[Lambert-Eaton myasthenic syndrome]. / Síndrome miasteniforme de Lambert-Eaton.

INTRODUCTION: Early diagnosis based on clinical findings, neurophysiological studies and serum antibody titres allows early initiation of symptomatic treatment and oncological screening. Reports of patients with LEMS in Latin America are scarce. AIM: This article aims to describe the characteristics of patients with LEMS from a private centre in Buenos Aires, Argentina, and to compare them with those of other series that have been published. PATIENTS AND METHODS: The medical records of 13 patients with LEMS with clinical findings, compatible electromyogram and/or positive antibodies were reviewed. Follow-up was performed until associated neoplasia was ruled out or confirmed according to the recommended algorithms. RESULTS: Four patients were diagnosed with T-LEMS, two of them with small-cell lung carcinoma. Of the nine patients with NT-LEMS, five had a DELTA-P score of 3 and 4. Nine patients presented with the classic clinical triad from the onset of the disease. All patients had electromyogram findings compatible with presynaptic neuromuscular plaque defect. Of the total, 70% improved symptomatically with pyridostigmine. CONCLUSIONS: The clinical findings, together with compatible neurophysiological studies, are sufficient for the diagnosis of LEMS. The relationship between the DELTA-P score and the risk of small-cell lung carcinoma could not be replicated. Symptomatic treatment with pyridostigmine represents an effective therapeutic alternative.

TITLE: Síndrome miasteniforme de Lambert-Eaton.Introducción. El síndrome miasteniforme de Lambert-Eaton (LEMS) es una patología paraneoplásica (T-LEMS) o idiopática autoinmunitaria (NT-LEMS) ocasionada por autoanticuerpos contra los canales de calcio dependientes del voltaje presinápticos de la unión neuromuscular. El 60% de los T-LEMS se asocia a carcinoma de pulmón de células pequeñas. Una puntuación Dutch-English LEMS Tumor Association Prediction (DELTA-P) mayor de 3 denota un riesgo elevado de dicha asociación. El diagnóstico precoz fundado en los hallazgos clínicos, estudios neurofisiológicos y dosificación de títulos de anticuerpos en el suero permite iniciar tempranamente el tratamiento sintomático y la búsqueda oncológica. Son escasos los informes de pacientes con LEMS en Latinoamérica. Objetivo. Describir las características de pacientes con LEMS de un centro privado de Buenos Aires, Argentina, y compararlas con las de otras series publicadas. Pacientes y métodos. Se revisaron historias clínicas de 13 pacientes con LEMS con hallazgos clínicos, electromiograma compatible y/o anticuerpos positivos. Se realizó seguimiento hasta descartar o confirmar una neoplasia asociada de acuerdo con los algoritmos recomendados. Resultados. Cuatro pacientes presentaron diagnóstico de T-LEMS, dos de ellos con carcinoma de pulmón de células pequeñas. De los nueve pacientes con NT-LEMS, cinco presentaron una puntuación DELTA-P de 3 y 4. Nueve pacientes presentaron la tríada clínica clásica desde el inicio. Todos los pacientes presentaron en el electromiograma hallazgos compatibles con defecto de placa neuromuscular presináptico. El 70% mejoró sintomáticamente con piridostigmina. Conclusiones. Los hallazgos clínicos, junto con los estudios neurofisiológicos compatibles, resultan suficientes para el diagnóstico de LEMS. No pudo replicarse la relación entre puntuación DELTA-P y riesgo de carcinoma de pulmón de células pequeñas. El tratamiento sintomático con piridostigmina representa una alternativa terapéutica eficaz.

Neuroendocrine tumors of the urinary bladder - Report of two cases

Primary paraganglioma and small cell neuroendocrine carcinoma of the urinary bladder are rare tumors, comprising 0.05% of all bladder tumors and <1% of all malignant bladder tumors, respectively. These tumors can be the cause of a diagnostic dilemma or misdiagnosis on morphology. Paraganglioma is often mistaken for urothelial carcinoma and small cell carcinoma for poorly differentiated carcinoma or lymphoma. Herein, we report a case of primary paraganglioma and another of a small cell carcinoma of the urinary bladder and discuss their closest differential diagnoses. The diagnostic pitfalls should be kept in mind so that correct, timely diagnosis of these entities can be made due to implications in the management and prognosis.

[Neuroendocrine carcinoma of the urinary bladder. A case report]. / Carcinoma neuroendocrino de vejiga. Reporte de un caso.

BACKGROUND: Small cell carcinoma of the urinary bladder is an infrequent lesion. CLINICAL CASE: We present the case of a 68-year-old male who arrived at the emergency room with a history of 24-h gross hematuria. Imaging studies show a urinary bladder tumor with a 218 cc volume that during a 20-day period increased to 426 cc. Histopathological images with hematoxylin-eosin show an infiltrating solid mass with uneven borders. It is composed of neoplastic cells with evident nuclei predominance and scant cytoplasm (small cells). Chromogranin immunohistochemical staining shows a diffusely positive cytoplasmic granular pattern on neoplastic cells. High molecular weight cytokeratin staining shows a negative pattern on neoplastic cells along with a positive pattern on reporsurrounding normal urothelium. Tumoral mass is positive for synaptophysin and CD-56 and negative for CK-7 and CK-20. Patient therapy was based on radiation plus chemotherapy. CONCLUSION: Small cell carcinoma of the urinary bladder represents 0.35-0.70% of urinary bladder tumors. Histological and immunohistochemical identification are key elements in the diagnosis. Treatment approach is based on cisplatin-based chemotherapy plus radical cystectomy, except when metastatic disease is present.

Antecedentes: el carcinoma neuroendocrino de células pequeñas primario de vejiga es una lesión maligna muy poco frecuente. Caso clínico: paciente masculino de 68 años de edad, que tuvo hematuria macroscópica de 24 horas de evolución. Estudios de imagen mostraron tumoración vesical de 218 cc, que en 20 días alcanzó un volumen de 426 cc. A la tinción con hematoxilina-eosina, histológicamente se apreció: placa sólida infiltrante de bordes irregulares, compuesta por células neoplásicas con claro predominio de núcleo y escaso citoplasma (células pequeñas). A la tinción inmunohistoquímica con cromogranina parecía difusamente positivo en células neoplásicas, en un patrón granular citoplasmático. A la tinción con citoqueratina de alto peso molecular se observó patrón negativo en células neoplásicas con control interno positivo en el urotelio acompañante en espécimen. De igual manera, la tumoración fue positiva para sinaptofisina y CD-56 y negativa para CK-7 y CK-20. El paciente recibió tratamiento a base de radioterapia y quimioterapia. Conclusión: el carcinoma neuroendocrino de células pequeñas primario de vejiga representa de 0.35 a 0.70% de los tumores vesicales primarios. Su diagnóstico se basa en el reconocimiento histológico e inmunohistoquímico. El tratamiento se fundamenta en quimioterapia con cisplatino más cistectomía radical, excepto cuando existe enfermedad metastásica.

Small cell neuroendocrine carcinoma of rectum with associated paraneoplastic syndrome: a case report.

Neuroendocrine carcinomas of the colon and rectum comprise fewer than 1% of all colorectal cancers. These aggressive tumors generally have a poor prognosis compared to that associated with colorectal adenocarcinoma. We describe herein the case of a 68-year-old female presenting with a bleeding rectal mass involving the anal canal, which case was associated with hyponatremia due to inappropriate serum levels of antidiuretic hormone. The histopathological examination was consistent with a small-cell neuroendocrine tumor. She was treated with combination chemotherapy and radiation therapy. The Syndrome of Inappropriate Antidiuretic Hormone (SIADH) was managed with vasopressin antagonists. After the completion of therapy, endoscopic ultrasound revealed evidence of residual disease, for which she underwent an abdominoperineal resection (APR). The patient died 4 months later of disease progression. To our knowledge, this is the first report of a small-cell neuroendocrine tumor involving the rectum and anal canal that presented with the paraneoplastic syndrome, SIADH.

Small cell carcinoma in Pancoast syndrome.

Pancoast syndrome consists of signs and symptoms resulting from a tumor affecting the pulmonary apex and adjacent structures. The process is typically caused by a neoplasm. The majority of cases of Pancoast syndrome are caused by bronchogenic carcinoma. The most commonly found histologic subtypes are adenocarcinoma and epidermoid carcinoma. There have been very few reports of small cell lung carcinoma in the genesis of Pancoast syndrome. We describe the case of a patient with Pancoast syndrome caused by small cell lung carcinoma and discuss the aspects related to the diagnosis and treatment.

Carcinoma de pequenas células na síndrome de Pancoast / Small cell carcinoma in Pancoast syndrome

A síndrome de Pancoast consiste de sinais e sintomas decorrentes do acometimento do ápice pulmonar e estruturas adjacentes por um tumor. Na maioria das vezes, o processo causal é uma neoplasia. O carcinoma broncogênico é a principal neoplasia causadora da síndrome. Os subtipos histológicos mais encontrados são o adenocarcinoma e o carcinoma epidermoide. A ocorrência de carcinoma de pequenas células de pulmão como gênese da síndrome de Pancoast é rara, com poucos relatos na literatura. Descrevemos o caso de um doente com síndrome de Pancoast causado por um carcinoma de pequenas células de pulmão, discutindo aspectos referentes ao diagnóstico e à terapêutica.

Pancoast syndrome consists of signs and symptoms resulting from a tumor affecting the pulmonary apex and adjacent structures. The process is typically caused by a neoplasm. The majority of cases of Pancoast syndrome are caused by bronchogenic carcinoma. The most commonly found histologic subtypes are adenocarcinoma and epidermoid carcinoma. There have been very few reports of small cell lung carcinoma in the genesis of Pancoast syndrome. We describe the case of a patient with Pancoast syndrome caused by small cell lung carcinoma and discuss the aspects related to the diagnosis and treatment.

Lung adenocarcinoma, dermatomyositis, and Lambert-Eaton myasthenic syndrome: a rare combination.

The incidence of lung neoplasms is increasing in Brazil and in the world, probably as a result of the increase in smoking. Due to the greater number of cases, atypical presentations appear. We report the case of a 66-year-old hypertensive male smoker who presented progressive proximal muscular weakness and, in two months, evolved to dysphagia, dysphonia, and V-shaped skin lesions on the chest. A chest X-ray showed a spiculated pulmonary nodule in the right upper lobe. The biochemical analysis revealed elevated creatine kinase levels. After complementary tests and biopsies, the patient underwent right upper lobectomy. Histopathology showed a moderately differentiated adenocarcinoma. The overall analysis of the case and a review of the literature allow us to suggest that the clinical profile of the patient was a result of an overlap of two paraneoplastic syndromes (dermatomyositis and Lambert-Eaton myasthenic syndrome) secondary to lung adenocarcinoma.

Adenocarcinoma pulmonar, dermatomiosite e síndrome miastênica de Lambert-Eaton: uma rara associação / Lung adenocarcinoma, dermatomyositis, and Lambert-Eaton myasthenic syndrome: a rare combination

A incidência das neoplasias pulmonares vem aumentando no Brasil e no mundo, provavelmente como resultado do aumento do tabagismo. Com o maior número de casos, surgem as apresentações atípicas. Relatamos o caso de um paciente do sexo masculino, 66 anos, tabagista e hipertenso, que apresentava quadro de fraqueza muscular proximal progressiva e, em dois meses, evoluiu com disfagia para alimentos sólidos, disfonia e lesões cutâneas em forma de "V" no tórax. O radiograma de tórax mostrou um nódulo pulmonar espiculado no lobo superior direito. A análise bioquímica revelou aumento da creatinoquinase. Após exames complementares e biópsias, o paciente foi submetido à lobectomia superior direita. A histopatologia evidenciou um adenocarcinoma moderadamente diferenciado. A análise global do caso e a revisão de literatura permitem sugerir que o quadro clínico do paciente era resultante da sobreposição de duas síndromes paraneoplásicas, a saber, a dermatomiosite e a síndrome miastênica de Lambert-Eaton, secundárias a um adenocarcinoma pulmonar.

The incidence of lung neoplasms is increasing in Brazil and in the world, probably as a result of the increase in smoking. Due to the greater number of cases, atypical presentations appear. We report the case of a 66-year-old hypertensive male smoker who presented progressive proximal muscular weakness and, in two months, evolved to dysphagia, dysphonia, and V-shaped skin lesions on the chest. A chest X-ray showed a spiculated pulmonary nodule in the right upper lobe. The biochemical analysis revealed elevated creatine kinase levels. After complementary tests and biopsies, the patient underwent right upper lobectomy. Histopathology showed a moderately differentiated adenocarcinoma. The overall analysis of the case and a review of the literature allow us to suggest that the clinical profile of the patient was a result of an overlap of two paraneoplastic syndromes (dermatomyositis and Lambert-Eaton myasthenic syndrome) secondary to lung adenocarcinoma.

Prognosis of lung cancer patients with life-threatening complications.

BACKGROUND: The management of patients with lung cancer has improved recently, and many of them will require admission to the ICU. The aims of this study were to determine hospital mortality and to identify risk factors for death in a large cohort of critically ill patients. METHODS: Cohort study in two ICUs specialized in the management of patients with cancer, in France and Brazil. RESULTS: Of the 143 patients (mean age, 61.6 +/- 9.9 years [+/- SD]), 25 patients (17%) had small cell lung cancer and 118 patients (83%) had non-small cell lung cancer. The main reasons for ICU admission were sepsis (44%) and acute respiratory failure (31%). Mechanical ventilation (MV) was used in 100 patients (70%), including 38 patients in whom lung cancer was considered a reason for MV. Hospital mortality was 59% overall and 69% in patients receiving MV. By multivariate logistic regression, airway infiltration or obstruction by cancer, number of organ failures, cancer recurrence or progression, and severity of comorbidities were associated with increased mortality. CONCLUSIONS: The improved survival previously reported in patients with cancer admitted to the ICU seems to extend to patients with lung cancer, including those who need MV. Mortality increased with the number of organ failures, severity of comorbidities, and presence of respiratory failure due to cancer progression. The type of the cancer per se was not associated with mortality and, therefore, should not be factored into ICU triage decisions.

Pontine metastases as a cause of dysphagia in lung carcinoma.

Dysphagia is an unusual symptom in the clinical course of lung carcinoma. When it appears, it is necessary to differentiate between regional dissemination, drug toxicity, opportunistic infection and, most rarely, metastatic dissemination to the brain stem. Magnetic resonance imaging (MRI) is the best diagnostic option to exclude this last possibility. We present a male patient with progressive dysphagia 15 months after the diagnosis of an oat-cell lung carcinoma. Cerebral MRI revealed a pontine lesion, probably of metastatic origin.

Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report.

Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and developed encephalopathy with cranial nerves signs and long tract spinal cord symptomatology. Brain CT scan and spinal MRI were normal. The CSF showed lymphocytic pleocytosis and no other abnormalities. EMG showed involuntary muscle activity with 2-6 seconds of duration, interval of 30-50 ms and a frequency of 2/second in the left lower limb. Anti-GAD antibodies were detected in the blood. We detected radiological signs of lung cancer during the follow-up, which proved to be an oat cell carcinoma. The patient died two weeks after the diagnosis of the cancer.

Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report

A encefalomielite progressiva com rigidez e mioclonia (PEWR) é doença neurológica rara, caracterizada por rigidez muscular, espasmos dolorosos, mioclonia e evidência de envolvimento de tronco cerebral e medula espinhal. Um paciente branco de 73 anos foi admitido com história de 10 dias de espasmos musculares dolorosos e rigidez muscular contínua no membro inferior esquerdo. Apresentava espasmos involuntários em membros inferiores e evoluiu com encefalopatia associada a sinais de nervos cranianos e sintomatologia de trato longo de medula espinhal. A tomografia computadorizada de crânio e a ressonância magnética de coluna foram normais. O LCR evidenciou pleocitose linfocítica, sem outras alterações. A EMG mostrou atividade muscular involuntária, de duração de 2-6 segundos, intervalo de 30-50 ms e uma freqüência de 2/segundo no membro inferior esquerdo. Foram detectados anticorpos anti-GAD no sangue. Na evolução, foram observados sinais radiográficos de neoplasia pulmonar, sendo posteriormente diagnosticado carcinoma de pequenas células de pulmão. O paciente faleceu duas semanas após o diagnóstico de câncer.

False positive troponin I in a case of metastatic small cell bronchogenic carcinoma complicated by pulmonary thromboembolism.

From the search for the ultimate cardiac marker have emerged the cardiac troponins, which have offered high sensitivity and specificity for myocardial damage. Troponin I has arguably been the best of this group, but even this marker is not infallible. We present the case of an elderly woman who died shortly after being diagnosed with acute myocardial infarction on the basis of elevated Troponin I. Autopsy revealed a small cell lung cancer complicated by pulmonary thromboembolism. There was no evidence of myocardial infarction. Explanations for false elevation of serum Troponin I are proposed.

[Partial non convulsive epileptic status as initial presentation of limbic encephalitis]. / Estado epiléptico no convulsivo como forma de presentación de encefalitis límbica.

INTRODUCTION: Limbic encephalitis is an unusual presentation of paraneoplastic syndrome, which includes among its symptoms seizures. CLINICAL CASE: We report a case with a rare presentation of limbic encephalitis as initial symptom of small cell lung carcinoma. A 69 year-old woman presented with partial non convulsive status epilepticus and neuropsychiatric disturbances. Chest radiography and computed tomography showed mediastinal lymphadenopathy and lung nodules. Subsequently, small cell lung carcinoma was diagnosed by lymph node biopsy. The cerebrospinal fluid study was normal. The electroencephalography and magnetic resonance imaging (MRI) findings had distinctive features compatible with temporo-limbic dysfunction. The anti-Hu antibodies were negative. The neuropsychiatric symptoms improved significantly after systemic chemotherapy and adjuvant radiotherapy. A serial follow-up MRI of the head showed no evidence of intracranial metastasis three months after the diagnosis of cancer. Limbic encephalitis may be an initial manifestation of lung cancer. Paraneoplastic limbic encephalitis is considered a remote effect of cancer commonly associated with anti-neuronal antibodies (anti-Hu) and small cell lung carcinoma. CONCLUSIONS: Status epilepticus could be an early sign of limbic encephalitis. The absence of anti-Hu antibodies does not rule out the presence of an underlying small cell lung carcinoma in patients with a clinical diagnosis of limbic encephalitis. Greater awareness for diagnosis and early treatment of the primary tumor offers the best chance for improvement in patients with lung cancer presenting with limbic encephalitis.

False positive troponin I in a case of metastatic small cell bronchogenic carcinoma complicated by pulmonary thromboembolism

From the search for the ultimate cardiac marker have emerged the cardiac troponin, which have offered high sensitivity and specificity for myocardial damage. Troponin I had arguably been the best of this group, but even this marker is not infallible. We present the case of an elderly women who died shortly after being diagnosed with acute myocardial infarction on the basis of elevated Troponin I. Autopsy revealed a small cell lung cancer complicated by pulmonary thromboembolism. There was no evidence of myocaridal infarction. Explanations for false elevation of serum Troponin I are proposed.(Au)

False positive troponin I in a case of metastatic small cell bronchogenic carcinoma complicated by pulmonary thromboembolism

From the search for the ultimate cardiac marker have emerged the cardiac troponins, which have offered high sensitivity and specificity for myocardial damage. Troponin I has arguably been the best of this group, but even this marker is not infallible. We present the case of an elderly woman who died shortly after being diagnosed with acute myocardial infarction on the basis of elevated Troponin I. Autopsy revealed a small cell lung cancer complicated by pulmonary thromboembolism. There was no evidence of myocardial infarction. Explanations for false elevation of serum Troponin I are proposed.

Síndrome de la vena cava superior: diagnóstico diferencial del edema angioneurótico / Superior vena caval syndrome: differencial diagnosis of angioneurotic edema

Presentamos el caso de un paciente, fumador de dos paquetes/día, que acudió a un servicio médico de urgencias refiriendo un cuadro de tres días de evolución de disnea y edema facial y palpebral. Con el diagnóstico de edema angioneurótico es remitido a nuestro servicio para estudio. A la exploración física el paciente presentaba un edema cérvico-facial y palpebral, así como una ingurgitación de las venas yugulares. En la parte superior del tórax se evidenciaron dilataciones varicosas de las venas superficiales. Con el diagnóstico de sospecha de síndrome de la vena cava superior (SVCS), se realizó una radiografía de tórax, en la que se objetivaron una masa pulmonar y un ensanchamiento mediastínico. Tras realizar una biopsia transbronquial mediante fibrobroncoscopía, se establece el diagnóstico de carcinoma broncogénico de células pequeñas (oat-cell). Se comentan la etiología, diagnóstico diferencial y tratamiento del SVCS

Síndrome de la vena cava superior: diagnóstico diferencial del edema angioneurótico / Superior vena caval syndrome: differencial diagnosis of angioneurotic edema

Presentamos el caso de un paciente, fumador de dos paquetes/día, que acudió a un servicio médico de urgencias refiriendo un cuadro de tres días de evolución de disnea y edema facial y palpebral. Con el diagnóstico de edema angioneurótico es remitido a nuestro servicio para estudio. A la exploración física el paciente presentaba un edema cérvico-facial y palpebral, así como una ingurgitación de las venas yugulares. En la parte superior del tórax se evidenciaron dilataciones varicosas de las venas superficiales. Con el diagnóstico de sospecha de síndrome de la vena cava superior (SVCS), se realizó una radiografía de tórax, en la que se objetivaron una masa pulmonar y un ensanchamiento mediastínico. Tras realizar una biopsia transbronquial mediante fibrobroncoscopía, se establece el diagnóstico de carcinoma broncogénico de células pequeñas (oat-cell). Se comentan la etiología, diagnóstico diferencial y tratamiento del SVCS (AU)