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Handbook of Clinical Neurology 2024Although they are relatively rare, the diagnosis of paraneoplastic neurologic syndromes (PNS) can be aided by the identification of neural autoantibodies in patients'... (Review)
Review
Although they are relatively rare, the diagnosis of paraneoplastic neurologic syndromes (PNS) can be aided by the identification of neural autoantibodies in patients' serum and cerebrospinal fluid (CSF). They often clinically manifest as characteristic syndromes, including limbic encephalitis, opsoclonus-myoclonus syndrome, paraneoplastic cerebellar degeneration, and paraneoplastic encephalomyelitis. The antibodies are directed either toward intracellular targets, or epitopes on the cell surface. As compared to cell surface antibodies, intracellular paraneoplastic autoantibodies are more classically associated with cancer, most often lung, breast, thymoma, gynecologic, testicular, and/or neuroendocrine cancers. The malignancies themselves tend to be small and regionally contained, attesting to the strength of the immune system in cancer immunosurveillance. Typically, the intracellular antibodies are not directly pathogenic and tend to be associated with PNS that are poorly responsive to treatment. With some notable exceptions, including patients with PNS associated with testicular cancer, patients with intracellular antibodies are typically older individuals, in their 7th decade of life and beyond. Many of them are current or former smokers. Treatment strategies include tumor removal as well as immunotherapy to treat the concomitant PNS. Newer technologies and the ever-broadening use of cancer immunotherapies are contributing to the continued identification of novel intracellularly targeted autoantibodies.
Topics: Male; Humans; Female; Testicular Neoplasms; Paraneoplastic Syndromes, Nervous System; Autoantibodies; Limbic Encephalitis; Immunotherapy
PubMed: 38494288
DOI: 10.1016/B978-0-12-823912-4.00021-9 -
Handbook of Clinical Neurology 2024Paraneoplastic neurologic syndromes (PNS) represent a rare group of immune-mediated complications associated with an underlying tumor. Ectopic protein expression in... (Review)
Review
Paraneoplastic neurologic syndromes (PNS) represent a rare group of immune-mediated complications associated with an underlying tumor. Ectopic protein expression in neoplastic cells or an aberrant immune regulation in the course of hematooncologic diseases or thymomas trigger an autoimmune response that may affect any part of the central and/or peripheral nervous system. Recent advances in drug therapies as well as novel animal models and neuropathologic studies have led to further insights on the immune pathomechanisms of PNS. Although the syndromes share common paths in pathogenesis, they may differ in the disease course, prognosis, and therapy targets, depending on the localization and type of antibody epitope. Neuropathologic hallmarks of PNS associated with antibodies directed against intracellular epitopes are characterized by T cell-dominated inflammation, reactive gliosis including microglial nodules, and neuronal degeneration. By contrast, the neuropathology of cell surface antibody-mediated PNS strongly depends on the targeted antigen and varies from B cell/plasma cell-dominated inflammation and well-preserved neurons together with a reduced expression of the target antigen in anti-NMDAR encephalitis to irreversible Purkinje cell loss in anti-P/Q-type VGCC antibody-associated paraneoplastic cerebellar degeneration. The understanding of different pathomechanisms in PNS is important because they strongly correspond with therapy response and prognosis, and should guide treatment decisions.
Topics: Animals; Humans; Autoantibodies; Paraneoplastic Syndromes, Nervous System; Nervous System Diseases; Neoplasms; Inflammation
PubMed: 38494287
DOI: 10.1016/B978-0-12-823912-4.00027-X -
Handbook of Clinical Neurology 2024Myasthenia gravis (MG) is an autoimmune disease characterized by dysfunction of the neuromuscular junction resulting in skeletal muscle weakness. It is equally prevalent... (Review)
Review
Myasthenia gravis (MG) is an autoimmune disease characterized by dysfunction of the neuromuscular junction resulting in skeletal muscle weakness. It is equally prevalent in males and females, but debuts at a younger age in females and at an older age in males. Ptosis, diplopia, facial bulbar weakness, and limb weakness are the most common symptoms. MG can be classified based on the presence of serum autoantibodies. Acetylcholine receptor (AChR) antibodies are found in 80%-85% of patients, muscle-specific kinase (MuSK) antibodies in 5%-8%, and <1% may have low-density lipoprotein receptor-related protein 4 (Lrp4) antibodies. Approximately 10% of patients are seronegative for antibodies binding the known disease-related antigens. In patients with AChR MG, 10%-20% have a thymoma, which is usually detected at the onset of the disease. Important differences between clinical presentation, treatment responsiveness, and disease mechanisms have been observed between these different serologic MG classes. Besides the typical clinical features and serologic testing, the diagnosis can be established with additional tests, including repetitive nerve stimulation, single fiber EMG, and the ice pack test. Treatment options for MG consist of symptomatic treatment (such as pyridostigmine), immunosuppressive treatment, or thymectomy. Despite the treatment with symptomatic drugs, steroid-sparing immunosuppressants, intravenous immunoglobulins, plasmapheresis, and thymectomy, a large proportion of patients remain chronically dependent on corticosteroids (CS). In the past decade, the number of treatment options for MG has considerably increased. Advances in the understanding of the pathophysiology have led to new treatment options targeting B or T cells, the complement cascade, the neonatal Fc receptor or cytokines. In the future, these new treatments are likely to reduce the chronic use of CS, diminish side effects, and decrease the number of patients with refractory disease.
Topics: Female; Humans; Male; Autoantibodies; Electromyography; Immunosuppressive Agents; Myasthenia Gravis; Neuromuscular Junction
PubMed: 38494283
DOI: 10.1016/B978-0-12-823912-4.00026-8 -
Seminars in Nephrology Jan 2024End-stage kidney disease patients who are referred for transplant undergo an extensive evaluation process to ensure their health prior to transplant due in part to the... (Review)
Review
End-stage kidney disease patients who are referred for transplant undergo an extensive evaluation process to ensure their health prior to transplant due in part to the shortage of available organs. Although management and surveillance guidelines exist for malignancies identified in the transplant and waitlist populations, less is written about the management of premalignant lesions in this population. This review covers the less common premalignant lesions (intraductal papillary mucinous neoplasm, gastrointestinal stromal tumor, thymoma, and pancreatic neuroendocrine tumor) that can be found in the transplant candidate population. High-level evidence for the management of these rarer premalignant lesions in the transplant population is lacking, and this review extrapolates evidence from the general population and should not be a substitute for a multidisciplinary discussion with medical and surgical oncologists.
Topics: Humans; Kidney Transplantation; Precancerous Conditions; Kidney Failure, Chronic; Gastrointestinal Stromal Tumors; Thymoma; Pancreatic Neoplasms; Thymus Neoplasms
PubMed: 38490902
DOI: 10.1016/j.semnephrol.2024.151495 -
Cancer Biotherapy & Radiopharmaceuticals Jun 2024The aim of this study was to evaluate the potential role of [F]FDG positron emission tomography/computed tomography (PET/CT) in the characterization of thymic...
The aim of this study was to evaluate the potential role of [F]FDG positron emission tomography/computed tomography (PET/CT) in the characterization of thymic epithelial tumors (TETs). A total of 73 patients who underwent preoperative [F]FDG PET/CT were included in this study. Visual total score (VTS), maximum standard uptake values (SUV), metabolic tumor volume (MTV), total lesion glycolysis (TLG), and heterogeneity index (HI) parameters were analyzed to investigate the prediction of histopathologic grade and advanced stage. The cohort included 26 patients with low-grade thymoma (LGT), 36 patients with high-grade thymoma (HGT), and 11 patients with thymic carcinoma (TC). Ninety-one percent of TC had VTS >2, whereas 31% of LGT and 75% of HGT had VTS >2. SUV, MTV, and TLG were statistically significantly higher in the TC group than in both thymoma and HGT. Using the cutoff value of 7.25 for SUV, TC was differentiated from thymomas with 91% sensitivity and 74% specificity. TC had significantly lower HI values than thymomas. HI parameters showed good diagnostic ability to differentiate TC from thymoma and TC from HGT. SUV, MTV, and TLG were significantly higher in advanced-stage disease than in early-stage disease. Visual and quantitative parameters can reliably predict both advanced disease and the grade of primary tumor in TETs. Therefore, as a promising metabolic imaging method, [F]FDG PET/CT makes important contributions to preoperative evaluation in routine clinical practice.
Topics: Humans; Thymus Neoplasms; Fluorodeoxyglucose F18; Male; Positron Emission Tomography Computed Tomography; Female; Middle Aged; Aged; Adult; Neoplasms, Glandular and Epithelial; Radiopharmaceuticals; Neoplasm Staging; Retrospective Studies; Aged, 80 and over; Thymoma
PubMed: 38484307
DOI: 10.1089/cbr.2023.0192 -
Thoracic Cancer Apr 2024Metastasis from one neoplasm to another is referred to as tumor-to-tumor metastasis (TTM). TTM is rarely observed. Here, we present a patient with TTM from a thymic...
Metastasis from one neoplasm to another is referred to as tumor-to-tumor metastasis (TTM). TTM is rarely observed. Here, we present a patient with TTM from a thymic carcinoma to an ovarian mature teratoma. A 25-year-old woman, diagnosed with unresectable thymic carcinoma, presented with a cyst with a solid tumor component in her right ovary. Laparoscopic cystectomy of the right ovary revealed that the solid tumor was a distant metastasis of the thymic carcinoma in an ovarian mature teratoma. The possibility of malignant transformation of the ovarian mature teratoma was ruled out, enabling accurate staging of the thymic carcinoma. This case emphasizes the need for clinicians to consider TTM and the importance of pathological confirmation of TTM when investigating potential distant metastases.
Topics: Female; Humans; Adult; Thymoma; Ovarian Neoplasms; Teratoma; Thymus Neoplasms
PubMed: 38468427
DOI: 10.1111/1759-7714.15277 -
Revue Des Maladies Respiratoires Apr 2024Primary thoracic cancers affect a large number of patients, mainly those with lung cancer and to a lesser extent those with pleural mesothelioma and thymic tumours.... (Review)
Review
Primary thoracic cancers affect a large number of patients, mainly those with lung cancer and to a lesser extent those with pleural mesothelioma and thymic tumours. Given their frequency and associated comorbidities, in patients whose mean age is high, these diseases are associated with multiple complications. This article, the last of a series dedicated to emergencies in onco-haematological patients, aims to present a clinical picture of the severe complications (side effects, immune-related adverse events) associated with systemic treatments, excluding infections and respiratory emergencies, with which general practitioners and specialists can be confronted. New toxicities are to be expected with the implementation of innovative therapeutic approaches, such as CAR-T cells, along with immunomodulators and antibody-drug conjugates.
Topics: Humans; Emergencies; Mesothelioma; Mesothelioma, Malignant; Thymoma; Lung Neoplasms; Pleural Neoplasms
PubMed: 38461088
DOI: 10.1016/j.rmr.2024.02.014 -
Journal of Robotic Surgery Mar 2024The conventional supine position for robotic thymectomy may present challenges in accessing the lower thymic horns, particularly in cases requiring extensive resection...
The conventional supine position for robotic thymectomy may present challenges in accessing the lower thymic horns, particularly in cases requiring extensive resection of mediastinal fat. To address this issue, the authors advocate for a lateral patient position during the procedure, emphasizing optimized access to the thymic horns and improved procedural efficacy. The lateral approach involves specific trocar placements and port arrangements to minimize conflicts between instruments. This report proposes an innovative approach to robotic thymectomy for patients diagnosed with thymoma or thymic hyperplasia associated with myasthenia gravis.
Topics: Animals; Humans; Thymectomy; Robotic Surgical Procedures; Thymus Gland; Posture; Thymus Neoplasms
PubMed: 38441746
DOI: 10.1007/s11701-024-01889-8 -
Folia Medica Feb 2024Resection and reconstruction of the superior vena cava (SVC) are required in a selected group of patients with anterior mediastinal tumors and lung neoplasms. We present...
Resection and reconstruction of the superior vena cava (SVC) are required in a selected group of patients with anterior mediastinal tumors and lung neoplasms. We present the case of a 63-year-old woman who underwent invasive type B2 thymoma resection and a rare type of reconstruction of the superior vena cava using a patch of the left brachiocephalic vein (LBV). The various types of reconstruction of the superior vena cava are discussed.
Topics: Female; Humans; Middle Aged; Vena Cava, Superior; Brachiocephalic Veins; Mediastinum; Thymoma; Thymus Neoplasms
PubMed: 38426478
DOI: 10.3897/folmed.66.e102981 -
CNS Neuroscience & Therapeutics Feb 2024This comprehensive review aimed to compile cases of patients with thymoma diagnosed with both autoimmune encephalitis (AE) and myasthenia gravis (MG), and describe their... (Review)
Review
OBJECTIVES
This comprehensive review aimed to compile cases of patients with thymoma diagnosed with both autoimmune encephalitis (AE) and myasthenia gravis (MG), and describe their clinical characteristics.
METHODS
Clinical records of 3 AE patients in the first affiliated hospital of Sun Yat-sen University were reviewed. All of them were diagnosed with AE between 1 November 2021 and 1 March 2022, and clinical evidence about thymoma and MG was found. All published case reports were searched for comprehensive literature from January 1990 to June 2022.
RESULTS
A total of 18 cases diagnosed with thymoma-associated autoimmune encephalitis (TAAE) and thymoma-associated myasthenia gravis (TAMG) were included in this complication, wherein 3 cases were in the first affiliated hospital of Sun Yat-sen University and the other 15 were published case reports. 5/18 patients had alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antibody (AMPAR-Ab) in their serum and cerebrospinal fluid (CSF). All of them had positive anti-acetylcholine receptor antibody (AChR-Ab). And 12/18 patients showed a positive response to thymectomy and immunotherapy. Besides, thymoma recurrences were detected because of AE onset. And the shortest interval between operation and AE onset was 2 years in patients with thymoma recurrence.
CONCLUSIONS
There was no significant difference in the clinical manifestations between these patients and others with only TAMG or TAAE. TAAE was commonly associated with AMPAR2-Ab. Significantly, AE more commonly heralded thymoma recurrences than MG onset. And the intervals of thymectomy and MG or AE onset had different meanings for thymoma recurrence and prognoses of patients.
Topics: Humans; Thymoma; Thymus Neoplasms; Myasthenia Gravis; Encephalitis; Hashimoto Disease
PubMed: 38421083
DOI: 10.1111/cns.14568