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1/134. carcinoid heart disease and carcinoid syndrome: successful surgical treatment.

    Tumor debulking can greatly improve quality of life for patients with malignant carcinoid syndrome, but hepatic cytoreduction is confounded by carcinoid heart disease, which can cause postsinusoidal portal hypertension, thereby increasing the risk of death from hemorrhage during hepatic resection. We describe a patient with metastatic carcinoid syndrome and carcinoid heart disease who had repair of his carcinoid heart disease and, after improvement of right-sided heart function, had successful hepatic debulking of carcinoid metastases. ( info)

2/134. Severe intrapulmonary shunting associated with metastatic carcinoid.

    A 37-year-old woman with a 10-year history of metastatic carcinoid presented to her oncologist with increased dyspnea. Further evaluation revealed hypoxemia and intrapulmonary vasodilatation. We describe a case of hepatopulmonary-like physiology associated with metastatic carcinoid in a patient with intact liver function. To our knowledge, this is the first documented case of intrapulmonary shunting and hepatopulmonary-like physiology associated with metastatic carcinoid. ( info)

3/134. Carcinoid syndrome with unknown primary: a case report.

    Carcinoid syndrome usually is associated with a classic presentation, the diagnosis of which is supported by elevations in neuroendocrine substances such as 5-hydroxyindoleacetic acid and by localization and pathologic identification of a neuroendocrine tumor. The authors report a patient for whom there was a 7-year delay in diagnosis and even then a primary tumor could not be localized. ( info)

4/134. Carcinoid constrictive pericarditis.

    A 78 year old man presented with diarrhoea, anorexia, and progressive lower limb oedema. He was in atrial fibrillation and had a right pleural effusion and ascites. Ultrasound of the abdomen and 24 hour urinary hydroxyindoleacetic acid output indicated metastatic carcinoid syndrome. Cardiac catheterisation revealed pericardial constriction, and pericardial exploration showed a greatly thickened pericardium with no evidence of tumour invasion. The patient died within 24 hours of surgery. Necropsy findings were consistent with a diagnosis of constrictive pericarditis secondary to metastatic carcinoid syndrome. ( info)

5/134. Sequential hypoglycemia, hyperglycemia, and the carcinoid syndrome arising from a plurihormonal neuroendocrine neoplasm.

    OBJECTIVE: To describe the first patient with a neuroendocrine tumor secreting serotonin, insulin, and glucagon and having the corresponding clinical syndromes. methods: We present a detailed case report, including serial laboratory and clinical findings, in a man with type 2 diabetes, symptomatic hypoglycemia, and the carcinoid syndrome and in whom a plurihormonal metastatic neuroendocrine neoplasm was ultimately diagnosed. RESULTS: In a 58-year-old man with type 2 diabetes, which was treated effectively with a sulfonylurea for 7 years, episodes of hypoglycemia developed. After discontinuation of the orally administered agent, the hypoglycemic episodes initially resolved but then recurred and were associated with inappropriately increased plasma insulin concentrations. In addition, the patient had symptoms and biochemical evidence of the carcinoid syndrome. Computed tomography of the abdomen showed multiple hypodense lesions in the liver, and ultrasound-guided fine-needle aspiration of a liver mass was performed. Undifferentiated neuroendocrine tumor with hepatic metastatic involvement was diagnosed. After a hepatic artery embolization procedure, the hypoglycemia was alleviated, but hyperglycemia soon recurred, associated with inappropriately increased serum glucagon concentrations. The patient's course strongly suggested the presence of a plurihormonal tumor secreting serotonin, insulin, and glucagon. CONCLUSION: To our knowledge, this is the first reported case of a patient with a neuroendocrine neoplasm secreting serotonin, glucagon, and insulin, manifested by the carcinoid syndrome, hyperglycemia, and hypoglycemia. ( info)

6/134. Carcinoid syndrome, acromegaly, and hypoglycemia due to an insulin-secreting neuroendocrine tumor of the liver.

    We report a patient with a hepatic neuroendocrine tumor showing an extraordinary change of the tumor's humoral manifestations from a clinically documented extrapituitary acromegaly and a typical carcinoid syndrome toward a hyperinsulinemic hypoglycemia syndrome. At the primary manifestation of the tumor, an increased serum level of insulin-like growth factor i due to overproduction of GHRH and an increased urinary excretion of 5-hydroxyindoleacetic acid were found. The clinical manifestation of the GHRH excess was an arthralgia, which resolved completely after operative tumor debulking and normalization of insulin-like growth factor i and GHRH serum levels. The secretion of serotonin from the tumor resulted in a typical carcinoid syndrome including right-sided valvular heart disease. On the later course of the disease, the humoral manifestations of the tumor were supplemented by the secretion of insulin, leading to recurrent severe hyperinsulinemic hypoglycemia. The hepatic origin of hyperinsulinism was demonstrated by selective arterial calcium stimulation. Moreover, tumor cells revealed insulin and c-peptide immunoreactivity in the immunohistochemical analysis. The patient died 8 yr after the initial diagnosis of the tumor, and a carefully performed autopsy procedure confirmed the absence of any extrahepatic tumor manifestation. ( info)

7/134. The carcinoid syndrome: an unusual cause of valvular heart disease.

    A 30-year-old woman presented to the Emergency Department with complaints of shortness of breath, orthopnea, and a severe reduction in exercise tolerance. The symptoms were the result of severe valvular heart disease that resulted from a bronchopulmonary carcinoid tumor. The carcinoid syndrome is a distinctive clinical syndrome seen in patients with carcinoid tumors. Cardiac valvular lesions are seen in the majority of patients with the carcinoid syndrome and represent the most clinically significant consequence of the carcinoid syndrome. This case report discusses carcinoid tumors, the carcinoid syndrome induced by these tumors, and the therapeutic options in the management of carcinoid tumors. ( info)

8/134. Perianaesthetic risks and outcomes of abdominal surgery for metastatic carcinoid tumours.

    patients with metastatic carcinoid tumours often undergo surgical procedures to reduce the tumour burden and associated debilitating symptoms. These procedures and anaesthesia can precipitate a life-threatening carcinoid crisis. To assess perioperative outcomes, we studied retrospectively the medical records of adult patients from 1983 to 1996 who underwent abdominal surgery for metastatic carcinoid tumours. Preoperative risk factors, intraoperative complications and complications occurring in the 30 days after surgery were recorded. Perioperative complications or death occurred in 15 of 119 patients (12.6%, exact confidence interval 7.2-19.9). None of the 45 patients who received octreotide intraoperatively experienced intraoperative complications compared with eight of the 73 patients (11.0%) who did not receive octreotide (P=0.023). The presence of carcinoid heart disease and high urinary output of 5-hydroxyindoleacetic acid preoperatively were statistically significant risk factors for perioperative complications. ( info)

9/134. Primary carcinoid of the testis associated with carcinoid syndrome.

    Testicular carcinoid is a rare disease accounting for less than 1% of all testicular neoplasms. It rarely manifests symptoms of carcinoid syndrome. Recent reports have noted that only 1.1-3.1% of testicular carcinoid tumors are complicated by carcinoid syndrome. In general, large tumor size and the presence of carcinoid syndrome are features associated with a malignant course. In the present case, pathological findings revealed pure carcinoid of the testis without metastasis. Moreover, watery diarrhea due to carcinoid syndrome disappeared and the serum serotonin level normalized following orchiectomy. The patient was followed up for 12 months with whole body computed tomography scan and assessment of serotonin levels. To date, there is no evidence of tumor recurrence. These findings suggest that monitoring serum serotonin levels may be useful as a marker during follow up of this type of tumor. ( info)

10/134. Malignant carcinoid in two brothers.

    Familial occurrence of malignant carcinoid is rare (about 3%). Authors describe occurrence of the malignant carcinoid in two brothers. In the older one the diagnosis was estimated in 1991. He had multiple intestinal carcinoid tumor with multiple liver metastases histological type III by Soga classification. Patient is intermittently treated with somatostatin analogue--lanreotid and with interferon alfa. By this therapy the disease is stabile. In the younger of brothers the diagnosis was estimated in 1999. The disease had rapid progression and in ten months patient died despite of the therapy. Definitive diagnosis was a malignant neuroendocrine tumor of pancreas-mixed low differentiated carcinoid with calcitonin overproduction. (Fig. 4, Ref. 15.) ( info)
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