Carcinoma neuroendocrine de célula pequeña en la amígdala. Un caso clínico / Small Cell neuroendocrine carcinoma of the tonsil. A case report

Primary small neuroendocrine carcinoma (SNEC) of the tonsil it’s a rarity. We present the case of a 64 year-old Caucasian male patient referred to our hospital due to a neck mass. A fixed non-tender rock hard supraclavicular right mass and ipsilateral cervical lymphadenopathy was palpable. Biopsy was performed and showed poor differentiated neoplasm with features of SNEC. A fludeoxyglucose positron emission tomography (FDG-PET) scan showed intense uptake in the right tonsil. The patient underwent right tonsillectomy and immunochemistry was positive for characteristic features of small cell neuroendocrine carcinoma. Right tonsil SNEC was diagnosed and the patient was proposed to chemotherapy. (AU)

De novo Gastrinoma: A Case Report.

Gastrinomas are neuroendocrine tumors characterized by gastrin overexpression - 80% are sporadic and 20% are associated with multiple endocrine neoplasia type 1. A 75-year-old male patient, surgically treated at the age of 50 years for gastrinoma, followed on an outpatient basis because of chronic non-bloody diarrhea, was admitted to our hospital because of abdominal pain, watery diarrhea, and nonbiliary vomits. He was hypotensive and showed no response to fluids. Blood cultures were positive for Salmonella, and a diagnosis of septic shock due to Salmonella infection was made. The patient's condition improved, but the history of chronic diarrhea was still not explained. To investigate chronic diarrhea, gastrinoma recurrence was considered. Serum gastrin measurement was five times higher than the upper limit of the normal range (536 pg/mL). A positive somatostatin receptor scintigraphy was diagnostic for neuroendocrine tumor. Metastases were excluded. The patient was proposed to curative surgery, and a diagnosis of a well-differentiated neuroendocrine tumor was made.

Gastrinomas são tumores neuroendócrinos caracterizados por hipersecreção de gastrina - 80% são esporádicos e 20% associados a neoplasia endócrina múltipla tipo 1 (MEN1). Apresenta-se o caso de um homem, de 75 anos, tratado cirurgicamente aos 50 anos por gastrinoma, seguido em consulta de Medicina Interna por diarreia crónica não-sanguinolenta, internado no nosso hospital por dor abdominal, diarreia aquosa e vómitos não biliares. À admissão, apresentava-se hipotenso e pouco responsivo a fluidoterapia. As hemoculturas foram positivas para Salmonella e foi feito o diagnóstico de choque séptico por Salmonella. O doente melhorou, mas a história prévia de diarreia crónica não estava esclarecida. Para investigar a diarreia crónica, foi colocada como hipótese recidiva de gastrinoma. A medição de gastrina sérica foi cinco vezes superior ao limite superior do normal (536 pg/mL). A cintigrafia com receptores de somatostatina foi diagnóstica para tumor neuroendócrino. Foram excluídas metástases. O doente foi proposto para cirurgia curativa e foi feito o diagnóstico de tumor neuroendócrino bem diferenciado.

New insights in cryoglobulinemic vasculitis.

Cryoglobulins are antibodies that precipitate at low temperatures and dissolve after rewarming. Cryoglobulinemia refers to the presence of circulating cryoglobulins and generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, ulcers, neuropathy and/or glomerulonephritis. The disease mainly involves small to medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes. Cryoglobulinemia is classified into three types (I, II and III) on the basis of immunoglobulin composition. Predisposing conditions include lymphoproliferative, autoimmune diseases and hepatitis C virus infection. The diagnosis of cryoglobulinemic syndrome is predominantly based on the presence of clinical features and laboratorial demonstration of serum cryoglobulins. The treatment strategy depends on the cause of cryoglobulinemia. For patients with chronic HCV infection, antiviral therapy is indicated. Immunosuppressive or immunomodulatory therapy, including steroids, plasmapheresis and cytotoxic agents, is reserved for organ-threatening manifestations. In this review, we discuss the main clinical presentations, diagnostic approach and treatment options.

An Unusual Case of Tricuspid Stenosis.

Tricuspid stenosis is an uncommon valvular abnormality commonly associated with other valvular lesions. Ebstein's anomaly is a rare congenital heart malformation characterized primarily by abnormalities of the tricuspid valve and right ventricle. Endomyocardial fibrosis is a restrictive cardiomyopathy observed in tropical and subtropical regions. It may cause right ventricular distortion with apparent apical displacement of the tricuspid valve, mimicking Ebstein's anomaly. Eosinophilia is the most commonly cited aetiological link in endomyocardial fibrosis. Here we report the case of 42-year-old male patient who presented with heart failure and severe tricuspid stenosis where a diagnosis of hypereosinophilic syndrome was also established. This case represented a diagnostic challenge in the search for the definitive cause of the tricuspid stenosis. LEARNING POINTS: Ebstein's anomaly is a rare congenital heart malformation characterized primarily by abnormalities of the tricuspid valve and right ventricle. The tricuspid valve is usually incompetent, and very rarely stenotic.Hypereosinophilic syndromes can be associated with heart damage. The fibrotic stage of eosinophil-mediated heart damage is characterized by altered cardiac function due to either compromise/entrapment of the cordae tendineae and/or restrictive cardiomyopathy.Endomyocardial fibrosis is a restrictive cardiomyopathy observed in tropical and subtropical regions that may be indistinguishable from the Loeffler's endocarditis observed in temperate climates. It may cause right ventricle distortion and apical displacement of the tricuspid valve, mimicking Ebstein's anomaly.