Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eight cases were treated by lateral skull base surgery. Three benign tumors (2: paraganglioma, 1: neurinoma) originated from parapharyngeal space and infiltrated into jugular foramen, and five malignant tumors (3: squamous cell carcinoma, 1: adenoid cystic carcinoma, 1: rhabdomyosarcoma) involved the skull base. Preoperative magnetic resonance imaging gives information as to whether tumors invaded to cranial bone or not, and also permits deciding surgical procedures. Surgical management of mandibular bone was performed in five cases for complete tumor resection and sufficient surgical view; hemimandibulectomy was carried out in four malignant cases, who simultaneously underwent radical neck dissection, and one benign case underwent mandiblotomy. Ligation of sigmoid sinus was carried out in four cases (1; benign, 3; malignant) to control bleeding from large vessels. The skull base was reconstructed with myocutaneous flaps which were fixed by fibrin tissue adhesive. No significant surgical complications were not noted. There were no recurrence of benign tumor during 8-13 years follow up period. Malignant tumors, except one (rhabdomyosarcoma), had no local recurrence during follow up period (4 months-2 years). We emphasize that the progress in preoperative assessment, as well as in reconstructive surgical techniques, will assure more adequate indication of lateral skull surgery for malignant tumors.
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PMID:[Clinical analysis of eight cases treated with lateral skull base surgery]. 166 77

We present a case report on a 35-year-old patient in whom a malignant sympathetic paraganglioma of the organ of Zuckerkandl was the cause of severe hypertension with excessive perspiration at night. Since curative surgery was not possible medical treatment was initiated. Interferon alfa 2b (Intron A, Essex Pharma) and the somatostatin-analogue SMS 201-995 (Sandostatin, Sandoz) had no effect on catecholamine production and progression of the tumor. Treatment with alpha-methyl-para-tyrosin (MPT, [Metyrosin], Demser, MSD) turned out to be an effective and well tolerable therapy in this patient with peritoneal carcinosis. Clinical and hormonal progression of the paraganglioma resumed only after two years of therapy, which constitutes the longest documented period of time of successful MPT treatment. The superior efficacy of MPT in our patient should encourage postoperative medical treatment with MPT in malignant pheochromocytoma or malignant paraganglioma, particularly when the tumor turns out to be resistent to alpha blocking drugs.
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PMID:[Therapy of a malignant sympathetic paraganglioma of the organ of Zuckerkandl--a case report]. 166 29

A case study of bilateral familial carotid body paragangliomas with DNA flow cytometric and cytogenetic analyses is presented. Analysis of tumor cell nuclear DNA content by flow cytometry revealed aneuploid cell populations in both tumors. Standard cytogenetic analysis (Giemsa-banding technique used) of the right carotid body paraganglioma showed no evidence of numerical or structural abnormalities. We describe parameters currently used to "predict" biological behavior in these tumors.
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PMID:Bilateral familial carotid body paragangliomas. Report of a case with DNA flow cytometric and cytogenetic analyses. 170 Dec 97

A case of vulvar paraganglioma is reported. Following presentation with vulvar pain, a 1-cm nodule was excised from the labium minus of a 58-year-old woman. Histologically, the tumor was composed of nests of round eosinophilic cells with moderately pleomorphic nuclei, beneath an intact squamous epithelium. Ultrastructural studies indicated two cell types within the neoplasm: chief cells with numerous small neurosecretory granules and peripheral slender sustentacular cells. A reticulin stain confirmed the "zellballen" nature of the neoplasm, and the neoplastic cells showed moderate argyrophilia on a Grimelius stain. The immunoperoxidase stains for chromogranin and neuron-specific enolase were strongly positive in the neoplastic chief cells. Immunostaining using anti-S-100 antibody confirmed the finding of sustentacular cells by identifying many slender cellular processes among the chief cells. These light and electron microscopic findings are diagnostic of paraganglioma, an entity not previously reported in the vulva to our knowledge.
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PMID:Paraganglioma of the vulva. 170 25

The authors describe clinical and pathologic features present in an adolescent girl who had a gastric tumor and mediastinal mass. The latter was shown to be a paraganglioma, and the gastric neoplasm was classified as malignant "leiomyoblastoma," with the use of current histologic criteria. This tumor had metastasized to the liver but not to the lungs. Although the histologic criteria for leiomyoblastoma were fulfilled, no definite evidence of smooth-muscle cell differentiation was present ultrastructurally or by immunostaining methods. Gastric tumors that form part of "Carney's triad" are known to differ clinically and pathologically in important ways from smooth-muscle cell malignant neoplasms that are not part of this syndrome. Some have been classified as gastrointestinal autonomic nerve tumors, but the current study did not confirm this contention. The nature of gastric leiomyoblastomas in Carney's multitumoral association remains undecided.
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PMID:The intriguing nature of gastric tumors in Carney's triad. Ultrastructural and immunohistochemical observations. 172 59

Sixteen cases of paragangliomas of the head and neck including 8 of the vagal body, 3 of the carotid body, 2 jugulotympanic, 2 vagal or jugulotympanic and 1 of the larynx were analysed. Clinically, 13 tumors were benign, 2 showed local aggressivity and 1 showed metastases. All tumors were tested with antisera directed against neuron-specific enolase (NSE), chromogranin A (CGA), S-100 protein, neurofilaments (NF), glial fibrillary acid protein (GFAP) and cytokeratin (CK). Immunohistochemical results were compared with those of 5 cases of neuroendocrine carcinoma (NC) (1 of the oral vestibule, 1 of the larynx, 1 Merkel-cell tumor of the skin and 2 medullary thyroid carcinomas). Immunoreactivity for NSE and/or CGA was always positive in all paragangliomas and NC. S-100 protein was positive in sustenticular cells in all cases of paragangliomas and focally in two cases of NC. NF and GFAP were focally positive in 3 and 2 paragangliomas respectively; and in 1 NC. CK was constantly negative in all cases of paraganglioma and constantly positive in all cases of NC. Antibody anti-CK is the single most useful immunomarker for differential diagnosis between paraganglioma, frequently benign neoplasms and NC commonly aggressive in the head and neck. These findings are consistent with the current concepts of the neuroendocrine system.
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PMID:Paragangliomas of the head and neck. Immunohistochemical analysis of 16 cases in comparison with neuro-endocrine carcinomas. 175 7

The authors report the case of a 40 years old woman with a fronto parietal tumefaction and intracranial hypertension caused by a paraganglioma of the cranial vault. The clinical follow up is marked by rapid local recurrence of the tumor after complete excision. Usually paragangliomas arise in any area of the body where paraganglionar structures are situated (glomus jugulare, carotid glomus, vagus nerve). Other rare locations have been reported and remain difficult to be explained. The location at the cranial vault is exceptional. this tumor would probably arise from the paraganglionar cells which migrate from the neural crest following the trigeminal branches particularly the ophtalmic division.
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PMID:[Paraganglioma of the cranial vault. Apropos of a case]. 175 67

Between january 1965 and november 1990, 32 operations for neck paraganglioma were performed: 29 chemodectomas (carotid body tumors) and 3 paragangliomas of the vagus nerve. Seven subjects were affected with bilateral chemodectomas and one of them showed concurrent unilateral vagal paraganglioma. Two paragangliomas were malignant, with invasion of the latero-cervical lymphnodes revealed at operation. Four individuals came to observation from two different families, suggesting familiarity. Preoperative diagnosis was correctly made in 12 of 18 asymptomatic chemodectomas (66.6%), ten of whom observed during the last decade: angiography is the gold standard for diagnosis but CT scan, ultrasound and NMR imaging are going to earn the confidence of physicians for precise evaluation of latero-cervical masses. Surgery is to date the treatment of choice, and the results are dependent on the size of the tumor and the involvement of the neighbouring vascular, nervous and visceral structures. According to the majority of the literature, the 29 chemodectomas were classified in the three groups of Shamblin: I: 4 cases; II: 10; III: 15. Twenty out of the 24 transient or permanent postoperative complications took place in the third group: in five instances some procedures of internal carotid artery reconstruction were needed. Fourteen complications for chemodectomas and 2 for vagal paragangliomas affected the cranial nerves; three transient and one permanent ischemic central neurological deficits occurred in the group III chemodectomas. Not any operative mortality was registered in this series.
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PMID:[Paraganglioma of the neck. Analysis of 32 operated cases]. 176 58

A rare paraganglioma of the cauda equina is reported. The clinical, radiological and histological features of this neoplasm are described. Diagnosis was resolved by the absence of glial fibrillary acid protein and electron microscopic evidence of neurosecretory features. Relevant literature is reviewed.
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PMID:Paraganglioma of the cauda equina: case report and review of the literature. 163 30

We report a 49-year-old man with a paraganglioma involving the nasopharynx, which according to the literature is the fourth case in Japan and the twentieth in the world. The initial histopathologic findings of the biopsy specimen from the nasopharyngeal tumor suspected a carcinoid tumor. However, the final histopathologic diagnosis from the surgically resected specimen was paraganglioma, determined by electron microscopy and using several stains, such as Grimelius and Neuron-specific enolase (NSE) stains. We wish to add this case of "primary" nasopharyngeal paraganglioma to the literature, and to report the pathologic findings and clinical course of this unusual case.
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PMID:Primary nasopharyngeal paraganglioma: a case report. 179 35


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