from other imitating lesions.
9 citations
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TL;DR: White matter periventricular contrast-enhancing single or multiple focal lesions were typical of non-AIDS PCNSL, and no or disseminated lesions heightened the risk of delayed or postmortem diagnosis.
Abstract: BACKGROUND AND PURPOSE: Studying imaging findings of non–acquired immunodeficiency syndrome (AIDS) primary central nervous system lymphoma (PCNSL), we hypothesized that the imaging presentation has changed with the increasing incidence of PCNSL and is related to clinical factors (eg, time to diagnosis and the patient9s being diagnosed alive or at postmortem examination). MATERIALS AND METHODS: Chart and histologic reviews of patients recorded as having PCNSL during 1989–2003 in the Norwegian Cancer Registry identified 98 patients with non-AIDS PCNSL; 75 had available imaging. CT and MR images from the first diagnostic work-up after onset of symptoms but before histologic diagnosis were reviewed. RESULTS: CT and/or MR imaging in the 75 patients revealed no lesion in 10 (13%), a single focal lesion in 34 (45%), multiple focal lesions in 26 (35%), and disseminated lesions in 5 (7%) patients. All together, we identified 103 focal lesions (single/multiple): 63% in white matter, 56% abutting the ventricular surface, and 43% in the frontal lobes); 100% (102/102 lesions evaluated with contrast) showed contrast enhancement. The median time from imaging to diagnosis for patients with no, single, multiple, or disseminated lesions was 32, 3, 5, and 3 weeks, respectively (P = .01). Patients with no or disseminated lesions were more often diagnosed at postmortem examination (P = .06). Imaging findings were practically unchanged during the consecutive 5-year periods. CONCLUSIONS: White matter periventricular contrast-enhancing single or multiple focal lesions were typical of non-AIDS PCNSL. No or disseminated lesions heightened the risk of delayed or postmortem diagnosis. Although the incidence of non-AIDS PCNSL has increased, its presentation at imaging remains unchanged.
120 citations
"Findings at presentation in primary..." refers background in this paper
...Diffusion within the tumor is considered a surrogate marker of tumor cellularity, and water diffusion is often restricted in PLCNSLs because they are highly cellular tumors ([11])....
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TL;DR: Signs and symptoms of increased intracranial pressure and personality change are most frequent, followed in frequency by ataxia and hemiparesis, and the definitive diagnosis of PCNSL requires biopsy.
Abstract: Immunocompetent patients with primary central nervous system lymphoma (PCNSL) present with a median age of 55 years, immunosuppressed patients with a median age of 40 years. They show a broad range of signs and symptoms. Symptoms of increased intracranial pressure and personality change are most frequent, followed in frequency by ataxia and hemiparesis. The median time from onset of symptoms to diagnosis is 3–5 months in immunocompetent patients and 2 months in immunodeficient patients. The time to diagnosis can be considerably longer in patients with slowly developing personality change or fluctuating symptoms due to spontaneous or steroid-induced remission of so-called sentinel lesions. Native CT scans show iso- or hyperdense lesions with homogenous contrast enhancement. T1-weighted MRI scans show hypointense and T2-weighted scans hyperintense lesions. The definitive diagnosis of PCNSL requires biopsy. In some cases, however, the definitive diagnosis may exclusively be made by the demonstration of malignant B-lymphocytes in the cerebrospinal fluid.
105 citations
Journal Article•
TL;DR: The findings of MR scans are sufficiently suggestive of the diagnosis of primary CNS lymphoma that a needle biopsy be performed based on these findings and appropriate therapy can then be instituted.
Abstract: PURPOSE To describe the MR findings of primary CNS lymphoma. METHODS MR scans of 20 patients with histologically proved primary CNS lymphoma were reviewed. We evaluated the size, multiplicity, signal intensities, and enhancement characteristics of the lesions. We divided the lesions into an enhancing area referred to as Zone 1 and abnormal signal surrounding this, referred to as Zone 2. RESULTS Primary CNS lymphoma presented as solitary enhancing lesions in 40% of the patients and multiple lesions in 40%. Thirty-three separate lesions were visible: 58% abutted the ventricular system, 76% showed a homogenous enhancement pattern, and 79% showed marked enhancement. In 64% of the lesions, Zone 1 and Zone 2 showed different signal intensities on T1-weighted images. CONCLUSIONS Primary CNS lymphoma usually presents as solitary or multiple dense homogenous enhancing lesions that abut an ependymal surface. These lesions can be divided into an enhancing area and an area of surrounding abnormal signal. These two areas often have different signal intensities on unenhanced T2-weighted images. These findings are sufficiently suggestive of the diagnosis of primary CNS lymphoma that a needle biopsy be performed based on these findings and appropriate therapy can then be instituted.
103 citations
TL;DR: The findings suggest that the previously described spectrum imaging characteristics of PCNSL has widened and neuroradiologists should be aware of the variable appearance in patients with AIDS.
Abstract: Diagnosis of primary central nervous system lymphoma (PCNSL) in patients with AIDS based on radiological findings is still a challenging problem. Our purpose was to review the CT and MRI findings in PCNSL in our patients with AIDS and compare them with those reported in the literature. CT and MRI of 28 patients with AIDS and pathologically confirmed PCNSL were analysed retrospectively for the number of lesions, their site, size, density, signal intensity, contrast enhancement, oedema and mass effect. We found 82 lesions. On CT 45 lesions were found in 22 patients, whereas MRI revealed 66 in 20 patients. The lymphoma was solitary in 20 patients (29 %) and multiple in 20 (71%). Spontaneous haemorrhage was seen in 7 patients. Contrast-enhanced MRI showed no enhancement in 27.3 % (18/66) of the lesions. In one patient diffuse signal abnormalities in the white matter were seen on T2-weighted images. Our findings suggest that the previously described spectrum imaging characteristics of PCNSL has widened. Neuroradiologists should be aware of the variable appearance in patients with AIDS. Spontaneous haemorrhage, a non-enhancing lesion, or diffuse white matter changes do not exclude lymphoma in an immunocompromised patient.
100 citations
TL;DR: The records and neuro-imaging studies of 8 cases of posttransplant primary CNS lymphoma diagnosed at Mayo Clinic Rochester between 1970 and 1998 were reviewed retrospectively, and PT-PCNSL presented nonspecifically and progressed rapidly and despite multimodal therapy, survival was poor.
Abstract: The records and neuro-imaging studies of 8 cases of posttransplant primary CNS lymphoma (PT-PCNSL) diagnosed at Mayo Clinic Rochester between 1970 and 1998 were reviewed retrospectively. All patients received organ transplantation. Patients who had hematologic transplantation were not included in the analysis. The median and mean age of the 4 men and 4 women was 45 years (range, 34 to 50 years). The median duration of symptoms before diagnosis was 36 days (range, 5 to 98 days). At diagnosis, the neurologic examination was focally abnormal in 6 of 8 patients. Compared with the initial computed tomographic study, MRI showed 25 additional brain lesions. Only 43.7% of lesions enhanced with contrast agent; of those that did, all but one were heterogeneous. Ependymal contact occurred in 5 patients. MRI lesion burden increased proportionally to the interval between scans. Diagnostic tissue was obtained by stereotactic biopsy from 6 patients and by open biopsy from 2. Hemorrhage occurred in the biopsy area in 4 patients who had stereotactic biopsy and 2 died (all had normal coagulation studies). Slides available for review (7 patients) showed that the tumors were of CD20-positive lineage and were positive for Epstein-Barr virus, using in situ hybridization. Six patients died. Median survival for the cohort was 13 weeks. PT-PCNSL has clinical and imaging features distinct from typical PCNSL. In our series, (1) PT-PCNSL presented nonspecie cally and progressed rapidly, (2) stereotactic brain biopsy had signie cant morbidity, and (3) despite multimodal therapy, survival was poor. NeuroOncology 2, 229‐ 238, 2000 (Posted to Neuro-Oncology
85 citations
"Findings at presentation in primary..." refers background in this paper
...This percentage increases for post-transplant immunodeficient patients ([26])....
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