Primary central nervous system lymphomas (PCNSL): MRI features at presentation in 100 patients
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.
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TL;DR: New CT and MR imaging techniques and metabolic imaging have demonstrated characteristic findings in CNS lymphoma, aiding in its differentiation from other CNS lesions, and advanced imaging techniques may, in the future, substantially improve the diagnostic accuracy of imaging, ultimately facilitating a noninvasive method of diagnosis.
Abstract: CNS lymphoma consists of 2 major subtypes: secondary CNS involvement by systemic lymphoma and PCNSL. Contrast-enhanced MR imaging is the method of choice for detecting CNS lymphoma. In leptomeningeal CNS lymphoma, representing two-thirds of secondary CNS lymphomas, imaging typically shows leptomeningeal, subependymal, dural, or cranial nerve enhancement. Single or multiple periventricular and/or superficial contrast-enhancing lesions are characteristic of parenchymal CNS lymphoma, representing one-third of secondary CNS lymphomas and almost 100% of PCNSLs. New CT and MR imaging techniques and metabolic imaging have demonstrated characteristic findings in CNS lymphoma, aiding in its differentiation from other CNS lesions. Advanced imaging techniques may, in the future, substantially improve the diagnostic accuracy of imaging, ultimately facilitating a noninvasive method of diagnosis. Furthermore, these imaging techniques may play a pivotal role in planning targeted therapies, prognostication, and monitoring treatment response.
365 citations
Cites background from "Primary central nervous system lymp..."
...Multiple lesions are reported in 20%– 40% of non-AIDS PCNSLs,(14,15,27,29,31) and ringlike enhancement, in 0%–13% (Figs 2 and 3).(14,29,31,32) Linear enhancement along perivascular spaces is highly suggestive of PCNSL....
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...The brain stem or cerebellum or both are affected in 9%– 13%,(15,32,35) and the spinal cord, in only 1%–2% of patients.(15,29) Hemorrhage or internal calcification within the tumor is quite a rare finding....
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...Most lesions show moderate-to-marked contrast enhancement on CT and MR imaging.(14,15,24-27,29) Isolated white matter hyperintensity on T2-weighted MR imaging or no contrast enhancement on T1weighted MR imaging has also been described in some rare cases of PCNSL....
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...In PCNSL, proton MR spectroscopy has demonstrated elevated lipid peaks combined with high Cho/Cr ratios.(29,42-44) These can, however, also be seen in glioblastoma multiforme(42)...
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TL;DR: Diagnosis of primary CNS lymphoma in immunocompetent patients is associated with unique diagnostic, prognostic, and therapeutic issues, and the management of this malignancy is different from that of other forms of extranodal NHL.
Abstract: Primary CNS lymphoma (PCNSL), an uncommon form of extranodal non-Hodgkin's lymphoma (NHL), has increased in incidence during the last three decades and occurs in both immunocompromised and immunocompetent hosts. PCNSL in immunocompetent patients is associated with unique diagnostic, prognostic, and therapeutic issues, and the management of this malignancy is different from that of other forms of extranodal NHL. Characteristic imaging features should be suggestive of the diagnosis, avoidance of corticosteroids, if possible, and early neurosurgical consultation for stereotactic biopsy. Because PCNSL may involve the brain, CSF, and eyes, diagnostic evaluation should include assessment of all of these regions as well as screening for possible occult systemic disease. Resection provides no therapeutic benefit and should be reserved for the rare patient with neurologic deterioration due to brain herniation. Whole-brain radiation therapy (WBRT) alone is insufficient for durable tumor control and is associated with a high risk of neurotoxicity in patients older than age 60. Neurotoxicity typically is associated with significant cognitive, motor, and autonomic dysfunction, and has a negative impact on quality of life. Chemotherapy and WBRT together improve tumor response rates and survival compared with WBRT alone. Methotrexate-based multiagent chemotherapy without WBRT is associated with similar tumor response rates and survival compared with regimens that include WBRT, although controlled trials have not been performed. The risk of neurotoxicity is lower in patients treated with chemotherapy alone.
322 citations
TL;DR: This review summarizes recent advances in the understanding of the major categories of RPD and outlines efficient approaches to the diagnosis of the various neurodegenerative, toxic‐metabolic, infectious, autoimmune, neoplastic, and other conditions that may progress rapidly.
Abstract: In contrast with more common dementing conditions that typically develop over years, rapidly progressive dementias can develop subacutely over months, weeks, or even days and be quickly fatal. Because many rapidly progressive dementias are treatable, it is paramount to evaluate and diagnose these patients quickly. This review summarizes recent advances in the understanding of the major categories of RPD and outlines efficient approaches to the diagnosis of the various neurodegenerative, toxic-metabolic, infectious, autoimmune, neoplastic, and other conditions that may progress rapidly.
322 citations
Cites background from "Primary central nervous system lymp..."
...Lesions may be isolated or multiple and generally show contrast enhancement [128]....
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TL;DR: The aim of this monograph is to provide a chronology of key events and milestones in the development of HIV/AIDS-related cancers over a 12-month period from 1989 to 2002, and to promote awareness of the importance of timely diagnosis and ART.
Abstract: M Bower, S Collins, C Cottrill, K Cwynarski, S Montoto, M Nelson, N Nwokolo, T Powles, J Stebbing, N Wales and A Webb, on behalf of the AIDS Malignancy Subcommittee Department of Oncology, Chelsea & Westminster Hospital, London, UK, HIV i-Base and UK-CAB, London, UK, St Bartholomew’s Hospital, London, UK, Royal Free Hospital, London, UK, Hammersmith Hospital, London, UK and Royal Sussex County Hospital, Brighton, UK
216 citations
TL;DR: This review will focus on PCNSL in the immunocompetent host, an uncommon variant of extranodal non-Hodgkin lymphoma that has been challenging to study and an effective standard of care has been difficult to establish.
Abstract: Primary central nervous system (CNS) lymphoma (PCNSL), an uncommon variant of extranodal non-Hodgkin lymphoma (NHL), can affect any part of the neuraxis including the eyes, brain, leptomeninges, or spinal cord. It accounts for approximately 3% of all the primary CNS tumors diagnosed each year in the United States. Congenital or acquired immunodeficiency is the only established risk factor for PCNSL, and individuals with human immunodeficiency virus (HIV) infection are at greater risk for developing this tumor. Infection with HIV likely accounted for the increased incidence in PCNSL observed from 1970 to 2000, but over the last decade the number of cases of PCNSL has stabilized or decreased to about 0.47 cases per 100 000 persons. Owing to the rarity of PCNSL, the disease has been challenging to study and an effective standard of care has been difficult to establish. Unfortunately, although durable remissions may be achieved for some patients with PCNSL, the tumor relapses in most cases. In this review, we will focus on PCNSL in the immunocompetent host.
180 citations
Cites background from "Primary central nervous system lymp..."
...In immunocompetent patients with PCNSL, lesions are solitary in 65% of cases and are located in a cerebral hemisphere (38%), thalamus/basal ganglia (16%), corpus callosum (14%), periventricular region (12%), and cerebellum (9%).(8) Isolated spinal cord involvement is rare and observed in less than 1% of cases, so spinal imaging is only necessary if warranted based on clinical suspicion or to screen for leptomeningeal involvement if lumbar puncture cannot be performed....
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References
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TL;DR: A correction method is proposed for proton spectroscopy, which uses the signal of prominent water protons as a reference for the water‐suppressed signal, and has a good resolution as shown by phantom measurements and brain and muscle spectra of volunteers.
Abstract: Spatially localized methods in spectroscopy often operate with magnetic field gradients for volume selection. The eddy currents induced by these gradients produce time-dependent shifts of the resonance frequency in the selected volume, which results in a distortion of the spectrum after Fourier transformation. In whole-body systems the complete compensation of eddy currents is a difficult procedure. To avoid this, a correction method is proposed for proton spectroscopy, which uses the signal of prominent water protons as a reference for the water-suppressed signal. The correction is performed in the time domain, dividing the water-suppressed signal by the phase factor of the water signal for each data point. The corrected spectra have a good resolution as shown by phantom measurements and brain and muscle spectra of volunteers.
585 citations
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...Further details have been reported elsewhere [14]....
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TL;DR: A vigorous multimodality approach to PCNSL was well tolerated, and survival is markedly improved over conventional therapy.
Abstract: Primary central nervous system lymphoma (PCNSL), an uncommon tumor, is occurring with increasing frequency. Conventional therapy with corticosteroids and cranial radiotherapy (RT) usually gives a dramatic initial response, but median survival is only 10 to 18 months. Chemotherapy is more successful in comparable systemic lymphoma and has been employed for PCNSL at relapse, causing remission but not cure. Between June 1985 and June 1988, we prospectively staged 32 patients with PCNSL at Memorial Sloan-Kettering Cancer Center and treated 28 on a new protocol that combined chemotherapy and radiotherapy at diagnosis. None had occult systemic lymphoma, but 19% had ocular and 69% had definite or probable leptomeningeal lymphoma. There were no complications in 19 stereotactic biopsies, but 4/10 patients who had a complete resection suffered a severe postoperative deficit. Four patients received RT alone, and 28 received chemotherapy and cranial RT, 17 of whom (group A) received a combination regimen using pre-RT systemic (1 g/m2) and intra-Ommaya methotrexate (MTX), 4,000 cGy whole-brain RT with a 1,440 cGy boost, and 2 courses of post-RT high-dose cytosine arabinoside; 5 other patients received an identical regimen but with a decreased dose of MTX (200 mg/m2). Sixty-three percent of assessable patients had a response to MTX independent of corticosteroid and prior to RT. Eighteen of 26 (69%) assessable patients who received combined therapy are alive with a median follow-up of 25.4 months. Twelve of 16 (75%) assessable group A patients are alive in the same period. Chemotherapy-related toxicity was minimal, and no late toxicities have occurred to date.(ABSTRACT TRUNCATED AT 250 WORDS)
264 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: Ocular lymphoma is an uncommon cause of chronic vitreitis or uveitis, often refractory to steroid treatment, and frequently representing another site of multifocal primary central nervous system lymphoma (PCNSL).
Abstract: Background. Ocular lymphoma is an uncommon cause of chronic vitreitis or uveitis, often refractory to steroid treatment, and frequently representing another site of multifocal primary central nervous system lymphoma (PCNSL).
Methods. The authors reviewed the medical and ophthalmologic records of 24 patients with ocular lymphoma; 23 had associated PCNSL.
Results. In half, the eyes were the initial site of disease; in the others central nervous system (CNS) lymphoma developed before, or concurrent with, ocular lymphoma. Most patients had bilateral ocular symptoms; slit-lamp examination revealed asymptomatic disease in four. Vitrectomy was not always diagnostic, particularly in patients who had received steroids. Ocular ultrasound, performed on seven patients, provided an objective measure of disease and treatment response. Patients received a variety of therapeutic combinations of steroids, radiation, and chemotherapy, including high-dose cytosine arabinoside. Despite therapy, eventual ocular or CNS relapse or both was common. Thirteen patients have died, 12 with known recurrent CNS disease.
Conclusions. Ocular lymphoma frequently is associated with PCNSL. The diagnosis should be considered in patients with steroid-resistant chronic vitreitis or uveitis. Patients with PCNSL should be carefully evaluated for ocular involvement, regardless of symptoms. Treatment can contribute to prolonged remission, but eventual ocular or CNS relapse is the rule.
233 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
"Primary central nervous system lymp..." refers background in this paper
...Perfusion-weighted MRI has also been applied for the differentiation of PCNSL and glioma in a small series [26], with promising results....
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