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At least 19 recordsLinked to original sources
PubMed1980

Malignant carcinoid syndrome: two case reports.

The malignant carcinoid syndrome (MCS) is a rare complication of malignant carcinoid neoplasms, fewer than 200 cases having been reported since its description by Thorson in 1954. Its signs and symptoms vary with the site of the primary neoplasm, and MCS is now recognized as one member of the large family of endocrine tumors--APUDOMAS). Although MCS remains uniformly fatal, significant palliation can be provided some patients with current surgical and chemotherapeutic management. All signs and symptoms in MCS, except evidence of the destruction of the tricuspid and pulmonic valves, can be reproduced experimentally. The factor causing this lethal complication has not been identified. Two previously unreported cases of MCS, each from the gastrointestinal (GI) tract, are presented. This brings the world's total report cases to approximately 210.

Aged↗
PubMed1989

[Symptomatic treatment of the malignant carcinoid syndrome with octreotide].

Intractable diarrhoea and flushing due to the malignant carcinoid syndrome is seldom relieved by conservative medical treatment. Octreotide (Sandostatin; Sandoz) is a long-acting analogue of somatostatin and a powerful inhibitor of endogenous peptide release. A patient with severe diarrhoea and flushing due to the malignant carcinoid syndrome, in whom symptomatic control with octreotide was achieved, is described, and the value of octreotide treatment in the malignant carcinoid syndrome is discussed.

Female↗
PubMed1991

A study of cyproheptadine in the treatment of metastatic carcinoid tumor and the malignant carcinoid syndrome.

Sixteen patients with metastatic neuroendocrine tumors and the malignant carcinoid syndrome were treated with cyproheptadine (Periactin, Merck, Sharp & Dohme, West Point, PA) at maximum tolerable doses that ranged from 12 to 48 mg daily. Usual side effects were mild sedation and dry mouth, but three patients found it impossible to sustain treatment due to nausea and vomiting. Most patients had significant relief of diarrhea, frequently associated with weight gain. Relief of flushing was uncommon. The therapeutic benefit produced by cyproheptadine would appear to be a peripheral effect because 5-hydroxyindoleacetic acid (5-HIAA) excretion in these patients was not reduced. Although there have been case reports of objective tumor regression with cyproheptadine therapy, this was not observed in any of these 16 patients. Cyproheptadine would appear to be a useful therapeutic tool for the management of diarrhea associated with the malignant carcinoid syndrome. An appropriate initial total daily dose is 0.4 mg/kg divided in three fractions with prompt modification to produce minimal and tolerable side effects.

Adult↗
PubMed2005

Cutaneous manifestations of the malignant carcinoid syndrome.

BACKGROUND: The incidence of carcinoid tumours is approximately 1.5 per 100 000 of the population. The malignant carcinoid syndrome, which is caused by circulating neuroendocrine mediators produced by the tumour, occurs in less than 10% of patients. Cutaneous involvement, although recognized in this rare syndrome, has rarely been reported. OBJECTIVES: To examine a series of patients with the malignant carcinoid syndrome, to establish the prevalence and types of cutaneous involvement and to assess whether these could be used as indicators of disease activity, progression and prognosis. METHODS: Patients with the malignant carcinoid syndrome who attended a multidisciplinary clinic for neuroendocrine tumours over a 23-month period between February 2001 and December 2002 were invited to participate in the study. This involved completion of a standard history proforma and a detailed cutaneous examination with findings recorded by clinical photography. RESULTS: Twenty-five patients were enrolled. All but one had experienced flushing, three had rosacea, five had clinical features of pellagra and two had scleroderma. Flushing was generally an early manifestation of the syndrome, whereas both the pellagra and scleroderma tended to occur in more advanced disease. CONCLUSIONS: This descriptive case series indicates that cutaneous features are not uncommon in the syndrome. They are readily diagnosed on clinical examination, and may be useful indicators of disease activity and prognosis.

Aged↗
PubMed2003

Sandostatin LAR (long-acting octreotide acetate) for malignant carcinoid syndrome: a 3-year experience.

BACKGROUND: Somatostatin analogues are the best therapy for controlling the symptoms of malignant carcinoid syndrome. Octreotide acetate given as subcutaneous injection up to three times daily, intramuscular Lanreotide injection given once per 1-2 weeks and monthly intramuscular Sandostatin LAR have demonstrated similar efficacy in short-term studies. AIM: To assess the long-term effect of Sandostatin LAR on the management of patients with malignant carcinoid syndrome. METHODS: This was a 3-year retrospective study. Twenty-seven patients were assessed with a median follow-up of 23 months. Thirteen patients were switched from subcutaneous octreotide and 14 patients were octreotide naive. All patients showed avid uptake on indium-111 octreotide imaging. RESULTS: Ten of the 13 patients previously on subcutaneous octreotide and 13 of the 14 patients who were octreotide naive had good symptom control on Sandostatin LAR. Over the period of follow-up, many patients showed progression of their tumour and required additional therapies. Patients expressed a preference for monthly intramuscular Sandostatin LAR as opposed to daily subcutaneous injections of octreotide. Although Sandostatin LAR was difficult to administer in certain instances, overall it was well tolerated. CONCLUSIONS: Sandostatin LAR provides good long-term symptomatic control in patients with malignant carcinoid syndrome; it is well tolerated and patients expressed improved satisfaction in their management.

Adult↗
PubMed1980

Effect of somatostatin infusion on jejunal water and electrolyte transport in a patient with secretory diarrhea due to malignant carcinoid syndrome.

The effect of intravenous somatostatin infusion was investigated in a patient with severe secretory diarrhea due to malignant carcinoid syndrome. Continuous somatostatin infusion for 48 hr (4 microgram/kg/hr) reduced stool volume from 2.2 to 0.7 liters/day. Intestinal perfusion studies before intravenous somatostatin infusion revealed decreased water and sodium absorption and jejunal chloride secretion. Somatostatin infusion for 2 hr (8 microgram/kg/hr) reversed chloride secretion to absorption and markedly enhanced water and sodium absorption. These results suggest that somatostatin inhibited a secretory component of water and ion movement in the small bowel of the patient with malignant carcinoid syndrome.

Aged↗
PubMed1993

Computed tomography during arterial portography in malignant carcinoid syndrome: a report of two patients.

Computed tomography during arterial portography (CTAP) and delayed high-dose iodine computed tomography (CT) have improved the preoperative localization of hepatic metastases from colon cancer. Nearly all patients presenting with malignant carcinoid syndrome have liver metastases, and removal of tumour bulk is considered the most effective means of management. To determine suitability for hepatic resection, CTAP and delayed high-dose iodine CT were used to evaluate the distribution of hepatic disease in two patients with malignant carcinoid syndrome. In both patients CTAP showed lesions not seen during recent dynamic incremented CT; the location of the lesions precluded resection. CTAP also demonstrated metastases less than 1 cm in diameter in one patient. Facial flushing (both patients) and hypotension (one) occurred during infusion of the contrast agent into the superior mesenteric artery. Because CTAP can demonstrate small hepatic metastases (less than 1 cm in diameter), it is recommended for patients with malignant carcinoid syndrome who are being considered for hepatic resection. The infusion of contrast media through the superior mesenteric artery may induce a carcinoid crisis, and prophylaxis with a somatostatin analogue is suggested.

Contrast Media↗
PubMed1993

Metastatic carcinoid tumors and the malignant carcinoid syndrome.

Patients with metastatic carcinoid tumors and the malignant carcinoid syndrome have benefited immensely from diagnostic and therapeutic advances during the past decade. Magnetic resonance imaging and whole body scintigraphy with radiolabelled analogues of somatostatin have improved our ability to diagnose, detect, stage and follow response to therapy. Surgical, medical, and radiation therapy may all contribute to the management of these patients. This disease is variable in its presenting symptoms and the biologic behavior of the tumor. The spectrum of clinical manifestations varies depending upon the type and quantity of polypeptide hormones or biogenic amines being produced. Although the tumors are usually indolent in their growth, the more dedifferentiated or anaplastic tumors can be quite aggressive. Thanks to new treatments that are very effective in the subgroup of anaplastic neuroendocrine carcinomas it is vital to recognize this subset. As research scientists and clinicians we must be aware of the natural history of the disease in order to optimize each patient's treatment. This highly selective review focuses on studies performed in collaboration with Dr. Charles Moertel along with other colleagues at the Mayo Clinic, have done in the past few years.

Carcinoid Tumor↗
PubMed1986

Primary duodenal carcinoid with malignant carcinoid syndrome: a case report.

Primary duodenal carcinoid causing a malignant carcinoid syndrome is rare. Herein a case is reported. In view of this case and a review of the literature, the carcinoid syndrome is almost pathognomonic of massive liver metastasis or involvement of organs whose venous drainage is not through the portal system. The right upper quadrant abdominal pains accompanied by symptoms that resemble even part of the carcinoid syndrome should initiate the investigational process including proximal gastrointestinal endoscopy to avoid delay in diagnosis of duodenal carcinoid.

Carcinoid Tumor↗
PubMed1984

Evaluation of combined cyclophosphamide and methotrexate therapy in the treatment of metastatic carcinoid tumor and the malignant carcinoid syndrome.

Sixteen patients with metastatic carcinoid tumor and the malignant carcinoid syndrome were treated with combined cyclophosphamide and methotrexate therapy in a regimen previously described as highly effective. Toxicity was relatively mild and consisted primarily of leukopenia. One patient experienced some symptomatic benefit and minor reduction in hepatomegaly and 5-hydroxyindoleacetic acid excretion. None met our criteria for an objective response. Combined cyclophosphamide and methotrexate therapy does not appear to offer significant benefit for this disease.

Adult↗