Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0007095 (carcinoid)
 6,990 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors reported a case of a carcinoid tumor of the ileum with nodal metastasis. Diagnosis was made by histological examination of the tumor after its removal. Immunohistochemistry demonstrated the presence of serotonin, synaptophysin, chromogranin and neuron-specific enolase (NSE) in the tumor cells. The patient was followed up for 15 months. Tumor ablation resulted in the total cessation of bronchospastic episodes in the patient, suggesting that these symptoms were mediated by tumor secretion. We also present a review of the literature concerning clinical, paraclinical and treatment aspects of gastrointestinal carcinoid tumors.
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PMID:[Digestive carcinoid tumors: often undiagnosed endocrine tumors (a case report)]. 1062 73

Retrorectal cysts are uncommon lesions of uncertain histogenesis, and primary carcinoid tumors arising in retrorectal cysts are extremely rare. We present the case of a 52-yr-old man who had a 22-cm partially cystic, partially solid mass in the presacral space. A computed tomography-guided fine-needle aspiration of the mass was performed. The smears contained abundant keratinous debris and rare groups of tumor cells. The tumor cells were cuboidal, with slightly granular cytoplasm and centrally located nuclei with speckled chromatin and inconspicuous nucleoli. Immunocytochemical analysis revealed strong reactivity for chromogranin and keratin, and focal reactivity for synaptophysin and neuron-specific enolase. The cytological diagnosis of a carcinoid arising in a tail-gut cyst was confirmed histologically. This is the first reported case of a carcinoid arising in a retrorectal cyst diagnosed preoperatively by cytology. This diagnosis is possible in the setting of consistent clinical, radiographic, and cytological findings.
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PMID:Aspiration cytology and core biopsy of a carcinoid tumor arising in a retrorectal cyst: a case report. 1082 May 32

Twenty-two cases of oncocytic thymic neuroendocrine carcinomas (carcinoid tumors) are presented. The patients were 17 men and 5 women between the ages of 26 and 84 years (median, 55 years). Nine were asymptomatic, and the tumor was found on routine examination; four patients presented with chest pain, two with weight loss, two with multiple endocrine neoplasia I syndrome, and one with Cushing's syndrome. Surgical resection of the mediastinal tumor was performed in all cases. The lesions were described as soft, light tan to brown, measuring from 3 to 20 cm in greatest diameter. On cut section, the tumors showed a homogeneous surface, soft consistency, and focal areas of hemorrhage. Microscopically, the lesions were characterized by nests or trabeculae of tumor cells that contained abundant granular to densely eosinophilic cytoplasm, with round to oval nuclei and in some areas prominent nucleoli. Mitotic figures ranged from 2 to 10 per 10 high-power fields; foci of comedonecrosis were seen in all cases. Immunohistochemical studies including broad spectrum keratin, CAM 5.2, chromogranin, synaptophysin, Leu-7, and p53 were performed in 12 cases. All of the tumors were strongly positive for CAM 5.2 low-molecular-weight cytokeratin, 11 showed strong positive reaction for Leu-7, 10 for broad-spectrum keratin, 8 for chromogranin, 7 for synaptophysin, and only 1 case showed focal positive staining of the tumor cells for p53. Clinical follow-up of 14 patients showed that 10 were alive between 2 and 11 years, and 4 patients had died of tumor from 4 to 11 years after diagnosis. Patients with good clinical outcome were those whose tumors showed low mitotic activity and minimal nuclear pleomorphism, whereas those who had died of their tumors were those whose tumors were characterized by marked nuclear atypia and higher mitotic rates. Oncocytic thymic carcinoids should be added to the differential diagnosis of anterior mediastinal neoplasms characterized by a monotonous population of tumor cells with prominent oncocytic features.
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PMID:Primary neuroendocrine carcinoma (thymic carcinoid) of the thymus with prominent oncocytic features: a clinicopathologic study of 22 cases. 1082 19

We studied 80 cases of primary thymic neuroendocrine carcinomas. Most patients had symptoms; approximately one third were asymptomatic. All cases were treated by surgical excision. The tumors were divided according to histopathologic features into low- (n = 29), intermediate- (n = 36), and high-grade (n = 15) types. The tumors displayed a variegated histologic appearance and unusual cytologic features. Some cases showed transition from low to high grade within the same tumor mass. Mitotic activity ranged from fewer than 3 to more than 10 mitotic figures per 10 high-power fields, and most tumors displayed marked cellular atypia and areas of necrosis. In 73 patients, the tumor was confined to the anterior mediastinum. Positive immunohistochemical reaction was observed using antibodies for CAM 5.2 low-molecular-weight cytokeratins, broad-spectrum keratin, chromogranin, synaptophysin, and Leu-7. The clinical follow-up obtained in 50 patients correlated well with tumor differentiation. Therefore, the behavior of these tumors seems to correlate with histologic grade, which seems directly proportional to degree of differentiation. We propose replacing the term thymic carcinoid with thymic neuroendocrine carcinoma, which better reflects the aggressive biologic behavior of these tumors in the mediastinal location.
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PMID:Neuroendocrine carcinomas (carcinoid tumor) of the thymus. A clinicopathologic analysis of 80 cases. 1088 5

Neuroendocrine and neuroectodermal tumors are interrelated, and comprise a neoplastic family including lesions formerly termed "carcinoid," "atypical carcinoid," "small cell undifferentiated carcinoma," "primitive neuroepithelioma," "chemodectoma," and "neuroblastoma," to name but a few entities. The nosology of these neoplasms has been simplified recently, in part as a result of a better understanding of their immunophenotypes and molecular biological attributes. This review considers those immunohistochemical markers that are now generally available for diagnostic evaluation of neuroendocrine and neuroectodermal differentiation, and provides information on the relative sensitivity and specificity of each of them. Intermediate filament proteins, chromogranins, synaptophysin, CD56, CD57, CD99, neuron-specific (gamma-dimer) enolase, protein gene product 9.5, and specific neuropeptide products are discussed. The application of such determinants in regional differential diagnosis is also summarized.
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PMID:Immunohistology of neuroendocrine and neuroectodermal tumors. 1096 5

We describe two cases of atypical carcinoid of the thymus. One was an 82-year-old woman with complaints of nonproductive cough and back pain, and the other a 64-year-old woman with no symptoms. Computed tomography scans of the chest in both cases revealed a large mass in the anterior mediastinum. Multiple metastases to bone and liver were also noted in the former case. Histological examination of their tumors revealed that the tumor cells were arranged in a nested, trabecular, or pseudorosette pattern, with increased numbers of mitoses, nuclear pleomorphism, and presence of necrosis. In addition, immunohistochemically, the cells stained for neuron-specific enolase, synaptophysin and chromogranin A. Combination chemotherapy consisting of carboplatin and etoposide was performed as initial chemotherapy in the former case and as adjuvant therapy in the latter. The former patient achieved a short-term partial response. It is important to differentiate atypical carcinoid from other thymic tumors, since such tumors including thymoma have a much better prognosis than does atypical carcinoid.
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PMID:Two cases of atypical carcinoid of the thymus. 1103 Feb 10

The authors report seven patients with carcinoid tumors of the extrahepatic bile ducts (EHBDs). All patients were women, with an average age at diagnosis of 49.8 years (range, 37-67 yrs). The most common presenting symptom was painless jaundice with or without pruritus. Although one patient had peptic ulcer disease before the onset of obstructive jaundice, none had systemic endocrine manifestations. These neoplasms were most often located in the common bile duct. Grossly, the carcinoid tumors were usually nodular and poorly demarcated, and ranged from 1.1 to 2.7 cm in size. Only one of the neoplasms was polypoid. Microscopically, the tumors had a trabecular or nesting pattern with occasional tubule formation, and were composed of relatively small cells with granular chromatin. All of the neoplasms expressed chromogranin and two expressed synaptophysin. Three expressed serotonin and two of the three were also immunoreactive for pancreatic polypeptide or somatostatin. Two tumors were focally positive for gastrin and one of these two tumors was also positive for serotonin and pancreatic polypeptide. All seven carcinoid tumors showed no immunoreactivity for p53, and assays for p53 loss of heterozygosity analysis were negative in two, suggesting that p53 mutations do not play a role in the pathogenesis of EHBD carcinoids. A mutation in codon 12 of K-ras was found in one carcinoid tumor whereas two of two showed immunoreactivity for Dpc4 protein. In view of the small number of carcinoids studied, the importance of these findings in the pathogenesis of these tumors is unclear. Ultrastructural examination of three of the tumors revealed numerous membrane-bound, round neurosecretory granules. Clinically, these lesions had an indolent course. Even in the presence of lymph node metastases (noted in two patients), all of the patients remained disease free 2 to 11 years (average follow up, 6.6 yrs) after segmental resection or pancreaticoduodenectomy (Whipple's procedure). Because carcinoid tumors of the EHBD are of low malignant potential, they should be separated from the more common adenocarcinomas in this location.
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PMID:Carcinoid tumors of the extrahepatic bile ducts: a study of seven cases. 1107 51

The category of large-cell neuroendocrine carcinoma (LCNEC) of the lung, proposed to expand the traditional scheme of typical carcinoid, atypical carcinoid (AC), and small-cell carcinoma (SCC), based on histologic features, has not been defined in cytology. We attempt to describe LCNEC cytologically. Cytologic features in 16 histologically confirmed LCNECs in fine-needle aspiration biopsies, cell blocks, bronchial brushes, washes, and sputum specimens stained with Diff-Quik, Papanicolaou, hematoxylin-eosin, chromogranin, and synaptophysin were analyzed. Three poorly differentiated nonsmall-cell carcinomas, 4 SCCs, and 2 atypical carcinoids were studied similarly. Twenty specimens from 16 histologically confirmed cases of LCNEC with original cytologic diagnoses including high-grade neuroendocrine carcinoma, large-cell carcinoma, nonsmall-cell carcinoma, poorly differentiated carcinoma, adenocarcinoma, and SCC, were examined. Features included flattened three-dimensional clusters with peripheral palisading, moderate to large single cells with scant (alcohol-fixed) or moderate (air-dried) cytoplasm; and large, oval, or polygonal nuclei with irregular contours, thickened nuclear membranes, and finely or coarsely granular chromatin, showing some molding and crush artifact. Nucleoli were generally present, and occasionally prominent. Mitosis and necrosis were apparent. Neuroendocrine stains were applied to all specimens, with at least one marker, commonly synaptophysin, positive in 18/20 specimens. LCNEC can be diagnosed in cytologic material, using morphology confirmed by immunocytochemistry. Treatment can be offered on the basis of cytologic examination.
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PMID:Large-cell neuroendocrine carcinoma of the lung: proposed criteria for cytologic diagnosis. 1113 71

The authors report a case of gangliocytic paraganglioma of the lung, which has not yet been described in a pulmonary neoplasm. A 75-year-old man underwent right middle and lower lobe lobectomy. A slightly yellowish mass was located at the bifurcation between the lower and middle lobe bronchus, protruding into the truncus intermedius. The neoplastic cells were composed of three cellular elements: uniform endocrine cells in a Zellballen arrangement, large ganglion-like cells within the nests of endocrine cells, and spindle-shaped cells arranged in streams to surround the nests. Each component exhibited the characteristic immunohistochemical properties, which were similar to those of the corresponding neuroendocrine neoplasms: Endocrine cells were positive for CAM 5.2, chromogranin A, and synaptophysin, like carcinoid tumor; ganglion-like cells were positive only for neurofilament, like ganglioneuroma; and spindle-shaped cells were positive for neurofilament and S-100 protein, like paraganglioma. These results agreed with those in gangliocytic paraganglioma of the duodenum. Pulmonary gangliocytic paraganglioma is similar to that in the duodenum, and is a hamartomatous proliferation of epithelial endocrine and neuronal cells of the bronchus.
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PMID:Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms. 1134 85

We report two cases of Gangliocytic Paraganglioma (GP) of the ampulla of Vater occurring in a 63-year-old and a 34-year-old individual. The patients were both admitted for a long history of intermittent gastrointestinal bleeding and abdominal discomfort, with no other symptoms. At endoscopy, the GP appeared as a polypoid, ulcerated mass in the ampullar region, measuring 2.5x1.8 and 2 cm, respectively. Microscopically, the tumors showed similar features and were composed of epithelial cells (more than 50%), spindle cells, and ganglion-like cells. The epithelial cells showed clear cytoplasm and formed nests (zellballen or paraganglioma-like groups), and less frequently, cords (carcinoid-like), extending to mucosa and submucosa. Ganglion cells were sparse, constantly associated with the spindle cells. Both epithelial and ganglion cells were synaptophysin, chromogranin A, and anti-neurofilament immunoreactive. The spindle cells were all S-100 positive. Ultrastructural studies revealed dark and light cells, rare elongated cellular processes, secretory granules, and fine fibrils resembling neurofilaments. The histogenesis of GP is still a matter of debate, however its neoplastic nature is supported by the occasionally reported malignant evolution.
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PMID:Duodenal periampullary gangliocytic paraganglioma: report of two cases with immunohistochemical and ultrastructural study. 1140 27


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