Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The current World Health Organization (WHO) classification of central nervous system tumors lists meningeal hemangiopericytomas (HPC) and meningeal solitary fibrous tumors (SFT) as separate entities. On the contrary, SFT and HPC of soft tissues are regarded in the WHO soft tissue fascicle as features of the same entity. The clinical data, histology, and immunohistochemistry of 18 cases of meningeal HPC and 12 cases of peripheral soft tissue HPC-SFT were compared. Both intracranial and soft tissue lesions had significant similarities that included staghorn vasculature, necrotic areas, cytologic atypia, and positivities for CD99, collagen IV, and reticulin. Nevertheless, intracranial tumors were more cellular than HPC-SFT of soft tissues and had fewer collagen bands. Meningeal HPC in addition had more mitoses, higher Ki67 index, stained less intensely for CD34 and B-cell lymphoma 2 (BCL2) than HPC-SFT of soft tissues. Meningeal HPCs recurred in 13 out of 14 cases (92.9%). One of the patients died in the postoperative period for a recurrent lesion 5 years after the diagnosis, and another patient developed an extracranial metastasis 13 years after surgery. None of the six cases of HPC-SFT of soft tissues available for follow-up recurred. Both meningeal and soft tissue tumors appear to represent different features of the same entity. A more aggressive phenotype of the tumor together with incomplete surgical resection of intracranial lesions might explain the noticeable clinical difference between HPC of the meninges and HPC-SFT of soft tissues.
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PMID:Meningeal hemangiopericytomas and hemangiopericytoma/solitary fibrous tumors of extracranial soft tissues: a comparison. 2016 66

Though rare, primary intracranial tumors can present with Parkinsonian symptoms, and diagnosis can be delayed unless there is a high index of suspicion. We herein present an 81-year-old man who was seen in our neurology clinic due to acute onset of unsteady gait and altered consciousness. Parkinsonism was initially diagnosed because of the typical manifestations. Levodopa was prescribed; however, there was a limited effect on his symptoms. Upon detail history and neurological examination, left sided hemiparesis was disclosed. Cerebral imaging studies revealed a solid mass over the right infratentorial para-midbrain area leading to reactive obstructive hydrocephalus. Work-up including chest and abdominal CT scanning, upper and lower GI endoscopy, and tumor marker studies failed to uncover any abnormalities. A neurosurgeon was consulted and a shunt procedure and biopsy of the infratentorial mass were performed. Histopathological examination of the biopsy tissue revealed tumor diffusely intermixed with large cells consistent with large B-cell lymphoma. The patient and his family declined further treatment. Though rare, cerebral tumors can present with Parkinsonian features and represent a diagnostic challenge. Clinicians should be aware of the possibility of cerebral neoplasms causing Parkinsonism, and include them in the differential diagnosis, especially for patients presenting with atypical Parkinsonian features, or those not responsive to initial therapy.
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PMID:Cerebral infratentorial large B-cell lymphoma presenting as Parkinsonism. 2020 13

OBJECT When intracranial tumors invade the overlying skull, gross resection typically includes removal of the involved bone. Methods used to repair the resulting structural defect in the cranium include artificial prostheses, allogeneic bone grafts, and autoclaving the autologous graft. The authors have previously reported a case involving high-dose extracorporeal ionizing radiation to treat the tumorous calvaria intraoperatively, followed by reimplantation of the treated bone flap. In this paper the authors report the long-term follow-up of that case, as well as results of using extracorporeal irradiation of tumorous calvaria (EITC) for an additional 20 patients treated similarly. METHODS The decision to undergo EITC was typically anticipated preoperatively, but determined intraoperatively, if upon inspection the bone flap was invaded by tumor. The bone flap was then delivered to the radiation oncology department, where a total dose of 120 Gy was delivered, using a clinical linear accelerator, over a period of approximately 15 minutes. After the intracranial tumor resection was completed, the irradiated craniotomy bone flap was reimplanted and the wound was closed in a standard fashion. A retrospective review of patients who had undergone EITC was performed for evidence of calvarial tumor recurrence or other complications. RESULTS Since the originally reported case, 20 additional patients have received EITC during craniotomy for invasive tumors. Eighteen (86%) of 21 patients were diagnosed with meningioma: 12 (67%) with WHO Grade I, 5 (28%) with WHO Grade II, and 1 with WHO Grade III (6%). The remaining 3 patients presented with dural-based B-cell lymphoma with extensive adjacent bone invasion (n = 2) and metastatic adenocarcinoma of the lung (n = 1). Follow-up of the 21 patients ranged from 1 to 132 months, with a mean of 41 months and a median of 23 months. No patients have experienced tumor recurrence, infection associated with the treated calvaria, or evidence of bone flap resorption. CONCLUSIONS Calvaria reconstructions represent an important component in structural and cosmetic outcome following craniectomy for tumorous bone. The authors' long-term experience with EITC has been excellent with no local tumor recurrence or complications. Therefore, EITC represents an excellent and efficient option for cranial reconstruction in such patients.
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PMID:Extracorporeal irradiation of tumorous calvaria: a case series. 2541 68

We describe an atypical man with diffuse large B cell lymphoma localized to the sphenoid wing and adjacent cavernous sinus, initially presenting with isolated ipsilateral facial pain mimicking trigeminal neuralgia due to invasion of Meckel's cave but subsequently progressing to intra-axial extension and having synchronous features of systemic lymphoma. Primary central nervous system lymphoma is uncommon, accounting for approximately 2% of all primary intracranial tumors, but its incidence has been steadily increasing in some groups [1]. It usually arises in the periventricular cerebral white matter, and reports of lymphoma in extra-axial regions are rare [2]. This man highlights the importance of maintaining lymphoma in the differential diagnosis of tumors of the skull base presenting with trigeminal neuralgia-like symptoms.
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PMID:Central nervous system lymphoma presenting as trigeminal neuralgia: A diagnostic challenge. 2586 26

A 27-year-old woman with acute lymphocytic leukemia, who underwent allogeneic hematopoietic stem cell transplantation, complained of nausea and blurred vision 288 days after the transplantation. Intracranial tumors were identified on brain MRI. She received whole brain radiation after open biopsy, but she died. The tumors had characteristics of diffuse large B cell lymphoma, and she was finally diagnosed with primary central nervous system post-transplant lymphoproliferative disorder. This disease is rare and has a poor outcome. Therefore, accumulation of cases and establishment of treatments for this condition are urgently needed.
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PMID:[Primary Central Nervous System Post-Transplant Lymphoproliferative Disorder in a Patient with Acute Lymphocytic Leukemia]. 2632 19

As the most common and lethal of intracranial tumors, glioma accounts for 81% of all malignant brain tumors. Research data have identified the role of microRNAs (miRs) as functional suppressors in the progression of Glioma. The present study aimed to, ascertain as to whether microRNA-499a (miR-499a) influences cell proliferation and apoptosis through the MAPK signaling pathway by targeting Notch1 in glioma. Both glioma and adjacent tissues between 2012-2016, were obtained from People's Hospital of Zhengzhou University (Henan Provincial People's Hospital). The collected glioma cells were treated with miR-449a mimic, miR-449a inhibitor, siRNA-Notch1, or SB230580 (an inhibitor of the MAPK signaling pathway). Verification of the targeting effect of miR-449a on Notch1 was provided by a dual-luciferase reporter gene assay. The expressions of miR-449a, Notch1, p38 mitogen-activated protein kinase (p38MAPK), extracellular regulated protein kinases (ERK1/2), B-cell lymphoma-2 (Bcl-2), Bcl-2 associated X protein (bax), CyclinD1, and phosphorylation of p38MAPK (p-p38MAPK) and ERK1/2 (p-ERK1/2) in tissues and cells were detected by means of reverse transcription quantitative polymerase chain reaction (RT-qPCR) and western blot analysis methods. Cellular processes of proliferation, cell cycle and apoptosis were evaluated by MTT and BrdU assays as well as flow cytometry, respectively. Notch1 was subsequently identified to be a target gene of miR-499a. After the cells were treated with miR-449a mimic, siRNA-Notch1 or SB230580, decreased expressions of Notch1, Bcl-2, CyclinD1, ERK1/2 and p-ERK1/2, cell proliferation as well as cells arrested at the S stage with elevated expressions levels of p38MAPK, p-p38MAPK, Bax, as well as increased cell apoptosis and number of cells arrested in G0/G1 stage were assessed. Taken together, based on the evidence obtained from the present study, assertions were subsequently made suggesting that MiR-499a targeted-inhibition of Notch1 may be a promising future therapeutic strategy for glioma treatment, by means of overexpressing of miR-499a resulting in the inhibition of glioma cell proliferation and promotion of cell apoptosis through suppression of the MAPK signaling pathway by decreasing Notch1.
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PMID:MicroRNA-499a decelerates glioma cell proliferation while accelerating apoptosis through the suppression of Notch1 and the MAPK signaling pathway. 2990 94

Radiation therapy is important for the comprehensive treatment of intracranial tumors. However, the molecular mechanisms underlying the pathogenesis of delayed cognitive dysfunction are not well-defined and effective treatments or prevention measures remain insufficient. In the present study, 60 adult male Wistar rats were randomly divided into three groups, which included a control, whole brain radiotherapy (WBRT) (single dose of 30 Gy of WBRT) and nimodipine (single dose of 30 Gy of WBRT followed by nimodipine injection intraperitoneally) groups. The rats were sacrificed 7 days or 3 months following irradiation. At 3 months, the Morris water maze test was used to assess spatial learning and memory function in rats. The results demonstrated that the WBRT group demonstrated a significantly impaired cognitive performance, decreased numbers of hippocampal Cornu Ammonis (CA)1 neurons and upregulated expression of caspase-3 in the dentate gyrus compared with those in the control and nimodipine groups. Reverse transcription-quantitative polymerase chain reaction analysis demonstrated that the WBRT group exhibited increased ratio of B-cell lymphoma 2 (Bcl-2)-associated X protein (Bax)/Bcl-2 compared with that in control and nimodipine groups on day 7 following irradiation. However, the WBRT group exhibited decreased levels of brain-derived neurotrophic factor (BDNF) compared with that in control and nimodipine groups at 3 months following brain irradiation. The levels of growth-associated protein 43 and amyloid precursor protein between the nimodipine group and WBRT group were not statistically significant. The present study demonstrated that neuron apoptosis may lead to delayed cognitive deficits in the hippocampus, in response to radiotherapy. The cognitive impairment may be alleviated in response to a calcium antagonist nimodipine. The molecular mechanisms involved in nimodipine-mediated protection against cognitive decline may involve the regulation of Bax/Bcl-2 and BDNF in the hippocampus.
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PMID:Delayed cognitive deficits can be alleviated by calcium antagonist nimodipine by downregulation of apoptosis following whole brain radiotherapy. 3001 47

Intravascular lymphoma (IVL) is a rare and clinically devastating subtype of extranodal diffuse large B-cell lymphoma with a distinct presentation. Diagnostic difficulty derives from marked variability in clinical presentations and nonspecific laboratory and radiological findings, especially when central nervous system (CNS) symptoms are the only manifestation. Establishing the diagnosis premortem thus remains a major challenge. We describe a 70-year-old male with CNS IVL. He presented with acute onset of neurocognitive impairments. Diffusion-weighted magnetic resonance imaging (MRI) showed multiple high-intensity areas suggesting occlusive cerebrovascular disease due to emboli, but extensive investigations detected no embolic sources. Intracranial neoplasm was included in a differential diagnosis based on elevated serum lactate dehydrogenase and interleukin 2 receptor levels. Gadolinium-enhanced MRI or 18-fluorodeoxyglucose positron emission tomography (PET) failed to demonstrate specific findings leading to a definite diagnosis, while 11C-methionine PET (MET-PET) distinctively demonstrated an area of focally increased MET uptake in the frontal cortex, suggesting the extent of tumor infiltration. Stereotactic biopsy was conducted under MET-PET imaging guidance and immunohistological examinations confirmed the proliferation and aggregation of CD20-positive lymphoma cells within the lumina of small blood vessels. The findings of the present case first suggest that MET-PET may provide important information on the diagnosis of CNS IVL and on the selection of the optimal site for brain biopsy. Further investigation is necessary to clarify whether positive findings on MET-PET are truly specific and pathognomonic for CNS IVL.
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PMID:Accurate Detection of Tumor Infiltration by 11C-Methionine Positron Emission Tomography in a Patient with Central Nervous System Intravascular Lymphoma: A Case Report. 3018 43