Primary central nervous system lymphoma in immunocompetent patients: A retrospective review of MRI features
10 Apr 2012-Journal of Medical Imaging and Radiation Oncology (J Med Imaging Radiat Oncol)-Vol. 56, Iss: 3, pp 295-301
TL;DR: The objective is to define the features of primary central nervous system lymphoma (PCNSL) on MRI in immunocompetent patients and show that they are similar to other types of lymphoma.
Abstract: Introduction: To define the features of primary central nervous system lymphoma (PCNSL) on MRI in immunocompetent patients.
Methods: A retrospective review of the authors' institutional database was performed to identify histologically proven cases of PCNSL. Images were retrieved and reviewed with respect to location, lesion number, size, signal intensity, enhancement characteristics, oedema and necrosis.
Results: Thirty-one cases of histologically proven PCNSL had available imaging. One patient was excluded due to immunosuppression. Of the 30 remaining cases, the average age was 65.5 years, and males and females were equally represented. A total of 68 lesions (average of 2.5 per patient) were identified. With diffusion-weighted imaging, all but two had restricted diffusion (40.3% mild and 55.6% marked) and all but one had enhancement (51.5% homogeneous, 42.6% heterogeneous and ring 4.4%). Most lesions were isointense to grey matter (75.8% on T2-weighted image (WI) and 82.5% on T1-WI). Oedema was mild in 43.4% and marked in 55.2%. Necrosis was seen in only five lesions (7.4%). On a per patient basis, 50% had bilateral lesions and 96.7% had lesions contacting a cerebrospinal fluid (CSF) surface. 16.7% of patients had posterior fossa involvement and 30% had lesions in the basal ganglia or thalami.
Conclusion: The vast majority of cases of PCNSL in immunocompetent patients have lesions contacting a CSF surface, enhancement and restricted diffusion with no necrosis. These features should alert radiologists to the diagnosis of PCNSL.
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TL;DR: The challenges in PCNSL diagnosis, assessment of response to treatments, and evaluation of potential neurotoxicity related to chemotherapy and radiotherapy are addressed.
Abstract: Primary Central Nervous System Lymphoma (PCNSL) is a rare neoplasm that can involve brain, eye, leptomeninges, and rarely spinal cord. PCNSL lesions most typically enhance homogeneously on T1-weighted magnetic resonance imaging (MRI) and appear T2-hypointense, but high variability in MRI features is commonly encountered. Neurological symptoms and MRI findings may mimic high grade gliomas (HGGs), tumefactive demyelinating lesions (TDLs), or infectious and granulomatous diseases. Advanced MRI techniques (MR diffusion, spectroscopy, and perfusion) and metabolic imaging, such as Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) or amino acid PET (usually employing methionine), may be useful in distinguishing these different entities and monitoring the disease course. Moreover, emerging data suggest a role for cerebrospinal fluid (CSF) markers in predicting prognosis and response to treatments. In this review, we will address the challenges in PCNSL diagnosis, assessment of response to treatments, and evaluation of potential neurotoxicity related to chemotherapy and radiotherapy.
69 citations
Cites background from "Primary central nervous system lymp..."
...In most cases, the lesions enhance homogeneously [Figure 2(a)], but sometimes the enhancement is mild or with a ring pattern or is even absent [13]....
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TL;DR: The primary objective of this study was to establish a baseline level of confidence that the once-in-a-lifetime treatment of glioblastoma multiforme is likely to be profitable and effective in the long-term.
Abstract: Abbreviations: ADC = apparent diffusion coefficient, AF = arcuate fasciculus, BOLD = blood oxygen level–dependent, CBV = cerebral blood volume, CNS = central nervous system, CST = corticospinal tract, CTV = clinical target volume, DSC = dynamic susceptibility contrast, DTI = diffusion tensor imaging, DW = diffusion-weighted, ESM = electrocortical stimulation mapping, FLAIR = fluid-attenuated inversion recovery, GBM = glioblastoma multiforme, GTV = gross tumor volume, NAA = N-acetylaspartate, TE = echo time
30 citations
Cites background from "Primary central nervous system lymp..."
...Compared with normal brain parenchyma, tumors with high cellularity often have reduced ADC values or greater diffusion restriction caused by decreased extracellular spaces (8)....
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TL;DR: The varied imaging features of CNS lymphoma, atypical presentations, and differential diagnoses are illustrated.
28 citations
TL;DR: A previously healthy 65-year-old man presented with a two-week history of weight loss, headaches, blurred vision, asthenia and quickly worsening walking impairment, leading to a diagnosis of diffuse large B-cell lymphoma, primarily located in the central nervous system (PCNSL).
Abstract: A previously healthy 65-year-old man presented with a two-week history of weight loss, headaches, blurred vision, asthenia and quickly worsening walking impairment. He denied photophobia, neck stiffness, fever, nausea or vomiting.Neurological examination showed global motor slowing, tendency to fall asleep during the clinical examination, generalized weakness against resistance to head and limbs, and osteotendon reflexes present in the upper limbs, but not evoked in the lower limbs. No sensitive deficit or focal neurologic sign was recognizable.Non-contrast multislice computed tomography (MSCT) of the head was performed in the emergency department, showing diffuse periventricular white matter and thalamic mild hyperdensity.Lumbar puncture, blood tests, including serology for HIV and other infections, were negative.On the third day the patient, showing decreased consciousness, underwent magnetic resonance imaging (MRI) with contrast medium injection. MRI revealed the presence of multiple pseudonodular avidly enhancing lesions, supra and infratentorial, crossing the midline, involving the ventricular system, including the fourth ventricle, with extension into the surrounding white matter, the corpus callosum, the thalamus and the hypothamalus.A stereotactic biopsy led to a diagnosis of diffuse large B-cell lymphoma, primarily located in the central nervous system (PCNSL).After the completion of the first phase of treatment (immunotherapy with intravenous Rituximab and corticosteroid), the MRI showed a marked regression of tumor masses.
18 citations
Cites background from "Primary central nervous system lymp..."
...After treatment, the fast regression of the enhanced areas was strongly suggestive of PCNSL.17...
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...The contact with a CSF surface, as in our case, is typical of PCNSL....
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...Contact with cerebrospinal fluid (CSF), ependymal or pial, is identifiable in nearly 97% of cases and highly suggestive for PCNSL.(4) Only a few cases of PCNSL have been reported in the hypothalamus and fourth ventricle....
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...Contact with cerebrospinal fluid (CSF), ependymal or pial, is identifiable in nearly 97% of cases and highly suggestive for PCNSL.4 Only a few cases of PCNSL have been reported in the hypothalamus and fourth ventricle.5–9...
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TL;DR: Knowing the unique imaging features of CNS-PTLD narrows the differential diagnosis, facilitates the diagnostic work-up, and optimizes making the diagnosis.
Abstract: Central nervous system post-transplant lymphoproliferative disorder (CNS-PTLD) is a rare disease that presents with non-specific signs and symptoms. The purpose of this article is to present the imaging appearances of CNS-PTLD by magnetic resonance imaging. We highlight the differential diagnostic considerations including primary central nervous system lymphoma, glioblastoma, cerebral abscess, and metastatic disease. This is an important topic to review since in daily practice the diagnosis of CNS-PTLD is often not initially considered when present due to its rarity and the lack of radiologists’ familiarity with the disease. Knowing the unique imaging features of CNS-PTLD narrows the differential diagnosis, facilitates the diagnostic work-up, and optimizes making the diagnosis. Advanced MRI data for CNS PTLD is limited but is promising for helping with narrowing the differential diagnosis.
17 citations
Cites background from "Primary central nervous system lymp..."
...The signal intensities on T1 and T2 have been reported as similar to CNS-PTLD [26]....
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References
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TL;DR: Pre-treatment MRI examinations of 100 immunologically competent patients with biopsy-proven PCNSL revealed a uniformly pathologic pattern of metabolite concentrations in all patients, and DW-MRI and proton spectroscopy may aid in differential diagnosis.
Abstract: To avoid an unnecessary extend of surgery in primary central nervous system lymphoma (PCNSL), the diagnosis should be suspected after MRI.
330 citations
TL;DR: Changes in the frequency of diagnosis, character of the tumors, and therapy for this disease prompted this study of the pathologic features, clinical data, and natural history of this tumor in 104 patients.
Abstract: Background. Primary central nervous system non-Hodgkin's lymphoma (NHL-CNS) is an enigmatic disease of uncertain origin. At the Massachusetts General Hospital, 104 patients with NHL-CNS were seen from 1958 through 1989. An impression of changes in the frequency of diagnosis, character of the tumors, and therapy for this disease prompted this study of the pathologic features, clinical data, and natural history of this tumor in these 104 patients.
Methods. Histologic slides (neurosurgical specimens and autopsy tissues) were available for 99 patients. The tumors were classified by the Working Formulation classification. Immunostaining data and all clinical data were retrieved from the relevant offices and hospital charts.
Results. Primary central nervous system non-Hodgkin's lymphoma tripled in frequency (5.66 cases per year in 1978-89 versus 1.75 cases per year in 1958-77) and now represents 6.6% of all primary brain neoplasms (versus 3.3% before 1978; x2 = 17.52, P < 0.01). For the 99 tumors histologically classified, 89% were high grade. Intermediate grade lymphomas, once the second most common subtype, have disappeared since 1983. All tumors had diffuse architecture; 77% (including all 11 patients with acquired immune deficiency syndrome) were large cell subtypes. Two cases were intravascular lymphoma. With one exception, all of the 41 tumors evaluated were B-cell types; 32 of 40 had monotypic surface immunoglobulin. There was 1 T-cell lymphoma. Of 64 tumor recurrences, 29 were at the initially defined site; 12 were in the leptomeninges, 29 were in other sites in the neuraxis, and 8 were in systemic sites. Systemic metastases have not occurred since 1984. Median survival for the 68 patients who survived after diagnostic surgery and for whom follow-up information could be obtained was 19 months; 9 months for those with high grade tumors and 30.5 months for those with intermediate grade tumors. This difference was not significant (P = 0.13). A separate set of seven patients had focal tumorlike lymphoid infiltrates composed of benign-appearing lymphocytes, which were associated with good long term survival. The differential histologic diagnosis of NHL-CNS was occasionally difficult, and the spectrum of this differential was broader than generally stated.
Conclusions. Primary central nervous system non-Hodgkin's lymphoma has increased in frequency even in nonimunocompromised patient populations. This increase has been accompanied by the disappearance of intermediate grade histologic types, suggesting a fundamental shift in the biology of the neoplasms. The introduction of chemotherapeutic regimens appears to have altered the natural history such that systemic metastases outside the central nervous system no longer occur, and there are now some long term survivors of this formerly uniformly fatal disease.
316 citations
TL;DR: Primary central nervous system lymphoma is an extranodal form of non‐Hodgkin lymphoma arising in the craniospinal axis and the incidence of PCNSL appears to be increasing.
Abstract: BACKGROUND
Primary central nervous system lymphoma (PCNSL) is an extranodal form of non-Hodgkin lymphoma arising in the craniospinal axis. The incidence of PCNSL appears to be increasing.
METHODS
PCNSL incidence data from 1973–1997 were obtained from the nine Surveillance, Epidemiology and End Results (SEER) registries. To limit the influence of the human immunodeficiency virus on incidence rates, data of never–married males and females and persons of unknown marital status were excluded. As a surrogate for new technology, SEER data were reviewed by dates of diagnosis (surrogate for imaging) and compared with glioma incidence (surrogate for stereotactic neurosurgery and improved diagnostic neuropathology). Age-adjusted incidence rates were estimated and compared for the period prior to computed tomography (CT) (1973–1984) and the magnetic resonance imaging (MRI) period (1985–1997). The estimated annual percent change was calculated based on linear regression analyses using SEER*STAT.
RESULTS
The incidence of PCNSL appears to be increasing in all SEER registries examined. All age groups demonstrated an increase over time. This increase was observed both in the CT era as well as in the MRI era. PCNSL age-adjusted incidence (0.15 to 0.48, a 3-fold increase) outpaced that of systemic lymphoma (14.1 to 18.5, a 33% increase) for the same registries over the same time periods. The rate of increase has begun to slow since 1985; the estimated annual percent change for PCNSL was three-fold higher during the period 1973–1985 compared with 1986–1997.
CONCLUSION
The incidence rate of PCNSL continues to rise. The increase is evident in all age groups and in both genders. Data from the current study suggest that improved diagnostic tools, such as CT or MRI, cannot explain this increase. Cancer 2002;95:1504–10. © 2002 American Cancer Society.
DOI 10.1002/cncr.10851
297 citations
TL;DR: MRI examinations of immunologically competent patients with primary CNS lymphoma found contrast-enhancing lesions in contact with the subarachnoid space and without necrosis are characteristic of PCNSL.
Abstract: Pretreatment MRI examinations of 40 immunologically competent patients with primary CNS lymphoma (PCNSL) were evaluated (24 men, 16 women, median age 63 years). Seventy lesions were found (mean size: 19.9 mm). The number of lesions ranged from one (n = 25) to six (n = 1). The most frequent locations were the cerebral hemispheres (n = 22), the corpus callosum (n = 11), and the basal ganglia (n = 11). Cerebellar manifestations were found in 10 patients. Ocular (n = 2) and medullary cord (n = 1) manifestations were rare. Contrast enhancement was encountered in all lesions. Although 39 patients had lesions adjacent to the CSF space, leptomeningeal spread was only present in five patients. Necrosis was seen in two lesions only. Edema was extensive in 24 patients, moderate in 11 patients, and absent in five patients. Contrast-enhancing lesions in contact with the subarachnoid space and without necrosis are characteristic of PCNSL.
261 citations
TL;DR: Comparing the intensity-time curves for the two tumor groups, the PCNSL showed a characteristic type of curve with a significant increase in signal intensity above the baseline due to massive leakage of contrast media into the interstitial space.
211 citations