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Extra-adrenal pheochromocytoma (paraganglioma) of the urinary bladder: a case report.

The case of a 49-year-old male patient with paraganglioma of the urinary bladder is presented here. The patient's only complaint was of gross hematuria: sustained hypertension and post-micturitional hypertension were not presented. Transurethral resection was performed to diagnose the bladder tumor. Pathological examination resulted in the diagnosis that the resected tissue was a paraganglioma. Computed tomography, magnetic resonance imaging and iodine-131-labeled metaiodbenzylguanidine scintigraphy revealed that the tumor was a primary paraganglioma in the urinary bladder. Plasma concentrations of the catecholamines were virtually within the normal limits. Hypertensive crisis was not revealed during the transurethral resection. The tumor was non-functional. Partial cystectomy was performed. The patient has remained disease-free for five months after surgery.

Biomarkers, Tumor↗

[A case of primary pulmonary paraganglioma].

A case of primary paraganglioma of the lung in a 48-year-old woman is presented. She was found to have a coin lesion in lower lobe on routine radiography. Results of bronchoscopic examination were negative, and exploratory right thoracotomy was performed. At operation, non-invasive encapsulated tumor found in posterior basal segment of the right lower lobe was enucleated. Histological analysis of the resected tumor proved to be compatible with pulmonary paraganglioma. Pulmonary paraganglioma is said extreme rare, and so only 21 cases were previously reported in the literature including this case.

Female↗

Pitfalls in imaging: differentiating intravagal and carotid body paragangliomas.

We report a case of an intravagal paraganglioma and a case of a carotid body tumor to illustrate a variable presentation of the former in which it mimicked the latter on preoperative imaging. The atypical imaging features of the intravagal paraganglioma included inferior extension to the level of the carotid bifurcation and splaying of the internal and external carotid arteries, features that are similar to those seen in a case of carotid body paraganglioma. Proper differentiation of these lesions permits more appropriate preoperative counseling and surgical preparation. When using magnetic resonance angiography rather than catheter angiography, we advocate the inclusion of gadolinium contrast and three-dimensional time-of-flight techniques to better demonstrate the position of the tumor relative to the carotid bifurcation.

Adult↗

[Paraganglioma of the urinary bladder: a case report].

A patient with paraganglioma of the urinary bladder is reported. A 65-year-old woman was referred to our hospital with the chief complaint of postvoiding headache, palpitation, and cold sweat. She had has no episodes of hematuria. On laboratory data, mild elevation was found in plasma neuron specific enolase (NSE), urinary noradrenaline and urinary metanephrine. The patient showed hypertension after urination with the elevation of plasma noradrenaline. 131I-MIBG scintigraphy showed abnormal accumulation in the bladder, and no abnormal accumulation in the other lesion. Pelvic magnetic resonance imaging (MRI) revealed a solid tumor of the urinary bladder, measuring 2 cm in diameter. Paraganglioma of the urinary bladder was diagnosed and the tumor was extirpated. The tumor measured 2 x 2 x 1.5 cm, and histopathologically diagnosed as paraganglioma. After extirpation, the patient became free of the postvoiding symptoms, and showed normal catecholamine levels. She has been followed up for 4 months after operation without any evidence of recurrence.

Aged↗

[Clinicopathologic study of four cases of vagal paraganglioma].

OBJECTIVE: To determine if pathologic examination can be useful in both diagnosing cervical paraganglioma and deducing its vagal origin. METHODS: Four cases of vagal paraganglioma were studied by light microscopy and immunohistochemistry, with clinical and radiologic (computerized tomography and/or magnetic resonance imaging) correlation. RESULTS: All patients were females and complained of upper neck mass with symptoms and signs of vagus nerve involvement, such as hoarseness of voice, ipsilateral vocal cord dysfunction and cough induced by drinking or local pressure. Radiological examination showed a tumor mass over the common carotid artery bifurcation, lying between the internal carotid artery and internal jugular vein. On gross inspection, the stump of the resected vagus nerve could be identified, with nerve bundles splaying over the tumor surface. Microscopically, the tumors showed a relatively uniform nesting arrangement (Zellballen pattern) of cells and were associated with rich sinusoidal vessels and fibrous stroma. Multiple myelinated nerve fibers were present in stroma and fibrous capsule of the tumor. Sometimes, invasion of nerve trunk by tumor cells was seen. Immunohistochemically, the tumor cells showed diffuse positivity for chromogranin A, neuron-specific enolase and synaptophysin. There was no expression of cytokeratin. The sustentacular cells and nerve bundles were highlighted by S-100 protein. CONCLUSION: In addition to the microscopic accurate diagnosis of paraganglioma, histopathologists can be of help in deducing the vagal origin of this tumor.

Cranial Nerve Neoplasms↗

Tissue culture, electron microscopic and enzyme histochemical investigations of extraadrenal paragangliomas.

Light and electromicroscopical as well as histochemical investigations were performed on three cases of extraadrenal paragangliomas. They were localized in the carotid body, tympanicum and cauda equina region. Tissue of two cases was cultivated in vitro in nutrient medium TCM 199. The tumours were classified as paragangliomas of the paraganglionic type with typical cell clusters, of the adenomatous and angiomatous type. The enzyme histochemistry showed a very high dehydrogenase activity. Ultrastructurally numerous typical osmiophilic granules could be observed in the cytoplasm of the tumour cells. In tissue culture only a minimal cellular proliferative activity could be detected. The few proliferating cell colonies showed mostly characteristics of epithelial tissue and sometimes a similar behaviour to cells of a ganglioneuroblastoma. The minimal proliferative activity in vitro is in good agreement with the proliferative behaviour of the extraadrenal paragangliomas in vivo.

Carboxylic Ester Hydrolases↗

[A case of paraganglioma in the posterior mediastinum].

A case of non-functioning aortico-sympathetic paraganglioma originating from left posterior mediastinum in a 34-year-old man is reported. He was found to have an abnormal shadow on the chest X-ray of an asymptomatic. His blood pressure and serological examination were within normal range on admission to our hospital. At thoracotomy a bronze 5 x 5 x 5 cm mass was recognized at the costovertebral sulcus related to the 1st sympathetic trunk. Histological examination of the surgical specimen showed that it was a paraganglioma, and neurosecretary granules were found on Grimelius staining microscopic examination. In the Japanese literature, only 13 cases of intrathoracic paraganglioma or pheochromocytoma is reported. The literature was reviewed.

Adult↗

Paragangliomas of the head and neck: immunohistochemical neuroendocrine and intermediate filament typing.

Twenty-nine paragangliomas of the head and neck region including 20 glomus jugulare (GJ) and nine carotid body (CB) tumors were evaluated for the presence of neuroendocrine and intermediate filament antigens. Immunohistochemistry on formalin-fixed, paraffin-embedded tissue was used to identify: S-100 protein (S-100); neuron-specific enolase (NSE); chromogranin A (CHA); serotonin (SER); synaptophysin (SYN); cytokeratin (CK); neurofilament (NF); desmin (DES); vimentin (VIM); and glial fibrillary acidic protein (GFAP). S-100 protein staining of sustentacular cell nuclei and cytoplasm was found in all tumors and was present in chief cells in 4 of 20 GJ and 3 of 9 CB tumors. All tumors stained with at least three neuroendocrine markers (29 of 29 NSE, 28 of 29 SYN, 26 of 29 CHA, 25 of 29 SER). CK was detected in 2 GJ and 1 CB tumor using anticytokeratins AE 1/3 and CAM 5.2. Neurofilament protein could not be demonstrated in fixed material, and all tumors were negative for GFAP and desmin. Vimentin was inconsistently detected in chief and sustentacular cells. We conclude that, in formalin-fixed material, paragangliomas have S-100 protein staining of sustentacular cells with chief cells containing antigens associated with neuroendocrine differentiation. The presence of CK in some paragangliomas is consistent with recent tissue culture studies demonstrating immunoblot confirmation of CK in pheochromocytomas and represents a potential source of immunohistologic misinterpretation in diagnosis, unless a panel of markers is utilized.

Antibodies↗

Gangliocytic paraganglioma in association with a duodenal diverticulum.

Gangliocytic paraganglioma is an extremely rare benign neurogenic tumor nearly exclusively located in the second portion of the duodenum, also the most common site of duodenal diverticula. A case is reported of a gangliocytic paraganglioma presenting in a 65-yr-old woman with a 1-yr history of postprandial cramping abdominal pain culminating in a single episode of melena leading to laparotomy. The tumor was identified with difficulty by endoscopy following negative barium studies. Histologically, the tumor is composed of carcinoid-like cells admixed with varying numbers of ganglion cells in a substratum of neuroid spindle cells. The epithelioid cells contain argyrophilic cytoplasmic granules confirmed by electron microscopy to be dense core membrane-bound secretory granules. Review of the literature suggests the tumor described is typical both clinically and pathologically of gangliocytic paraganglioma of the duodenum, except for its unprecedented occurrence in association with a duodenal diverticulum.

Aged↗

[Vagal paraganglioma associated with a rare malformation of the aortic arch. Apropos of a case and review of the literature].

The authors report about a very peculiar case of cervical paraganglioma which had two specific features. This is a familial case with 2 sisters presenting with bilateral paragangliomas. In addition, this patient's aortic trunk is very peculiar, with a retroesophageal right subclavian artery and, above all, a divided intrathoracic right common carotid artery, producing an ascending pharyngeal supply for the right paraganglioma, which can be embolized without any risks of reflux.

Adult↗

Histomorphometric and morphologic studies of the carotid body and aortic paragangliomas.

Morphometric and morphological parameters have been evaluated in tumours of carotid body paraganglia and of aorticopulmonary and aorticosympathetic paraganglia. Paragangliomas are characterized by a higher cell density and rounder nuclei (roundness factor) than non-neoplastic carotid body paraganglia. The nuclear area has similar mean values in tumours and normal paraganglia; however, the former group shows higher standard deviation values and coefficients of variation, reflecting the fact that paragangliomas have nuclei of variable size; the variation is more pronounced in aorticopulmonary and aortico-sympathetic paragangliomas. However, subgroups on the basis of morphological features (architectural patterns and degree of intensity of argyrophilia), site of origin, and clinical behaviour (metastases and recurrences) show no morphometric differences.

Aorta↗

[An autopsied case of malignant paraganglioma of the posterior thoracic cavity].

An autopsied case of a malignant paraganglioma of the posterior thoracic cavity is reported. A 68-year-old man had complained of chest discomfort, and serial examinations revealed a functioning paraganglioma with bone metastasis. After death a pathological examination revealed that the tumors consisted of alveolarly arranged cells and well developed capillary vessels. Numerous neurosecretory granules were observed on viewing by electron microscopy. An immunohistochemical examination showed that most of the tumor cells were positive for NSE, while only a few cells were positive for the S-100 protein. These results indicate that a paraganglioma originating from the aortic sympathetic paraganglia had similar features of a carcinoid and a neuroblastoma.

Adrenal Gland Neoplasms↗

[An autopsy case of malignant retroperitoneal paraganglioma].

A case of an extra-adrenal malignant paraganglioma of the retroperitoneum is reported. A 33-year-old woman with a complaint of a dull pain in the right thigh was admitted. A CT scan revealed a large retroperitoneal mass situated between the right kidney and the aorta. A biopsied specimen of the right femur uncovered a metastatic focus of a paraganglioma. Subsequent chemotherapy and irradiation ultimately proved ineffective, and the patient died at the age of 37. Autopsy revealed a retroperitoneal mass 11 X 9 X 4 cm, and its metastatic foci in the vertebrae, ribs, sternum, and liver. Six cases of extra-adrenal malignant paragangliomas of the retroperitoneum that have been reported in Japan also are briefly reviewed.

Adult↗

[The risk of endocrine activation in interventional procedures on paraganglioma of the head and neck].

In the literature on paragangliomas or glomus tumors of the head and neck, the question rarely arises as to whether in endocrine inactive paragangliomas the tumor necrosis resulting from the embolization can release the stored substances. Based on 34 paragangliomas after surgical treatment, the angiographic and immunohistological characteristics of the paraganglionic, angiomatous and adenomatous tumors are presented. No catecholamine secretion occurred as a result of interventional or surgical treatment.

Antibodies, Neoplasm↗

[A case of malignant paraganglioma within the renal sinus].

A case of malignant paraganglioma within the renal sinus is presented. A 51-year-old woman underwent left transabdominal nephrectomy for renal tumor. Pathologically, the tumor was malignant nonchromaffin paraganglioma. This tumor seemed to originate from parasympathetic paraganglions around the left renal artery. Paraganglioma is rare in the literature. Especially, this may be the first report of such a lesion in the renal sinus.

Female↗

Non-chromaffin paraganglioma of the orbit. Case report.

The non chromaffin paraganglioma of the orbit is a relatively rare tumor. To our knowledge only 25 cases have been reported in the world literature. We report on the case of a 39-year-old woman who was treated surgically for the removal of an orbital paraganglioma 10 years ago. She had complained of proptosis and her right eyeball was slightly displaced upwards and laterally. A transcranial operation was performed and the tumor, located medically and weighing 9'5 grs., was completely removed; it was encapsulated. 10 years after this total excision there was no evidence of recurrence. From a histological point of view the tumor cells closely resembled those of paragangliomas of the carotid body and glomus jugulare.

Adult↗

Paraganglioma of the larynx.

A patient with a laryngeal paraganglioma is presented in which overtly malignant behavior occurred despite a four-year period of symptoms prior to diagnosis. A review of the world literature reveals 52 reported cases of laryngeal paragangliomas. The incidence of malignancy in these neoplasms is often underestimated, as at least 25% of reported cases exhibited a clinically malignant natural history. We believe that the treatment of laryngeal paragangliomas should be based on the assumption that all tumors have a potential for malignant growth. Wide-field surgical excision is the applicable treatment for tumors still localized to the larynx. The results of radical neck dissection for established nodal disease are discouraging. In those patients with palpable lymph nodes, widespread dissemination has usually taken place.

Humans↗

[Vagal paraganglioma. Apropos of 2 cases. Review of the literature].

The authors relate the case of two patients of 61 and 64 years old, each one having a high latero-cervical tumor, arching the soft palate, developed from the right vagus nerve and which was a chromaffin paraganglioma. After a recall about the APUD system tumors and the "paraganglioma" concept, they relate their observations at state of the vagal paraganglioma histology, pathology and treatment.

Female↗