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.

Metabolic Super Scan in 18F-FDG PET/CT Imaging

A 50-yr-old man presented with intermittent hemoptysis and was diagnosed small cell lung cancer. 18F-FDG PET/CT for staging demonstrated extensive hypermetabolic lesions throughout the skeleton and liver. Interestingly, skeletal muscles of limbs, mediastinum, bowel, and especially brain showed very low FDG uptake. Because of some characteristics in common with super scan on skeletal scintigraphy, this case could be considered as 'metabolic super scan'.

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.

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.

Bone marrow involvement at presentation in pediatric non-haematological small round cell tumours.

Pediatric small round cell tumors (SRCT) are a group of neoplasms occurring in children, which have in common a cytomorphology of groups of small round cells with scanty cytoplasm. The common SRCT encountered are neuroblastoma, retinoblastoma, Ewing's sarcoma/peripheral neuroectodermal tumor (PNET), rhabdomyosarcoma and lymphoma which show varying degrees of bone marrow involvement and bone marrow evaluation forms a part of the initial staging procedure. This study was undertaken to evaluate marrow involvement at presentation in pediatric non hematological SRCT. 7833 bone marrow aspirates done over a period of three years in different malignancies were analysed and of these 180 aspirates were performed in patients of pediatric non hematological SRCT at presentation. These cases were evaluated in detail for incidence of marrow involvement. Thirty two (17.7%) cases showed marrow involvement and these cases have been analysed with respect to the primary tumor. The SRCT showing involvement of bone marrow included neuroblastoma (48.8%), retinoblastoma (11.1%), Ewing's sarcoma/PNET (8.6%) and rhabdomyosarcoma (3.2%).These findings are discussed in the light of available world literature.

A Case of Fulminant Hepatic Failure Secondary to Hepatic Metastasis of Small Cell Lung Carcinoma / 대한간학회지

Although liver metastasis is commonly found in cancer patients, fulminant hepatic failure secondary to diffuse cancer infiltration into the liver is rare. Liver metastasis-induced fulminant hepatic failure has been reported in patients with primary cancer of the gastrointestinal tract, breast and uroepithelium, and in patients with melanoma and hematologic malignancy. Small cell lung cancer is so highly invasive that hepatic metastasis is common, but rapid progression to fulminant hepatic failure is extremely rare. We report here on a case of a patient who died because of rapid progression to fulminant hepatic failure as a result of hepatic metastasis of small cell lung carcinoma.

Pancreatic Metastasis and Obstructive Jaundice in Small Cell Lung Carcinoma

Primary lung cancer frequently metastasizes to distant organs. The pancreas is a relatively infrequent site of metastasis. Furthermore, obstructive jaundice resulting from pancreatic metastasis is extremely rare. This paper examines the case of a 65-year-old woman with small cell lung cancer initially presenting with extrahepatic biliary obstruction. The patient underwent percutaneous transhepatic biliary drainage. The obstruction was relieved with a stent placement, then the woman was treated with combination chemotherapy (irinotecan, cisplatin) and a complete remission achieved in six months.

A Case of Primary Small Cell Carcinoma Arising from the Common Bile Duct / 대한소화기학회지

Small cell carcinoma is usually seen in the lung, but rarely involves the gastrointestinal tract including biliary tract. A 65 year-old man was admitted because of obstructive jaundice. A smooth-surfaced round intraluminal mass with proximal bile duct dilatation was seen in the proximal common bile duct on endoscopic retrograde cholangiogram. Under the diagnosis of bile duct cancer, pylorus-preserving pancreatoduodenectomy was done. Pathology revealed a 2 cm sized small cell carcinoma in the proximal common bile duct and distal common hepatic duct. On immunohistochemical stain, the tumor cells were positive for neuroendocrine markers CD56 and synaptophysin. After surgery, the patient received 5 cycles of adjuvant chemotherapy with VIP (etoposide, ifosfamide, and cisplatin) regimen. However, the patient died of liver metastasis 12 months after the diagnosis. We report a case of extrapulmonary small cell carcinoma arising from the common bile duct.

Pancreatitis from Metastatic Small Cell Lung Cancer: Successful Treatment with Endoscopic Intrapancreatic Stenting

Lung cancer metastases can occur in almost any organ. However, metastasis of small cell lung cancer to the pancreas is rare. Moreover, not all cases present with clinically diagnosed pancreatitis. We recently treated a patient with small cell lung carcinoma that invaded the pancreatic duct causing acute pancreatitis. Generally, the treatment for tumor-induced acute pancreatitis is initially supportive followed by aggressive chemotherapy or surgery. If the patient can tolerate the insertion of an endoscopic intrapancreatic stent, this is performed in addition to chemotherapy and surgery; this approach offers a safe and effective treatment modality for such patients.

(18)FDG PET in primary oat cell carcinoma of the esophagus.

The role of FDG-PET in oat cell carcinoma of the esophagus is hitherto unexplored. A MEDLINE search using the terms "small cell carcinoma" or "oat cell carcinoma" combined with "FDG-PET" yielded no report on this issue till date. We report, in this article, two cases depicting the usefulness of this modality in the management of this uncommon neoplasm. While reevaluation of unsuspected metastatic sites missed by other modalities suggest its role in M staging, whole body FDG PET (both baseline as well as post treatment) may find important role in treatment monitoring and evaluation in residual viable disease, taking into account the systemic nature of the disease.

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.

Acute onset paraneoplastic cerebellar degeneration in a patient with small cell lung cancer.

A patient with small cell lung cancer presented with a rare presentation of an acute onset pancerebellar dysfunction. His clinical condition markedly improved following the surgical removal of the tumor and chemo- and radiotherapy.

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.

Clinical analysis of 12 Korean Lambert-Eaton myasthenic syndrome (LEMS) patients

The Lambert-Eaton myasthenic syndrome (LEMS) heralds the occurrence of malignancy, especially small-cell lung cancer (SCLC), but it can also occur in the absence of cancer. Twelve patients were diagnosed as LEMS by clinical features and the classical electrophysiological triad, which includes a low amplitude of compound muscle action potentials (CMAP), decremental responses on low-rate stimulation, and incremental responses on high-rate stimulation on the repetitive nerve stimulation (RNS) test. There were 6 male and 6 female patients, ranging in age from 49 to 66 years. Malignancy(all were SCLC) was found in 7 patients. Males predominantly expressed the paraneoplastic form; whereas the primary autoimmune form was found only in women, who showed a good response to corticosteroid treatment. The neurological features were similar in both groups: proximal lower limb weakness, depressed muscle stretch reflexes, and dryness of mouth in nearly all patients. Bulbar dysfunction and limb paresthesia were a little more frequent in the paraneoplastic form. In RNS tests, the characteristic electrophysiological abnormalities were found in all patients and were more profound in the paraneoplastic form. We concluded that LEMS is commonly associated with malignancy, especially SCLC, but it should also be stressed that there are many female LEMS patients who do not harbor any malignancy at all, and that other treatment strategies such as immunotherapy should be considered for these patients.

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

Carcinoma en el esófago, de células pequeñas / Small-cell carcinoma in the esophagus

The oat-cell carcinoma is more frequently found in the lungs, but has been described in other organs. It is a rare but aggressive tumor and its prognosis is dismal. The longest survival time reported is 22 months. In our patient was 15 months. We treated at the National Cancer Institute of Panama a 52-year-old patient with oat-cell carcinoma of the esophagus and described his symptoms, pathology, treatment and results