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PubMed1980

Midgut carcinoid tumor with carcinoid heart disease. Its presence in the absence of hepatic metastases--a case report.

A 56-year-old man with a midgut carcinoid tumor had extensive metastatic involvement of lymphatic spaces and carcinoid heart disease. Unlike similar previously reported cases with carcinoid heart disease, hepatic metastases and history of the carcinoid syndrome were absent in this patient. Tumor involvement of cardiac lymphatic spaces may have been responsible for the development of carcinoid heart disease due to a local fibrogenic effect similar to the extensive fibrosis associated with tumor seen within the abdominal cavity at autopsy.

Heart Neoplasms↗
PubMed1997

[Gastrointestinal carcinoids and carcinoid syndrome: classification, diagnosis and therapy].

Historically, carcinoids have long been known as a morphologically distinct class of rare intestinal tumors which behave less aggressively than the more common intestinal adenocarcinomas. In recent decades refined methods in pathology improved our knowledge on the diffuse neuroendocrine system, leading to some confusion in classification and nomenclature of carcinoids. The World Health Organization (WHO) classification of endocrine tumors has cleared the definition of carcinoids. The Authors report on 7 cases of midgut carcinoid tumors and 1 case of carcinoid syndrome focusing on the newer approaches to the diagnosis, localization, and role of surgery and peptide therapy.

Adult↗
PubMed1986

Metastatic carcinoid tumors and the carcinoid syndrome. A selective review of chemotherapy and hormonal therapy.

Malignant carcinoid tumors are remarkably varied in their biologic behavior. The disease may be indolent for years with minimal or no symptoms. On the other hand, an acute carcinoid crisis with severe diarrhea, dehydration, and hypotension may develop in the patient. Patients with flushing and/or diarrhea, not responsive to standard symptomatic measures, may benefit from chemotherapy or hormonal therapy. Chemotherapy with single agents or combination chemotherapy may be associated with response rates ranging from 20 to 40 percent. Hepatic de-arterialization by ligation or occlusion is an effective means of inducing rapid tumor shrinkage for patients who have carcinoid tumors and hepatic dominant metastases. The addition of chemotherapy after induction of a partial remission with hepatic de-arterialization may prolong the duration of response, but this remains to be proven in prospective clinical trials. Hormonal therapy with the antiestrogen tamoxifen has been unsuccessful, but treatment of the carcinoid syndrome with a long-acting analogue of somatostatin has been strikingly effective.

Antineoplastic Agents↗
PubMed2006

Carcinoid crisis and reversible right ventricular dysfunction after embolization in untreated carcinoid syndrome.

A 59-year-old white male with carcinoid tumor and hepatic metastases underwent hepatic artery embolization. The patient developed carcinoid crisis and a subsequent transthoracic echocardiogram showed classic findings of carcinoid heart disease along with a dilated hypertrophied right ventricle and severely depressed right ventricular ejection fraction. After treatment with octreotide the patient's clinical condition improved and a repeat transthoracic echocardiogram showed a significant improvement and normalization of right ventricular systolic function. Serotonin levels showed a progressive decline that correlated well with the patient's improved clinical condition. These findings suggest that the acute right ventricular dysfunction was secondary to acute carcinoid crisis and resolution resulted in a significant improvement of both right ventricular systolic function and clinical condition.

Acute Disease↗
PubMed2000

Ileal carcinoid tumor complicating carcinoid heart disease and secondary retroperitoneal fibrosis.

A 70-year-old man with metastatic liver tumors showed carcinoid syndrome with clinical symptoms of facial flushing, palpitation, dyspnea, and an itching sensation. Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine excretion of serotonin metabolites were confirmed. Autopsy confirmed a serotonin-immunoreactive 1 cm ileal carcinoid tumor with metastasis to the liver, bone and peritoneum. The tumor cells were argyrophilic but not argentaffin, and showed erythrophagocytosis in the primary lesion. All the four heart valves and bilateral atrial endocardium showed fibromyxoid thickening, indicating the association of carcinoid heart disease. Desmoplastic reaction with deposition of sulfated acid mucopolysaccharides was also observed in the retroperitoneal space (secondary retroperitoneal fibrosis). Stenosis of the bilateral ureters and inferior mesenteric artery provoked hydronephrosis and lethal ischemic necrosis of the left-sided colon, respectively. Rarity of functioning ileal carcinoid tumor and pathogenesis of systemic fibroplasia are discussed.

Aged↗
PubMed2000

Carcinoid heart disease in association with a primary ovarian carcinoid tumor: diagnostic role of echocardiography.

We describe a patient with carcinoid heart disease secondary to a primary ovarian carcinoid tumor who initially presented with unexplained right-sided heart failure. The patient did not exhibit any of the typical clinical manifestations of the carcinoid syndrome. Echocardiography demonstrated typical features of carcinoid heart disease and played an important role in the detection and management of a condition that was previously unrecognized.

Aged↗
PubMed1983

Treatment of the carcinoid tumor and the malignant carcinoid syndrome.

Certainly in treatment of the carcinoid tumor surgery has a well-established curative and palliative potential. The primary challenge is a knowledgable marriage between stage of disease and aggressiveness of operative procedure. Nonsurgical treatment of the malignant disease per se has thus far not produced optimum results and, in the opinion of this author, should still be confined to a clinical research setting. It would seem very doubtful, however, that the more mundane types of trials, empirically testing drug after drug and arbitrarily concocted drug combinations, is the most productive road to follow. There is an evident need for more sophisticated approaches to this tumor, which has unique metabolic characteristics that should be pharmacologically exploitable. Past experience has indicated that such a setting may permit dramatic therapeutic accomplishment. There is a strong need for animal models or established cell lines which would facilitate preclinical therapeutic exploration. The carcinoid syndrome presents an unparalleled opportunity for fundamental physiologic observations and for experimental therapeutic study that can have applicability not only in palliating the syndrome itself but also in the management of other pathophysiologic states that may involve more subtle abnormalities of the same hormonal mechanisms. The patient with the carcinoid tumor should not just be a fascinating curio for grand rounds exhibition. He should be a focal point for research involving an experienced, multidisciplinary clinical team supported by devoted basic scientists. If our patient resources and efforts can be concentrated in this manner, the carcinoid should be a strong candidate for the next medically curable human cancer.

Adult↗
PubMed1989

Therapy of metastatic carcinoid tumor and the malignant carcinoid syndrome with recombinant leukocyte A interferon.

Twenty-seven patients with metastatic carcinoid tumor, 24 of whom had the malignant carcinoid syndrome, were treated with recombinant leukocyte A interferon at a planned dose of 24 x 10(6) U/m2. Twenty percent of patients with measurable tumor experienced an objective regression and 39% of those with the carcinoid syndrome experienced a reduction of more than 50% in urine 5-hydroxyindoleacetic acid (5HIAA) excretion. Flushing was partially or completely relieved in 65% of patients and diarrhea was relieved in 33%. Regrettably, these favorable treatment effects were transient in nature, with objective regressions persisting for a median of only 7 weeks and hormonal responses for a median of only 4 weeks. Any therapeutic gain experienced by these patients seemed to be outweighed by the frequency and severity of toxic reactions, which consisted primarily of chills and fever, fatigue, anorexia, weight loss, leukopenia, and abnormalities of liver function. Whereas other interferons, administration by alternative dosages and regimens, or incorporation of interferons into drug combinations may merit future study, we cannot recommend recombinant leukocyte A interferon, administered by the methods we employed, for routine therapy of the carcinoid tumor or syndrome.

Adolescent↗
PubMed1995

[A functioning carcinoid tumor with liver involvement without the associated carcinoid syndrome].

The carcinoid tumor, the most frequent variety of the neuroendocrine or amine precursor uptake and decarboxylation (APUD) tumors, typically produces biogenic amines and polypeptide hormones and occasionally gives rise to a striking clinical presentation called carcinoid syndrome. The severity of this syndrome is directly related to the tumor bulk draining into the systemic circulation. This implies almost always hepatic metastases. We present a case in which a carcinoid tumor with extensive hepatic involvement and remarkable biologic activity did not produce any clinical features of the carcinoid syndrome. We believe that such a case enlightens the clinical and biochemical complexity of this entity.

Aged↗
PubMed1985

Argyrophilic prostatic carcinoma. Case report with literature review on prostatic carcinoid and "carcinoid-like" prostatic carcinoma.

An unusual prostatic neoplasm characterized by a carcinoid-like light microscopic pattern together with argyrophilia of tumor cells is described. Immunoperoxidase stain for prostatic specific antigen, however, was positive, indicating that this neoplasm was an argyrophilic prostatic carcinoma. Although the clinical significance of a carcinoid-like pattern and/or argyrophilia in prostatic carcinoma is currently unknown, pathologic recognition of these features is of paramount importance because prostatic carcinoma with these features has to be distinguished from true primary prostatic carcinoid tumor or, in areas of metastasis, from metastatic carcinoid originating from other sites such as the gastrointestinal or respiratory tracts. The value of the immunoperoxidase technique for prostatic specific antigen as well as prostatic acid phosphatase in this differentiation is stressed.

Adenocarcinoma↗
PubMed1989

Composite carcinoid-adenocarcinoma of the stomach associated with multiple gastric carcinoids and nonantral gastric atrophy.

A case of multiple gastric carcinoids and nonantral atrophic gastritis in which the larger tumor was a composite carcinoid-adenocarcinoma is presented. The two components of the composite tumor immunohistochemically showed clear-cut diverging functional differentiations although the available evidence supported a common histogenesis from the metaplastic intestinal epithelium of the gastric mucosa. The carcinoid tissue of the composite tumor, which showed "atypical" features, also differed from the other, pure carcinoids, in which the histologic appearance was "typical." Total gastrectomy performed 1 month after the original gastric resection with antrectomy disclosed regressive changes in the endocrine cell proliferations of the gastric stump consistent with the withdrawal of a stimulating effect of the antral gastrin.

Adenocarcinoma↗
PubMed2006

Familial rectal carcinoid: report of two first-degree relatives with rectal carcinoid and review of the literature.

Two brothers with familial carcinoid tumors of the rectum are presented. A few cases documenting the occurrence of carcinoid tumors in first-degree relatives in the absence of the multiple endocrine neoplasia (MEN) syndromes have been reported in the literature. Consistent with these previous reports, in this case both patients had gastrointestinal carcinoid tumors that are located in identical anatomic locations. The current literature on carcinoid tumors outside the setting of any known genetic syndrome is reviewed. Clinical relevance and screening recommendations are discussed.

Adult↗
PubMed2004

Carcinoid heart disease associated with primary ovarian carcinoid tumor.

Because the venous drainage of the ovary bypasses the portal circulation, carcinoid heart disease in patients with primary ovarian carcinoid tumors may develop in the absence of liver metastasis. We describe 4 patients who presented with symptomatic carcinoid heart disease in association with primary ovarian carcinoid tumor.

Adult↗
PubMed1997

Bronchopulmonary carcinoid tumors associated with Cushing's syndrome: a more aggressive variant of typical carcinoid.

OBJECTIVES: Our objectives were to delineate the clinicopathologic characteristics of adrenocorticotropin-secreting bronchopulmonary carcinoid tumors causing Cushing's syndrome and to derive from these findings a rational approach to diagnosis and surgical management of this unusual condition. METHODS: We conducted a retrospective, chart-review analysis of seven consecutive patients treated at the Massachusetts General Hospital over a 16-year period. RESULTS: The patients uniformly had symptoms of marked hypercortisolism, and the underlying lung lesions remained clinically occult for a mean of 24 months. Standard endocrine testing was misleading in 83% of patients, reinforcing the need for an alternative diagnostic strategy based on petrosal sinus catheterization and computed tomography of the chest. Although 72% of the tumors were typical carcinoids by standard criteria, 57% demonstrated microscopic evidence of local invasiveness, and 43% were associated with mediastinal lymph node metastases. Eighty-six percent of patients have been cured by pulmonary resection a mean of 59 months after the operation, but 50% of these required a second operation for resection of involved lymph nodes after an initial relapse. CONCLUSIONS: These data suggest that adrenocorticotropin-secreting bronchopulmonary carcinoid tumors represent a distinct, more aggressive subtype of the usual, typical carcinoid. The high rate of lymphatic and local spread demands a surgical approach consisting of anatomic resection and routine mediastinal lymph node dissection.

ACTH Syndrome, Ectopic↗
PubMed2004

[Solitary hepatic carcinoid tumor in a man with prior treatment for rectal adenocarcinoma. Rapid growth without carcinoid syndrome].

We describe a case of hepatic carcinoid tumor that was diagnosed after right hepatectomy. The tumor was initially believed to be metastases from a rectal adenocarcinoma for which surgery had been performed seven years earlier. We highlight the localization of the tumor in the liver only, its large size and rapid growth, as well as the absence of carcinoid syndrome. Diagnostic studies of function and localization are presented. The absence of neoplasia in other locations suggested that this neoplasm was probably a primary hepatic carcinoid tumor. The association of carcinoid tumors with other neoplasms is well-known. However, because of our patient's history and the absence of symptoms, the preoperative diagnosis was incorrect. The definitive diagnosis was confirmed by pathological analysis, allowing specific studies of function and localization to be performed and therapeutic and follow-up measures to be adopted. Although surgery plays a crucial role in the treatment of these tumors, it should be performed in the context of the multidisciplinary management recommended for this disease.

Adenocarcinoma↗
PubMed1989

[Preoperative diagnosis of carcinoid of the terminal ileum in the absence of carcinoid syndrome].

Carcinoid of the small intestine, usually found in the terminal ileum, presents a diagnostic challenge when the classic clinical and laboratory findings are absent. The commonest symptom, marked abdominal pain due to intussusception, may arouse suspicion of carcinoid. The precise preoperative diagnosis in the absence of the classic syndrome is impossible and the only way to diagnose it is by colonoscopic biopsy of the terminal ileum. The case described illustrates such a preoperative diagnosis in a 59-year-old woman with severe abdominal pain, nausea, vomiting and weight loss. X-ray studies aroused suspicion of tumor intussusception as the cause of the intestinal obstruction. Colonoscopic biopsy revealed the presence of a carcinoid tumor. However, there had been no symptoms of the carcinoid syndrome, nor was there increased urinary 5-hydroxy indoleacetic acid. On operation the tumor was found to be disseminated and unresectable, so surgical intervention was limited to palliative ileo-transversostomy.

Biopsy↗
PubMed2001

Ovarian strumal carcinoid tumor responsible for carcinoid heart disease.

We report a case of right ovarian strumal carcinoid tumor responsible for tricuspid regurgitation. Valve replacement and salpingo-oophorectomy were performed. Serotonin level and tomodensitometry were normal at 3-year follow-up. Rarity of strumal carcinoid tumor explains why this tumor has never been reported with carcinoid heart disease before.

Aged↗
PubMed1996

An 18-year follow-up of primary hepatic carcinoid with carcinoid syndrome.

Primary hepatic carcinoid is extremely rare. Although one of the 18 previously reported cases was accompanied by clinical features of carcinoid syndrome, no patient manifested these features as the presenting complaint, as was true in our case. During the 18 years this patient has been followed, she has been treated with most of the major therapeutic methods, including systemic chemotherapy, hepatic artery chemoembolus injection, extended right hepatic lobectomy, and, eventually, more systemic chemotherapy and octreotide. She continues to be nearly asymptomatic and is still working. We present the results of extensive chemical and hormonal assays, briefly summarize the primary hepatic carcinoids reported previously, and review therapy of this disease.

Adult↗