Is a “vanishing tumor” always a lymphoma?

Bromberg, Jacoline E C; Siemers, Maaike D.; Taphoorn, M. J. B.

doi:10.1212/wnl.59.5.762

Cited by 72 publications

(38 citation statements)

References 11 publications

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“…Glucocorticoids should be withheld if possible, since lesions may disappear within a few days after treatment with steroids and biopsies may subsequently result as 'nondiagnostic'. Response to steroid treatment is not diagnostic of PCNSL, since many types of brain infiltrates improve after steroid therapy, for instance sarcoidosis, multiple sclerosis plaques and infectious lesions [23]. Optimum treatment of PCNSL remains challenging.…”

Section: Discussionmentioning

confidence: 99%

Diagnostic delay and prognosis in primary central nervous system lymphoma compared with glioblastoma multiforme

et al. 2015

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Glioblastoma multiforme (GBM) and primary central nervous system lymphoma (PCNSL) are malignant cerebral neoplasms associated with poor prognosis. Early diagnosis and subsequent planning of adequate treatment strategy are relevant to improve survival and reduce neurological deficit. Two groups of patients affected by GBM and PCNSL were compared to identify: (1) factors influencing the time necessary to obtain a correct diagnosis; (2) the influence of the interval time from clinical onset to diagnosis on the prognosis. Fifty-six patients (28 PCNSL and 28 GBM, 23 females and 33 males) referred to the same hospital setting were retrospectively evaluated. The mean age at diagnosis was 61 years. The two groups were comparable in terms of age, sex, clinical symptoms at onset and performance status. There was no relevant difference in time span from clinical onset to first neuroimaging examination, while time span from first neuroimaging to final morphological diagnosis was much longer in PCNSL patients (p = 0.008). Multivariate Cox regression analysis, including both PCNSL and GBM cases, showed a significant association of the overall survival with: time to diagnosis (HR 0.06), age at onset (HR 1.04). Our results show a significant diagnostic delay in PCNSL cases. Age at onset of disease and time to diagnosis emerge as clinical factors affecting overall survival in both groups. Stereotactic-guided biopsy should be chosen as routine method to early diagnose PCNSL. The clinical relevance of early diagnosis in GBM and PCNSL needs to be emphasized to maximize the overall survival in both neoplasms.

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Section: Discussionmentioning

confidence: 99%

Diagnostic delay and prognosis in primary central nervous system lymphoma compared with glioblastoma multiforme

et al. 2015

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“…As many others, I believe that detection of a "vanishing tumor" should not be considered as diagnostic of PCNSL because sarcoidosis, multiple sclerosis, acute encephalomyelitis, and other malignancies can also exhibit a dramatic response to steroids. 8 Steroid effects can interfere with histopathologic diagnosis. Although some authors reported that PCNSL can be successfully diagnosed without stopping steroids, 9 there is consensus that these drugs should be withheld in patients with a presumptive diagnosis of PCNSL until tissue is obtained, limiting its use to cases of osmotherapy inefficacy.…”

Section: Should Steroids Be Interrupted Before Biopsy?mentioning

confidence: 99%

How I treat primary CNS lymphoma

Ferreri¹

2011

Blood

163

109

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Primary CNS lymphoma (PCNSL) is a rare malignancy with peculiar clinical and biologic features, aggressive course, and unsatisfactory outcome. It represents a challenge for multidisciplinary clinicians and scientists as therapeutic progress is inhibited by several issues. Molecular and biologic knowledge is incomplete, limiting the identification of new therapeutic targets, and the particular microenvironment of this malignancy, and sanctuary sites where tumor cells grow undisturbed, strongly affects treatment efficacy. Moreover, active treatments are known to be associated with disabling neurotoxicity, posing the dilemma of whether to intensify therapy to improve the cure rate or to de-escalate treatment to avoid sequels. The execution of prospective trials is also difficult because of the rarity of the tumor and the impaired general condition and poor performance status of patients. Thus, level of evidence is low, with consequent uncertainties in therapeutic decisions and lack of consensus on primary endpoints for future trials.Despite this unfavorable background, laboratory and clinical researchers are coordinating efforts to develop new ideas, resulting in the recent publication of studies on PCNSL's biology and molecular mechanisms and of the first international randomized trials. Herein, these important contributions are analyzed to provide recommendations for everyday practice and the rationale for future trials. (Blood. 2011;118(3):510-522) IntroductionPrimary CNS lymphoma (PCNSL) is an aggressive malignancy arising exclusively in the CNS, that is, the brain parenchyma, spinal cord, eyes, cranial nerves, and/or meninges. 1 This lymphoma represents 4% of intracranial neoplasms and 4%-6% of all extranodal lymphomas but some registry studies suggest that its incidence in immunocompetent patients is progressively increasing. 2 PCNSL exhibits peculiar clinical and biologic features and constitutes a diagnostic and therapeutic challenge for multidisciplinary clinicians and scientists. Its outcome remains unsatisfactory if compared with that of patients with extra-CNS lymphomas of a similar stage and histotype, and several factors prevent therapeutic progress. First, molecular and biologic knowledge is insignificant compared with other lymphomas, which limits the identification of new therapeutic targets. Second, patients with PCNSL usually exhibit impaired general condition and poor performance status (PS) more often than subjects with other lymphomas. 1 This usually interferes with the enrollment of patients in prospective trials. In fact, current therapeutic knowledge is only based on 3 randomized trials, some single-arm phase 2 trials, anecdotal meta-analyses, and a few multicenter retrospective studies. Thus, available level of evidence is low, with consequent uncertainties in therapeutic decisions and lack of consensus on primary endpoints for future trials.In this manuscript, I discuss available clinical and biologic data on PCNSL in immunocompetent patients and provide recommendations for everyday pract...

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“…2 Relapse generally occurs within 18 months in 80% of patients with PCNSL vanishing tumors, with a median remission duration of 7 months for PCNSL (range 1-54 months). 9 Late recurrence ($5 years from initial diagnosis) in appropriately treated PCNSL is a rare event. One large series identified a late relapse rate of 4% in patients who achieved CR following appropriate treatment.…”

Section: Sectionmentioning

confidence: 99%

Clinical Reasoning: Vanishing tumor

et al. 2015

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In 2005, a 60-year-old man presented to the hospital complaining of a 2-month history of headaches and lethargy. His medical history included osteoarthritis and hypertension. From a social perspective, he was married with children, drove a truck for a living, and had never traveled abroad. His family history was unremarkable. Investigations included a brain CT, which highlighted an enhancing, spaceoccupying lesion in the left basal ganglia with associated edema and mass effect (figure, A). He was prescribed high-dose dexamethasone while awaiting a neurosurgical review. One week later, a prebiopsy, contrast-enhanced CT showed a reduction in the size of the caudate head mass (figure, B). MRI brain with gadolinium showed small white matter changes and no mass was evident. He did not proceed to biopsy and was subsequently observed. CSF analysis was abnormal, revealing mature, lymphoid cells and some larger immature cells, some of which appeared plasmacytoid (B cell). Although suspicious for lymphoma, this result was nondiagnostic. The CSF protein level was 331. CSF low-density lipoprotein, Epstein-Barr virus, and flow cytometry were not performed. The b2-microglobulin level and serum low-density lipoprotein were normal. Serology for cytomegalovirus and toxoplasma were negative, as were ELISA for Toxocara and HIV screen. Slit-lamp examination was unremarkable. He was discharged, but readmitted 2 weeks later with a deep vein thrombosis. Repeat CSF cytology was again nondiagnostic. CSF was not examined for oligoclonal bands or myelin basic protein. A bone marrow biopsy and staging CT had normal results. Whole body PET/CT was unavailable.Questions for consideration:

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Is a “vanishing tumor” always a lymphoma?

Cited by 72 publications

References 11 publications

Diagnostic delay and prognosis in primary central nervous system lymphoma compared with glioblastoma multiforme

Diagnostic delay and prognosis in primary central nervous system lymphoma compared with glioblastoma multiforme

How I treat primary CNS lymphoma

Clinical Reasoning: Vanishing tumor

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