scholarly journals Various Neurological Symptoms by Neurolymphomatosis as the Initial Presentation of Primary Testicular Lymphoma

2015 ◽  
Vol 8 (1) ◽  
pp. 200-204 ◽  
Author(s):  
Yoshitaka Sunami ◽  
Akihiko Gotoh ◽  
Yasuharu Hamano ◽  
Yuriko Yahata ◽  
Hiroko Sakurai ◽  
...  

Neurological symptoms induced by the infiltration of malignant lymphoma into the nervous systems are subsumed under the term neurolymphomatosis (NL). Here, we report the case of a 30-year-old Japanese man with primary testicular lymphoma complicated, as seen in various neurological findings, by secondary NL prior to testicular swelling. Painless right scrotal enlargement was noticed more than 1 month after the appearance of neurological complications such as right upper extremity numbness, dysarthria, facial palsy, and diplopia. Proactive investigation and biopsies of extranodal sites at high risk of central nervous system infiltration of malignant lymphoma, such as the testes, should be considered when secondary NL is suspected based on imaging findings.

2020 ◽  
Vol 35 (11) ◽  
pp. 753-762
Author(s):  
Ronald R. Seese ◽  
Daniel Glaser ◽  
Andre Furtado ◽  
Kavita Thakkar ◽  
Kathryn S. Torok

Objective: Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Methods: Neuroimaging and neurological symptoms of children evaluated at our institution with a diagnosis of PRS or ECDS were retrospectively reviewed. Laterality, location, stability, and number of lesion(s) were evaluated, as was the presence of susceptibility lesion(s) and contrast enhancement. History of seizures or headaches was noted. Results: From 2003 to 2019, 80 patients with PRS or ECDS were followed at our institution. Neuroimaging was completed in 73 and found to be abnormal in 25. In 12 (48%) of these 25 cases, headaches and/or seizures were present. In the vast majority of these cases (22/25, 88%), lesions were ipsilateral to skin findings. White matter was involved in 19 (76%) patients. MRI abnormalities preceded a rheumatological diagnosis in 7 (28%). Susceptibility lesions were noted in 11 (44%), and 8 (73%) of these patients endorsed a history of headaches. Most lesions were in the supratentorial compartment, did not enhance, and were stable at 1-year follow up imaging. Of those with progression, susceptibility findings were present at baseline. Conclusions: Neuroimaging findings in pediatric PRS and ECDS are often supratentorial, stable, unilateral, and ipsilateral to skin findings, and they can precede cutaneous findings.


2013 ◽  
Vol 154 (42) ◽  
pp. 1666-1673
Author(s):  
János László Iványi ◽  
Éva Marton ◽  
Márk Plander ◽  
Zoltán Vendel Engert ◽  
Csaba Tóth

Introduction: Primary testicular lymphoma constitutes a rare subgroup among extranodal non-Hodgkin’s lymphomas. Because of its aggressive clinical behaviour due to high grade histological features developing mainly in older population, patients with this disease usually have a poor prognosis. Orchidectomy followed by combination immunochemotherapy is a traditional treatment method with a rather inferior outcome. Aim: In this retrospective survey the authors analysed the clinical presentation, pathological features and treatment results of patients with primary testicular lymphoma diagnosed and treated in their haematology centre between 2000–2012 Method: During this period 334 patients with aggressive non-Hodgkin’s lymphomas were treated, of whom 8 patients (2.39%; age between 23 and 86 years; median, 60 years) underwent semicastration for primary testicular lymphoma (7 patients had diffuse, large B-cell lymphoma and one patient had Burkitt-like lymphoma). According to the Ann Arbor staging system a limited stage I-IIE was diagnosed in 7 patients and advanced stage was found in one patient. All but one patients were treated with rituximab added to CHOP regimen (6 or 8 cycles in every 21 or 28 days), whereas one patient received radiotherapy only. Central nervous system intrathecal prophylaxis was used in one case and no preventive irradiation of the contralateral testis was used. Results: With a median follow-up of 50 months complete remission was observed in 7 patients. However, two patients died (one due to progression and one in remission from pulmonary solid tumour). Complete remission rate proved to be 87.5%, disease-free survival was between 13 and 152 months (median 38 months) and overall survival rates were between 17 and 156 months (median 43 months). The 5-year disease-free and overall survival rates were 37.5 %. Conclusions: The relatively favourable treatment outcome could be mainly explained by the high number of patients with early-stage of the disease, early surgical removal of testicular lymphomas and the use if immunochemotherapy. This therapeutic regimen was effective to prevent localized and distant relapses. Despite omission of regular prophylaxis of the central nervous system, no relapse was detected. Orv. Hetil., 154 (42), 1666–1673.


2020 ◽  
pp. 186-187
Author(s):  
O.A. Loskutov

Background. Coronavirus disease (COVID-19) affects not only the alveoli but also the central nervous system. The pathogenesis of neurological complications of COVID-19 is based on the receptor damage, cytokine-mediated injury, damage of the nervous system due to hypoxia, and neuronal damage due to the retrograde transport of the virus through nerve fibers. Objective. To determine the mechanisms of coronavirus neuroinvasion and treatment of neurological complications COVID-19. Materials and methods. Analysis of literature sources and own research on this topic. Results and discussion. In 3 days after infection viral antigens are detected in the olfactory sensory neurons of the nasal cavity epithelium, which confirms the transnasal invasion of coronaviruses. According to various authors, 36.4-84 % of patients with COVID-19 have neurological manifestations. In general, neurological symptoms of COVID-19 can be divided into three categories: symptoms of the central nervous system disorders (headache, dizziness, consciousness disorders, nausea and vomiting, ataxia, acute cerebrovascular disease, and epilepsy), symptoms of the peripheral nervous system disorders (hypogeusia, hyposmia, hypopsia, and neuralgia) and musculoskeletal symptoms. As viral infections cause cytokine storm, inflammation and hyperactive oxidation, it is advisable to use antioxidants in their treatment. According to the Japanese clinical guidelines for the treatment of acute respiratory distress syndrome, edaravone may be used as a free radical scavenger in such patients. In the experiment, edaravone reduced the area of infarction and promoted functional recovery in cerebral thrombosis. Other properties of edaravone include the prevention of excessive permeability of the pulmonary vessels’ endothelium. Own research has shown that edaravone (Ksavron, “Yuria-Pharm”) reduces the severity of inflammation and mortality in patients with COVID-19. In the control group, the level of the proinflammatory mediator interleukin-6 exceeded the upper limit of normal values by 1652.40 %, and in the Ksavron group – only by 269.97 %. Mortality in the control group was 14.3 %, and in the Ksavron group – 0 %. Conclusions. 1. Coronaviruses affect not only the lungs but also the nervous system. 2. Neurological symptoms of COVID-19 include headache, dizziness, consciousness disorders, nausea and vomiting, ataxia, acute cerebrovascular disease, hypogeusia, hyposmia, hypopsia, neuralgia, and musculoskeletal symptoms. 3. Edaravone (Ksavron) reduces the severity of inflammation and has an antioxidant effect, which justifies its use in COVID-19.


2015 ◽  
Vol 37 (3) ◽  
pp. 223-226 ◽  
Author(s):  
Y Kemal ◽  
F Teker ◽  
G Demirag ◽  
I Yucel

Aim: Primary testicular lymphoma (PTL) is an uncommon and aggressive form of extranodal non-Hodgkin’s lymphoma (NHL). We aimed to analyse the clinicopathological characteristics and outcomes of our PTL cases. Materials and Methods: A review was made of the medical records of 339 NHL patients who were treated in the Medical Oncology Department between January 2005 and December 2013. Results: 8 PTL patients were identified from the 339 NHL patients. The average age of the patients was 67.7 ± 7.9 years (range 53–79 years). The mean follow-up time was 24.8 months (range 7–98 months). Inguinal orchiectomy was performed as a diagnostic and initial therapy and all the patients underwent 4–6 cycles of chemoimmunotherapy consisting of cyclophosphamide, doxorubicin, vincristine and prednisone plus rituximab. 4 of 8 patients received intrathecal prophylactic chemotherapy and 6 of 8 patients continued contralateral testis irradiation. Relapse occured in only 1 patient in central nervous system after 6 months who had not received intrathecal prophylaxis. No contralateral testis relapse was observed. Conclusions: Primary testicular NHL is an uncommon entity and we evaluated 8 patients; with one relapse in central nervous system and no relapse in the contralateral testis.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 930-930 ◽  
Author(s):  
Lakshmi Nayak ◽  
Fabio Iwamoto ◽  
Ann S. LaCasce ◽  
Srinivasan Mukundan ◽  
Margaretha G M Roemer ◽  
...  

Abstract Background: Primary central nervous system lymphoma (PCNSL) and primary testicular lymphoma (PTL) are rare and aggressive extranodal non-Hodgkin lymphomas with shared molecular features and a poor prognosis. PCNSL is currently treated with high-dose methotrexate (HD-MTX) based induction chemotherapy. Younger patients who are healthy and eligible for consolidation treatment are considered for whole brain radiation (WBRT) or high-dose chemotherapy with autologous stem-cell transplant (ASCT). Despite this, nearly 50% of patients with PCNSL relapse within 2 years of diagnosis and one third of patients have primary refractory disease. PTL is treated with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP). However, almost 50% of patients with PTL progress after induction treatment, frequently with CNS or contralateral testicular involvement. Treatment options are limited for patients with recurrent and refractory PCNSL or PTL, and the majority of patients ultimately die of their disease. We recently identified frequent 9p24.1/ PD-L1(CD274)/ PD-L2 (PDCD1LG2) copy number alterations and additional translocations in PCNSL and PTL (Chapuy and Roemer et al Blood 2016; 127:869-81). These genetic alterations are associated with increased expression of the programmed cell death protein 1 (PD-1) ligands, PD-L1 and PD-L2. Activation of PD-1 signaling by PD-L1 or PD-L2 binding leads to a reversible state of T-cell "exhaustion" characterized by decreased T-cell receptor signaling, reduced T-cell proliferation and survival, perturbed metabolism and altered transcription factor expression. The activity of PD-1 blockade in other lymphomas with 9p24.1 alterations such as classical Hodgkin's lymphoma prompted us to test the efficacy of this approach in PCNSL and PTL. Patients and Methods: We treated 5 patients with recurrent/refractory PCNSL and PTL with off-label Nivolumab, a humanized IgG4 monoclonal antibody checkpoint inhibitor that targets PD-1 and blocks binding by PD-L1 or PD-L2. Median age was 64 (range, 54-85) years with a median Karnofsky Performance Score (KPS) of 70 (40-80) %. Four patients had multiply recurrent disease (3 patients with PCNSL and 1 patient with PTL and CNS relapse); 1 patient had primary refractory PCNSL. All patients had been treated with standard of care treatments and had no other available options. Prior therapies included HD-MTX based chemotherapy, pemetrexed, high-dose cytarabine, WBRT and thiotepa-based chemotherapy followed by ASCT. All patients signed informed consent and were treated with nivolumab at 3mg/kg intravenously every 2 weeks. Only 1 patient received corticosteroids (dexamethasone 2mg daily) during initial nivolumab treatment; steroids were rapidly tapered and discontinued in 4 weeks. Additionally, 1 patient received 3 doses of rituximab and 2 others received WBRT. All patients had objective radiographic responses to treatment with nivolumab, including 4 complete responses and 1 partial response. The patient with CNS relapse from PTL had complete radiographic resolution of CNS abnormalities with persistent intraocular disease that required vitrectomy. All responses were confirmed at repeat restaging. The median number of nivolumab treatments to objective radiographic response was 3 (range, 2-4). The 4 patients who were symptomatic at the initiation of nivolumab therapy had complete or near-complete resolution of neurologic signs/symptoms and a marked improvement in KPS. All patients are alive and remain progression-free at this time. Four patients continue to receive treatment. The median progression-free survival is 9 (range, 7-11) months. Conclusion: Our pilot series indicates that nivolumab is active in primary refractory and relapsed/refractory PCNSL and PTL with CNS relapse. Based on our preclinical molecular data and these pilot clinical results, a multi-institutional phase 2 open-label, single-arm trial of nivolumab in recurrent and refractory PCNSL and PTL patients (CA209-647) is scheduled to open shortly. Disclosures LaCasce: Forty Seven: Consultancy; Seattle Genetics: Consultancy. Armand:Infinity Pharmaceuticals: Consultancy; Merck: Consultancy, Research Funding; Pfizer: Research Funding; Roche: Research Funding; Sequenta Inc: Research Funding; Bristol-Myers Squibb: Consultancy, Research Funding. Shipp:Bayer: Research Funding; Merck, Gilead, Takeda: Other: Scientific Advisory Board; Cell Signaling: Honoraria; Bristol-Myers Squibb: Consultancy, Research Funding.


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