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At least 19 recordsLinked to original sources
PubMed1982

Neurohormonal peptides in ovarian carcinoids: an immunohistochemical study of 81 primary carcinoids and of intraovarian metastases from six mid-gut carcinoids.

Eighty-one primary ovarian carcinoids and intraovarian metastases from six mid-gut carcinoids were examined for the presence of tumor cells immunoreactive with antisera raised against various neurohormonal peptides, mostly of gastroenteropancreatic (GEP) origin. Twenty of the primary and two of the metastatic carcinoids contained such tumor cells. The incidence of tumors with any kind of neurohormonal peptide immunoreactive tumor cells was 53% in the trabecular carcinoids, and 42% in the strumal carcinoids, whereas the incidence was much lower (7%) in the insular type. Immunoreactive pancreatic polypeptide (PP), glucagon, enkephalin, and somatostatin were those neurohormonal peptides most commonly observed in the tumor cells of the primary carcinoids. Those less commonly found were substance P, calcitonin, VIP, neurotensin, beta-endorphin, and ACTH. Four metastatic carcinoids were nonreactive with all the antisera used. Cells storing immunoreactive insulin, glucagon, PP, VIP, gastrin, substance P, or enkephalin were found in one of the two remaining metastatic carcinoids; in the other only gastrin-immunoreactive tumor cells were observed. The occurrence and distribution of tumor cells storing the neurohormonal peptides in ovarian carcinoids are discussed in relation to their possible origin in the ovary and to carcinoids in the gut.

Carcinoid Tumor↗
PubMed1985

The carcinoid syndrome: comparison of 21 necropsy subjects with carcinoid heart disease to 15 necropsy subjects without carcinoid heart disease.

Carcinoid heart disease is a morphologically specific type of cardiac disorder that involves the mural and valvular endocardium on the right side of the heart. Twenty-one subjects (57 percent) (Group I) with carcinoid heart disease and 15 subjects (43 percent) (Group II) without carcinoid heart disease were studied at necropsy. The two groups were similar in mean age (54 years versus 55 years), duration of clinical illness (4.7 years versus 6.3 years), body weight (50 kg versus 52 kg), systemic blood pressure (117/77 mm Hg versus 128/77 mm Hg), blood hematocrit levels (37 percent versus 36 percent), total serum protein levels (6.0 g/dl), and serum albumin levels (2.2 g/dl versus 2.6 g/dl). The two groups were different in the frequency of the presence of precordial murmurs consistent with tricuspid regurgitation and/or pulmonic stenosis (95 percent versus 13 percent), cardiomegaly by chest radiography (38 percent versus 0), low voltage on electrocardiography (47 percent versus 0), and location of the primary site of the carcinoid tumor. Total electrocardiographic 12-lead QRS voltage was similar in each group (105 mm versus 132 mm) (10 mm = 1 mV). Of Group I subjects, 43 percent died of cardiac causes; none of the Group II subjects died of cardiac causes. Of the 21 subjects with carcinoid heart disease, seven had left-sided cardiac involvement, but in none was it of functional significance. Thus, although carcinoid heart disease frequently is the cause of death in patients with the carcinoid syndrome, the development of carcinoid heart disease is not related to the duration of symptoms of the carcinoid syndrome.

Adult↗
PubMed2005

Primary hepatic carcinoid tumor with carcinoid syndrome and carcinoid heart disease: a case report of a patient on long-term follow-up.

We report a 57-year-old woman with a primary hepatic carcinoid tumor who had been suffering from symptoms due to carcinoid syndrome and carcinoid heart disease for more than 9 years. Long-term injection of octreotide acetate was fairly effective to relieve the symptoms due to carcinoid syndrome, but its tumor contraction effect was not confirmed. The massive ascites in the follow-up period appeared to be diminished to a certain extent by timely chemotherapy including cisplatin. The patient died of multi-organ failure about 9 years after the onset of disease. Autopsy confirmed a primary hepatic carcinoid tumor with limited distant metastases.

Antineoplastic Agents, Hormonal↗
PubMed2004

The use of Alu-PCR to distinguish between typical pulmonary carcinoids versus classic midgut carcinoids.

Although histologically 'typical' pulmonary and 'classic' midgut carcinoids are similar, the small intestine tumors are more aggressive than their pulmonary counterpart. We believe that in contrast to pulmonary carcinoids arising from Kulchitsky cells, 'classical' midgut carcinoids develop from crypt stem cells that differentiate into endocrine ('classical') or exocrine-endocrine ('non-classical' adenocarcinoid) tumors. The different progenitor cells may determine different malignant potentials between these types of carcinoids. To identify genetic differences between 'classical' midgut and typical pulmonary carcinoids using an Alu-PCR genomic profiling method, we reviewed 54 cases of carcinoid tumors that were surgically removed at Hartford Hospital from 1996 through 2001. Histologically these cases were selected into three groups: i) foregut or pulmonary carcinoids, ii) 'classic' midgut carcinoids of small intestine and iii) multiple typical pulmonary carcinoids. Genomic-profiling of DNA from these cases was performed using an Alu-PCR method. Metastases were observed in 18/20 'classical' intestinal carcinoid tumors, 3/30 pulmonary carcinoids, and 0/4 multiple pulmonary carcinoids. These results confirm that pulmonary carcinoids behave in a more benign fashion than intestinal carcinoid tumors. Using Alu-PCR to profile tumor cell genomic DNA, we showed that 68% of small intestine carcinoids and 58% of pulmonary carcinoids had allelic banding patterns suggestive of either amplification or deletion of gene sequences. Alu-PCR demonstrated loss or gain of genetic sequences that were unique for each examined group. These findings strongly suggest that pulmonary carcinoids differ from their intestinal counterparts.

Alu Elements↗
PubMed1992

Rectal carcinoids: the most frequent carcinoid tumor.

One hundred seventy patients with gastrointestinal carcinoid tumors were treated at Ochsner Clinic from 1958 to 1990. Ninety-four rectal carcinoid tumors were diagnosed and treated during this time. Carcinoid tumors of the rectum represented the most frequent primary site (55 percent), followed by carcinoids of the ileum (12 percent), appendix (12 percent), colon (6 percent), stomach (6 percent), jejunum (2 percent), pancreas (2 percent), and other (5 percent). One-half of rectal carcinoids were discovered during anorectal examination of asymptomatic patients. The remainder were found primarily by examination of patients for symptoms of benign anorectal conditions. The diagnosis of rectal carcinoid was made at the time of initial examination in 61 patients. This allowed definitive treatment in a single session by local excision and fulguration in 48 patients. The remainder were treated by repeat biopsy and fulguration (25 patients) or by transanal excision (12 patients). Overall, 85 carcinoid tumors of the rectum measuring less than 2 cm were treated by local excision and fulguration or by transanal excision, with an average five-year follow-up. There were no local recurrences. Ten patients with metastasizing rectal carcinoids averaging 4 cm were treated. All were symptomatic at presentation and fared poorly despite radical surgery. Three were alive at three years but only one survived five years. At our institution, rectal carcinoids were the most frequently detected carcinoid tumor. Small carcinoids of the rectum were adequately treated by local excision and fulguration or by transanal excision, with no local recurrence. The true incidence of rectal carcinoids is detected only with careful and complete rectal examination of the asymptomatic screening population by experienced surgeons. With more widespread screening of the well population, rectal carcinoids may become recognized as the most frequent human carcinoid tumor.

Aged↗
PubMed2002

Different beta-catenin immunoexpression in carcinoid tumors of the appendix in comparison to other gastrointestinal carcinoid tumors.

Carcinoid tumor of the appendix is an endocrine tumor that is histologically similar to, but biologically less aggressive than carcinoids arising from other parts of the gastrointestinal tract. In this study, we examined E-cadherin, beta-catenin, DCC, p53 and Ki67 immunoexpression in cases of carcinoid of the appendix and made a comparison with non-appendiceal carcinoid tumors. Nine cases of appendiceal carcinoid and 11 biopsies of carcinoid of other parts of the gastrointestinal tract, five cases of the small intestine and six of the stomach were immunohistochemically evaluated for Ki67, p53, DCC, E-cadherin and beta-catenin. Two main patterns of beta-catenin staining were observed. The first pattern was characterized as membranous and cytoplasmic, and was seen mainly in the peripheral cells of the nests. The second pattern was diffuse, predominantly membranous. Most (five of seven) appendiceal carcinoids and only three of 11 non-appendiceal cases showed the first staining pattern (p < 0.05). Immunoexpression of E-cadherin and DCC was similar in both groups. p53 and Ki-67 immunostaining revealed stronger nuclear positivity in the non-appendiceal carcinoid tumors (statistically not significant). We found a pattern of beta-catenin immunostaining in typical carcinoid tumors of the appendix that was different from the pattern seen in non-appendiceal carcinoid tumors. This alteration suggests that carcinoid of the appendix may represent a different subtype of carcinoid tumors with different immunohistochemical and biological behavior.

Adult↗
PubMed2001

Carcinoid syndrome symposium on treatment modalities for gastrointestinal carcinoid tumours: symposium summary.

OBJECTIVE: To develop a collaborative approach for the treatment of gastrointestinal carcinoid tumours and carcinoid syndrome. PARTICIPANTS: Leaders in the medical, endocrine, radiologic and surgical treatment of carcinoid disease were selected to present papers at the Carcinoid Syndrome Symposium on Treatment Modalities for Gastrointestinal Carcinoid Tumours and participate in the workshop that followed. EVIDENCE: A multidisciplinary symposium with experts in the field of carcinoid syndrome was organized at the University of Calgary. Data presented, participation of the attendees and a review of the literature were used in the workshop to develop a collaborative approach to the management of carcinoid tumours. BENEFITS: Carcinoid tumours are rare and few centres have large experiences in their treatment. Before the development of this collaboration, patients with carcinoid tumours received a unidisciplinary approach depending on referral patterns. The development of a multidisciplinary neuroendocrine clinic helped to unify the approach to these patients, yet a consensus on the treatment of carcinoid tumours was lacking. The expertise at the symposium allowed for consensus and the development of treatment algorithms, including biochemical screening, radiographic localization and surgical intervention, for gastrointestinal carcinoid tumours. The role of medical and hormonal therapy after cytoreducion is presented. RECOMMENDATION: Patients with carcinoid tumours require a multidisciplinary approach to their care.

Algorithms↗
PubMed2003

Carcinoid tumor and carcinoid syndrome.

PURPOSE OF REVIEW: Carcinoid tumors secrete many different types of substances (e.g. serotonin, bradykinin) that may produce potentially fatal intraoperative reactions such as hypotension and bronchoconstriction. The most effective treatment for the deleterious cardiovascular and pulmonary effects of serotonin and bradykinin is octreotide, a somatostatin analogue. Carcinoid heart disease, which develops in the majority of patients with carcinoid syndrome, presents the anesthesiologist with more diagnostic and therapeutic dilemmas. RECENT FINDINGS: The incidence of carcinoid tumors is much greater than previously recognized. New diagnostic techniques permit identification and localization of carcinoid tumors with greater accuracy. Short term and long term therapy with octreotide significantly improves survival and reduces the severity of 'carcinoid crises'. Echocardiographic studies have demonstrated a very high incidence of carcinoid heart disease (tricuspid and pulmonary insufficiency) in patients with carcinoid syndrome. Cardiac valve surgery is, consequently, becoming much more common in patients with carcinoid syndrome. SUMMARY: More patients with carcinoid tumors and carcinoid syndrome are requiring anesthesia and surgery. Specific therapy with somatostatin analogues (octreotide) has replaced older less specific drugs (e.g. antihistamines) for the treatment of adverse effects caused by products of carcinoid tumors.

Journal Article↗
PubMed1994

Progressive carcinoid heart disease after resection of primary ovarian carcinoid.

Primary ovarian carcinoid tumors are uncommon, and carcinoid heart disease is a rare complication. Although carcinoid syndrome and carcinoid heart disease typically occur in the setting of metastatic carcinoid tumor, particularly involving the liver, this is not necessarily the case in patients with primary ovarian carcinoid tumors. After surgical resection of an ovarian carcinoid tumor, the prognosis is excellent; however, carcinoid heart disease can continue to progress. The following is a case report of a patient who, despite having complete resection of a primary ovarian carcinoid tumor, went on to develop progressive, debilitating carcinoid heart disease. This is an important scenario to recognize, because proper management and surgical intervention in carcinoid heart disease can be lifesaving.

Aged↗
PubMed2002

The occurrence of malignant carcinoid syndrome in patients with carcinoid tumors.

BACKGROUND: The carcinoid syndrome occurs in about 10% of patients with carcinoid tumors. OBJECTIVES: The main objective of the present study was to determine the presence of the carcinoid syndrome in a group of patients with carcinoid tumor. SUBJECTS AND METHODS: 35 pts with carcinoid tumors (group A--24 pts with metastatic disease, group B--11 pts after resection of the primary tumor with no metastases detected at the time of the investigation) were included into the study. The localisation and the diameter of the primary tumor were ascertained. Somatostatin receptor scintigraphy--Octreoscan and echocardiography were done. The urinary excretion of 5-HIAA was performed. We estimated the presence of the carcinoid syndrome in all patients. RESULTS: We confirmed the difference in the diameter of the primary carcinoid tumor in patients with metastases--group A 3.0 (2.5-4.2) cm by comparison with group B 0.7 (0.6-0.8) cm, (p < 0.001). Patients in the metastatic group are older 64 year (42-70) than pts without metastases--33 y (20-54) (p < 0.01). The amount of the 5-hydroxyindolacetate acid in urine in pts with metastatic carcinoid was statistically significantly higher than in the pts without metastases (p < 0.001). The carcinoid syndrome in the metastatic group was found in 6 patients (25%), with maximum (12.5%) localised in midgut. Out of the whole sample it counts up to 15.1%. During the follow up period, 8 patients from group A died. CONCLUSION: The large diameter of the primary carcinoid tumor and the higher age are the bad prognostic factors in patients with carcinoid tumors. (Tab. 4, Ref. 27.)

Adult↗
PubMed2003

Genetic alterations in goblet cell carcinoids of the vermiform appendix and comparison with gastrointestinal carcinoid tumors.

Goblet cell carcinoid is a relatively rare neuroendocrine tumor of the vermiform appendix with poorly understood molecular pathogenesis. We studied the clinicopathologic features and genetic alterations, including allelic loss of chromosomes 11q, 16q, and 18q; sequencing of the K-ras, beta-catenin, and DPC4 (SMAD4) genes; and p53 overexpression and loss of DPC4 by immunohistochemistry; in 16 goblet cell carcinoids. We compared the allelic loss in goblet cell carcinoids to those in 18 gastrointestinal carcinoid tumors. For goblet cell carcinoids, appendiceal perforation was the most common (70%, 7/10) clinical presentation. The mean tumor size was 2.0 +/- 1.5 cm (range, 0.4 to 4.5 cm). The tumor invaded to appendiceal serosa in 50% (8/16) of patients, and two patients had metastasis in lymph nodes or adjoining viscera. With mean follow-up of 24 +/- 14 months (median, 23 mo), 1 of 10 patients had died of disease, and 2 others had tumor recurrence. All four patients with metastases, recurrences, and/or death from disease had serosal involvement at presentation (P =.02). Loss of heterozygosity of chromosome 11q was present in 25% of goblet cell carcinoids, 14% of ileal carcinoid tumors, and 9% of nonileal carcinoid tumors; of chromosome 16q in 38%, 29%, and 0 (P =.02); and of chromosome 18q in 56%, 86%, and 9% (P =.002), respectively. No mutations of K-ras, beta-catenin, or DPC4 genes; p53 overexpression; or loss of staining for DPC4 was present in any tumors. These findings suggest that allelic loss of chromosomes 11q, 16q, and 18q in goblet cell carcinoids and ileal carcinoids may have an important role in the pathogenesis of these tumors.

Adult↗
PubMed1989

Clinical and echocardiographic comparison of patients with the carcinoid syndrome with and without carcinoid heart disease.

To correlate clinical and laboratory variables in carcinoid heart disease, clinical data, echocardiograms, 24-hour urinary 5-hydroxyindoleacetic acid levels and liver function tests were evaluated in 30 patients with the carcinoid syndrome. The dominant cardiac lesion of carcinoid heart disease by echocardiography and Doppler was severe tricuspid regurgitation with right ventricular volume overload. A characteristic finding was thickened, retracted tricuspid valve leaflets that were fixed in a partially open position. Carcinoid heart disease was progressive and often fatal. The 17 patients with echocardiographic evidence of carcinoid heart disease had higher peak levels of urinary 5-hydroxyindoleacetic acid (331 +/- 231 vs 58 +/- 78 mg, p less than 0.001) and more severe hepatic dysfunction than the 13 patients without carcinoid heart disease. Although duration of symptoms of the carcinoid syndrome before echocardiography was similar for patients with and without carcinoid heart disease (5.4 +/- 6.4 vs 6.2 +/- 5.9 years, respectively, p greater than 0.1), survival after echocardiography was shorter for those with carcinoid heart disease (1.9 +/- 1.4 vs 3.8 +/- 2.9 years, p = 0.05). The findings support the concept that long-term exposure of the endocardium to serotonin in the right side of the heart leads to the development of heart lesions; in addition, progressive hepatic dysfunction may allow more serotonin to bypass liver enzymes and reach the right side of the heart.

Adult↗
PubMed1982

Carcinoid syndrome from gastrointestinal carcinoids without liver metastasis.

Although patients with bronchial and ovarian carcinoid tumors can develop the carcinoid syndrome (diarrhea and/or flushing) in the absence of hepatic metastasis, it is believed that development of the carcinoid syndrome in patients with carcinoid tumors of gastrointestinal origin occurs only after the patient has hepatic metastasis. This is explained by hepatic inactivation of most of the serotonin in the portal circulation or by the fact that hepatic metastases are larger than the primary tumor in the gastrointestinal tract. Three patients with ileal and jejunal carcinoid tumors who developed the carcinoid syndrome without obvious hepatic metastasis are described. Two of the patients had intra-abdominal, but extrahepatic, metastasis that probably drained directly into the systemic circulation. The third patient had an ileal carcinoid with clinical involvement limited to adjacent mesenteric lymph nodes. Following resection of her tumor, her urinary 5-HIAA excretion and platelet serotonin level returned to normal, and her attacks of carcinoid flushing virtually ceased. She has occasional spells of "blushing" that are thought to be benign; however, further close follow-up study will be needed to be certain that she is free of disease. It is suggested that each patient with the carcinoid syndrome be evaluated with CT and technetium-99 pertechnetate liver scans. If there is no liver involvement detected with these studies, one should consider hepatic arteriogram or laparotomy to determine if the patient's tumor might be totally resectable.

Adult↗
PubMed2004

[Occurrence of carcinoid syndrome in patients with carcinoid tumors].

BACKGROUND: Carcinoid syndrome occurs in about 10% of patients with carcinoid tumors. OBJECTIVES: The main objective of the present study was to determine the presence of the carcinoid syndrome in the group of patients with the carcinoid tumor. SUBJECTS AND METHODS: 43 pts. (22 w/21 m) with carcinoid tumors (M group--26 pts. with metastatic disease, B group--17 pts. after resection of the primary tumor without known metastases at the time of the investigation) were included in the study. We estimated the presence of the carcinoid syndrome in all patients. The urinary excretion of 5-HIAA was performed and echocardiography were done. RESULTS: The amount of the 5-hydroxyindolacetate acid in urine in pts. with metastatic carcinoid was statistically significant higher than in the pts. without metastases (p < 0.001). The carcinoid syndrome in metastatic group we found in 10 patients. Flush had 9, diarhoe 7 pts., and cardiac involvement with endocardial fibrosis had 3 pts. During the treatment with lanreotide the amount of 5-HIAA in urine decreased from 1155 (207.6-1406) to 471 (96.6-1000) mumol/24 h (p < 0.05). During the follow up period 3 patients with carcinoid syndrome died.

Adult↗
PubMed1990

Bronchial carcinoids with S-100 positive sustentacular cells. A comparative study with gastrointestinal carcinoids, pheochromocytomas and paragangliomas.

Fourty-six bronchial carcinoids, twelve tumourlets and twenty areas of neuroendocrine cell dysplasia (NED) were immunohistochemically evaluated for various neuroendocrine markers, S-100 protein (S-100), myelin basic protein, intermediate filaments, actin, Leu-7 and several neurohormonal polypeptides. Eighteen of the bronchial carcinoids (39.1%) showed a biphasic cell pattern, with abundant stellate-shaped S-100 positive cells (SC). SC were not reactive for chromogranin A, myelin basic protein, cytokeratins, neurofilaments, glial fibrillary acidic protein or actin, and were only occasionally weakly positive for vimentin. SC were not detected in the tumourlets nor in the NED observed. For comparison a group of other neuroendocrine tumours (11 gastrointestinal carcinoids, 4 pheochromocytomas and 4 paragangliomas) were immunostained for S-100, chromogranin A and actin. SC similar to the ones detected in the bronchial carcinoids could be detected in appendiceal carcinoids, paragangliomas and in two out of four pheochromocytomas. Our present data are in keeping with a Schwannian/sustentacular nature of SC rather than that of a histiocytic or myoepithelial nature. We suggest that SC-rich bronchial carcinoids are biphasic tumours, which could be designed "paraganglioid" bronchial carcinoids. The relationship between SC-rich bronchial carcinoids and tumourlets/NED is a matter of further investigation: SC-rich bronchial carcinoids may either differentiate in a biphasic pattern during tumoural growth or may not be histogenetically related to tumourlets.

Adrenal Gland Neoplasms↗
PubMed1997

Ileal carcinoid tumors stimulating Crohn's disease: incidence among 176 consecutive cases of ileal carcinoid.

OBJECTIVE: There are seven published cases of ileal carcinoid tumor misdiagnosed and treated as Crohn's disease. We evaluated 176 consecutive ileal carcinoid patients to determine the frequency with which this occurs. Clinical, hormonal, and radiographic data of those patients misdiagnosed were also analyzed to determine whether there were clinical lessons to be learned. METHODS: Records of 176 patients with ileal carcinoid tumors were retrospectively reviewed. It was determined whether patients were diagnosed and treated for Crohn's before the diagnosis of carcinoid was established. Radiographic, hormonal, and clinical data were reviewed and comparison was made to those who were correctly diagnosed as having carcinoid. RESULTS: Of 176 ileal carcinoid patients, 4 were initially diagnosed and treated as having Crohn's; their mean age was 51.5 +/- 8.9 yr. The hormonal data of patients treated as Crohn's was indistinguishable from patients with known carcinoid. Two of four patients had colonoscopy performed, neither had terminal ileal intubation. None responded to medical therapy and all required surgery for diagnosis. The mean time from symptom onset to the establishment of the correct diagnosis was 24 +/- 6.9 months with a range of 6 to 45 months. CONCLUSIONS: Although rare, ileal carcinoid tumors may be misdiagnosed as Crohn's disease. In our study approximately 2.3% of patients with ileal carcinoid were initially diagnosed and treated as having Crohn's disease. The presence of disease refractory to medical therapy should alert clinicians to this possibility and urinary 5-HIAA levels should be obtained as a screening test.

Adult↗