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Journal ArticleDOI

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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Journal ArticleDOI
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

Journal ArticleDOI
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

References
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Journal ArticleDOI
TL;DR: The data show that PCNSL has a variable enhancement pattern on MR images, similar to other central nervous system lymphoma cases, which typically have larger sized tumors with mild or moderate enhancement.
Abstract: Objective: To record the MR imaging features of primary central nervous system lymphoma (PCNSL) and compare these features in monofocal and multifocal disease. Materials and Methods: Twenty-one cases of monofocal disease were compared to five cases of multifocal disease. All patients were examined by nonenhanced and contrast-enhanced MRI. Tumor location, tumor size, signal intensity, enhancement characteristics, age distribution, peritumoral edema, cystic changes, and the presence of calcifications were assessed. The MRI features were compared between the monofocal and multifocal disease cases. Results: The 26 cases, including both the monofocal and multifocal cases, exhibited 37 lesions. Contrast-enhanced images showed variable enhancement patterns: homogeneous enhancement (33 lesions), ring-like enhancement (2), and ‘open-ring-like’ enhancement (2). The ‘notch sign’ was noted in four of 33 homogeneously enhancing lesions. One case of hemorrhage and three cases of cystic formation were observed. Intra-tumoral calcification was not found. The frontal lobe, the corpus callosum and the basal ganglia were commonly affected in both the monofocal and multifocal groups. Tumor size differed significantly between the two groups (t = 3.129, p 0.05) and the signal characteristics (p > 0.05) between the two groups. Conclusion: Our data show that PCNSL has a variable enhancement pattern on MR images. We first reported two lesions with an ‘open-ring’ enhancement as well as four cases with a ‘notch sign’. Monofocal PCNSL cases typically have larger sized tumors with mild or moderate enhancement.

79 citations


"Findings at presentation in primary..." refers background in this paper

  • ...That it is rare to find serious edema is due to the infiltrative nature of the tumor ([6]), which also engenders a slight mass effect....

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  • ...The peak incidence is in the sixth decade [5] and primarily affects men ([6-8])....

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Journal ArticleDOI
TL;DR: Brain biopsy sampling remains the gold standard for PCNSL diagnosis in all patients, although the possibility of establishing routine, minimally invasive diagnostic procedures in which Epstein-Barr virus polymerase chain reaction (PCR) analysis of the CSF and nuclear imaging are used is currently under investigation in the population of patients with AIDS.
Abstract: Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that affects the brain, spinal cord, leptomeninges, and eyes. The clinical presentation and neuroimaging appearance of PCNSL differ in immunocompetent patients and in those with acquired immunodeficiency syndrome (AIDS). A magnetic resonance (MR) image of the brain in immunocompetent patients with PCNSL typically demonstrates one or more homogeneously enhancing lesions located in the periventricular white matter, characteristically spanning the corpus callosum. In patients with AIDS, multiple ring-enhancing lesions are more common. After neuroimages raising the suspicion of PCNSL are obtained, a definitive diagnosis should be established in both immunocompetent and AIDS patients by performing pathological analysis of cerebrospinal fluid (CSF), vitreous fluid, or a biopsy specimen. Brain biopsy sampling remains the gold standard for PCNSL diagnosis in all patients, although the possibility of establishing routine, minimally invasive diagnostic procedures in which Epstein-Barr virus polymerase chain reaction (PCR) analysis of the CSF and nuclear imaging are used is currently under investigation in the population of patients with AIDS. At the time of diagnosis, the patient should undergo further evaluation, which should include a physical examination, ophthalmic evaluation with a slit-lamp examination, serum lactate dehydrogenase levels, human immunodeficiency virus testing, computed tomography scans of the chest/abdomen/pelvis, bone marrow biopsy sampling, contrast-enhanced brain MR imaging, and lumbar puncture (LP). Testicular ultrasonography studies should be considered in men. In patients who cannot undergo LP or in those with evidence of spinal cord dysfunction, contrast-enhanced MR imaging of the entire spine should be considered.

74 citations

Journal ArticleDOI
TL;DR: There is a considerable delay in the diagnosis of PCNSL and strategies for earlier diagnosis are thus needed, and Physicians should consider early neuroimaging in patients with personality changes or visual disturbance, early renewed imaging in Patients with persistent neurological symptoms but no tumour on initial imaging, and early/repeated biopsy of focal brain lesions in both AIDS patients and non-AIDS patients.
Abstract: This study investigates delay in diagnosing primary central nervous system lymphoma (PCNSL), which has a variable clinical and radiological presentation. Early diagnosis and treatment may improve survival and cause less sequela in PCNSL. Medical records of all new cases of PCNSL morphologically verified while alive or by autopsy in Norway in 1989-1998 were reviewed (n = 74). The time from initial symptom to final morphological diagnosis of PCNSL had a median (mean, range) of 70 (106, 22-330) days in 16 AIDS patients and 75 (157, 8-1285) days in 58 non-AIDS patients. Among non-AIDS patients, the time to diagnosis was longer in patients with no tumour in the first neuroimaging report after initial symptom (p = 0.001). Median (mean, range) time from initial symptom to neuroimaging was 14 (25, 1-60) days in AIDS patients and 21 (88, 1-1095) days in non-AIDS patients. In the non-AIDS group, those presenting with personality change or visual disturbance had more delayed imaging than the others. The time from first neuroimaging examination to final diagnosis in non-AIDS patients had a median (mean, range) of 28 (69, 1-845) days, and was longer when no tumour was indicated in the imaging report (p = 0.005) and if first biopsy did not confirm the diagnosis (p = 0.02). All AIDS patients had their diagnosis of PCNSL first established by autopsy. The time from first neuroimaging to autopsy had a median (mean, range) of 48 (81, 10-270) days. There is a considerable delay in the diagnosis of PCNSL and strategies for earlier diagnosis are thus needed. Physicians should consider early neuroimaging in patients with personality changes or visual disturbance, early renewed imaging in patients with persistent neurological symptoms but no tumour on initial imaging, and early/repeated biopsy of focal brain lesions in both AIDS patients and non-AIDS patients.

60 citations

Journal ArticleDOI
Philipp Kiewe1, Lars Fischer1, Peter Martus1, Eckhard Thiel1, Agnieszka Korfel1 
TL;DR: No impact on outcome was seen for MD, diagnosed by any of the methods used; however, patients with cytomorphologic proof of MD had a significantly shorter median EFS and OAS.
Abstract: The frequency of meningeal dissemination (MD) in primary CNS lymphoma (PCNSL), its prognostic impact, and optimal management have not been defined thus far. In 69 of 92 (75%) immunocompetent patients, primarily diagnosed with PCNSL at our institution between January 1994 and February 2007, cerebrospinal fluid was analyzed for MD. MD was found by cytomorphology in 7/63 (11%), by immunophenotyping in 1/32 (3%), and by PCR of the IgH CDR III region in 6/37 (16%). Neuroradiologic examination revealed MD in 3 of 69 patients (4%). Median event-free survival (EFS) of patients with MD diagnosed by any of the methods was 26 months, of those without MD 34.1 months (P = .24); median overall survival (OAS) of these two patients' groups was 45.5 and 42.5 months, respectively (P = .34). Patients with cytomorphologic proof of MD had a median EFS of 15.4 months and OAS of 18.5 months, those without MD 34.3 and 45 months (P = .018 and .017, respectively). We found a low frequency of MD despite the use of putatively sensitive diagnostic methods. No impact on outcome was seen for MD, diagnosed by any of the methods used; however, patients with cytomorphologic proof of MD had a significantly shorter median EFS and OAS.

58 citations


"Findings at presentation in primary..." refers background in this paper

  • ...Only a small percentage of lumbar puncture-diagnosed meningeal involvement is confirmed radiologically ([32])....

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Journal ArticleDOI
TL;DR: Compared with several other populations, PCNSL is not becoming significantly more common in Alberta, and comparisons between Albertans and other populations in whom the incidence is rising may provide clues regarding the etiology ofPCNSL.

52 citations