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: The differential diagnosis of PCNSL includes central nervous system gliomas, metastatic tumors, demyelinating disorders, subacute infarcts, and space-occupied lesions due to an infectious etiology.
Abstract: Primary central nervous system lymphoma (PCNSL) is an uncommon extranodal non-Hodgkin lymphoma. Its incidence has increased during the last 3 decades and has been reported in both immunocompromised and immunocompetent patients. Immunocompromised patients are affected at a younger age compared with immunocompetent patients. It presents with raised intracranial pressure and focal neurologic and neuropsychiatric symptoms. The lesions are typically solitary. The majority of the lesions are located in the periventricular area, whereas in a few cases they are located in the supratentorial area. Diffuse large B-cell lymphomas constitute most PCNSLs, whereas T-cell, low-grade, anaplastic, and Hodgkin lymphomas are rarely encountered. The morphology of PCNSL shows a characteristic angiocentric pattern and is positive for B-cell markers by immunohistochemistry. The differential diagnosis of PCNSL includes central nervous system gliomas, metastatic tumors, demyelinating disorders, subacute infarcts, and space-occupying lesions due to an infectious etiology. The understanding of the molecular mechanisms involved in the pathogenesis of PCNSL and the identification of molecular biomarkers have lagged behind that of systemic nodal lymphomas. Primary central nervous system lymphomas are treated with combined radiotherapies and chemotherapies. The prognosis for PCNSL is worse than for other extranodal lymphomas.
448 citations
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
"Findings at presentation in primary..." refers background in this paper
...Currently, newer advanced imaging techniques, such as DWI, perfusion MRI, perfusion CT, MR spectroscopy, positron emission tomography (PET) and single photon emission computed (SPECT) metabolic imaging, may potentially aid in the differentiation of CNS lymphoma from other brain lesions ([33])....
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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
"Findings at presentation in primary..." refers result in this paper
...The mean number of lesions was approximately 2 per patient, which is similar to the numbers cited in a previous study ([13])....
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Book•
01 Jun 2004
TL;DR: Each ' original' diagnosis has been revised to include the most recent information, updated references, and new image galleries in this Second Edition of the bestselling "Diagnostic Imaging: Brain".
Abstract: In this Second Edition of the bestselling "Diagnostic Imaging: Brain", each 'original' diagnosis has been revised to include the most recent information, updated references, and new image galleries. Moreover, the book features more than 100 new diagnoses. You'll find thousands of new images, all crisply annotated to reinforce the most important points. Richly colored graphics pop off the page, and both typical and variant findings are lavishly illustrated in more than 300 diagnoses. This updated volume will surely become the new standard reference textbook for neuroradiologists, general radiologists, neurologists, and neurosurgeons. A new companion eBook offers the fully searchable expanded text, hundreds of additional images, and extensive linked references.
323 citations
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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