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Las manifestaciones cutáneas de los tumores malignos comprenden un grupo de dermatosis que pueden ser marcadores de la presencia de neoplasias ocultas y permiten su diagnóstico oportuno. El objetivo de este informe es presentar las características clínicas de una acantosis nigricans asociada a carcinoma de mamario. Para ello, se describe el caso clínico de una paciente de 50 años, color de piel negro, asistida en la consulta de Dermatología del Policlínico Universitario Raúl Sánchez, por tener una placa única eritematosa localizada en la mama izquierda, acompañada de dolor, aumento de la temperatura local y máculas hipercrómicas en las axilas; así como adenopatías axilares múltiples. Los exámenes complementarios mostraron la presencia de acantosis nigricans maligna asociada a un carcinoma inflamatorio de la mama izquierda. Fue intervenida quirúrgicamente, con la consiguiente desaparición de las lesiones cutáneas, pero con un pronóstico reservado. La mayoría de los síndromes paraneoplásicos son inespecíficos; existe una necesidad urgente de sospechar una correlación entre los cambios cutáneos y la posibilidad de una neoplasia interna, por lo que es de suma importancia derivar a estos pacientes para su identificación y el diagnóstico precoz de la enfermedad de base. Esto mejoraría el pronóstico y atenuaría en gran medida las consecuencias.

The skin manifestations of malignant tumors include a group of dermatoses that can be the sign of the occult neoplasms presence and allow their timely diagnosis. The objective of this report is to present the acanthosis nigricans' clinical characteristics associated with breast carcinoma. The clinical case of a 50-years-old black-skinned patient is described, assisted in the Dermatology consultation of the Raúl Sánchez University Polyclinic, for having a single erythematous plaque located in the left breast, accompanied by pain, increased local temperature and hyperchromic macules in the armpits; as well as multiple axillary lymphadenopathy. Complementary examinations showed the presence of malignant acanthosis nigricans associated with an inflammatory carcinoma of the left breast. She underwent surgery, with the consequent disappearance of the skin lesions, but with a reserved prognosis. Most paraneoplastic syndromes are nonspecific; there is an urgent need to suspect a correlation between skin changes and the possibility of an internal neoplasia, so it is of utmost importance to refer these patients for identification and early diagnosis of the underlying disease. This would improve the prognosis and greatly mitigate the consequences.

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Abstract Background Autoimmune encephalitis (AE) consists of a group of acquired diseases that affect the central nervous system. A myriad of phenotypes may be present at the onset. Due to the heterogeneity of clinical presentations, it is difficult to achieve uniformity for the diagnostic and therapeutic processes and follow-up strategies. Objective To describe a series of patients diagnosed with AE in a resource-limited public hospital in southern Brazil and to analyze therapeutics and outcomes. Methods We retrospectively reviewed the electronic medical records of patients diagnosed with AE at the Hospital de Clínicas de Porto Alegre from 2014 to 2022. Data collected included clinical presentation, neuroimaging, cerebrospinal fluid testings, electroencephalogram, autoantibodies, treatments, outcomes, follow-up time, degree of neurological impairment, and mortality. Results Data from 17 patients were retrieved. Eleven cases were classified as definite AE and 6 as possible AE. Autoantibodies were identified in 9 patients. Timing for diagnosis was impacted by the high costs associated with autoantibody testing. Most patients became functionally dependent (82.4%) and most survivors remained with autoimmune-associated epilepsy (75%). Five patients died during hospitalization, and one after a 26-month of follow-up. Conclusion In this resource-limited hospital, patients with AE had a worse clinical outcome than that previously described in the literature. Development of epilepsy during follow-up and mortality were greater, whilst functional outcome was inferior. Autoantibody testing was initially denied in most patients, which impacted the definitive diagnosis and the use of second-line therapies.

Resumo Antecedentes A encefalite autoimune (EA) consiste em um grupo de doenças adquiridas que afetam o sistema nervoso central. Objetivo Descrever uma série de pacientes diagnosticados com EA em um contexto de atenção terciária à saúde com recursos limitados e analisar a terapêutica e os resultados. Métodos Revisamos retrospectivamente os prontuários eletrônicos de pacientes diagnosticados com EA no Hospital de Clínicas de Porto Alegre de 2014 a 2022. Os dados coletados incluíram apresentação clínica, neuroimagem, exames de líquido cefalorraquidiano, eletroencefalograma, autoanticorpos, tratamentos, resultados, tempo de acompanhamento, grau de comprometimento neurológico e mortalidade. Resultados Dados de 17 pacientes foram coletados. Onze casos foram classificados como EA definitivo e seis como EA possível. Autoanticorpos foram identificados em nove pacientes. O tempo para o diagnóstico foi afetado pelos altos custos associados ao teste de autoanticorpos. A maioria dos pacientes tornou-se funcionalmente dependente (82,4%), e a maioria dos sobreviventes permaneceu com epilepsia autoimune associada (75%). Cinco pacientes faleceram durante a internação, e um após 26 meses de seguimento. Conclusão No hospital em questão, os pacientes com EA tiveram um desfecho clínico pior do que o previamente descrito na literatura. O desenvolvimento de epilepsia durante o acompanhamento e a mortalidade foram maiores, enquanto o desfecho funcional foi inferior. Os testes de autoanticorpos foram inicialmente negados para a maioria dos pacientes, o que impactou o diagnóstico definitivo e o uso de terapias de segunda linha.

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Resumen El linfoma de Hodgkin (LH) comprende un grupo heterogéneo de neoplasias linfoides cuyo origen radica en linfocitos B. Las manifestaciones neurológicas de dicha enfermedad son infrecuentes, pudiendo tener su origen por invasión directa de las células neoplásicas en el sistema nervioso, o indirectamente, a través de síndromes paraneoplásicos o como complicación del tratamiento. Dentro de los síndromes neurológicos paraneoplásicos que afectan a pacientes con LH, la degeneración cerebelosa paraneoplásica es la más frecuente. Otros reportados con menor frecuencia en series de casos o casos aislados incluyen encefalitis límbica, neuronopatía sensitiva, motora y autonómica. Estos pueden ser la manifestación inicial de la enfer medad neoplásica, y la falta de conocimiento de dicha asociación puede retrasar el diagnóstico, con inicio tardío del tratamiento y peor pronóstico. Reportamos el caso de una mujer con LH que presentó al inicio de su enfermedad neuronopatía sensitiva y autonómica como manifestaciones neurológicas paraneoplásicas. Una vez iniciado el tratamiento específico para su lin foma, la neuronopatía autonómica tuvo resolución casi completa a diferencia de la neuronopatía sensitiva, la cual demostró escasa recuperación.

Abstract Hodgkin lymphoma (HL) comprises a heterogeneous group of lymphoid neoplasms whose origin lies in B lym phocytes. The neurological manifestations of this pathol ogy are infrequent, and may arise from direct invasion of neoplastic cells to the nervous system, or indirectly, through paraneoplastic syndromes or as a complication of treatment. Among the neurological paraneoplastic syndromes that affect patients with HL, paraneoplastic cerebellar degeneration is the most common. Other few cases include limbic encephalitis, sensory, motor, and autonomic neuronopathy. These syndromes can be the initial manifestation of neoplastic disease, and the lack of information regarding this association can lead to a delay in diagnosis and consequently in the initiation of therapy worsening the prognosis. We report the case of a woman with HL who presented sensory and autonomic neuronopathy at the onset of her disease as paraneo plastic neurological manifestations. After the initiation of the specific treatment for the lymphoma, the autonomic neuronopathy had almost complete resolution, unlike the sensory neuronopathy, which showed limited recovery.

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Hodgkin lymphoma (HL) comprises a heterogeneous group of lymphoid neoplasms whose origin lies in B lymphocytes. The neurological manifestations of this pathology are infrequent, and may arise from direct invasion of neoplastic cells to the nervous system, or indirectly, through paraneoplastic syndromes or as a complication of treatment. Among the neurological paraneoplastic syndromes that affect patients with HL, paraneoplastic cerebellar degeneration is the most common. Other few cases include limbic encephalitis, sensory, motor, and autonomic neuronopathy. These syndromes can be the initial manifestation of neoplastic disease, and the lack of information regarding this association can lead to a delay in diagnosis and consequently in the initiation of therapy worsening the prognosis. We report the case of awoman with HL who presented sensory and autonomic neuronopathy at the onset of her disease as paraneoplastic neurological manifestations. After the initiation of the specific treatment for the lymphoma, the autonomic neuronopathy had almost complete resolution, unlike the sensory neuronopathy, which showed limited recovery.

El linfoma de Hodgkin (LH) comprende un grupo heterogéneo de neoplasias linfoides cuyo origen radica en linfocitos B. Las manifestaciones neurológicas de dicha enfermedad son infrecuentes, pudiendo tener su origen por invasión directa de las células neoplásicas en el sistema nervioso, o indirectamente, a través de síndromes paraneoplásicos o como complicación del tratamiento. Dentro de los síndromes neurológicos paraneoplásicos que afectan a pacientes con LH, la degeneración cerebelosa paraneoplásica es la más frecuente. Otros reportados con menor frecuencia en series de casos o casos aislados incluyen encefalitis límbica, neuronopatía sensitiva, motora y autonómica. Estos pueden ser la manifestación inicial de la enfermedad neoplásica, y la falta de conocimiento de dicha asociación puede retrasar el diagnóstico, con inicio tardío del tratamiento y peor pronóstico. Reportamos el caso de una mujer con LH que presentó al inicio de su enfermedad neuronopatía sensitiva y autonómica como manifestaciones neurológicas paraneoplásicas. Una vez iniciado el tratamiento específico para su linfoma, la neuronopatía autonómica tuvo resolución casi completa a diferencia de la neuronopatía sensitiva, la cual demostró escasa recuperación.

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Introducción: El melanoma uveal, es el cáncer intraocular más común en adultos, representa cerca del 3 % al 5 % de todos los melanomas; con un pico de incidencia en hombres en la sexta década de la vida. Objetivo: Describir la presentación clínica, diagnóstico y tratamiento de una paciente con melanoma uveal. Caso clínico: Paciente femenina de 39 años de edad, con antecedentes de salud, quien acudió a consulta por presentar dolor intenso de doce días de evolución localizado en el ojo derecho, asociado a pérdida de la visión, dolor a los movimientos oculares, astenia y anorexia. Al examen físico se encontró edema palpebral severo, abundantes secreciones mucopurulentas, quémosis conjuntival en 360 grados marcado e hiperemia cilio-conjuntival en el ojo derecho. Se realizaron estudios analíticos e imagenológicos. Al analizar el resultado de la exploración física y los complementarios se decidió la intervención quirúrgica consistente en la exenteración orbitaria derecha, esfenoidectomía y etmoidectomía transorbitaria derecha. Con una evolución satisfactoria fue egresada del centro hospitalario. La biopsia informó un melanoma coroideo. Conclusiones: El melanoma coroideo es una enfermedad ocular poco frecuente en mujeres jóvenes de piel negra. La sintomatología es extremadamente variable con las masas coroideas. Es importante conocer sus características, pues estos efectos clínicos sirven como un recordatorio para los oftalmólogos para incluirla en el diagnóstico diferencial de otras patologías oculares.

Introduction: Uveal melanoma is the most common intraocular cancer in adults, represents approximately 3 % to 5 % of all melanomas; with a peak incidence in men in the sixth decade of life. Objective : To describe the clinical presentation, diagnosis and treatment of a patient with uveal melanoma. Clinical case: A 39-year-old female patient, with a health history, who came to the consultation due to intense pain of twelve days of evolution located in the right eye, associated with loss of vision, pain with eye movements, asthenia and anorexy. Physical examination revealed severe palpebral edema, abundant mucopurulent secretions, marked 360-degree conjunctival chemosis, and cilio-conjunctival hyperemia in the right eye. Analytical and imaging studies were performed. When analyzing the results of the physical and complementary examination, the surgical intervention consisting of right orbital exenteration, sphenoidectomy and right transorbital ethmoidectomy was decided. With a satisfactory evolution, she was discharged from the hospital. Biopsy reported choroidal melanoma. Conclusions: Choroidal melanoma is a rare ocular entity in young black women. Symptomatology is extremely variable with choroidal masses. It is important to know its characteristics, since these clinical effects serve as a reminder for ophthalmologists to include it in the differential diagnosis of other ocular pathologies.

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Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge.

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Mielopatia inflamatória ou mielite transversa é uma síndrome neurológica potencialmente incapacitante com uma variedade de etiologias. Episódios únicos ou recorrentes podem resultar em dependência de cadeira de rodas. O quadro clínico de fraqueza, alteração de sensibilidade e disfunção autonômica de início agudo ou subagudo é marca dessa síndrome. Esse cenário é comum às diferentes etiologias, que podem ser de natureza desmielinizante, por doença autoimune sistêmica, paraneoplásica ou infecciosa. A ressonância magnética de coluna é o exame de neuroimagem de escolha. Exames complementares como avaliação do líquido cefalorraquidiano, testes sorológicos e pesquisa de anticorpos dão suporte à investigação. A depender da etiologia, há tratamentos específicos a fim de reduzir incapacidade e chance de novos surtos, além de diferentes prognósticos. Este trabalho objetiva uma revisão de literatura sobre mielopatias inflamatórias e suas principais etiologias, a partir de dados obtidos na plataforma eletrônica PubMed. Para a discussão, foram revisadas as etiologias desmielinizantes (encefalomielite disseminada aguda, esclerose múltipla, doença do espectro, neuromielite óptica e neurite óptica, encefalite e mielite associadas ao MOG-IgG); doenças autoimunes (lúpus eritematoso sistêmico e síndrome de Sjögren); síndromes paraneoplásicas e mielopatias infecciosas (neuroesquistossomose, mielite por HIV e por HTLV-1 e neurossífilis). Concluiu-se com este estudo que a mielopatia inflamatória é uma condição de gravidade variável que produz potencial incapacidade, causada por diferentes etiologias, porém com quadro clínico comum entre elas. Por isso, é importante conhecer cada uma dessas causas, a fim de promover o melhor e mais precoce tratamento e reduzir sequelas.

Inflammatory myelopathy or transverse myelitis is a potentially disabling neurological syndrome with various etiologies. Single or recurrent episodes can result in wheelchair dependence. A clinical picture of weakness, altered sensitivity, and autonomic dysfunction with acute or subacute onset is characteristic of this syndrome. This scenario is common to different etiologies, which can be of a demyelinating nature, due to systemic, paraneoplastic, or infectious autoimmune disease. Spine MRI is the neuroimaging test of choice. Complementary tests such as cerebrospinal fluid evaluation, serological tests and antibody research support the investigation. Depending on the etiology, there are specific treatments to reduce disability and the chance of new episodes, and different prognoses. This study is a literature review on inflammatory myelopathies and their main etiologies, based on data obtained from the PubMed database. Demyelinating etiologies (acute disseminated encephalomyelitis, multiple sclerosis, neuromyelitis optic spectrum disease and optic neuritis, MOG-IgG-associated encephalitis and myelitis), autoimmune diseases (systemic lupus erythematosus and Sjögren's syndrome), paraneoplastic syndromes and infectious myelopathies (neuroschistosomiasis, HIV and HTLV-1 myelitis, and neurosyphilis) were reviewed for discussion. In conclusion, inflammatory myelopathy is a condition of variable severity that produces potential disability, caused by different etiologies, but with a common clinical picture between them. Thus, knowledge on each of these causes is important to promote the best and earliest treatment and reduce sequelae.

La mielopatía inflamatoria o mielitis transversa es un síndrome neurológico potencialmente incapacitante con una variedad de etiologías. Los episodios únicos o recurrentes pueden tener como consecuencia dependencia de silla de ruedas. El cuadro clínico de debilidad, sensibilidad alterada y disfunción autonómica de inicio agudo o subagudo es distintivo de este síndrome. Esto es común a diferentes etiologías, que pueden ser de naturaleza desmielinizante, debido a enfermedades autoinmunes sistémicas, paraneoplásicas o infecciosas. La resonancia magnética de columna es la prueba de neuroimagen de elección. Las pruebas complementarias, como la evaluación del líquido cefalorraquídeo, las pruebas serológicas y la investigación de anticuerpos respaldan la investigación. Dependiendo de la etiología, existen tratamientos específicos para reducir la discapacidad y la posibilidad de nuevos brotes, además de diferentes pronósticos. Este trabajo tiene como objetivo revisar la literatura sobre mielopatías inflamatorias y sus principales etiologías desde los datos obtenidos de la base de datos electrónica PubMed. Se revisaron las etiologías desmielinizantes (encefalomielitis aguda diseminada, esclerosis múltiple, enfermedad del espectro, neuromielitis óptico y neuritis óptica, encefalitis y mielitis asociadas a MOG-IgG), las enfermedades autoinmunes (lupus eritematoso sistémico y síndrome de Sjögren), los síndromes paraneoplásicos y mielopatías infecciosas (neurosquistosomiasis, mielitis por VIH y HTLV-1 y neurosífilis). Se concluyó que la mielopatía inflamatoria es una condición de severidad variable, que produce potencial discapacidad causada por diferentes etiologías, pero tiene un cuadro clínico común entre ellas. Por ello, es importante conocer cada una de las causas para promover el mejor y más precoz tratamiento, además de reducir las secuelas.

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The tumor microenvironment is a dynamic, complex, and redundant network of interactions between tumor, immune, and stromal cells. In this intricate environment, cells communicate through membrane-membrane, ligand-receptor, exosome, soluble factors, and transporter interactions that govern cell fate. These interactions activate the diverse and superfluous signaling pathways involved in tumor promotion and progression and induce subtle changes in the functional activity of infiltrating immune cells. The immune response participates as a selective pressure in tumor development. In the early stages of tumor development, the immune response exerts anti-tumor activity, whereas during the advanced stages, the tumor establishes mechanisms to evade the immune response, eliciting a chronic inflammation process that shows a pro-tumor effect. The deregulated inflammatory state, in addition to acting locally, also triggers systemic inflammation that has repercussions in various organs and tissues that are distant from the tumor site, causing the emergence of various symptoms designated as paraneoplastic syndromes, which compromise the response to treatment, quality of life, and survival of cancer patients. Considering the tumor-host relationship as an integral and dynamic biological system, the chronic inflammation generated by the tumor is a communication mechanism among tissues and organs that is primarily orchestrated through different signals, such as cytokines, chemokines, growth factors, and exosomes, to provide the tumor with energetic components that allow it to continue proliferating. In this review, we aim to provide a succinct overview of the involvement of cancer-related inflammation at the local and systemic level throughout tumor development and the emergence of some paraneoplastic syndromes and their main clinical manifestations. In addition, the involvement of these signals throughout tumor development will be discussed based on the physiological/biological activities of innate and adaptive immune cells. These cellular interactions require a metabolic reprogramming program for the full activation of the various cells; thus, these requirements and the by-products released into the microenvironment will be considered. In addition, the systemic impact of cancer-related proinflammatory cytokines on the liver-as a critical organ that produces the leading inflammatory markers described to date-will be summarized. Finally, the contribution of cancer-related inflammation to the development of two paraneoplastic syndromes, myelopoiesis and cachexia, will be discussed.

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BACKGROUND: Urticaria is one of the most common causes of emergency room visits. It is defined as an acute inflammatory dermatosis, characterized by localized degranulation of mast cells, with consequent dermal microvascular and formation of edematous and pruritic plaques called hives. Urticaria affects the skin and tissues of the superficial mucosa. Sometimes it is accompanied by angioedema, which is characterized by deeper edema of the dermis and subcutaneous cellular tissue known as the urticarial-angioedema syndrome. About 15%-25% of the general population has suffered at least one type of urticaria at some point during their lifetime and hyperpermeability estimated at 7.6%-16% and has experienced acute urticaria that is usually self-limited and spontaneously resolves without requiring medical attention. CASE SUMMARY: We present the case of a young male patient who was referred to our department with a clinical picture of 4 mo of pruritus associated with hives of variable sizes, irregular borders, with interlesional confluence, that were non-painful, without involvement of the palms and soles of the feet but with a tendency to progression in a generalized manner. He had multiple emergency room visits and poor response to antihistamines and systemic corticosteroids. Imaging studies demonstrated nodules in the lower lingula segment, at the level of the greater fissure and in the anterior contour of the left anterior basal segment associated with parahiliar adenopathies in the absence of findings suggestive of infectious or autoimmune etiology. Segmental lobectomy was performed by thoracoscopy with resection of a lung nodule in the lingula and biopsy of the para-aortic mediastinal ganglion. The histopathological report showed the presence of poorly differentiated invasive adenocarcinoma with a solid morphological and acinar pattern with immunohistochemical description of lung tissue that expresses strong positive and diffuse reaction for thyroid transcription factor 1 (TTF-1) with negativity to P40 for a histopathological diagnosis of malignant epithelial neoplasia with expression of infiltrating adenocarcinoma. Spontaneous chronic urticaria is considered possibly secondary to lung adenocarcinoma. CONCLUSION: Chronic spontaneous urticaria is considered a paraneoplastic dermatosis with a controversial association in the literature. In the presented case, a young patient presented with chronic refractory urticaria and after an exhaustive clinical work-up was found to have a diagnosis of poorly differentiated lung adenocarcinoma with high expression of TTF-1. According to the Curth criteria, the urticaria presented by the patient is related to the oncological diagnosis. In addition, the high expression of TTF-1 documented in this case could be acting as an autoantigen that would cause chronic spontaneous urticaria. Further research evaluating a causal relationship between the TFF-1 protein and urticaria in lung cancer is needed.

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Lipase hypersecretion syndrome (LHS) is a rare paraneoplastic syndrome, associated with acinar cell carcinoma of the pancreas (ACCP) in 10% to 15% of patients. Clinically, LHS manifests itself with the appearance of subcutaneous fibrocystic nodules, associated with trophic changes in the overlying skin, such as ulcers or fistulas that are difficult to manage, mainly affecting the lower extremities. Additionally, lipolysis near the joints and in the intraosseous adipose tissue can cause bilateral arthralgias, especially of the knees and ankles. We report a 57-year-old man, with a history of insulin resistance and allergic rhinitis, who presented in June 2019 with multiple subcutaneous nodules in the lower extremities, predominantly in both ankles, associated with arthralgia in that region. Additionally, a CT scan of the abdomen revealed a significant abdominal mass, measuring approximately 17 cm and in contact with the body and tail of the pancreas, pathologically compatible with an ACCP. Treatment with capecitabine was started with a favorable progression. The patient currently presents a small left lateral retro malleolar fistula, which, given the analyzes, studies and reviewed literature is concluded to be a lesion in the context of LHS.

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ABSTRACT Autoimmune encephalitis (AE) comprises a group of diseases mediated by antibodies against neuronal cell surface or synaptic antigens, such as ion channels or neurotransmitter receptors. New clinical syndromes and their associated antibodies were and are still being characterized over the last two decades. The fact that their main clinical features are interdisciplinary, - encompassing neuropsychiatric symptoms, cognitive dysfunction, epileptic seizures, movement and sleep disorders - has led to a surge of interest in this field. Some of these diseases present with a well-defined syndrome, being recognizable on clinical grounds. Correct diagnosis is important since AE are potentially treatable diseases, despite their severity. On the other hand, an increasing number of neuronal antibodies being described casts doubt upon the way we should utilize antibody testing and interpret results. In this article we review, summarize and update the current knowledge on antibody mediated encephalitis.

RESUMO As encefalites autoimunes compreendem um grupo de doenças mediadas por anticorpos contra antígenos de superfície neuronal ou sinapse, como canais iônicos ou receptores de neurotransmissores. Novas síndromes clínicas e os anticorpos a elas associados foram e ainda estão sendo caracterizados ao longo das últimas duas décadas. Dado que suas principais características clínicas são interdisciplinares, isto é, incluem -se sintomas neuropisquiátricos, disfunção cognitiva, crises epilépticas, distúrbio do movimento e do sono, há uma grande onda de interesse sobre esse campo de conhecimento. Algumas dessas doenças apresentam-se com uma síndrome bem definida, sendo possível reconhecê-las clinicamente. Diagnosticá-las corretamente é importante uma vez que se trata de doenças potencialmente tratáveis apesar da gravidade que lhes é característica. Por outro lado, o número crescente de anticorpos sendo descritos causam dúvida frequente sobre qual o melhor teste a solicitar e como interpretá-los. Nós aqui apresentamos uma revisão atualização resumida do conhecimento atual sobre as encefalites mediadas por anticorpos.

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RESUMEN: El eritema multiforme es una enfermedad autoinmune vesicular-ampollosa y autolimitada, se presenta de forma aguda afectando la piel y mucosa. En 2015, Lee E. y Freer J., incluyeron esta enfermedad dentro de los síndromes dermatológicos paraneoplásicos, pero actualmente no existen muchas publicaciones de esta entidad como manifestación a una neoplasia subyacente. El objetivo de esta investigación es presentar el eritema multiforme como una manifestación paraneoplásica inusual. En este caso una paciente de 64 años de edad que cursaba con adenocarcinoma de colon exhibió el eritema multiforme oral; cuyas manifestaciones bucales involucionaron gracias a la farmacoterapia local y sistémica en conjunto con la excéresis de la lesión cancerosa en el colon. Es importante reconocer la presencia de eritema multiforme como una manifestación paraneoplásica cuando no se exhiben los factores etiológicos frecuentes de esta patología y, por lo tanto, centrarse en la búsqueda subyacente de neoplasias malignas que no son muy evidentes.

ABSTRACT: Erythema multiforme is a self-limiting, blistering, autoimmune disease that presents acutely, affecting the skin and mucosa. In 2015, Lee E. and Freer J. included this disease in paraneoplastic dermatological syndromes, but there are currently not many publications about this entity as a manifestation of an underlying malignancy. The objective of this research is to present the erythema multiforme as an unusual paraneoplastic manifestation. In this case, a female 64-year-old patient with colon adenocarcinoma exhibited oral multiform erythema; whose oral manifestations regressed thanks to local and systemic pharmacotherapy in conjunction with the exeresis of the cancerous lesion in the colon. It is important to recognize the presence of erythema multiforme as a paraneoplastic manifestation when the frequent etiological factors of this pathology are not exhibited and, therefore, to focus on the underlying search for malignant neoplasms that are not very evident.

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RESUMEN Fundamento Las dermatosis paraneoplásicas son poco frecuentes, y acerca de ellas no existen estudios previos en la provincia de Cienfuegos. De su correcto reconocimiento depende en ocasiones el diagnóstico oportuno de una posible neoplasia interna. Objetivo describir el comportamiento de las dermatosis paraneoplásicas en pacientes oncológicos. Métodos estudio descriptivo, realizado en pacientes con neoplasias malignas, atendidos durante el período de junio de 2016 a diciembre de 2017, en el Hospital Dr. Gustavo Aldereguía Lima, de Cienfuegos. Fueron incluidos 59 pacientes con dermatosis paraneoplásicas, para los cuales se analizó tipo de dermatosis, momento de aparición y su asociación con cada tipo de neoplasia. La información se obtuvo a través del exámen clínico dermatológico y las historias clínicas. Resultados se identificaron 17 tipos de dermatosis paraneoplásicas, con destaque del signo Leser Trélat (21,0 %), seguido del Síndrome de Sweet (16,1 %) y el pioderma gangrenoso (11,3 %). En 25 (42,4 %) pacientes estas fueron detectadas antes del diagnóstico de neoplasias, entre las cuales el linfoma (86,7 %) fue la más representativa, seguido por las neoplasias de colon y mamas, aunque también se asociaron a Síndrome de Sweet y a pioderma gangrenoso. Conclusión Reconocer las lesiones características de las dermatosis paraneoplásicas facilita la detección precoz de un cáncer oculto, cuyas manifestaciones propias, probablemente, aparecerían meses después, incluso años.

ABSTRACT Background Paraneoplastic dermatoses are rare, and about them there are no previous studies in the Cienfuegos province. The timely diagnosis of a possible internal neoplasm sometimes depends on its correct recognition. Objective to describe the behavior of paraneoplastic dermatoses in cancer patients. Methods descriptive study, carried out in patients with malignant neoplasms, treated from June 2016 to December 2017, at the Dr. Gustavo Aldereguía Lima Hospital, in Cienfuegos. Fifty-nine patients with paraneoplastic dermatosis were included, for which the type of dermatosis, time of appearance and its association with each type of neoplasia were analyzed. The information was obtained through the clinical dermatological examination and the medical records. Results 17 types of paraneoplastic dermatoses were identified, highlighting the Leser Trélat sign (21.0%), followed by Sweet's syndrome (16.1%) and pyoderma gangrenosum (11.3%). In 25 (42.4%) patients these were detected before the diagnosis of neoplasms, among which lymphoma (86.7%) was the most representative, followed by colon and breast neoplasms, although they were also associated with Syndrome of Sweet and a pyoderma gangrenosum. Conclusion Recognizing the characteristic lesions of paraneoplastic dermatoses facilitates the early detection of a hidden cancer, whose own manifestations would probably appear months later, even years.

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Rapidly progressive cerebellar ataxia is a classical paraneoplastic neurological syndrome associated with different autoantibodies and typical demographic characteristics, extracerebellar signs, tumor association, and prognosis. Anti-Tr/anti-Delta/Notch-like epidermal growth factor-related receptor (DNER) antibody is one of the associated antibodies. Given the rarity of this condition, our current knowledge is based on case reports and small case series. In order to improve our understanding of these conditions, we conducted a systematic review of the literature. Our study followed the PRISMA reporting guidelines. Studies of patients with the presence of anti-Tr/DNER antibodies in serum or cerebrospinal fluid (CSF) were included. We extract data information related to study characteristics, demographics, clinical symptoms, tumor association, neuroimaging, and cerebrospinal fluid analysis. Out of 131 records, we analyzed 17 papers, including a total of 85 patients with anti-Tr/DNER antibody-associated cerebellar ataxia. We confirmed that this disease occurred mostly in middle-aged males. Isolated cerebellar ataxia was the most common presentation. Extracerebellar features were rare (8%). Ninety-one percent of the patients presented an associated tumor, being Hodgkin lymphoma the most common. Abnormal neuroimaging patterns included cerebellar atrophy (19%) and cerebellar hypersignal (6%). Cerebrospinal fluid was inflammatory in 64% of the patients. Oncological response was complete in 88%, but neurological prognosis was poor with only 41% of the patients presenting significant neurological improvement at the last follow up. Anti-Tr/DNER antibodies should be tested in rapid progressive cerebellar ataxia. Oncological response is excellent; however, many patients do not improve from their cerebellar ataxia.

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ABSTRACT Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge.

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INTRODUCCIÓN: El teratoma del ovario es el tumor de células germinales más frecuente. Entre sus complicaciones se describen algunos síndromes paraneoplásicos, como la encefalitis por anticuerpos contra el receptor N-metil-D-aspartato (NMDA). CASO CLÍNICO: Mujer de 22 años sin antecedentes de importancia que consulta por cuadro clínico de 4 días de evolución caracterizado por síntomas psiquiátricos y un episodio convulsivo. Se considera un cuadro de meningoencefalitis y se indica manejo antibiótico de amplio espectro y antiviral. Ante el deterioro se sospecha una encefalitis autoinmunitaria, se identifica un tumor anexial en los estudios imagenológicos compatible con teratoma y se confirma el diagnóstico con el hallazgo de anticuerpos NMDA en el líquido cefalorraquídeo. A pesar de su resección y manejo sistémico, fallece a los 5 meses. DISCUSIÓN: La encefalitis autoinmunitaria asociada a un teratoma es inusual, pero es una complicación que debe sospecharse como diagnóstico de exclusión. La mayoría tienen un pronóstico favorable, aunque hasta una cuarta parte de los casos puede asociarse a daño irreversible en la corteza del hipocampo e incluso la muerte, principalmente cuando el diagnóstico y el tratamiento son tardíos. CONCLUSIONES: Este caso es un reto clínico que representa un vacío en la evidencia actual, puesto que no existe un estándar de manejo de los teratomas. Se propone que, una vez diagnosticado un teratoma, se realice una cistectomía ovárica. Son necesarios más estudios para validar esta recomendación.

INTRODUCTION: The ovarian teratoma is the most common cell germ tumor. Some paraneoplastic syndromes have been described, including the anti-N-methyl-D-aspartate (NMDA) receptor encephalitis as part of its complications. CASE REPORT: A 22 years old female patient with no important medical history, consults due to an acute psychotic disorder and a convulsion. A meningoencephalitis was considered and broad-spectrum antibiotics and antivirals were started. Faced with deterioration, an autoimmune encephalitis is considered as well. Imaging studies revealed an ovarian teratoma and diagnosis was confirmed with antibodies against NMDA receptor in cerebrospinal fluid. Despite its resection and systemic management, the patient dies after 5 months. DISCUSSION: The autoimmune encephalitis associated with an ovarian teratoma is rare, its a complication that must be suspected as an exclusion diagnosis and most have a favorable prognosis, however up to a quarter of cases can be associated with irreversible damage to the hippocampal cortex and even death, mainly when late diagnosis and treatment are made. CONCLUSIONS: This case is a clinical challenge, no evidence is available since there is no standard for teratoma management. It is proposed that once a teratoma is diagnosed, an ovarian cystectomy is performed. Further studies are necessary to validate this recommendation.

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BACKGROUND: Canine multicentric lymphomas are lymphoproliferative malignancies that have increased in recent decades. The patient's treatment and prognosis are determined by the grade, histological type, and lymphoma immunophenotyping. AIM: To investigate the paraclinical signs and survival time in canines with different lymphoma immunophenotypes. METHODS: Over 2 and a half years, 47 untreated dogs were diagnosed with multicentric lymphoma at the Veterinary School Hospital of Uruguay. The disease was clinically and cytologically diagnosed, and immunophenotyping was determined by flow cytometry. After the immunophenotyping, most of the patients were grouped into the following: B (LB), T aggressive (LTCD45+), or T-zone lymphoma (LTCD45-). The patients' haematological values, calcemia, lactate dehydrogenase (LDH) levels, and plasmatic electrophoretic profiles were all determined immediately after that. RESULTS: Of all canine lymphomas, 55.3% were B, 31.9% were LTCD45+, and 10.6% were TCD45-. Only 2.2% were classified as nonB/nonT, and survival time differed between groups. Patients with LTCD45- lymphomas had a mean life span of 641 days after diagnosis, followed by LB (166 days) and LTCD45+ (62 days). Red blood cell count, hematocrit, and hemoglobin levels did not differ between groups. However, the LTCD45- group had significantly higher lymphocyte levels than the LTCD45+ and LB groups (p = 0.01 and 0.006, respectively). Levels of albumin, alpha-1, and alpha-2 globulins did not differ between groups. On the other hand, gamma globulins levels in the LTCD45- were higher than in the other lymphoma groups. The presence of hypercalcemia and high plasma LDH levels were associated with patient severity. Only the TCD45+ group had hypercalcemia although both the LB and TCD45+ groups had elevations in LDH activity. Interestingly, there was a direct relationship between high LDH values (greater than 500 IU/l) and lower survival in TCD45+ lymphomas. CONCLUSION: Survival time and hematological and biochemical patterns differed among canine lymphomas immunophenotypes. Patients of LTCD45- phenotype showed higher lymphocyte counts and gamma globulin levels and more prolonged survival. Serum LDH activity may provide additional prognostic information in high-grade T-cell lymphoma.

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Introducción: La dermatomiositis es una inflamación muscular autoinmune con presencia de rash, se manifiesta con debilidad muscular proximal, asociado a complicaciones cardiacas como miocarditis y/o trastornos de la conducción. En algunas ocasiones puede ser la única manifestación clínica de una neoplasia maligna oculta, por lo cual su detección temprana puede tener grandes repercusiones en el pronóstico del tumor oculto. Objetivo: Describir el caso de una paciente con un síndrome paraneoplásico dado por una dermatopolimiositis como presentación inusual de un cáncer de mama. Caso clínico: Se trata de una paciente en quinta década de la vida sin antecedentes personales de importancia, que debuta con una debilidad en miembros superiores de predominio proximal incapacitante, fiebre y elevación importante de la creatinquinasa (CPK), bajo un diagnóstico de una dermatopolimiositis como manifestación principal de un cáncer de mama infiltrante. Conclusiones: La dermatomiositis en mujeres de mediana edad debe hacer sospechar en una patología neoplásica oculta. Lo más importante es descastar el cáncer de mama(AU)

Introduction: Dermatomyositis is an autoimmune muscle inflammation exhibited rash, and proximal muscle weakness, associated with cardiac complications such as myocarditis and / or conduction disorders. In some cases, it can be the only clinical manifestation of hidden malignancy, so its early detection can have great repercussions on the prognosis of the hidden tumor. Objective: To describe the case of a patient with a paraneoplastic syndrome caused by dermatopolymyositis as an unusual presentation of breast cancer. Clinical case report: This is a patient in her fifties with no significant clinical personal history, who had disabling proximal weakness, predominantly in her upper limbs, fever and significant elevation of creatine kinase (CPK), under a diagnosis of dermatopolymyositis as the main manifestation of an infiltrating breast cancer. Conclusions: Dermatomyositis in middle-aged women should make us suspect an occult neoplastic pathology. The most important is to rule out breast cancer(AU)

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RESUMEN Los síndromes paraneoplásicos neurológicos se presentan en menos del 1% de los tumores sólidos y son infrecuentes en linfomas. Se asocian a tumores con alta actividad biológica y condicionan deterioro funcional y discapacidad. La dermatomiositis se asocia a cáncer, por tanto obliga al estudio de neoplasias ocultas; su diagnóstico como síndrome paraneoplásico se establece con criterios específicos. El pronóstico funcional depende del diagnóstico oportuno, control del cáncer y de la regulación de la respuesta inmunológica. Se presenta el caso de una mujer de 65 años con dermatomiositis en el curso de un linfoma B marginal variante convencional de primario cutáneo.

ABSTRACT Neurological paraneoplastic syndromes occur in less than 1% of solid tumours and are uncommon in lymphomas. They are related to tumours with high biological activity and cause functional impairment and disability. Dermatomyositis is associated with cancer, and requires the study of hidden neoplasms. Its diagnosis as a paraneoplastic syndrome is established with specific criteria. Functional prognosis depends on early diagnosis, cancer control, and regulation of the immune response. The case is presented of a 65 year-old woman with dermatomyositis during the course of a conventional variant of a primary cutaneous B marginal lymphoma.

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