Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Carcinoids”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources

Carcinoids and the carcinoid syndrome.

The many manifestations of carcinoids will continue to puzzle and intrigue surgeons. Because these are dynamic tumors which produce multiple hormones with many potential side effects, an aggressive surgical debulking of them is advocated. Even in the presence of metastatic disease, some long-term survival can be anticipated. The most debillitating aspect of this tumor to the patient is the carcinoid syndrome. The symptoms occur erratically and are often not well controlled by presently available medication. Although metastatic disease present at diagnosis is most commonly seen with jejunoileal carcinoids, colorectal carcinoids appear to be the most lethal tumors. However, they are rarely associated with the symptoms of carcinoid syndrome. Carcinoids may often be associated with other types of tumors, some of which are known to produce their own hormones. Survival of the patients seems to be related to their other tumor type rather than the primary carcinoid. The clinician should be aware of the strong association of carcinoids with peptic ulcer disease. The ulcer diathesis is probably related to ectopic histamine production and can usually be controlled by cimetidine and antacids, although surgical intervention may be required.

Adult↗

TTF-1 expression is specific for lung primary in typical and atypical carcinoids: TTF-1-positive carcinoids are predominantly in peripheral location.

Thyroid transcription factor (TTF)-1 expression in neuroendocrine tumors (NETs) has not been studied as widely as that in non-NETs, with the exception of small cell carcinomas, in which TTF-1 is highly sensitive but not specific for a primary lung tumor. The reported incidence of TTF-1 expression in pulmonary carcinoids has also been highly variable in the literature. To evaluate the expression of TTF-1 in NETs and potential value of TTF-1 in distinguishing pulmonary NETs from those of extrapulmonary origin, we performed an immunohistochemical study by using semiquantitative analysis on formalin-fixed, paraffin-embedded sections from 111 NETs, including 80 pulmonary (11 carcinoid tumorlets [TLs] or foci of neuroendocrine cell hyperplasia [NEH], 36 typical carcinoids [TCs], 17 atypical carcinoids [ACs], 16 large cell neuroendocrine carcinomas [LCNECs]), 13 thymic (3 TCs, 8 ACs, 2 LCNECs), 17 gastrointestinal or pancreatic (13 TCs, 4 ACs), and 1 ovarian (LCNEC). Pulmonary carcinoids were subdivided into those with central and those with peripheral location. TTF-1 positivity was seen exclusively in pulmonary NETs and was significantly higher in NEH or TLs (72.7%) than in TCs (27.8%), ACs (29.4%), and LCNECs (37.5%; P = 0.03). All extrapulmonary NETs were uniformly negative for TTF-1 staining. Interestingly, 12 of 14 TTF-1-positive pulmonary TCs and ACs had a peripheral location with spindle cell morphology, as did all cases of TL, a purported precursor of peripheral carcinoids. In conclusion, TTF-1 expression was 100% specific, though not so sensitive, for the lung primary in TCs and ACs and possibly also in LCNECs. Prevalent TTF-1 positivity in TLs and peripheral carcinoids suggest that they may be histogenetically distinct from the central carcinoids, which are typically composed of TTF-1-negative, more rounded cells.

Aged↗

Carcinoids of unknown origin: comparative analysis with foregut, midgut, and hindgut carcinoids.

BACKGROUND: Carcinoids are rare neuroendocrine tumors typically arising in the gastrointestinal tract. A significant percentage of these tumors present as metastatic disease of unknown primary site. The aim of this study was to better define the functional and clinical characteristics of carcinoids of unknown primary (CUP) site. METHODS: This study examines the hormonal activity, clinical characteristics, and survival of 434 patients with carcinoids originating in the foregut, midgut, hindgut, or unknown location. The 143 patients with CUP were compared with the other groups with regard to presenting characteristics, diagnostic tests and therapeutic modalities used, hormonal activity, and survival. RESULTS: The hormone levels (urinary 5-hydroxyindoleacetic acid and serotonin, serum and platelet serotonin) of CUP were not significantly different from midgut carcinoids with metastatic disease. Although survival with CUP was shorter than with carcinoids with identified primaries (10-year survivals of 22% vs 62%, 50%, and 48% for foregut, midgut, and hindgut, respectively), the survival curve for CUP was quite similar to that of patients with midgut carcinoids with distant disease (10-year survival of 22% vs 28%). CONCLUSIONS: CUP are similar to midgut carcinoids presenting with metastatic disease with regard to hormone production and survival. Like other carcinoids, CUP can be an indolent disease process with gradual progression over decades.

Adolescent↗

Morphologic study of carcinoid-like tumors and their relation to true carcinoids, using tumors of the breast as a model.

Two cases of an unusual type of infiltrating ductal carcinoma of the breast are presented. Both cases demonstrated a carcinoid-like pattern and were indistinguishable from carcinoid tumors of the breast by light microscopy. However, Grimelius stains and electron microscopic evaluation showed no evidence of membrane bound secretory granules. In regard to the prognostic significance and proper classification of carcinoids of the breast, awareness of carcinoid-like morphologic variants of infiltrating ductal or lobular carcinoma is important. It is also apparent that there is a spectrum of tumors which demonstrate some properties of true carcinoids, however, only the true carcinoids show a better prognosis and it serves no useful purpose to separate the rest of these tumors. The diagnosis of carcinoid tumors requires demonstration of secretory granules on electron microscopic examination or in special stains; conventional light microscopic studies alone are insufficient for this diagnosis. All these principles may be applied to carcinoid like tumors of other sites.

Aged↗

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.

Adult↗

Primary carcinoid tumor of the bilateral testis associated with carcinoid syndrome.

Carcinoid tumors derived from neuroendocrine cells can release serotonin and other vasoactive substances into the systemic circulation, resulting in carcinoid syndrome. Testicular carcinoid, a rare disease accounting for less than 1% of all testicular neoplasms, rarely manifests symptoms of carcinoid syndrome. We describe a case of carcinoid syndrome arising from a primary testicular carcinoid tumor. A 21-year-old male patient presented with facial flushing and diarrhea for 5 years. He had an enlarged left testis and a 1-cm, ill-defined, hard, non-tender mass in his right testis. His 24 h urinary excretion of 5-hydroxyindoleacetic acid was elevated (16.1 mg/day). Somatostatin receptor scintigraphy correlated with carcinoid tumor in both testes. Following bilateral orchiectomy, the patient's facial flushing and diarrhea disappeared.

Adult↗

Statistical evaluation of 2001 carcinoid cases with metastases, collected from literature: a comparative study between ordinary carcinoids and atypical varieties.

This study aimed at obtaining a better understanding of the malignant characteristics of carcinoids (gut endocrinomas) of the digestive system. After excluding cases with no possible individual identification as for age and sex, 2001 carcinoid cases with metastases among 5647 of the digestive system computerized in the Niigata Registry were thus subject to the present analysis. These cases were divided into two groups: [A] 1719 with ordinary carcinoid histology and [B] 282 with atypical varieties including argyrophil (endocrine) cell carcinomas, small/oat cell carcinomas of endocrine type, composite carcinoid-adenocarcinoma varieties and other neuroendocrine tumors. In many aspects, a comparative analysis demonstrated statistical differences between [A] and [B]: average age (55.7 vs 60.1), the rates of metastases (34.4% vs 55.6%), the association of carcinoid syndrome (21.4% vs 2.8%), tumor size 20mm or less (42.2% vs 22.5%), depth of invasion down to the submucosa (21.6% vs 11.7%) and transmural invasion (34.3% vs 47.7%), immunohistochemistry of neuron-specific enolase (86.5% vs 66.7%), chromogranin (86.1% vs 67.6%), vasointestinal polypeptides, ACTH and alpha-fetoprotein, and the 5-year survival rate (61.4% vs 17.9%). No statistical differences between these two groups were found in the male to female ratio, serotonin activities and Grimelius argyrophilia. Although no generally acceptable definite criteria and definition for atypical carcinoid varieties have yet established, neoplasias of this category seem to have a wide range of histological, histochemical, immunohistochemical, ultrastructural and biochemical features that may mostly be placed in between ordinary carcinoids and ordinary carcinomas.

Adenocarcinoma↗

Strumal carcinoid of the ovary: an analysis of 50 cases of a distinctive tumor composed of thyroid tissue and carcinoid.

Strumal carcinoid of the ovary is a type of germ-cell tumor characterized by an intimate mixture of thyroid tissue and carcinoid. Fifty patients with this type of tumor ranged in age from 21 to 77 years. The tumors measured up to 26 cm in diameter and were always unilateral. In 10% of the cases, the contralateral ovary contained another type of neoplasm, usually a dermoid cyst. Three-fifths of the strumal carcinoids arose in dermoid cysts or in mature solid teratomas. Birefringent calcium oxalate monohydrate crystals were commonly identified in the thyroid colloid, and argentaffin granules, in the carcinoid cells, thus substantiating the identity of the neoplastic components. Although 31% of the tumors were accompanied by focal stromal luteinization, only 8% of the cases exhibited clinical signs of steroid hormone production (endometrial hyperplasia, hirsutism, or virilism). In 8% of the cases, there was evidence suggesting functioning of the thyroid component, but no patient had the carcinoid syndrome. Only one woman died of tumour; five others died of unrelated causes. The remaining patients remained alive and well; 23, for at least five years, and 15, for at least ten years postoperatively. Although the carcinoid component of the strumal carcinoid has been considered a malignant transformation of struma ovarii, it is almost always benign, and treatment with a simple oophorectomy or salpingo-oophorectomy is effective.

Adult↗

Cushing's syndrome caused by ectopic corticotropin secretion by multiple peripheral pulmonary carcinoids and tumorlets of carcinoid type.

Multiple peripheral pulmonary carcinoid tumors or their smaller counterparts (tumorlets of carcinoid type) are the most unusual form of carcinoids as a cause of ectopic corticotropin syndrome. Only three case reports were found in the literature. We describe a 35 year-old female patient with ectopic corticotropin secretion due to multiple peripheral pulmonary carcinoid tumors and tumorlets. A high-dose dexamethasone suppression test result led to the diagnosis of Cushing's disease in our case. But no tumor was identified on sella imaging and bilateral inferior petrosal sinus sampling was non-diagnostic. Computed tomography of the lungs revealed multiple acinar-nodular parenchymal infiltrations confined to the left lung. Corticotropin-dependent hypercortisolism persisted after bilateral adrenalectomy. A second operation was necessary to remove the hyperplastic adrenal remnants. Meanwhile, computed tomography findings of the thorax were unchanged. We decided to explore these nodules by open lung biopsy. During the procedure multiple nodules ranging 12 to 3 mm in diameter scattered throughout the left lung were observed and left pneumonectomy was performed. Histopathological diagnosis was multiple peripheral carcinoid tumors and tumorlets of carcinoid type showing positive immunostaining with corticotropin. This observation emphasizes a rare form of carcinoids as a cause of ectopic corticotropin secretion and its unusual response to high dose dexamethasone suppression test.

ACTH Syndrome, Ectopic↗

Morphological-histochemical study of intestinal carcinoids and K-ras mutation analysis in appendiceal carcinoids.

Intestinal carcinoids are potentially malignant neoplasms. Their histogenesis and pathogenesis are currently uncertain. The morphological and histochemical characteristics of twenty intestinal carcinoids are studied. The primary sites of three mucin-producing tumors were examined by electron microscope. Furthermore 11 appendiceal carcinoids were analysed by the polymerase chain reaction (PCR) for the detection of ras and p53 point mutations. Microscopically all carcinoids were of mixed type. Focal mucin production was evident in three carcinoids that metastasised to regional lymph nodes. HID-Alcian blue staining proved that mucin in both primary and secondary foci did not belong to the sulphated group. The secretory granules and mucin droplets found in a single neoplastic cell suggest that carcinoids of the small intestine and some of the appendix arise from the endoderm. Neither ras nor p53 mutations were detected. It seems that ras oncogenes are probably not involved in the pathogenesis of appendiceal carcinoids.

Adolescent↗

Evidence of hybrid cell of thyroid follicular cell and carcinoid cell in strumal carcinoid.

A strumal carcinoid associated with mature cystic teratoma of the ovary in a 59-year-old was investigated immunohistochemically and electron microscopically. Histologically it was composed largely of trabecular and partly of insular carcinoid and individual thyroid follicles. Intensive argyrophilia was shown in both the cells of carcinoid tumor and follicular structure. Thyroglobulin was strongly positive in the follicular lining epithelium and weakly positive in the carcinoid cells adjacent to the follicular area. Immunoreactive cells for somatostatin and prostatic acid phosphatase were strongly detected in the carcinoid area and gradually blended to the follicular epithelium. Methionine-enkephalin, glicentin, and pancreatic polypeptide were focally detected in the carcinoid area. Whereas calcitonin-positive cells were sparsely observed in the follicular area, carcinoembryonic antigen and serotonin were absolutely negative. Electron microscopic findings revealed abundant neurosecretory granules, microfilaments, and colloid-like droplets in the same cells. We suggest that these hybrid cells are the origin of strumal carcinoid.

Carcinoid Tumor↗

Metastatic carcinoid to the conducting system-is it a rare or merely unrecognized manifestation of carcinoid cardiopathy?

We report a unique case of carcinoid cardiopathy. The patient, a 59-year-old white woman with a known carcinoid tumor of the terminal ileum, presented with complete heart block and subsequently died. Because of her clinical presentation, we examined the conducting system at autopsy. The atrioventricular node was heavily infiltrated by metastatic carcinoid. The tumor was also seen in the surrounding atrial myocardium. No other metastatic lesions were seen in the heart. Carcinoid cardiopathy in the form of right-sided endocardial and valvular thickening has been well documented in the literature, and we found these features in our case as well. To our knowledge, carcinoid has never been documented in the cardiac conducting system until now. We found 2 cases of possible involvement of the conducting system reported in the literature. Unfortunately, the conducting system was not examined postmortem in either of these cases. If conducting system involvement is not unique to the present case, patients with metastatic carcinoid and cardiac problems may require different management. We hope that this case will stimulate further postmortem study of the conducting system in patients with metastatic carcinoid tumor.

Carcinoid Heart Disease↗

Evaluation of 342 cases of mediastinal/thymic carcinoids collected from literature: a comparative study between typical carcinoids and atypical varieties.

This study is aimed to provide extensive and useful information based on an analysis of a large and reliable series of cases regarding mediastinal/thymic carcinoids, both typical and atypical. It also serves to assist investigators to improve their research activities in this particular field. In the present study, 342 patients (3.8%) with mediastinal/thymic carcinoids from amongst a total series of 8,970 patients with carcinoids, registered in the Niigata Registry for gut-pancreatic endocrinomas, were analyzed to clarify their detailed clinicopathological characteristics. Added to an analysis of mediastinal/thymic series including a comparison between typical and atypical carcinoid varieties, another comparative study was performed between the present series and a bronchopulmonary series. Mediastinal/thymic carcinoids may be briefly characterized by 1) no significant difference in major factors statistically demonstrable between typical carcinoids and atypical varieties, 2) a male preponderance, 3) difficult (delayed) preoperative diagnosis, 4) a high rate of metastasis, 5) a large tumor size on average (delayed detection), 6) a low rate of the carcinoid syndrome, 7) a high rate of association of the Cushing syndrome, and 8) low postoperative survival rates.

Adolescent↗

[Satellite cells in pulmonary carcinoid tumorlets and carcinoids].

Pulmonary tissue of twenty-four patients with carcinoid tumorlets and twenty-three typical carcinoid tumors was studied immunohistochemically for stellate-shaped S-100 protein positive (sustentacular) cells. Sustentacular cells (SCs) have been calculated per 10,000 tumorlet or carcinoid elements. The presence of SCs was proved in 18 subjects (75%) of all examined tumorlet cases with quantitative frequency between 13 and 196 SCs per 10,000 tumorlet cells. These elements were also found in 18 carcinoid tumors (79%) of all 23 investigated cases with quantitative frequency between 5 and 927 SCs per 10,000 carcinoid cells. The cluster analysis showed two separate clusters in both groups of lesions with strikingly high frequency of SCs, i.e., from 66 up to 196 SCs/10,000 tumorlet elements and from 138 up to 923 CSs/10.000 carcinoid cells, respectively. SCs may not be used as an indicator of biological behaviour of pulmonary neuroendocrine tumors for their a broad frequency spectrum in examined benign lesions, i.e. tumorlets and typical carcinoid tumors.

Adolescent↗

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.

Adult↗

Carcinoid crisis after biopsy of a bronchial carcinoid.

Pulmonary carcinoids are rarely associated with carcinoid syndromes and even less commonly with carcinoid crisis. Somatostatin analogues can control carcinoid syndrome or crisis with tumors of gastrointestinal origin. We report the successful use of a somatostatin analogue in preventing carcinoid crisis at the time of resection of an "active" bronchial carcinoid tumor.

Biopsy↗

Angioedema as a single manifestation of carcinoid syndrome in a bronchial carcinoid tumor.

BACKGROUND: The association of bronchial carcinoid tumours with carcinoid syndrome is extremely rare especially in the absence of metastasic disease, and the angioedema is not a typical sign of this syndrome. METHODS AND RESULTS: We report the case of a 39 year-old woman referred to our allergy department with recurrent episodes of angioedema. The aetiological study of angioedema did not show evidence of hypersensitivity to common inhalants, food allergens and latex. C1-inhibitor, C3, C4, C1q, proteinogram and immunoglobulins (IgA, IgG, IgM) all were normal. TSH determination gave normal results, too. Faecal analyses for parasites were negative. The haemogram showed moderate leucocytosis and hypocromic mycrocitic anaemia. The thoracic radiography showed a mediastinal node image in the right paratracheal region. Histology analyses of the samples were diagnostic of a typical carcinoid tumor. Levels of 5-hydroxyindolacetic acid (5-HIIA) were slightly increased. A superior lobectomy was performed and no new episodes of angioedema appeared after surgical intervention. CONCLUSIONS: We report the first case of typical bronchial carcionid tumour, without metastasic disease, with angioedema as a single manifestation of carcinoid syndrome. In our knowledge, only one case of Quincke's edema as part of typical carcinoid syndrome has been reported, in a case of primary midgut carcinoid tumor with metastasic disease to liver. It is very important to include complementary tests, as thoracic radiography, in the routine study of angioedema to reject malignant diseases.

Adult↗