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Cureus May 2023Kikuchi-Fujimoto disease (KFD) is an autoimmune condition that is more common in females and occurs in the third decade of life. The condition is usually benign and...
Kikuchi-Fujimoto disease (KFD) is an autoimmune condition that is more common in females and occurs in the third decade of life. The condition is usually benign and self-resolving and is characterized by fever, cervical lymphadenopathy, night sweats, myalgia, and rashes. The disease can be misdiagnosed as reactive follicular hyperplasia, tuberculous lymphadenitis, systemic lupus erythematosus, and malignant lymphoma. The diagnosis of KFD involves the excision of the affected lymph node. Although there is no specific treatment for the disease, usually symptomatic and supportive measures are effective; however, steroids and immunosuppressive therapies are considered in more severe cases. The disease lasts for around one to four months. The neurological complications include cerebellar ataxia, meningoencephalitis, and aseptic meningitis. Here, we describe the case of a 36-year-old male who presented with complaints of fever, malaise chills, anorexia, and fatigue associated with a tender right axillary lymph node. The patient underwent a biopsy which confirmed KFD and responded well to supportive therapy.
PubMed: 37332401
DOI: 10.7759/cureus.39098 -
Oncology Letters Jul 2023Mucosa-associated lymphoid tissue (MALT) lymphoma involving meningeal tissue is rare condition, easily mistaken for meningiomas upon imaging. In this report, a case of...
Mucosa-associated lymphoid tissue (MALT) lymphoma involving meningeal tissue is rare condition, easily mistaken for meningiomas upon imaging. In this report, a case of primary left temporal lobe MALT lymphoma that was initially misdiagnosed as temporal meningioma is presented, with subsequent investigation into the mechanism and treatments. Clinically, MALT lymphomas can be easily confused with meningiomas based solely on imaging and clinical manifestations. MALT lymphomas are indolent, localized lesions that can be cured through surgical resection and radiotherapy. Currently, radiotherapy is the most commonly used treatment; however, the patient in the present report did not receive any chemotherapy or radiotherapy after surgery, and recent related examinations revealed a recurrence of lymphomas that had metastasized throughout the body. As a result, future patients may benefit from chemotherapy or radiotherapy, and clinicians should be more meticulous regarding patient follow-up.
PubMed: 37274477
DOI: 10.3892/ol.2023.13863 -
Journal of Integrative Neuroscience May 2023Lesions of the central nervous system (CNS) can present with numerous and overlapping radiographical and clinical features that make diagnosis difficult based... (Review)
Review
Lesions of the central nervous system (CNS) can present with numerous and overlapping radiographical and clinical features that make diagnosis difficult based exclusively on history, physical examination, and traditional imaging modalities. Given that there are significant differences in optimal treatment protocols for these various CNS lesions, rapid and non-invasive diagnosis could lead to improved patient care. Recently, various advanced magnetic resonance imaging (MRI) techniques showed promising methods to differentiate between various tumors and lesions that conventional MRI cannot define by comparing their physiologic characteristics, such as vascularity, permeability, oxygenation, and metabolism. These advanced MRI techniques include dynamic susceptibility contrast MRI (DSC), diffusion-weighted imaging (DWI), dynamic contrast-enhanced (DCE) MRI, Golden-Angle Radial Sparse Parallel imaging (GRASP), Blood oxygen level-dependent functional MRI (BOLD fMRI), and arterial spin labeling (ASL) MRI. In this article, a narrative review is used to discuss the current trends in advanced MRI techniques and potential future applications in identifying difficult-to-distinguish CNS lesions. Advanced MRI techniques were found to be promising non-invasive modalities to differentiate between paraganglioma, schwannoma, and meningioma. They are also considered promising methods to differentiate gliomas from lymphoma, post-radiation changes, pseudoprogression, demyelination, and metastasis. Advanced MRI techniques allow clinicians to take advantage of intrinsic biological differences in CNS lesions to better identify the etiology of these lesions, potentially leading to more effective patient care and a decrease in unnecessary invasive procedures. More clinical studies with larger sample sizes should be encouraged to assess the significance of each advanced MRI technique and the specificity and sensitivity of each radiologic parameter.
Topics: Humans; Brain Neoplasms; Magnetic Resonance Imaging; Central Nervous System Neoplasms; Glioma; Meningeal Neoplasms
PubMed: 37258452
DOI: 10.31083/j.jin2203073 -
Case Reports in Oncological Medicine 2023Primary central nervous system lymphoma (PCNSL) is a rare, aggressive form of non-Hodgkin lymphoma contained in the brain and the spinal cord as well as the meninges,...
BACKGROUND
Primary central nervous system lymphoma (PCNSL) is a rare, aggressive form of non-Hodgkin lymphoma contained in the brain and the spinal cord as well as the meninges, cranial nerves, eyes, and cerebrospinal fluid (CSF). Due to its variable presentation and lack of associated B-symptoms, it is quite challenging to diagnose PCNSL, if there is not a high level of suspicion.
METHODS
This is a retrospective case series examining 13 human immunodeficiency virus- (HIV-) negative patients with PCNSL and DLBCL type, with a median age of 75 years old.
RESULTS
The most common presenting symptom was altered mental status. The frontal lobes, basal ganglia, cerebellum, and corpus callosum were most affected. Prior to brain biopsy, 4/13 patients were on steroids, which did not affect biopsy results and the average time to diagnosis was 1 month. 9/13 patients who did not receive steroids had an average time to diagnosis of less than 1 month.
CONCLUSION
Although steroid administration did not appear to diminish the yield of the biopsy, it is a best practice to withhold steroids prior to biopsy to decrease the time to diagnose PCNSL.
PubMed: 37228706
DOI: 10.1155/2023/8815502 -
Journal of Hematology Apr 2023Primary central nervous system lymphoma (PCNSL) is an aggressive form of extranodal non-Hodgkin lymphoma that arises in the brain parenchyma, eyes, meninges, or spinal...
Primary central nervous system lymphoma (PCNSL) is an aggressive form of extranodal non-Hodgkin lymphoma that arises in the brain parenchyma, eyes, meninges, or spinal cord in the absence of systemic disease. Primary dural lymphoma (PDL), in contrast, arises from the dura mater of the brain. PDL is usually a low-grade B-cell marginal zone lymphoma (MZL), whereas other types of PCNSL are usually high-grade large B-cell lymphoma. This specific pathological subtype has important therapeutic and prognostic implications, making PDL a distinct subtype of PCNSL. Herein, we report a case of PDL in an African American patient, in her late thirties, who presented to our emergency room with chronic headaches. An emergent magnetic resonance imaging (MRI) of the brain showed a dural-based homogeneously enhancing extra-axial mass along the left hemisphere, which was contained within the anterior and parietal dural mater. A surgical specimen was collected after an emergency debulking procedure. The flow cytometry, done on the surgical specimen obtained, was positive for CD19, CD20, and CD22, but negative for CD5 and CD10. These findings were consistent with a clonal B-lymphoproliferative disorder. The surgical pathology specimen immunohistochemistry was positive for CD20 and CD45, but negative for Bcl-6Cyclin D1 and CD56. The Ki67 was 10-20%. These findings were consistent with extranodal MZL. Given the location and pathology, the patient was diagnosed with PDL. Due to MZL's indolent nature, location outside the blood-brain barrier, and known efficacy to bendamustine-rituximab (BR), we decided to treat our patient with BR. She completed six cycles without major complications, and her post-therapy brain MRI showed complete remission (CR). Our case adds to the sparse literature about PDL and highlights the efficacy of BR systemic chemotherapy on MZLs.
PubMed: 37187500
DOI: 10.14740/jh1113 -
Indian Journal of Nuclear Medicine :... 2023Neurolymphomatosis is rarely encountered in high-grade lymphomas. In this case series, we retrospectively analyzed six neurolymphomatosis cases to look for possible risk...
Neurolymphomatosis is rarely encountered in high-grade lymphomas. In this case series, we retrospectively analyzed six neurolymphomatosis cases to look for possible risk factors, common and uncommon presentations, and the lessons learned. Neuropathic pain was the most common symptom with mono or polyradiculopathy in this series. However, all lymphomatous infiltrated nerves diagnosed on fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET/CT) were not symptomatic. The lumbar, brachial plexus, and trigeminal nerve were the most common sites and were depicted well on FDG PET/CT. Magnetic resonance imaging (MRI) of the brain better delineates cranial nerves and meningeal involvement. Cerebrospinal fluid flow cytometry was normal until meninges were involved. FDG PET/CT incrementally evaluated extra-neural disease sites, thus helping in deciding biopsy sites and further management. We concluded that a whole-body FDG PET/CT including limbs with MRI brain was the appropriate investigation for evaluating suspected neurolymphomatosis in advanced-stage diffuse large B-cell lymphoma.
PubMed: 37180178
DOI: 10.4103/ijnm.ijnm_165_22 -
Cureus Apr 2023Sarcoidosis is a systemic disease of unknown etiology with multi-system affection. It typically involves the skin, eyes, hilar lymph nodes, and pulmonary parenchyma....
Sarcoidosis is a systemic disease of unknown etiology with multi-system affection. It typically involves the skin, eyes, hilar lymph nodes, and pulmonary parenchyma. However, as any organ system could be involved, one has to be aware of its atypical manifestations. We present three uncommon manifestations of the disease. Our first case presented with fever, arthralgias, and right hilar lymphadenopathy with a history of tuberculosis in the past. He was treated for tuberculosis but had a relapse of symptoms three months after completion of treatment. The second patient presented with a headache for two months. On evaluation, cerebrospinal fluid examination showed evidence of aseptic meningitis, while an MRI of the brain demonstrated enhancement of the basal meninges. The third patient was admitted with a mass on the left side of the neck for one year. On evaluation, he was found to have left cervical lymphadenopathy, with its biopsy showing non-caseating epitheloid granuloma. Immunofluorescence did not show evidence of leukemia or lymphoma. All the patients had negative tuberculin skin tests and elevated serum angiotensin-converting enzyme levels supporting the diagnosis of sarcoidosis. They were treated with steroids with complete resolution of symptoms and no recurrence at follow-up. Sarcoidosis is an underdiagnosed entity in India. Thus, awareness of the atypical clinical features could lead to early recognition of the disease and its treatment.
PubMed: 37159764
DOI: 10.7759/cureus.37208 -
Journal of Clinical Medicine Apr 2023Invasive lobular carcinoma accounts for 10 to 15% of all breast cancers. The first objective of this retrospective study was to assess the diagnostic performance of...
BACKGROUND
Invasive lobular carcinoma accounts for 10 to 15% of all breast cancers. The first objective of this retrospective study was to assess the diagnostic performance of FDG-PET/CT scanning in women previously treated for invasive lobular carcinoma with suspected first recurrence. The secondary objectives were to evaluate the impact of PET/CT in a change in treatment and its prognostic value on specific survival.
METHODS
Patients in whom a PET/CT scan was performed from January 2011 to July 2019 in our Cancer Research Center were enrolled. Recurrence was suspected based on clinical symptoms, abnormal findings on conventional imaging, and/or elevated tumor markers. The diagnosis of recurrence was established by the oncologist after integration of all clinical, biological, histological, imaging, and follow-up data. Prognostic factors of recurrence as predicted by PET were determined using univariate logistic regression. KI67, mitotic index, or grade of mitosis were tested. Survival curves were compared using the log-rank test. Sixty-four patients (mean age: 60.3; SD = 12.4 years) were enrolled. The average time from initial diagnosis of the primary tumor to suspicion of recurrence was 5.2 ± 4.1 years. Forty-eight patients (75%) were judged to have recurrence by the oncologist: 7 local and 41 metastatic, with mainly bone ( = 24), lymph node ( = 14) and liver ( = 10) metastases.
RESULTS
Sensitivity, specificity, and positive and negative predictive values of PET/CT to predict recurrence were, respectively: 87%, 87%, 95%, and 70%. SUVmax at recurrence sites was generally high (mean: 6.4; SD = 2.9). False negative PET/CT results occurred with local ( = 2), peritoneal ( = 2), meningeal ( = 1), or bladder ( = 1) recurrences. In 40 patients with available histopathological data from suspected sites of recurrence, 30 PET/CT were true positive. In four patients, primary lung ( = 1) or gastric ( = 1) tumors or lymphomas ( = 2) were found. The detection of a recurrence resulted in a change in treatment in 44/48 patients (92%). No association between recurrence predicted by PET and biological biomarkers was found. Median specific survival appears shorter in patients with metastatic recurrence versus patients with local or no recurrence on PET/CT ( = 0.067).
CONCLUSIONS
FDG-PET/CT is an effective and reliable tool for the detection of invasive lobular carcinoma recurrence, although certain recurrence sites specific to this histological type can impair its diagnostic performance.
PubMed: 37109252
DOI: 10.3390/jcm12082916 -
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 -
Practical Laboratory Medicine Mar 2023Monoclonal protein bands are present mainly in blood and secondary in urine representing specific antibody produced in excess by abnormal lymphocytes or plasma cells.We...
INTRODUCTION
Monoclonal protein bands are present mainly in blood and secondary in urine representing specific antibody produced in excess by abnormal lymphocytes or plasma cells.We describe a case of a patient with acute encephalitis associated with an unexpected finding of a monoclonal protein band present in blood, urine and in cerebrospinal fluid (CSF).
CASE PRESENTATION
This 50-year-old woman with no significant past medical history, with the exception of unintentional weight loss exceeding 5 kg over the last 3 months, presented to the emergency department with seizures and altered mental status, after 3 days of vomiting and headaches. Magnetic Resonance Imaging showed lesions suspicious for infectious encephalitis/meningitis and for ischemia possibly related to central nervous system (CNS) autoimmune vasculopathy/vasculitis. The patient died the following day after losing brainstem reflexes. Testing for the previously mentioned etiologies returned negative with the exception of high protein concentration and increased immunoglobulin gamma (IgG) concentration in the CSF. Protein electrophoresis, ordered in error, showed a well-defined IgG with lambda light chain monoclonal protein band running in similar positions in serum, urine and in CSF. Due to SARS-CoV-2 PCR positivity no autopsy was performed.
CONCLUSION
The presence of this monoclonal protein band produced in the CNS suggests the diagnosis of CNS myeloma. The accelerated course in this case could be the result of the CNS myeloma or lymphoma responding to SARS-CoV-2 infection. Testing for monoclonal protein bands in CSF, in patients with pertinent clinical presentation would boost the awareness of this these diseases improving patient care.
PubMed: 37090932
DOI: 10.1016/j.plabm.2023.e00313