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Waldenstrom macroglobulinaemia (WM), the predominant subtype of lymphoplasmacytic lymphoma with bone marrow involvement and serum IgM paraprotein, is a haematological condition commonly associated with renal parenchymal involvement. However, antineutrophil cytoplasmic antibody (ANCA)‐negative pauci‐immune crescentic glomerulonephritis (PICGN) in kidney infiltrated by lymphoma is very rare, with only two cases described in chronic lymphocytic leukaemia in English literature so far. We herein report the first patient with WM developing ANCA‐negative PICGN. He was a 76‐year‐old male who presented with elevated serum globulin level and bilateral groin lymph node enlargement, subsequently diagnosed to have WM after pathologic examination of the bone marrow and groin lymph node. One month later, he was found to have acute kidney injury and proteinuria. Renal biopsy confirmed the presence of parenchymal involvement by WM accompanied by PICGN; while ANCA testing was negative. He was treated with pulse methylprednisolone followed by oral prednisolone. In addition, six courses of intravenous rituximab and oral cyclophosphamide were given. There was significant improvement in both his renal and haematological conditions. The clinical course of this case suggested that ANCA‐negative PICGN may represent a paraneoplastic syndrome and a rare manifestation of WM‐associated renal lesion. Early kidney biopsy and prompt treatment may improve the outcome of patients. [ABSTRACT FROM AUTHOR]

PurposeMethodResultsConclusionTo report a case of asymmetric melanoma-associated retinopathy (MAR) associated with metastatic melanoma which was thought to be in remission for 6 years. Identification of MAR led to the discovery of recurrent malignancy.A man in his 60s presented with monocular visual disturbances with a large relative afferent pupillary defect, rapidly progressing visual field defect and otherwise normal eye examination. Initial work-up for retrobulbar optic neuropathy was inconclusive. After a few months, similar symptoms developed in his fellow eye and a full-field electroretinogram revealed a reduced b:a wave ratio suspicious for MAR.Visual field defects were present in both eyes at initial examination, but the visual field of one eye progressed rapidly while the fellow eye did not develop symptoms or progress until roughly 3 months later. Visual field defects and symptoms improved following resection of the lymph node with active metastatic disease and serum plasmapheresis.This report highlights a case of MAR with asymmetric objective findings and progression of visual field defects. It also demonstrates the success of plasmapheresis, in combination with treating recurrent metastatic disease, in improving visual function. [ABSTRACT FROM AUTHOR]

Key clinical message: Small cell lung cancer (SCLC), a neuroendocrine aggressive subtype of lung cancer, is associated with paraneoplastic disorders in about 9% of patients. In this report, we describe a middle‐aged man who presented with chronic bowel obstruction caused by chronic intestinal pseudo‐obstruction (CIPO) due to SCLC. [ABSTRACT FROM AUTHOR]

Opsoclonus-myoclonus syndrome (OMS) is a rare neuroinflammatory disorder characterized by ataxia, opsoclonus, and myoclonus. Clinical diagnosis of OMS has been challenging; therefore, we sought to determine the clinical and treatment profiles of patients with OMS at the largest pediatric hospital in Latin America. We analyzed the data of patients diagnosed with OMS between 2010 and 2020 at Pequeno Principe Hospital (Brazil) to determine the corresponding clinical profile more accurately. Of the approximately 50,000 visitors to our pediatric neurology department from 2010 to 2020, 10 patients with OMS were observed. Five nontumor cases included three parainfectious and two idiopathic cases. The median time from symptom onset to diagnosis was 34 days. All patients with diagnostic OMS criteria in the idiopathic, nontumor group underwent whole-exome sequencing, with potentially pathogenic mutations identified in two cases. Nine patients were treated with methylprednisolone pulse, followed by oral steroids; eight received one or more intravenous immunoglobulin treatments; and six received azathioprine and cyclophosphamide. Complete symptomatic recovery was observed in only one patient. OMS diagnosis remains challenging. Diagnostic suspicion is necessary to improve the management of these patients and allow early immunosuppressive treatment. Paraneoplastic etiology is the most prevalent. In idiopathic patients who do not respond to immunosuppressive treatment, tests, such as whole-exome sequencing, may reveal a differential diagnosis. Genetic alterations that increase the risk of tumors may be an important clue to the pathophysiology of OMS. [ABSTRACT FROM AUTHOR]

Pruritus, defined as an unpleasant sensation that compels scratching, is a common occurrence in dermatological disorders. Chronic pruritus is defined as itching that persists for a period exceeding six weeks. Lichen simplex chronicus (LSC) is a chronic dermatological condition that presents with dry, scaly, and thickened skin lesions, which typically arise from habitual scratching. Additionally, paraneoplastic pruritus may serve as an early indicator of the presence of solid tumours. This case report outlines the diagnostic and therapeutic process of a 73-year-old male diagnosed with lichen simplex chronicus (LSC), confirmed by skin biopsy. Subsequent malignancy screening revealed the presence of solid nodules in the lungs and multiple metastatic masses in the liver. Biopsies of the lung nodule and liver mass demonstrated positivity for TTF-1 and synaptophysin, leading to a diagnosis of mixed component lung adenocarcinoma and small cell lung cancer. In order to ascertain the underlying cause of the patient's chronic itching, a comprehensive investigation was conducted, which considered a range of potential factors, including haematological diseases, endocrine disorders, liver diseases, infections, drug-related factors, and neurological and psychiatric causes. Despite a comprehensive evaluation, the precise etiology of the chronic itching remained elusive. The initiation of carboplatin and etoposide therapy for lung cancer resulted in a significant reduction in the patient's itching, which may suggest a possible link between chronic itching and malignancy. Unfortunately, the patient succumbed to hypoxia, pneumonia, and multiple organ failure two months post-diagnosis. This case serves to illustrate the potential of chronic pruritus as a symptom of cancer. While paraneoplastic pruritus is often associated with haematological malignancies, this report highlights that it can also occur with solid tumours. It is important for clinicians to be aware of this potential association, as it allows for timely diagnosis and treatment. [ABSTRACT FROM AUTHOR]

PNS are uncommon manifestations of cancer. The current literature about these syndromes in the setting of cHL is disintegrated. A systematic literature review of all published literature was conducted. One hundred twenty-eight patients from 115 publications met the inclusion/exclusion criteria. Eight-five patients were of the NS subtype (66.4%). The most frequent clinical presentation of the PNS was CNS manifestation (25.8%). The majority of patients were diagnosed with the cHL and PNS simultaneously (42.2%). In 33.6% of patients, the lymphoma diagnosis preceded the PNS diagnosis. In 16.4% of patients, the PNS diagnosis preceded the lymphoma diagnosis. The presence of PNS antibodies was reported in 35 patients (27.3%). Age older than 18 was associated with higher prevalence of PNS. The CR rate of the lymphoma was 77.3%. The complete resolution rate of the PNS was 54.7%. Relapse of lymphoma was reported in 13 patients, and recurrence of the PNS upon relapse was reported in 10/13 patients. [ABSTRACT FROM AUTHOR]

Neuroendocrine prostate cancer (NEPC) is a rare subtype of prostate cancer (PCa) that usually results in poor clinical outcomes and may be accompanied by paraneoplastic syndromes (PNS). NEPC is becoming more frequent. It can initially manifest as PNS, complicating diagnosis. Therefore, we reviewed the literature on the different PNS associated with NEPC. We systematically reviewed English-language articles from January 2017 to September 2023, identifying 17 studies meeting PRISMA guidelines for NEPC and associated PNS. A total of 17 articles were included in the review. Among these, Cushing's Syndrome (CS) due to ectopic Adrenocorticotropic hormone (ACTH) secretion was the most commonly reported PNS. Other PNS included syndrome of inappropriate Anti-Diuretic Hormone secretion (SIADH), Anti-Hu-mediated chronic intestinal pseudo-obstruction (CIPO), limbic encephalitis, Evans Syndrome, hypercalcemia, dermatomyositis, and polycythemia. Many patients had a history of prostate adenocarcinoma treated with androgen deprivation therapy (ADT) before neuroendocrine features developed. The mean age was 65.5 years, with a maximum survival of 9 months post-diagnosis. NEPC is becoming an increasingly more common subtype of PCa that can result in various PNS. This makes the diagnosis and treatment of NEPC challenging. Further research is crucial to understanding these syndromes and developing standardized, targeted treatments to improve patient survival. [ABSTRACT FROM AUTHOR]

This article discusses a case of paraneoplastic stiff-leg syndrome, a rare immune-mediated disorder associated with breast cancer. The patient, a 45-year-old female, experienced progressive stiffness and painful cramps in her lower extremities, along with other symptoms. After extensive testing, including imaging and electromyography, the patient was diagnosed with paraneoplastic stiff-leg syndrome due to the presence of anti-amphiphysin antibodies. Treatment included chemotherapy, physical rehabilitation, and symptomatic management with medication. The patient showed improvement and remained free of muscular spasms after completing oncological treatment. [Extracted from the article]

Sarcoid-like reaction (SLR) has been reported in patients with solid tumor malignancies, lymphomas, and patients receiving immunotherapy. SLR is often incidentally found during positron emission tomography/computed tomography scans as hilar and/or mediastinal lymphadenopathy. SLR has also been found in the lung, spleen, bone marrow, and skin. Biopsy of these lesions shows noncaseating granulomas. When systemic criteria are not met for sarcoidosis, these noncaseating granulomas are termed SLR. We present the first case in the literature of a case of orbital SLR in a patient with concomitant diffuse large B-cell lymphoma and inverted papilloma of the maxillary sinus. This case highlights the importance of including malignancy in the differential for the presence of a noncaseating granuloma in the orbit. [ABSTRACT FROM AUTHOR]

The clinical category of immune-mediated cerebellar ataxias (IMCAs) has been established after 3 decades of clinical and experimental research. The cerebellum is particularly enriched in antigens (ion channels and related proteins, synaptic adhesion/organizing proteins, transmitter receptors, glial cells) and is vulnerable to immune attacks. IMCAs include various disorders, including gluten ataxia (GA), post-infectious cerebellitis (PIC), Miller Fisher syndrome (MFS), paraneoplastic cerebellar degeneration (PCD), opsoclonus myoclonus syndrome (OMS), and anti-GAD ataxia. Other disorders such as multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), Behçet disease, and collagen vascular disorders may also present with cerebellar symptoms when lesions are localized to cerebellar pathways. The triggers of autoimmunity are established in GA (gluten sensitivity), PIC and MFS (infections), PCD (malignancy), and OMS (infections or malignant tumors). Patients whose clinical profiles do not match those of classic types of IMCAs are now included in the spectrum of primary autoimmune cerebellar ataxia (PACA). Recent remarkable progress has clarified various characteristics of these etiologies and therapeutic strategies in terms of immunotherapies. However, it still remains to be elucidated as to how immune tolerance is broken, leading to autoimmune insults of the cerebellum, and the consecutive sequence of events occurring during cerebellar damage caused by antibody- or cell-mediated mechanisms. Antibodies may specifically target the cerebellar circuitry and impair synaptic mechanisms (synaptopathies). The present Special Issue aims to illuminate what is solved and what is unsolved in clinical practice and the pathophysiology of IMCAs. Immune ataxias now represent a genuine category of immune insults to the central nervous system (CNS). [ABSTRACT FROM AUTHOR]

We report the case of a 58-year-old patient with a multiple myeloma who experienced an inflammatory shoulders and hands pain with progressive irreducible fingers retractions. After further examinations, the diagnoses of associated paraneoplastic polymyalgia rheumatica and palmar fasciitis were made, revealing at the same time a relapse of his haemopathy. The inclusion of these two pathologies under the same diagnostic label cannot be excluded. Despite the use of corticosteroids at an appropriate dose, symptomatic improvement could only be noted following tocilizumab treatment, suggesting a potential role of the interleukin-6 in the pathophysiology of paraneoplastic palmar fasciitis. [ABSTRACT FROM AUTHOR]

Background: Anti-Hu antibody neuropathy is considered a rare acquired peripheral neuropathy, but common among paraneoplastic syndromes. Typically, is described as subacute sensory neuronopathy and electrophysiological findings are usually suggestive of a sensory axonal neuropathy. Case presentation: We report the case of a 67-year-old man referred to our clinic with a 4-month history of progressive pain and paresthesias of distal lower limbs. He had a 30-year history of alcohol abuse and smoking. Alcoholic neuropathy was considered the most likely diagnosis, considering his history and evaluation. The patient's neurological examination revealed symmetric bilateral superficial and deep sensory loss in the lower extremities, reduced Achilles tendon reflexes and wide based gait. Electrophysiological testing was suggestive of axonal sensory-motor polyneuropathy and small fiber involvement. Even though alcohol consumption was discontinued, symptoms gradually worsened. Further testing was performed and the patient was found seropositive for anti-Hu antibody. Small-cell lung cancer was detected later, but patient passed away before treatment for cancer was administrated. Conclusions: The aim of our paper is to report a case of a rare paraneoplastic syndrome that can cause progressive sensory-motor neuropathy with large and small fiber involvement, which should be rapidly differentially diagnosed from other neuropathies, so that the underlying cause can be identified and, potentially, treated. [ABSTRACT FROM AUTHOR]

Objective: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy with evidence of neuroinflammation and demyelination that affects the central nervous system and is mediated by aquaporin-4 (AQP4) immunoglobulin (IgG). AQP4-IgG may also be present in paraneoplastic syndromes secondary to malignancy such as breast cancer. Methods: A systematic review and meta-analysis of the literature were completed using PubMed, Scopus, and ScienceDirect databases (CRD42022352109). Results: A total of 12 publications, which included 19 cases, met the inclusion criteria and were assessed in both the qualitative and quantitative synthesis. The mean age was 51.26 years (SD: 13.12, SEM: 3.01), and 100% of the cases were reported in women. Speech abnormalities and symptoms of myelopathy were the most observed neurological manifestations. MRI often revealed longitudinally extensive transverse myelitis (LETM) involving the cervical spine. Three of 19 (15.9%) cases were diagnosed with NMOSD and breast cancer within the same month. Five of 19 (26.1%) cases had a diagnosis of breast cancer preceding that of NMOSD. Eight of 19 (42.1%) cases were diagnosed with breast cancer after NMOSD. The median time of breast cancer diagnosis was 1.0 months (range 216 months) after NMOSD. Conclusions: The diagnosis of breast cancer most often occurs after the onset of the paraneoplastic NMOSD symptoms. However, a wide time range for the diagnosis of breast cancer was observed both before and after the onset of neurological symptoms. Older women with a new diagnosis of NMOSD should be considered for frequent breast cancer screening. [ABSTRACT FROM AUTHOR]

Simple Summary: Morphea and eosinophilic fasciitis (EF) are cutaneous autoimmune fibrosing diseases. We conducted a scoping review following PRISMA-ScR guidelines to ascertain the association between cancer and morphea/EF, focusing specifically on the paraneoplastic phenomenon, risk of subsequent cancer and development of morphea/EF as a consequence of cancer treatment. We identified that morphea patients, particularly those with generalized disease, might be at an increased risk of secondary malignancy, notably skin and pancreatic cancer. EF, on the other hand, occurred as a paraneoplastic disease in 10% of patients, primarily associated with hematologic malignancies. While reports of radiotherapy and chemotherapy-induced morphea are numerous, immunotherapy-induced morphea/EF cases are emerging. Interestingly, all immunotherapy-induced cases occurred with PD-1 inhibitors. Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review assessing the risk of secondary cancer among morphea/EF patients, paraneoplastic morphea/EF and morphea/EF developing secondary to cancer therapy. The search was conducted using MEDLINE, Embase, Cochrane databases for articles published from inception to September 2022 following the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines with no language or date restrictions. Two hundred and one studies were included. Of these, 32 studies reported on secondary cancer in morphea/EF patients, 45 on paraneoplastic morphea/EF and 125 on cancer-treatment-induced morphea/EF. While the current evidence remains limited, data suggest an increased risk of secondary cutaneous and possibly pancreatic malignancy in morphea patients, particularly the generalized subtype. There were insufficient data for EF. On the other hand, paraneoplastic morphea was anecdotal, whereas several observational studies suggested that ~10% of EF cases may be paraneoplastic, primarily in the context of hematologic malignancies. Radiotherapy-induced morphea is rare, seen in ~0.2% of treated patients and is usually localized to the treatment site, except in patients with pre-existing autoimmunity. While chemotherapy-induced cases are reported, immunotherapy morphea/EF cases are emerging and are preferentially seen with PD-1 and not CTLA-4 inhibitors. This study is limited by the type of articles included (case reports, case series and observational studies), and hence, additional research on this important topic is needed. [ABSTRACT FROM AUTHOR]

Paraneoplastic cerebellopathies are immune-mediated disorders targeting primarily the cerebellar circuitry, often presenting in a subacute course. The syndrome often develops before the cancer. Therefore, its identification often leads secondarily to a diagnosis of cancer, a critical step to stabilize symptoms. Two categories of antibodies have been identified these last 30 years: (a) onconeuronal antibodies which are directed against intracellular antigens, and (b) antibodies which are directed against synaptic and cell surface proteins. These latter impact on the location and function of the antigens, causing a genuine neuronal dysfunction. Appropriate and fast tumor screening has emerged as a recommendation facing a subacute cerebellar syndrome suspected to be paraneoplastic. Search for antibodies is now a milestone for the diagnosis. [ABSTRACT FROM AUTHOR]

Acute Disseminated Encephalomyelitis (ADEM) is a monophasic demyelinating disease most often triggered by infection or immunization, though associations with malignancy and stem cell transplant have been described. We described the case of a four-year-old boy with new-onset neurological symptoms associated with ADEM, acute leukemia, and equivocal evidence of Mycoplasma pneumoniae infection. He responded to a short course of antibiotics and chemotherapy for B cell acute lymphoblastic leukemia (B-ALL) that included a prolonged course of high dose steroids with immunosuppressive therapy. This case illustrated a possible association between paraneoplastic ADEM and leukemia in pediatric patients. [ABSTRACT FROM AUTHOR]

Granuloma annulare (GA) is a benign, self-limiting granulomatous inflammatory disease that exhibits different histologic patterns. Infrequently, granuloma annulare can be associated with malignancy, the so-called malignancy-associated granuloma annulare (MGA). In this study, we aimed to compare the clinical and histopathological differences between GA and MGA. We retrospectively reviewed patient charts and identified 35 patients diagnosed with GA and concurrent hematological or solid organ malignancies as a case group. Additionally, we identified 33 patients without any known solid organ or hematological malignancy as a control group. MGA is commonly seen in the seventh decade of life, while GA affects the younger population. MGA is most commonly presented in the extremities of the body. The most common malignancy associated with MGA was chronic lymphocytic leukemia. Prostate cancer was the most common type of solid organ malignancy that was associated with MGA. The most common histopathological pattern seen in MGA was interstitial, comprising half of the cases. Multinucleated giant cells were present in half of the MGA cases and in most of the control group. In the literature, there are no established features that distinguish MGA from GA. Although MGA and GA have overlapping features, in our series, we found that the interstitial pattern was more common in MGA, while the necrobiotic pattern was more common in GA. [ABSTRACT FROM AUTHOR]

Introduction: Immune checkpoint inhibitors (ICIs) have become an important part of the treatment of multiple cancers, especially for advanced melanoma and non-small cell lung cancer. Some tumors are capable of escaping immunosurveillance by stimulating checkpoints on T-cells. ICIs prevent activation of these checkpoints and thereby stimulate the immune system and indirectly the anti-tumor response. However, the use of ICIs is associated with various adverse events. Ocular side effects are rare but may have a major impact on the quality of life of the patient. Methods: A comprehensive literature search of the medical databases Web of Science, Embase and PubMed was performed. Articles that provided a comprehensive description of a case report containing 1) cancer patient(s) treated with (a combination of) immune checkpoint inhibitors, and 2) assessed occurrence of ocular adverse events, were included. A total of 290 case reports were included. Results: Melanoma (n = 179; 61.7%) and lung cancer (n = 56; 19.3%) were the most frequent reported malignancies. The primary used ICIs were nivolumab (n = 123; 42.5%) and ipilimumab (n = 116; 40.0%). Uveitis was most the common adverse event (n = 134; 46.2%) and mainly related to melanoma. Neuro-ophthalmic disorders, including myasthenia gravis and cranial nerve disorders, were the second most common adverse events (n = 71; 24.5%), mainly related to lung cancer. Adverse events affecting the orbit and the cornea were reported in 33 (11.4%) and 30 cases (10.3%) respectively. Adverse events concerning the retina were reported in 26 cases (9.0%). Conclusion: The aim of this paper is to provide an overview of all reported ocular adverse events related to the use of ICIs. The insights retrieved from this review might contribute to a better understanding of the underlying mechanisms of these ocular adverse events. Particularly, the difference between actual immune-related adverse events and paraneoplastic syndromes might be relevant. These findings might be of great value in establishing guidelines on how to manage ocular adverse events related to ICIs. [ABSTRACT FROM AUTHOR]

Objectives: Radiology receives a large volume of referrals for systemic scans and neuroimaging in suspected cases of paraneoplastic neurological syndrome (PNS) patients. To date, there have been no guidelines to define imaging pathways in diagnosis or surveillance of such patients. This article aims to evaluate diagnostic utility of imaging in detecting positive results as well as ruling out significant pathologies in suspected cases of PNS and strategize vetting requests. Materials and Methods: Retrospectively evaluated scan records, onconeuronal antibody results of 80 patients (separated into below and over 60s age group) referred with suspected PNS (categorized as classical or probable PNS after neurological assessment). Imaging findings and final diagnoses were classified into three groups: Normal (N), non-neoplastic significant findings (S), and malignancies (M) after evaluating histopathology results/perioperative findings and treatment notes. Results: There were ten cases of biopsy-proven malignancies and 18 cases of non-neoplastic significant conditions (predominantly neurological) with malignancies dominating in the elderly age group, demyelinating neurological conditions in below 60s group and patients suspected of classical PNS on neurological evaluation. Staging computed tomography (CT) had 50%, positron emission tomography CT (PETCT) had 80%, sensitivity had 93%, and negative predictive value in ruling out malignancy had 96%. Magnetic resonance of brain and spine was reported abnormal in 68% of finally diagnosed positive cases while only 11% cases demonstrated onconeuronal antibody positivity. Conclusion: Complete neuroimaging before systemic scans, categorization of referral requests in probable and classical cases of PNS with prioritization of PET in cases of high clinical concern might help in better detection of pathologies and reduce unnecessary CTs. [ABSTRACT FROM AUTHOR]

We report an 80‐year‐old male developing linear IgA bullous dermatosis (LAD) in the setting of angioimmunoblastic T‐cell lymphoma (AITL). This phenomenon is rare, as only three cases have been described in the literature. The pathophysiologic process can be attributed to dysregulation in somatic hypermutation and the expression of chemokine receptor 5 in AITL, contributing to increased IgA. Immunoglobulin production resulting from clonal plasma cell expansion may be because of the B‐cell promotional effect by neoplastic follicular helper T‐cells. Beyond providing a pathophysiologic platform for AITL‐associated LAD, we also briefly summarized prior cases. This report demonstrates the importance of considering LAD in the differential diagnosis for patients with a bullous eruption in the setting of AITL. [ABSTRACT FROM AUTHOR]

Background: Aquaporin‐4 IgG seropositive neuromyelitis optica spectrum disorder (AQP4‐IgG NMOSD) might occur in association with cancer. According to diagnostic criteria, a probable paraneoplastic NMOSD can be diagnosed only in patients with isolated myelitis and adenocarcinoma or tumors expressing AQP4. The aim of this study was to explore the features of paraneoplastic NMOSD through a data‐driven approach. Methods: A systematic literature review was performed. Patients with AQP4‐IgG positivity in association with tumor in the absence of history of checkpoint inhibitors administration/central nervous system metastases were included. Demographic, clinical, and oncological data were collected. A hierarchical cluster analysis (HCA) was performed and data were compared between resulting clusters. Results: A total of 1333 records were screened; 46 studies (72 patients) fulfilled inclusion criteria. Median age was 54 (14–87) years; adenocarcinoma occurred in 41.7% of patients, and 44% of cases had multifocal index events. Cancer and NMOSD usually co‐occurred. HCA classified patients in three clusters that differed in terms of isolated/multifocal attacks, optic neuritis, pediatric onset, and type of underlying tumor. Age, time from neoplasm to NMOSD onset, and tumor AQP4 staining did not differ between clusters. Conclusions: Our data‐driven approach reveals that paraneoplastic NMOSD does not present a homogeneous phenotype nor peculiar features. Accordingly, cancer screening may be useful in AQP4‐IgG NMOSD regardless of age and clinical presentation. [ABSTRACT FROM AUTHOR]

The rapidly expanding spectrum of autoimmune encephalitis in the last fifteen years is largely due to ongoing discovery of many neuronal autoantibodies. The diagnosis of autoimmune encephalitis can be challenging due to the wide spectrum of clinical presentations, prevalence of psychiatric features that mimic primary psychiatric illnesses, frequent absence of diagnostic abnormalities on conventional brain MR-imaging, non-specific findings on EEG testing, and the lack of identified IgG class neuronal autoantibodies in blood or CSF in a subgroup of patients. Early recognition and treatment are paramount to improve outcomes and achieve complete recovery from these debilitating, occasionally life threatening, disorders. This review is aimed to provide primary care physicians and hospitalists who, together with neurologist and psychiatrists, are often the first port of call for individuals presenting with new-onset neuropsychiatric symptoms, with up-to-date data and evidence-based approach to the diagnosis and management of individuals with neuropsychiatric disorders of suspected autoimmune origin. [ABSTRACT FROM AUTHOR]

Movement disorders as well as peripheral neuropathies are extremely frequent in the general population; therefore, it is not uncommon to encounter patients with both these conditions. Often, the coexistence is coincidental, due to the high incidence of common causes of peripheral neuropathy, such as diabetes and other age-related disorders, as well as of Parkinson disease (PD), which has a typical late onset. Nonetheless, there is broad evidence that PD patients may commonly develop a sensory and/or autonomic polyneuropathy, triggered by intrinsic and/or extrinsic mechanisms. Similarly, some peripheral neuropathies may develop some movement disorders in the long run, such as tremor, and rarely dystonia and myoclonus, suggesting that central mechanisms may ensue in the pathogenesis of these diseases. Although rare, several acquired or hereditary causes may be responsible for the combination of movement and peripheral nerve disorders as a unique entity, some of which are potentially treatable, including paraneoplastic, autoimmune and nutritional aetiologies. Finally, genetic causes should be pursued in case of positive family history, young onset or multisystemic involvement, and examined for neuroacanthocytosis, spinocerebellar ataxias, mitochondrial disorders and less common causes of adult-onset cerebellar ataxias and spastic paraparesis. Deep phenotyping in terms of neurological and general examination, as well as laboratory tests, neuroimaging, neurophysiology, and next-generation genetic analysis, may guide the clinician toward the correct diagnosis and management. [ABSTRACT FROM AUTHOR]

Introduction: Lambert–Eaton myasthenic syndrome (LEMS) is characterized by autoantibodies against voltage-gated calcium channels (VGCC) at the neuromuscular junction causing proximal muscle weakness, decreased tendon reflexes, and autonomic changes. The European LEMS registry aimed to collate observational safety data for 3,4-diaminopyridine phosphate (3,4-DAPP) and examine long-term outcomes for patients with LEMS. Methods: Thirty centers across four countries participated in the non-interventional European LEMS registry. Any patients diagnosed with LEMS by means of clinical assessment and abnormal neurophysiological testing, or clinical assessment and positive for VGCC antibodies were eligible to participate. Patients were monitored using standard assessments for LEMS-related clinical manifestations. Results: Among 96 evaluable participants, 50 (52.1%) were being treated with 3,4-DAPP, 21 (21.9%) with 3,4-diaminopyridine (3,4-DAP), and 25 (26.0%) with other treatments (e.g., pyridostigmine, corticosteroids, immunoglobulins, and azathioprine); 74 participants (77.1%) were exposed to 3,4-DAPP at any time. Quantitative myasthenia gravis scores were similar across treatment groups. Muscle strength was generally good and maintained during follow-up. Cerebellar ataxia, defined as a negative Romberg's test and at least one other positive ataxia test, was observed in 30 (56.6%) patients. Most participants had reduced reflex tone and limited functioning. Sustained or improved functioning was observed in participants administered 3,4-DAPP. Inconsistent and sporadic functional improvement and regression was observed with 3,4-DAP and other treatments. Fifty-five treatment-related adverse events (AEs) were reported by 32 (33.3%) participants. Eight (8.3%) participants reported nine treatment-related serious AEs. No new safety signals were identified. Conclusion: No new safety signals were observed following long-term management of LEMS with 3,4-DAPP. [ABSTRACT FROM AUTHOR]

Background: Autoimmune autonomic ganglionopathy is a rare, immune-mediated disorder associated with anti-ganglionic α3-subunit nicotinic acetylcholine receptor (anti-α3gAChR) antibodies, which bind to acetylcholine receptor in autonomic ganglia (parasympathetic and sympathetic) leading to autonomic failure. This disorder is mostly associated with viral infections, but it can also be associated with systemic malignancies. Here, we report the case of a paraneoplastic autonomic ganglionopathy as the first symptom of bladder cancer.Method: Case report.Results: A 47-year-old man, without medical history of interest, stated to the emergency department for progressive blurry vision with eye and mouth dryness, constipation, and dizziness upon standing for the last 2 weeks. Orthostatic hypotension was demonstrated by a drop in 13.3 mmHg mean blood pressure (BP) from supine (100/60 mmHg) to 45° reclining sitting position (80/50 mmHg). Blood tests, chest X-ray, brain MRI, and electroneuronography were unremarkable. Electrochemical skin conductance was reduced. Serological examination was positive for anti-α3gAChR antibodies. A full-body CT scan revealed a bladder tumor, which was treated by transurethral bladder resection. The pathologic study demonstrated a low-grade non-muscle-invasive bladder urothelial carcinoma. After tumor resection, and treatment with intravenous immunoglobulins and corticoids, a gradually improvement was observed. Today, the patient remains asymptomatic.Conclusion: Subacute panautonomic failure can be the first symptom for systemic malignancies. This case reports a paraneoplastic autonomic ganglionopathy as the first symptom of bladder cancer. This case highlights the importance of a systemic study to rule out the presence of cancer when autoimmune autonomic ganglionopathy is present. [ABSTRACT FROM AUTHOR]

Lung cancer is one of the most fatal types of cancer and paraneoplastic syndromes are commonly associated with lung cancer. Paraneoplastic Cushing can be associated with different types of tumors, especially small cell lung cancer (SCLC). The aim of this report was to describe the status of a patient presenting with progressive weakness, who did not have a previous medical history. Clinical examination revealed bilateral edema of the lower limbs. Lab results exhibited severe hypokalemia and metabolic alkalosis. physical examination showed elevated blood pressure. Imaging revealed a large mass with necrosis. Bronchoscopy was done and biopsy pathology showed SCLC. The patient underwent chemotherapy with cisplatin and etoposide. Following SCLC, the patient developed a type of paraneoplastic syndrome called Cushing syndrome. He experienced a severe decrease in saturation in the second session of chemotherapy and eventually died. With respect to the fact that most paraneoplastic syndromes are associated with poor response to treatment, high clinical suspicion is essential for the rapid diagnosis of these syndromes to improve outcomes. [ABSTRACT FROM AUTHOR]

Inclusion body myositis (IBM) is an acquired, late-onset inflammatory myopathy, with both inflammatory and degenerative pathogenesis. Although idiopathic inflammatory myopathies may be associated with malignancies, IBM is generally not considered paraneoplastic. Many studies of malignancy in inflammatory myopathies did not include IBM patients. Indeed, IBM is often diagnosed only after around 5 years from onset, while paraneoplastic myositis is generally defined as the co-occurrence of malignancy and myopathy within 1 to 3 years of each other. Nevertheless, a significant association with large granular lymphocyte leukemia has been recently described in IBM, and there are reports of cancer-associated IBM. We review the pathogenic mechanisms supposed to be involved in IBM and outline the common mechanisms in IBM and malignancy, as well as the therapeutic perspectives. The terminally differentiated, CD8+ highly cytotoxic T cells expressing NK features are central in the pathogenesis of IBM and, paradoxically, play a role in some cancers as well. Interferon gamma plays a central role, mostly during the early stages of the disease. The secondary mitochondrial dysfunction, the autophagy and cell cycle dysregulation, and the crosstalk between metabolic and mitogenic pathways could be shared by IBM and cancer. There are intermingled subcellular mechanisms in IBM and neoplasia, and probably their co-existence is underestimated. The link between IBM and cancers deserves further interest, in order to search for efficient therapies in IBM and to improve muscle function, life quality, and survival in both diseases. [ABSTRACT FROM AUTHOR]

Wernicke's encephalopathy (WE) and paraneoplastic limbic encephalitis (PLE) can both present with acute-to-subacute memory impairment and cognitive dysfunction. Both can lead to significant morbidity and mortality without rapid identification and treatment. Often patients with WE may not have the typical clinical triad of ophthalmoplegia, gait ataxia, and altered mental status. Furthermore, both WE and PLE may share similar MRI findings. Here, we present a case of a patient with a history of seronegative PLE presenting with acute-to-subacute cognitive changes and gait imbalance. Initially, it was felt to be a relapse of PLE but upon further history and testing may potentially have represented WE in the setting of a recent dietary change. [ABSTRACT FROM AUTHOR]

Keywords: kidney; paraneoplastic; partial nephrectomy; RCC; small renal mass; symptoms; fever EN kidney paraneoplastic partial nephrectomy RCC small renal mass symptoms fever 434 436 3 03/24/23 20230401 NES 230401 In January 2015, a 62-year-old age woman arrived at the San Raffaele Emergency Department complaining of fever of unknown origin (FUO) and two recent syncopal episodes. Out of 3769 patients submitted to active treatment for RCC registered in our prospectively maintained database from 1987 until 2021, 46 (1.2%) had a SRM with paraneoplastic systemic symptoms. These 46 patients represented 16.6% of all the patients with systemic symptoms at presentation, and 2.8% of all the patients with a SRM. [Extracted from the article]

Introduction: Patients with small‐cell lung cancer (SCLC) have a very poor prognosis. However, a subset of SCLC achieves long‐term survival. The objective of this study was to investigate factors and pattern of long‐term survival in patients with limited‐stage small cell lung cancer (LS‐SCLC) who achieved a complete response (CR) after chemoradiotherapy. Patient and Methods: This was a single‐center retrospective study. The analysis of hazard ratio (HR) and 95% confidence interval (CI) was performed using Cox proportional hazards model. For pattern analysis, the date of recurrence was used as the endpoint. The nominal categorical variables were analyzed by the χ2 test. Survival was estimated using the Kaplan–Meier model, and the results were reported as the median and interquartile range. Results: We identified 162 patients, median age was 64.7 (56.2–70.2) years, and 94 (58%) were females. Eighty‐one patients (50%) had recurrence during follow‐up. Gastroesophageal reflux disease (GERD) (HR, 0.65; 95% CI, 0.45–0.93; p = 0.016) and neurological paraneoplastic syndrome (PNS) (HR, 0.46; 95% CI, 0.29–0.72; p < 0.001) were independent factors associated with improved overall survival (OS). Patients with GERD had prolonged recurrence free survival (RFS) compared to patients without GERD (median, 29.1 months vs. 13.9 months, p < 0.001), whereas patients with neurological PNS had a reduced recurrence rate compared to those patients without neurological PNS (No. [%], 8 [20.5] vs. 73 [59.3], p < 0.001). Conclusions: Patients with LS‐SCLC achieving a CR after chemoradiotherapy, GERD, and neurological PNS were associated with improved OS. GERD and neurological PNS were associated with longer RFS and lower recurrence rate, respectively. [ABSTRACT FROM AUTHOR]

Objectives: This work aimed to report the demographic and clinical characteristics of two new cases with non-paraneoplastic anti-Hu-associated gut motility impairment, and perform a thorough revision covering anti-Hu-associated paraneoplastic (PGID) and non-paraneoplastic (nPGID) gastrointestinal dysmotility. Background: Several case series have clearly established a relationship between certain type of cancers, the development of circulating anti-Hu antibodies, and the concomitant usually severe gastrointestinal dysmotility; in contrast, a few studies focused on anti-Hu-associated nPGID. Methods: We searched for studies regarding anti-Hu-associated gastrointestinal manifestations and extracted data concerning clinical characteristics of patients, including specific demographic, oncological, neurological, gastrointestinal, histological, and treatment response features. Results: Forty-nine articles with a total of 59 cases of anti-Hu-associated gastrointestinal dysmotility were analyzed. The patients' age at symptom onset significantly differed between PGID and nPGID (median 61 vs 31 years, p < 0.001). Most cancers (95%) in PGID were detected within 24 months from the beginning of gastrointestinal symptoms. The impairment of gastrointestinal motility was generalized (i.e., involving the whole gut) in 59.3% of patients, with no significant differences between PGID vs nPGID group. nPGID patients showed a better response to immunomodulatory/immunosuppressive treatment and a longer life expectancy. Conclusions: Anti-Hu-associated gastrointestinal dysmotility covers a wide clinical spectrum. Patients with otherwise unexplained gastrointestinal dysmotility, especially when associated with other neurological symptoms, should be tested for anti-Hu antibodies regardless age of onset and disease duration. Compared to PGID, nPGID occurs in younger patients with a long duration of disease. [ABSTRACT FROM AUTHOR]

Hypertrophic osteoarthropathy (OAH) is a primary or secondary clini coradiological entity. OAH is most often secondary to pulmonary neoplasia. We report a rare case of OAH secondary to thymic carcinoma. A 60-year-old Men with no pathological history, he was a nonsmoker. He has been suffering from distal polyarthritis for 2 months, in a context of asthenia and weight loss. The clinical examination revealed a digital clubbing, synovitis of the knees and ankles bilateral and symmetrical. His balance sheet showed periosteal appositions of the long bones of the upper and lower limbs, without pinching or erosion, and an inflammatory syndrome. The chest CT SCAN shows a heterogeneous tissue mass of the thymic compartment, the biopsy has concluded that there is undifferentiated thymic carcinoma. The occurrence of OAH during thymic carcinoma may be a sign of poor prognosis, given the possible relationship between VEGF and OAH. [ABSTRACT FROM AUTHOR]

Neuromyelitis optica spectrum disorder is an inflammatory condition of the central nervous system typically manifesting as myelitis, optic neuritis, and/or area postrema syndrome. Here, we present a pediatric patient who developed symptoms consistent with area postrema syndrome with positive anti-aquaporin-4 (AQP4) antibodies who was also found to have an ovarian teratoma. Pathological specimens revealed the presence of aquaporin-4. This was felt to be the antigenic trigger that led to the patient's condition. She suffered no further clinical attacks and seroconverted to negative AQP4 status upon teratoma removal. This case varies from others, in that the paraneoplastic presentation occurred in a pediatric patient and in that the patient has not required maintenance immunotherapy after teratoma removal. [ABSTRACT FROM AUTHOR]

Context: Dermatomyositis is a rare autoimmune disease characterized by noninfectious inflammatory damage of skin and predominant muscles in the belts. It is believed to be associated with about 1 in 1000 cases of nasopharyngeal carcinoma. This association has been described for locally advanced stages II and III nasopharyngeal carcinoma. It has rarely been described in the early stages (stage I).Case Presentation: A 65-year-old Moroccan patient residing in Casablanca, with no particular history was referred to the Mohamed VI Center for the treatment of cancers of the University Hospital Center IBN ROCHD in Casablanca, for treatment of nasopharyngeal cancer. He was admitted in poor general condition, performance status 3, with erythema on the face, neck, and extremities. The diagnosis of paraneoplastic dermatomyositis was made owing to progressive muscle weakness and elevation of muscle enzymes associated with the typical rash of the face and hands. He received corticosteroid therapy and then radiotherapy to the nasopharynx with good clinical outcome, disappearance of skin lesions, and recovery of muscle strength.Conclusions: We report this case of dermatomyositis in early-stage nasopharyngeal carcinoma, which is a rarely described entity. Rapid treatment of dermatomyositis improved the patient's quality of life and enabled him to support specific cancer treatments. This can be used as an element of early diagnosis and monitoring after treatment. [ABSTRACT FROM AUTHOR]

Autoantigen discovery is a critical challenge for the understanding and diagnosis of autoimmune diseases. While autoantibody markers in current clinical use have been identified through studies focused on individual disorders, we postulated that a reverse approach starting with a putative autoantigen to explore multiple disorders might hold promise. We here targeted the epidermal protein transglutaminase 1 (TGM1) as a member of a protein family prone to autoimmune attack. By screening sera from patients with various acquired skin disorders, we identified seropositive subjects with the blistering mucocutaneous disease paraneoplastic pemphigus. Validation in further subjects confirmed TGM1 autoantibodies as a 55% sensitive and 100% specific marker for paraneoplastic pemphigus. This gene-centric approach leverages the wealth of data available for human genes and may prove generally applicable for biomarker discovery in autoimmune diseases. [ABSTRACT FROM AUTHOR]

Necrolytic migratory erythema (NME) is a rare cutaneous paraneoplastic manifestation of glucagonoma. We report a case of a woman with a 6‐year history of delayed diagnosis of glucagonoma. This case highlights the atypical clinical features of NME which makes the diagnosis difficult. [ABSTRACT FROM AUTHOR]

• Paraneoplastic cerebellar degeneration (PCD) symptoms include ataxia and nystagmus. • Holmes tremor is a large amplitude, 3–4 Hz tremor associated with PCD. • PCD associated with non-Hodgkin's lymphoma has rarely been reported. • Malignancy workup and repeat biopsy is recommended if suspicion for PCD is high. • Treating the underlying malignancy is the most likely to improve PCD symptoms. [ABSTRACT FROM AUTHOR]

Neurologic symptoms are commonly seen in patients with cancer and can be among the most challenging to diagnose and manage. It is often difficult to determine if new neurologic symptoms are secondary to direct effects of a malignant lesion, systemic complications of disease, paraneoplastic disorders, or side effects of cancer treatment itself. However, early diagnosis and treatment of each of these conditions can improve patients' quality of life and long-term functional outcomes. In this review, we describe a systematic approach to the diagnosis of new neurologic symptoms in patients with known malignancy. We have categorized the neurologic complications of cancer through a mechanistic approach, with an emphasis on ascertaining underlying pathophysiology to guide treatment choice. This review focuses on the acute neurologic complications of cancer that require hospital admission. [ABSTRACT FROM AUTHOR]

Ataxia is a frequent symptom in neurological cases with many causes. Sensory ataxia (due to involvement of the proprioceptive pathways) is observed in conditions affecting the central nervous system (spinal cord disorder) and the peripheral nervous system (peripheral neuropathy). The latter correspond to what we refer to as 'ataxic neuropathies'. Ataxic neuropathies represent a wide and heterogeneous spectrum of disorders that may affect dorsal root nerves, dorsal root ganglia, nerve trunks, distal nerve endings or all of them together. The identification of a predominant sensory ataxia in a case of peripheral neuropathy should raise the possibility of some specific etiologies. We propose here to present the main causes of ataxic neuropathies, which are identified with diagnostic workflows that are dictated by the topography of the likely sites of lesions in the proprioceptive pathway together with the timing of their occurrence (acute, subacute, or chronic). [ABSTRACT FROM AUTHOR]

Background/aim: Ganglioside antibodies are identified not only in patients with inflammatory neuropathies but also several central nervous system disorders and paraneoplastic neuropathies. Our aim was to investigate whether ganglioside antibodies are found in autoimmune encephalitis patients and may function as a diagnostic and prognostic biomarker. Materials and methods: Sera and cerebrospinal fluid (CSF) samples of 33 patients fulfilling the criteria for probable autoimmune encephalitis were collected within the first week of clinical manifestation. None of the patients had evident symptoms and findings of peripheral polyneuropathy. Well-characterized antineuronal and paraneoplastic antibodies were investigated in sera and CSF and antiganglioside (antiGM1, GM2, GM3, GD1a, GD1b, GT1b, and GQ1b) IgG and IgM antibodies were measured in sera using commercial immunoblots. Results: Twenty-eight of 33 autoimmune encephalitis patients displayed antibodies against neuronal surface or onco-neural antigens with N-methyl-D-aspartate receptor (NMDAR), glutamic acid decarboxylase (GAD) and Hu antibodies being the most prevalent. While no antiganglioside IgG antibodies were found, 4 patients (2 anti-NMDAR+, 1 anti-GAD+ and 1 antibody negative) with autoimmune limbic encephalitis displayed anti-GM1, anti-GM2, anti-GM3 or anti-GQ1b IgM antibodies. There was no apparent association between antiganglioside positivity and clinical and demographic features. Conclusion: Serum ganglioside IgM antibodies may infrequently emerge during the clinical course of autoimmune limbic encephalitis without evident polyneuropathy. Absence of the IgG response suggests that these antibodies might have developed as a hyperacute immune response to neuro-axonal destruction. Nevertheless, potential impact of ganglioside antibodies on axonal degeneration and neuronal loss in limbic encephalitis pends to be further investigated. [ABSTRACT FROM AUTHOR]

Introduction: Doege-Potter syndrome is a rare paraneoplastic syndrome presenting as hypoinsulinemic hypoglycemia due to ectopic secretion of prohormone: insulin-like growth factor-2 (IGF-2) from a solitary fibrous tumor (SFT). It is essentially a non-islet cell tumor hypoglycemia (NICTH) or in more recent terms, an 'IGF-2oma'. In case of delay in diagnosis, tumor-induced hypoglycemia could cause hypoxic cerebral injury or death. We hereby present a case of 45 years old male who presented with hypoglycemia due to pleural SFT, which is a very rare and unique presentation. Results: Upon biochemical and radiological investigations, Doege-Potter syndrome was diagnosed and the patient was planned for surgical resection of the tumor. Conclusion: This case stresses upon thorough evaluation of patients presenting with non-diabetic hypoglycemia to rule out underlying malignancy. [ABSTRACT FROM AUTHOR]

Generalized granuloma annulare can be associated with breast cancer. Atypical granuloma annulare especially in older patients should alert physicians to the possibility of an occult malignancy. [ABSTRACT FROM AUTHOR]

Introduction: Neuromyelitis optica spectrum disorder (NMOSD; also known as Devic syndrome) is a clinical syndrome of central nervous system characterized by immune mediated attacks of acute optic neuritis and myelitis. Paraneoplastic neurological syndrome is a group of nervous system disorders resulting from the remote immune effects of malignant neoplasm. NMOSD occurs mostly in young people, and tumor is not a common cause, especially recurrent tumor. Methods: We reported a case of a 59‐year‐old man who developed anti‐aquaporin‐4 IgG positive longitudinally extensive myelitis. We also summarized and analyzed previously reported cases of paraneoplastic NMOSD. Results: Among these 43 patients, 88.4% patients are female. The largest number of patients is between 60 and 69 years old. Breast cancer and lung cancer are the most common types. The most common lesions were located in the cervicothoracic region with patchy gadolinium enhancement. The existing treatment can only delay rather than stop the progress of the disease. Conclusion: It is necessary to perform tumor screening in patients with NMOSD, especially patients over 50 years. Wereported a rare case of AQP4‐IgG positive paraneoplastic NMOSD with recurrent rectal cancer. We summarized the previously reported cases of paraneoplastic NMOSD from demographics, clinical features, neuroimaging findings, laboratory examination, therapy, outcome, and other aspects and highlighted the necessity to perform tumor screening in patients with NMOSD, especially patients over 50 years. We analyzed the pathogenesis of AQP4‐IgG positive paraneoplastic NMOSD and discussed the phenomenon of paraneoplastic NMOSD with intractable hyponatremia. [ABSTRACT FROM AUTHOR]

Melanoma-associated retinopathy (MAR) is a paraneoplastic syndrome that involves the production of autoantibodies which can cross-react with retinal epitopes leading to visual symptoms. Autoantibodies can target intracellular proteins, and only a few are directed against membrane proteins. This discrepancy in autoantibody–protein target can translate into different immune responses (T-cell mediated vs B-cell mediated). Historically, treatment of MAR has focused on surgical reduction or immunosuppressive medication, mainly glucocorticoids. However, tumor resection is not relevant in metastatic melanoma in which MAR is mostly encountered. Moreover, the use of glucocorticoids can reduce the efficacy of immunotherapy. We report the first case to our knowledge with subjective resolution of visual symptoms and objective evidence of normalization of electroretinogram of MAR with undetectable autoantibodies after administration of programmed death-1 (PD-1) inhibitor (pembrolizumab) without the use of surgical reduction or systemic immunosuppression. This case highlights the potential improvement and resolution of negative autoantibody MAR with the use of PD-1 inhibitors and emphasizes the importance of multidisciplinary approach and team discussion to avoid interventions that can decrease immunotherapy-mediated anti-tumor effect. [ABSTRACT FROM AUTHOR]

Paraneoplastic limbic encephalitis (PLE) is an immunopathologic syndrome associated with malignancy and represents a rare remote outcome of tumor on the nervous system. We report a case of PLE caused by a regressed testicular germ cell tumor in an otherwise healthy young man, who presented with acute-onset confusion, memory impairment and anterograde amnesia. Prompt recognition of PLE is critical as it allows early treatment of both the primary tumor and PLE-related neurologic impairments, which can be severe and irreversible if treatment is delayed. Complete tumor treatment response offers the best chance for neurologic recovery in patients with PLE. To our knowledge, this is the second reported case of PLE with radiologic-pathologic correlation in the setting of a regressed testicular germ cell tumor. • PLE associated with regressed testicular germ cell tumor is exceedingly rare. • Anti-Ma2/Ta antibody is a paraneoplastic antibody associated with testicular tumors in men less than 50 years of age. • Early recognition of PLE allows early diagnosis and treatment of the primary tumor. [ABSTRACT FROM AUTHOR]

Oncological emergencies can occur at any time during an neoplastic pathology or during specialized treatment, and must be identified and properly addressed as early as possible, so as to determine a maximum favorable response. Emergencies in cancer can be represented by iatropathic disease (Cringanu, 2013), paraneoplastic syndromes or the cancer itself. The earlier we diagnose, the better we can establish a treatment with a successful result. This article aims to present treatment protocols in the most common cases of veterinary oncological emergencies. The main objective of our research was to establish a unitary conduct for a fast and correct diagnosis and an efficient treatment of oncological emergencies, before their effects became irreversible. [ABSTRACT FROM AUTHOR]

Solid malignant neoplasms have the capability of disturbing the fibrinolytic system, leading to primary hyperfibrinolysis, a paraneoplastic syndrome that potentially results in severe bleeding. Yet, the full extent of primary hyperfibrinolysis in solid malignant neoplasms is unknown. Thus, the purpose of this study was to systematically review the current literature regarding clinical manifestations, biochemical diagnosis, and treatment of primary hyperfibrinolysis in patients with solid malignant neoplasms. The review was performed in agreement with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The databases PubMed, Embase, Scopus, and Web of Science were searched on December 5, 2019, without time limits. Studies were included if they comprised at least one biochemical marker of fibrinolysis in addition to fibrinogen degradation products such as D-dimer, and furthermore included a correlation between biochemical marker and clinical outcome. In total, 12 studies were included. All studies were case reports including a total of 21 patients. Prostate cancer was the most frequently represented cancer type (76%), and the majority of cancer patients had metastatic disease (81%). Spontaneous bleeding was the clinical presentation in the majority of patients (76%), and the most frequently localization for the bleedings was subcutaneous. Antifibrinolytic agents were the most commonly used treatment and ceased bleedings in 80% of patients. Three patients died of uncontrolled bleedings. In conclusion, primary hyperfibrinolysis induced by solid malignant neoplasms is a rare but potentially life-threatening condition that should be considered, especially in patients with metastatic disease presenting with serious, spontaneous subcutaneous bleedings. A standardized diagnostic strategy is strongly needed. [ABSTRACT FROM AUTHOR]

Myelopathy is a broad term used to describe a heterogeneous group of disorders that affects the spinal cord; the focus of this article will be a subgroup of these disorders with an autoimmune and inflammatory-based pathology. Symptoms typically develop over hours or days and then worsen overa matter of days to weeks, but sometimes can have a more insidious or subacute presentation, which can make the diagnosis more puzzling. Despite relatively low incidence rates, almost a third of affected patients are left with severelydisablingsymptoms. Promptrecognition of the underlyingetiologyis essential sothataspecifictargetedtherapycan be implemented foroptimal outcomes. The authors discuss a systematic approach to immune-mediated myelopathies, with a focus on the unique characteristics of each that may aid in diagnosis. [ABSTRACT FROM AUTHOR]

Introduction. Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) mainly characterized by subacute muscle weakness and skin rash sometimes associated with malignancy. Case Presentation. A 61-year-old female was admitted to our hospital because of progressive proximal muscular weakness, heliotropic rash and left breast rash. Muscle biopsy findings were consistent with dermatomyositis (DM). A full panel of myositis associated (MAA) and specific antibodies (MSA) revealed the presence of anti-nuclear antibodies (1:160, speckled), Anti-Ro52 and anti TIF1-γ antibodies. A whole body Computed Tomography Scan showed three left mammary nodules and homolateral axillary lymphadenopathy. The breast biopsy confirmed the diagnosis of ductal carcinoma. Patient was initiated to neoadjuvant chemotherapy followed by surgery for cancer, and corticosteroid and intravenous immunoglobulins for DM with a complete resolution of muscle weakness and pathological complete response of breast cancer. Discussion and conclusion. Similar cases in literature are commonly referred to a first-line surgery and the role of neoadjuvant chemotherapy is debatable. [ABSTRACT FROM AUTHOR]