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Revue Neurologique Mar 2023PCNSL is a non-Hodgkin lymphoma (NHL) affecting brain, spinal cord, eyes and leptomeninges. In the past two decades, its prognosis significantly improved due to... (Review)
Review
PCNSL is a non-Hodgkin lymphoma (NHL) affecting brain, spinal cord, eyes and leptomeninges. In the past two decades, its prognosis significantly improved due to therapeutic advances but it remains a highly aggressive tumor and early diagnosis is necessary for optimal management. Diagnosis relies on the identification of lymphoma cells in brain tissue obtained by stereotactic biopsy. Alternatively, lymphoma cells may be found in CSF through lumbar puncture (LP) or by a vitrectomy. For several reasons, the diagnosis of PCNSL may be challenging. Misleading radiological presentations are frequent. Dramatic response to steroids may bias histological analysis and deep brain location or frail health status can contraindicate brain biopsy. In the follow-up of patients who have been previously treated, differential diagnosis between tumor relapse and post-treatment may be also difficult. Therefore, the development of complementary reliable diagnostic tools is needed. This review will summarize several diagnostic or prognostic CSF biomarkers which have been proposed in PCNSL, their interests and limits.
Topics: Humans; Central Nervous System Neoplasms; Neoplasm Recurrence, Local; Lymphoma, Non-Hodgkin; Biomarkers; Prognosis
PubMed: 36336490
DOI: 10.1016/j.neurol.2022.06.014 -
Cancer Control : Journal of the Moffitt... Jan 2017Leukemic and lymphomatous meningitis is a major presentation of primary or secondary central nervous system (CNS) involvement by aggressive lymphomas or acute leukemia. (Review)
Review
BACKGROUND
Leukemic and lymphomatous meningitis is a major presentation of primary or secondary central nervous system (CNS) involvement by aggressive lymphomas or acute leukemia.
METHODS
The medical literature and ongoing clinical trials were reviewed on the clinical presentation, diagnosis, prognosis, prevention, and treatment of leukemic and lymphomatous meningitis.
RESULTS
Treatment for secondary leukemic and lymphomatous meningitis remains unsatisfactory, and efforts should be made to prevent and treat subclinical disease. Intrathecal and systemic chemotherapy remain the main therapeutic approaches for this disease. Outcomes have improved in patients with primary CNS lymphoma and meningeal involvement.
CONCLUSIONS
Appropriate selection of patients at high risk for leukemic and lymphomatous meningitis is important so that preventive strategies can decrease the incidence of this complication of leukemia and lymphoma. Use of chemotherapy agents that cross the blood-brain barrier and the adoption of high-dose chemotherapy with autologous hematopoietic stem cell transplantation have increased the proportion of patients whose primary disease is cured.
Topics: Disease Management; Humans; Leukemia; Lymphoma; Meningitis; Prognosis
PubMed: 28178710
DOI: 10.1177/107327481702400105 -
Veterinary Journal (London, England :... Oct 2016In cats, lymphoma (lymphosarcoma) is the most common neoplasm affecting the spinal cord and the second most common intracranial tumour. Although lymphoma commonly... (Review)
Review
In cats, lymphoma (lymphosarcoma) is the most common neoplasm affecting the spinal cord and the second most common intracranial tumour. Although lymphoma commonly develops in the spinal cord as a part of a multicentric process, a primary form may occur. Lymphoma can exhibit a wide range of morphological patterns, including intraparenchymal brain mass, lymphomatosis cerebri, intravascular lymphoma, lymphomatous choroiditis and meningitis, extradural, intradural-extramedullary or intramedullary lymphoma in the spinal cord, or neurolymphomatosis in the peripheral nerves. Lymphoma may occur as a paraneoplastic disorder associated with peripheral neuropathies. Magnetic resonance imaging (MRI) and computed tomography (CT) are the techniques of choice for morphological assessment of nervous system lesions in vivo. However, biopsy should be performed to achieve a definitive diagnosis. Knowledge of the different morphological patterns expressed by lymphoma in the nervous system of cats allows veterinary clinicians to suspect lymphoma and to arrange appropriate diagnostic procedures, including immunophenotype and clonality studies, along with therapeutic protocols and prognostic evaluations.
Topics: Animals; Cat Diseases; Cats; Central Nervous System Diseases; Lymphoma, Non-Hodgkin; Magnetic Resonance Imaging; Peripheral Nervous System Diseases; Tomography, X-Ray Computed
PubMed: 27687936
DOI: 10.1016/j.tvjl.2016.07.013 -
Neuroradiology Aug 2021Meningiomas are the most common extra-axial intracranial neoplasms with typical radiological findings. In approximately 2% of cases, histopathological reports reveal...
PURPOSE
Meningiomas are the most common extra-axial intracranial neoplasms with typical radiological findings. In approximately 2% of cases, histopathological reports reveal different neoplasms or non-neoplastic lesions that can closely mimic meningiomas. We describe radiological features of meningioma mimics highlighting imaging red flags to consider a differential diagnosis.
METHODS
A total of 348 lesions with radiological diagnosis of meningiomas which underwent to surgical treatment or biopsy between December of 2000 and September of 2014 were analyzed. We determined imaging features that are not a typical finding of meningiomas, suggesting other lesions. The following imaging characteristics were evaluated on CT and MRI: (a) bone erosion; (b) hyperintensity on T2WI; (c) hypointensity on T2WI; (d) bone destruction; (e) dural tail; (f) leptomeningeal involvement; (g) pattern of contrast enhancement; (h) dural displacement sign.
RESULTS
We have a relatively high prevalence of meningioma mimics (7.2%). Dural-based lesions with homogeneous contrast enhancement (52%) are easily misdiagnosed as meningiomas. Most lesions mimic convexity (37.5%) or parafalcine (21.9%) meningiomas. We have determined five imaging red flags that can alert radiologists to consider meningioma mimics: (1) bone erosion (22.2%); (2) dural displacement sign (36%); (3) marked T2 hypointensity (32%); (4) marked T2 hyperintensity (12%); (5) absence of dural tail (48%). The most common mimic lesion in our series was hemangiopericytomas, followed by lymphomas and schwannomas.
CONCLUSION
The prevalence of meningioma mimics is not negligible. It is important to have awareness on main radiological findings suggestive of differential diagnosis due to a wide range of differentials which lead to different prognosis and treatment strategies.
Topics: Hemangiopericytoma; Humans; Magnetic Resonance Imaging; Meningeal Neoplasms; Meningioma; Neurilemmoma
PubMed: 33459822
DOI: 10.1007/s00234-021-02632-y -
Frontiers in Neurology 2023Chimeric antigen receptor (CAR) T-cell systemic immunotherapy has revolutionized how clinicians treat several refractory and relapsed hematologic malignancies. Due to... (Review)
Review
Chimeric antigen receptor (CAR) T-cell systemic immunotherapy has revolutionized how clinicians treat several refractory and relapsed hematologic malignancies. Due to its peculiar mechanism of action, CAR T-cell-based therapy has enlarged the spectrum of neurological toxicities. CAR T-cell-associated neurotoxicity-initially defined as CAR T-cell-related encephalopathy syndrome (CRES) and currently coined within the acronym ICANS (immune effector cell-associated neurotoxicity syndrome)-is perhaps the most concerning toxicity of CAR T-cell therapy. Importantly, hematologic malignancies (especially lymphoid malignancies) may originate in or spread to the central nervous system (CNS) in the form of parenchymal and/or meningeal disease. Due to the emergence of deadly and neurological adverse events, such as fatal brain edema in some patients included in early CAR T-cell trials, safety concerns for those with CNS primary or secondary infiltration arose and contributed to the routine exclusion of individuals with pre-existing or active CNS involvement from pivotal trials. However, based primarily on the lack of evidence, it remains unknown whether CNS involvement increases the risk and/or severity of CAR T-cell-related neurotoxicity. Given the limited treatment options available for patients once they relapse with CNS involvement, it is of high interest to explore the role of novel clinical strategies including CAR T cells to treat leukemias/lymphomas and myeloma with CNS involvement. The purpose of this review was to summarize currently available neurological safety data of CAR T-cell-based immunotherapy from the clinical trials and real-world experiences in adult patients with CNS disease due to lymphoma, leukemia, or myeloma. Increasing evidence supports that CNS involvement in hematologic disease should no longer be considered as an absolute contraindication to CAR T-cell-based therapy. While the incidence may be high, severity does not appear to be impacted significantly by pre-existing CNS status. Close monitoring by trained neurologists is recommended.
PubMed: 37090983
DOI: 10.3389/fneur.2023.1144414 -
Medicine Dec 2022Primary central nervous system lymphoma (PCNSL) is a rare extranodal non-Hodgkin lymphoma, and isolated meningeal PCNSL, without evidence of parenchymal involvement, is... (Review)
Review
RATIONALE
Primary central nervous system lymphoma (PCNSL) is a rare extranodal non-Hodgkin lymphoma, and isolated meningeal PCNSL, without evidence of parenchymal involvement, is even less common, occurring in only 10% to 15% of cases.
PATIENT CONCERNS
A 65-years-old female presented to our hospital with progressive lower extremity motor dysfunction and blurred vision. The initial neurological examination revealed decreased muscle strength in both lower extremities and sensory dysfunction of lower extremities, saddle area, and buttocks. Brain magnetic resonance imaging showed no abnormalities. Lumbar enhanced magnetic resonance imaging showed T11 to L3 horizontal meningeal enhancement. Cerebrospinal fluid (CSF) cytology revealed lymphoma cells. Immunohistochemistry and flow cytometry of the CSF were performed as auxiliary methods to establish the diagnosis of lymphoma.
DIAGNOSES
The patient was diagnosed primary meningeal central nervous system lymphoma.
INTERVENTIONS
During hospitalization, the patient was treated with 2 courses of high-dose intrathecal methotrexate and rituximab combined with intrathecal chemotherapy and supportive treatment.
OUTCOMES
After 2 years of follow-up, the patient was able to walk and take care of herself.
LESSONS
Cases of PCNSL involving only the meninges are rare. Multimodal analysis of the CSF comprises an important component of the diagnostic work-up for patients with primary meningeal central nervous system lymphoma.
Topics: Humans; Female; Aged; Central Nervous System Neoplasms; Lymphoma; Meninges; Rituximab; Brain
PubMed: 36596043
DOI: 10.1097/MD.0000000000032567 -
Critical Reviews in Oncology/hematology Nov 2021Non-Hodgkin lymphomas with T-cell immunophenotype encompass a heterogeneous group of infrequent neoplasms that follow variable clinical courses but prevalently include... (Review)
Review
Non-Hodgkin lymphomas with T-cell immunophenotype encompass a heterogeneous group of infrequent neoplasms that follow variable clinical courses but prevalently include aggressive behavior and high mortality rates. The involvement of the central nervous system (CNS) is an uncommon event in T-cell lymphomas, with wide variability among the different disease entities. CNS can be affected either at initial diagnosis or at recurrence, and both forms are considered "secondary CNS T-cell lymphoma". Given the low incidence of secondary CNS T-cell lymphoma, related literature is sparse, contradictory, and primarily constituted by small case series and single case reports. However, reported studies uniformly suggest high mortality rates related to this event. Therefore, to improve our ability to identify high-risk patients and offer them successful CNS prophylaxis or timely and effective treatment once the event has occurred may prevent CNS-related T-cell lymphomas deaths. For example, some entities like aggressive adult T-cell leukemia/lymphoma, extranodal natural killer/T-cell lymphoma, and other peripheral T-cell lymphomas with involvement of two or more extranodal organs are prone to CNS dissemination and should be considered for personalized CNS prophylaxis. The level of evidence suggesting an increased risk of CNS recurrence for other T-cell lymphomas and for other risk factors is lower. Published case series show that, following the example of aggressive B-cell lymphomas, patients with T-cell lymphomas and putative increased CNS risk receive different forms of prophylaxis, mostly methotrexate and cytarabine delivered by intrathecal and/or intravenous routes, with varied success. To date, achievements in the treatment of CNS involvement in patients with aggressive B-cell lymphoma were not replicated in secondary CNS T-cell lymphomas, and identification of effective therapies remains an urgent research target. This review is focused on clinical findings, diagnosis, treatment, and prognosis of patients with T-cell lymphoma experiencing CNS dissemination either at presentation or relapse. It aims to provide logical and, oftentimes, evidence-based answers to the most common questions on the most probable risk factors to CNS involvement in patients with T-cell lymphoma, the indications and strategies to prevent this life-threating event, and the management of patients with CNS disease.
Topics: Central Nervous System; Central Nervous System Neoplasms; Humans; Lymphoma, T-Cell, Peripheral; Methotrexate; Prognosis
PubMed: 34653598
DOI: 10.1016/j.critrevonc.2021.103496 -
Der Ophthalmologe : Zeitschrift Der... Oct 2021There are various options for the conservative treatment of the most frequent orbital tumors. These can delay, complement or be superior to the surgical approach, which... (Review)
Review
BACKGROUND
There are various options for the conservative treatment of the most frequent orbital tumors. These can delay, complement or be superior to the surgical approach, which is often prone to complications.
OBJECTIVE
This article gives a summary of the possible treatment options for the most common orbital tumors in childhood and adulthood.
METHODS
A literature search was carried out and the possible treatment pathways are presented.
RESULTS
1. Frequent orbital tumors in childhood: a systemic treatment with noncardioselective beta blockers is the primary treatment for capillary orbital hemangiomas. In cases of no response, steroids, interferon alpha or cyclophosphamide are treatment options. Observation is a possible option for smaller dermoid cysts, in cases of progression excision can become necessary. Symptomatic optic nerve gliomas can also be observed and in cases of progression treated with chemotherapy, mTOR/MEK inhibitors or radiotherapy (children > 5 years). Rhabdomyosarcomas are biopsied and subsequently treated by radiotherapy and chemotherapy. 2. Frequent orbital tumors in adulthood: asymptomatic cases of cavernous hemangiomas of the orbit can just be observed. Symptomatic hemangiomas can be surgically excised or treated with radiotherapy. For meningiomas of the optic nerve sheath radiotherapy is a very effective treatment. Surgical excision should be reserved for cases with no prognosis of visual acuity. There is also the option to treat with antiprogesterone. Orbital lymphomas with purely orbital involvement can be treated with radiotherapy, chemotherapy or the application of rituximab.
CONCLUSION
There are now very effective conservative treatment options for many orbital tumors. In some cases a surgical procedure can be avoided and a good visual function can be retained.
Topics: Adult; Child; Humans; Meningeal Neoplasms; Meningioma; Orbit; Orbital Neoplasms; Retrospective Studies; Treatment Outcome
PubMed: 34406460
DOI: 10.1007/s00347-021-01471-9 -
World Neurosurgery Feb 2022The diagnosis of a contiguous, synchronous meningioma and central nervous system B-cell lymphoma is rare and associated with paradoxical treatment paradigms. We... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The diagnosis of a contiguous, synchronous meningioma and central nervous system B-cell lymphoma is rare and associated with paradoxical treatment paradigms. We performed a scoping review of contiguous meningioma and B-cell lymphoma and included an additional illustrative case.
METHODS
The OVID Medline and PubMed databases were systematically searched using the Preferred Reporting Items of Systematic Reviews and Meta-Analysis guidelines. Only human clinical reports of contiguous, synchronous meningioma and B-cell lymphoma were included. We concurrently detailed a representative case from our institution.
RESULTS
Nine case reports met our criteria, including the present case. The average age at diagnosis was 67.4 years. Patients showed a female-to-male predominance of 7:2. The diagnosis of synchronous intracranial tumors was not suspected or discovered until after surgical resection in 100% of cases. All meningiomas were grade I on histopathologic diagnosis, while lymphomas were distributed between diffuse large B-cell lymphoma (56%), metastatic lymphoma (22%), Burkitt lymphoma (11%), and follicular lymphoma (11%). All patients underwent surgical resection. Patients (n = 5) treated with adjuvant chemotherapy had evidence of longer progression-free survival (median 12 months; range, 3-18 months) than patients without adjuvant chemotherapy (n = 2; median 2 months; range, 1-3 months).
CONCLUSIONS
Contiguous, synchronous meningioma/B-cell lymphoma is a rare diagnosis that may appear as an inconspicuous solitary intracranial neoplasm on imaging. Based on the limited cases and current treatment of lymphoma, progression-free survival may be contingent on the prompt initiation of chemotherapy targeting the lymphoma rather than surgical resection of the meningeal mass. Providers should prioritize prompt medical management.
Topics: Brain Neoplasms; Burkitt Lymphoma; Central Nervous System Neoplasms; Female; Humans; Lymphoma, Large B-Cell, Diffuse; Male; Meningeal Neoplasms; Meningioma; Neoplasms, Multiple Primary; Systematic Reviews as Topic
PubMed: 34798339
DOI: 10.1016/j.wneu.2021.11.042 -
Mycoses Sep 2016Cryptococcosis is an opportunistic invasive fungal infection that is well described and easily recognised when it occurs as meningitis in HIV-infected persons.... (Review)
Review
Cryptococcosis is an opportunistic invasive fungal infection that is well described and easily recognised when it occurs as meningitis in HIV-infected persons. Malignancy and its treatment may also confer a higher risk of infection with Cryptococcus neoformans, but this association has not been as well described. A case of cryptococcosis in a cancer patient is presented, and all cases of coincident C. neoformans infection and malignancy in adults published in the literature in English between 1970 and 2014 are reviewed. Data from these cases were aggregated in order to describe the demographics, type of malignancy, site of infection, clinical manifestations, treatment and outcomes of cryptococcosis in patients with cancer. Haematologic malignancies accounted for 82% of cases, with lymphomas over-represented compared to US population data (66% vs. 53% respectively). Cryptococcosis was reported rarely in patients with solid tumours. Haematologic malignancy patients were more likely to have central nervous system (P < 0.001) or disseminated disease (P < 0.001), receive Amphotericin B as part of initial therapy (P = 0.023), and had higher reported mortality rates than those with solid tumours (P = 0.222). Providers should have heightened awareness of the possibility of cryptococcosis in patients with haematologic malignancy presenting with infection.
Topics: AIDS-Related Opportunistic Infections; Amphotericin B; Antifungal Agents; Cryptococcosis; Cryptococcus neoformans; Female; Hematologic Neoplasms; Humans; Lymphoma; Meningitis, Cryptococcal; Middle Aged; Neoplasms; Opportunistic Infections
PubMed: 26932366
DOI: 10.1111/myc.12496