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Novel crystalloid structures in suprasellar paraganglioma.

A-52-year-old woman was admitted to a hospital because of 2-year history of abnormal behavior and impaired visual acuity. Magnetic resonance imaging delineated a sizable mass at the suprasellar region. The partially removed tumor was arranged in irregular lobules composed of an admixture of clusters of cobblestone-like small cells and process-bearing cells with ovoid nuclei, surrounded by a fine, neuropil-like matrix. The Zellballen structure was inconspicuous, and mitosis was absent. Immunohistochemically, the tumor cells were positive for chromogranin A, synaptophysin, class III beta-tubulin and neurofilament, while negative for glial fibrillary acidic protein, cytokeratin and all 6 pituitary hormones. S100 protein expression was limited to cells adjacent to stroma. The MIB-1 labeling index was 0.5%. Histopathological diagnosis was paraganglioma of abortive architecture. Ultrastructurally, numerous dense-cored vesicles were found within the processes and cytoplasm. Synapse formation was not demonstrated. Interestingly, crystalloids up to 3 microm in size were frequently found. They had hexagonal or quadrilateral architecture without limiting membranes. The interval between periodically arranged fibrils was variable, ranging from approximately 20 - 50 nm. Retrospective examination by light microscopy failed to reveal corresponding structures. Crystalloids are rare manifestation of paragangliomas, yet undescribed in those of intracranial origin. Furthermore, the ultrastructure of the present case differs from those of previous cases.

Biomarkers, Tumor↗

[Aorticopulmonary paraganglioma. A case report].

Paraganglioma is a tumor which develops from the paraganglion system, generally in the adrenal medulla (90%). Thoracic localisations are exceptional and arise essentially for the aortic and subaortic bodies, leading, in this case, to an anterior and posterior localisation. We report a case of non-chromaffin non-secreting aorticopulmonary paraganglioma, discovered fortuitously in a 64-year-old man.

Humans↗

Preaortic paraganglioma mimicking a hypervascular tumor of the pancreas.

Paragangliomas are rare tumors that arise from neuroepithelial cells. They are most frequently located in the para-aortic region and they may be confused with other retroperitoneal tumors, especially pancreatic tumors. We present a case of a secreting preaortic paraganglioma in a young patient which was mimicking a hypervascular tumor of the pancreas, and that was completely resected 5 years after the failure of a first attempt to remove the tumor.

Adult↗

[Combined surgical treatment for carotid paraganglioma].

The paper gives a clinical example of embolization of the vascular network of carotid paraganglioma with subsequent en-block tumor removal with bifurcation of the common carotid artery and with recovery of great blood flow along the internal carotid artery through alloprosthesis of the latter. The positive effects of a combination of endovascular embolization of the vascular network of the tumor with its subsequent removal are noted. Indications for endovascular embolization and its procedure, intraoperative evaluation of cerebral collateral circulation are discussed. Those for carotid repair during en-block removal of carotid paraganglioma with bifurcation of the common carotid artery are considered.

Adult↗

[Galanin immunoreactivity in a laryngeal paraganglioma: case report and literature review].

The authors describe a case of laryngeal paraganglioma (LP) occurring in a 57-year-old-woman. To date, 70 cases have been described in the literature. It is benign and recurrences are infrequent. The differential diagnosis with typical and atypical carcinoids, hemangiopericytomas, alveolar soft-part sarcomas, medullary thyroid carcinomas, malignant melanomas and metastatic renal cell carcinomas is supported by immunohistochemistry. Moreover, this tumor shows the immunohistochemical expression of galanin, a variably expressed marker of paragangliomas that it is not expressed in carcinoid tumors. Nevertheless, our observations militate against its role as a solitary marker but advocate its use in conjunction with other antibodies for the differential diagnosis of neuroendocrine neoplasms of larynx.

Female↗

[Posterior mediastinal paraganglioma with a 60-year history; report of a case].

We report a case of a 68-year-old man with a posterior mediastinal tumor. He remembered having difficulty in food passage through his esophagus in childhood. The preoperative thoracic computed tomography (CT) revealed a 5 x 3 cm mass with strong enhancement in the posterior mediastinum. A right thoracotomy was performed to resect this indeterminate mass. Complete resection was achieved. The pathologic diagnosis was paraganglioma. Postoperatively, he experienced more comfortable food passage through his esophagus than ever before. There has been no sign of recurrence 3 years after the operation. The tumor was clinically diagnosed as benign paraganglioma because of its long silent course.

Aged↗

[Head and neck paraganglioma--neoplasm of nervous origin].

The authors of the present paper demonstrate the head and neck paraganglioma, discuss its location, clinical picture, diagnostics and treatment. Paragangliomas are relatively rarely occurring tumors, stemming from the autonomic nervous system. Initially asymptomatic, they do not present any characteristic clinical picture during the further development. Not large size of the tumor contributes to the late diagnosis which is most regrettable due to its potential malignancy (10%). Diagnostic and therapeutic problems cause it's medication reasonable only in specialist centers. Perioperative mortality is estimated at 5 to 13%. Postoperative neurological lesions in a form of the cranial carebro-vascular failure occur in 8 to 20% of cases. A contemporary advanced angiosurgical and anesthesiologic technology allow reducing a postoperative mortality to 5% and neurological complications by 10%. During the previous years many centers carried a preoperative embolization of a tumor during angiographies. An alternative to the large tumor vessels obliteration may be a radium therapy, although several reports confirm its low radiosensitivity.

Head and Neck Neoplasms↗

[Retroperitoneal paraganglioma: case report].

Paraganglioma are rare neoplasms arising from undifferentiated cells of the primitive neural crest. We report a case of a 53 years old patient presented with a large tissular retropéritoneal tumour situated above the left kidney. Serum Adrenaline, Nor drenaline and VMA levels were normal. Surgical complete resection of the tumour was performed without intra-operative incident. The histopathological examination and the immunohistochemical analyses concluded to a non secreting retroperitoneal paraganglioma. Postoperative course was uneventful.

Follow-Up Studies↗

[A difficult intubation in a case of endolaryngeal paraganglioma].

In a female patient aged 71, a tumor of unknown histology almost completely occupied the whole of the supraglottic space. Because of the valve-like behaviour of the tumor, the patient could not be ventilated by mask. Therefore, preoxygenation and preservation of spontaneous breathing were essential. Anaesthesia was induced by titration of etomidate, and no muscle relaxant or opioid was administered. The patient was successfully intubated by means of direct laryngoscopy. In all probability the blind nasal technique or the use of a fiberoptic device would not have been helpful, because the tumor had to be luxated before the endotracheal tube could be placed. Figure 1 shows the tumor (a) with a diameter of approximately 3 cm, which has displaced the epiglottis (b) to the left. A part of the endotracheal tube is visible at the bottom left. The spiral tube had to be replaced by a tube suitable for laser surgery. After resection (see Fig. 2) the coagulated tumor base (a) and the right vocal cord (b) can be seen with the laser tube still in place. Postoperatively extubation was possible. The histological examination revealed a paraganglioma without signs of malignancy. Apart from occlusion of the upper airway, the main danger stems from the considerable vascularization of such a paraganglioma. Not only the surgical procedure, but also minor manipulations during the endotracheal intubation may cause significant blood loss. In a small percentage of cases hormone-mediated cardiocirculatory complications may occur. If a difficult intubation is expected because of an endolaryngeal tumor, management should be based on the diagnostic findings and the planned surgical procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Paraganglioma of the lumbar spinal canal -- case report.

We present the case of a 46-year-old female with a lumbar spine paraganglioma. The patient complained of a right-sided lumboischialgia. Preoperative MRI revealed an intradural tumour at the vertebra L3 level, located in the midline, 7 mm in diameter. The tumour was totally removed by laminectomy. Histopathological examination showed nests of chief cells (zellballen), surrounded by reticulin fibres. Immunohistochemistry showed a positive reaction for chromogranin A, neuronal specific protein, synaptophysin and cytokeratin in the chief cells. The sustentacular cells displayed immunopositivity for S-100 protein, single cells were also positive for GFAP. We found no proliferative activity in the tumour cells (Ki-67 index = 0%). In the two years follow-up the patient remains without clinical or radiological signs of recurrence. Spinal paraganglioma is a rare, surgically curable tumour with low proliferative potential. This entity should be taken into consideration in the differential diagnosis of extramedullary spinal lesions.

Diagnosis, Differential↗

[Clinical study of paragangliomas in the thorax].

Four cases of paraganglioma in the thorax, consisted of 3 cases originating from the mediastinum and a case of pulmonary metastasis from the posterior peritoneum, were clinically investigated. Three were malignant and one was benign with noradrenaline secretion. On surgical excision, preoperative arterial embolization was assumed to be useful for reducing hemorrhage during procedure. Pre and intra-operative circulatory management was also mandatory in case of functioning paraganglioma. Of the 3 malignant cases, 2 survived for more than 10 years with radiotherapy alone or surgical resection of multiple metastatic nodules in the bilateral lungs. The other one, though inoperable due to extensive invasion to right and left atria, showed highly sensitivity to intra-arterial infusion chemotherapy of CDDP. These results suggested that long term survival might be expected even in metastatic or inoperable cases. There were no reliable histologic features distinguishing malignant from benign tumors, however 2 malignant cases with local invasion showed aneuploid patterns in the DNA histograms, which might offer a adjunct possibility in the assessment of malignancy.

Adult↗

Paraganglioma of the cerebellopontine angle. Case presentation and pathological considerations.

Paragangliomas (glomus tumors) arise from the extra-adrenal neuroendocrine system. They are benign but locally aggressive tumors, causing bone destruction and compression related symptoms. We present a case of paraganglioma of the cerebellopontine angle. Emphasis on possible difficulties in clinical, imaging or pathological identification, and surgical removal is done. To the best of our knowledge, only one more case was reported arising in the cerebellopontine angle.

Cerebellar Neoplasms↗

Retroperitoneal para-aortic paraganglioma.

Paragangliomas or chemodectomas are neoplasms that arise from neural crest cells and histologically resemble their adrenal counterpart, the pheochromocytoma. Seventy-one percent of the extra-adrenal paragangliomas are located in the superior or inferior paraaortic area. This tumor usually presents as an abdominal mass producing back pain. Tumor localization has improved remarkably through the use of computed tomography and I131 metaiodobenzyl-guanidine scintigraphy, particularly when tumors are hormonally active. Surgical excision remains the mainstay of treatment, although vascularity can at times make excision difficult.

Abdominal Pain↗

[Non-functioning retroperitoneal paraganglioma].

Extra-adrenal pheochromocytoma or paraganglioma are rare, usually benign tumours, which clinical behaviour varies in function of whether they are able to secrete vasopressor hormones. The case of a 66 year-old male with non-functional retroperitoneal paraganglioma (chemodectoma) accidentally found with an echography is presented here. The paper analyzes the most relevant features from a pathological, clinical, evolutive, diagnostic and therapeutic point of view.

Aged↗

Retroperitoneal para-aortic paraganglioma.

Paragangliomas or chemodectomas are neoplasms that arise from neural crest cells and histologically resemble their adrenal counterpart, the pheochromocytoma. Seventy-one percent of the extra-adrenal paragangliomas are located in the superior or inferior para-aortic area. This tumor usually presents as an abdominal mass producing back pain. Tumor localization has improved remarkably through the use of computed tomography and I131MIBG (metaiodobenzylguanidine) scintigraphy, particularly when tumors are hormonally active. Surgical excision remains the mainstay of treatment, although vascularity can at times make excision difficult.

Adult↗

[Gangliocytic paraganglioma of the duodenum].

Gangliocytic paraganglioma in a female of 64 years of age with stones in the gall bladder, atrophic liver cirrhosis and gastro-intestinal polyposis is described in the Russian literature for the first time. Polyps of the rectum and sygmoid were removed within 15 years. The same pathology was described by other authors. It is possible that the occurrence of paraganglioma and polyps was of genetical nature. The tumor was detected at gastroduodenoscopy. Biopsy diagnosis varied from polymorphocell liposarcoma and malignant fibrous histiocytoma. Endoscopic tumor electroexcision was complicated by intestinal bleeding that followed by an urgent laparatomy, duodenotomy and suture of the bleeding vessel in the duodenal wall. Bleeding zone in the duodenum was 3 cm from papilla Vateri. Analysis of difficulties in clinical and morphological differential diagnosis is performed.

Diagnosis, Differential↗

Paragangliomas of the cauda equina. Report of one case and review of the literature.

The authors report one case of cauda equina paraganglioma and review the neurosurgical, radiological and pathological literatureon this rare tumour. Although it is difficult to distinguish preoperatively the paraganglioma from other tumours of the cauda equina, like neurinoma or ependymoma, this neuroendocrine tumour should be included in differential diagnosis.

Cauda Equina↗

[Paraganglioma of the cranial vault. Apropos of a case].

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.

Adult↗