Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Vagal paragangliomas are rare tumors of neural crest origin. This neoplasm is generally located at various points along the peripheral distribution of the vagus nerve, in the region of the jugular and nodose ganglia, just beneath the perineurium of the nerve, but may arise anywhere along the course of the vagus nerve. Histologically, vagal paragangliomas are similar to carotid body tumors, and occasionally can undergo malignant transformation and metastasize (10-19%) or infiltrate the base of the skull. Selective arteriography can distinguish vagal paraganglioma from carotid body tumor, because the first lies above carotid bifurcation without widening of the vessels. CT scanning and MR are useful for preoperative evaluation of the jugular foramen for neoplastic involvement. Surgical resection is the treatment of choice, but usually it isn't possible to exercise these lesions without sacrifice of the vagus nerve. A careful follow-up examination for the development of metastases and multicentric paragangliomas is necessary. Two cases of vagal paragangliomas one of them with cervical node metastases, are reported. Diagnostic and surgical features of these rare neoplasms are outlined.
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PMID:[Vagal paraganglioma]. 817 50

Between October 1960 and January 1991, 5 male and 9 female patients (mean age, 44 +/- 19 years) were seen at the Mayo Clinic because of mediastinal paraganglioma. Nine patients presented with hypertension. In these and an additional patient, the tumors were biochemically active. In 13 patients, the mediastinal mass was localized preoperatively. All patients with functioning tumors received alpha-adrenergic and beta-adrenergic blockade preoperatively. Total resection was performed in 11 patients and partial resection in 2. In 1 patient, the tumor was found at postmortem examination. Five lesions were grossly invasive and nine were noninvasive. Blood pressure returned to normal in 6 of the 8 hypertensive patients whose tumors were totally removed. In 4, catecholamine excretion normalized as well. Tumor multicentricity occurred in 6 patients. In 7 patients, the disease took a malignant course (metastases in 5 and progression of unresectable lesions in 2). The probability of 10-year survival after resection was 50% in the group with malignant tumors, and there were no deaths in the group with benign lesions. Analysis of the DNA cellular content showed no correlation between the ploidy pattern and the behavior of the disease.
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PMID:Mediastinal paraganglioma: a surgical experience. 823 7

Paragangliomas have neuroendocrine characteristics. We previously described successful in vivo visualization of various tumors of neuroendocrine origin after injection of the radiolabeled somatostatin analogue octreotide. In this study, we report the results of 111In-octreotide scintigraphy in 34 patients referred because of known paragangliomas or in whom a paraganglioma was suspected and compared the results of octreotide scintigraphy with the outcomes of other imaging techniques used in the diagnosis or follow-up of these patients. Fifty of 53 (94%) known localizations in 25 patients with paragangliomas were visualized. In two patients, three localizations were missed during octreotide scintigraphy. Unexpected additional paraganglioma sites, not detected or not investigated with conventional imaging techniques, were found in 9 of 25 patients (36%) with known paragangliomas. In four of them, the supposed tumor localizations were thereafter also demonstrated with other imaging modalities. In eight of nine patients who were referred because of symptoms consistent with paraganglioma or follow-up after surgical removal of a paraganglioma, neither routine imaging nor octreotide scintigraphy revealed any abnormalities indicative of paraganglioma. We conclude that: (1) virtually all paragangliomas can be visualized using in vivo 111In-octreotide scintigraphy and (2) because conventional imaging is usually limited to the site where a paraganglioma is clinically suspected, octreotide scintigraphy, because of the information it provides on potential tumor sites in the whole body, may be useful in detecting multicentricity or metastases in patients with paraganglioma.
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PMID:Octreotide scintigraphy for the detection of paragangliomas. 838 41

From 1980 until 1991 10 patients were operated upon a paraganglioma: 5 patients with an encapsulated solitary tumor, two with infiltrating tumors and 3 with metastatic disease. The diagnosis of a catecholamine-producing tumor was most reliably made by norepinephrine analysis in urine and serum. The localization of the tumor was diagnosed in 9 of 10 cases by sonography and CT-scan. In 9 patients the tumor was excised completely, 4 times by extended resections. Whereas 4 out of 5 patients with an encapsulated solitary paraganglioma live to date without recurrence, all 3 patients suffering from metastatic disease had recurrent disease within one year postoperatively. A differentiated operative therapy of paragangliomas based on a clinical characterization is proposed: Encapsulated paragangliomas should always be excised. For solitary infiltrating tumors an extended resection may be necessary. This seems not to be justified for multiple infiltrating or metastasizing paragangliomas. Thus, only a reduction in size may be adequate for these tumors.
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PMID:[Diagnosis and surgical treatment of extra-adrenal pheochromocytoma]. 843 47

We report a case of splenic peliosis imitating metastases on CT. The patient was a 44-year-old man who previously had a retroperitoneal paraganglioma surgically removed. Peliosis must be considered a potential differential diagnosis of hypodense foci of the spleen seen on CT.
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PMID:Peliosis of the spleen associated with a paraganglioma. Case report. 845 21

A case of cauda equina paraganglioma is described; subsequent intracranial and intraspinal metastases occurred after partial resection and adjunctive radiotherapy. Cerebrospinal fluid dissemination is a rare complication of spinal paragangliomas. Factors predictive of this unusual biological behaviour are discussed.
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PMID:Cauda equina paraganglioma with subsequent intracranial and intraspinal metastases. 873

A papillary neoplasm that was presumed to originate from the endolymphatic sac was described by Heffner in 1989. This tumor was considered to be a "low-grade adenocarcinoma" because of its behavior: slow growth, local destruction and failure to metastasize. The clinical manifestations are hearing loss, vertigo, facial nerve paralysis and/or cerebellar disorders. Endolymphatic sac tumors have previously been mistaken for such neoplasms as paraganglioma, choroid plexus papilloma and carcinoma, adenomatous tumor of the middle ear and secondary metastases. The diagnosis of this neoplasm is facilitated by CT and MRI. The treatment of choice is total removal of tumor as soon as possible and requires clinical awareness of this rare but important pathologic entity.
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PMID:[Papillary tumor of the endolymphatic sac]. 876 30

Distant metastases due to paraganglioma are a rare occurrence. We report a case of paraganglioma with pulmonary metastases and review the literature on paragangliomas. A 29-year-old female presented with cervical lymphadenopathy, excision biopsy of which confirmed the diagnosis of paraganglioma. She subsequently underwent tumour embolisation and received a course of radiotherapy. Seven years later, she was investigated for multiple pulmonary nodules noted on her chest roentgenogram. Histology of the wedge excision of the lung showed metastatic paraganglioma. Increased catecholamine production was documented; this was however not associated with symptoms of catecholamine excess. Reports concerning the treatment of paragangliomas with pulmonary metastases are few, given their rarity.
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PMID:Paraganglioma with pulmonary metastases: a case report. 889 37

Pheochromocytoma and paraganglioma of childhood are rare neuroendocrine tumors. Urinary catecholamine measurements, metaiobenzylguanidine (MIBG) scanning, computed tomographic scanning, and magnetic resonance imaging have greatly facilitated diagnosis. Prognosis after surgical resection is excellent. In this retrospective series collected from French oncology centers, the risk of tumor progression was studied in order to assess prognostic factors and the optimal diagnostic and therapeutic management. Medical records of 24 children with paraganglioma were reviewed. This tumor occurred at a median age of 12.5 years and in most cases was revealed by arterial hypertension. The diagnosis was made by the demonstration of urinary excretion of catecholamines and their metabolites. Six patients had bilateral adrenal pheochromocytomas; two patients had extra-adrenal paragangliomas. In eight patients, the paraganglioma occurred as a familial disease. Surgical excision was the only therapeutic procedure. With a follow-up of 5.2 years, 14 of the patients are still in first complete remission and 6 have developed metastases or shown tumor progression. Despite a high long-term survival rate, the risk of malignancy and of multifocal involvement is of concern and is associated with a significant rate of late events. The outcome depends on adequacy of tumor resection and must be serially assessed.
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PMID:Pheochromocytoma and paraganglioma in children: a report of 24 cases of the French Society of Pediatric Oncology. 926 73

Of a total of 5,700 surgical procedures on the neck performed at our Institution between 1984 and 1995, 13 operations (0.22%) have been done on 11 patients with 16 cervical paragangliomas. A woman underwent resection of synchronous bilateral carotid body tumours and of an intravagal paraganglioma. Ten years later, after preoperative angiographic embolization, she underwent resection of a paraganglioma of the left hypoglossal nerve. Her sister, at age 21, underwent resection of a carotid body tumour and, respectively 19 and 20 years later, of a right and left intravagal paraganglioma. An interposition graft for replacement of the carotid bifurcation was necessary in one patient only. During resection of a left carotid body tumour, acute hypotension occurred resulting in an ischemic lesion of the right temporal lobe. Postoperatively, she also complained of respiratory distress that responded to medical therapy with difficulty. The related neurologic symptoms completely resolved three months after surgery. The operation for a paraganglioma of the left hypoglossal nerve resulted in a temporary motor deficit of the tongue and in permanent considerable difficulty in swallowing. Unilateral recurrent nerve palsy occurred in two patients. No patients during the postoperative follow-up showed signs of local recurrence or metastatic disease. In conclusion, surgery is an effective treatment for cervical paraganglioma, but because of the high surgical complication rate, an experienced and skilled surgeon is called on to optimize outcome. An adequate perioperative care is advisable.
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PMID:Surgical treatment of paragangliomas of the neck. 941 39


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