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Neurofibromatosis-1 and Ampullary Gangliocytic Paraganglioma Causing Biliary and Pancreatic Obstruction.

Gangliocytic paragangliomas (GPs) are exceedingly rare tumors that arise in close proximity of the papillaof Vater. Nevertheless, jaundice is an uncommon presenting symptom, reported in only 3 of 125 casesdescribed in the literature to date, with gastrointestinal bleeding being more common.Association between GPs and neurofibromatosis 1 (NF-1), described in two patients, may be more thancasual, being the frequency in normal population 1:3000.We report an additional case of this association, presenting for the first time with simultaneous obstructionof the common biliary duct, and of the main pancreatic duct. Previous cases of GPs with jaundice and/orNF-1 are reviewed, and updating of the histogenesis presented.

Journal Article↗

[Intubation using an illuminating stylet in a patient with a catecholamine-secreting paraganglioma].

Laryngoscopy and tracheal intubation produce intense noxious stimuli and are associated with an adrenergic response that can be deleterious in patients with concomitant diseases. Illuminating stylets effectively aid blind intubation by lighting the trachea, and using such devices has been associated with lower incidences of sore throat, dysphonia, and adverse hemodynamic events in comparison with rigid laryngoscopy. We report the case of a female patient with a catecholamine-secreting vagal paraganglioma. She developed multiple hypertensive episodes that were difficult to control during surgical resection of the tumor. It was decided to perform orotracheal intubation with a Light Wand (Vital Signs, Inc., Totowa, NJ, USA) to avoid oropharyngeal stimulation and to attenuate the hypertension and arrhythmias associated with laryngoscopy in such patients. The procedure was carried out without difficulty on the first attempt, in spite of tracheal displacement. The maximum increases in blood pressure and heart rate were observed 1 minute after intubation and were less than 30% of basal levels. We believe that using this light wand while intubating this patient helped keep the expected hemodynamic response to a minimum during laryngoscopy.

Carotid Body Tumor↗

[Arteriovenous shunting of cerebral blood flow in basal meningo- cerebral developmental anomalies and paraganglioma of the glomus jugulare].

The authors describe a case of angiographically verified arteriovenous shunting of the cerebral blood flow coupled with basal ++meningo-cerebral developmental abnormality and paraganglioma of the glomus jugulare in a 52-year-old patient. Such a unique combination made it possible to suggest the existence of potential mechanisms of blood flow shunting either via tumorous vessels or vessels (arteriovenous shunts) of the basal ++meningo-cerebral developmental abnormality. In the latter case, the shunts were likely to occur latently while the clinical manifestations of the disease might be related to the tumor growth, impairment of the venous outflow and disclosure of arteriovenous shunts.

Abnormalities, Multiple↗

[Tumor of the carotid body (paraganglioma)].

Carotid body tumours (paragangliomas) are uncommon but rather an interesting pathology, due to the controversy that still remains regarding their histopathologic behaviour (malignancy?), as well as their diagnosis and management. Because of its nature being composed of chemoreceptor paraganglionic tissue, the carotid body responds to lower partial pressure of oxygen and can cause severe cardiovascular disturbances. Based upon a series of 12 cases, the authors reviewed this pathology in detail, and discussed the methods of diagnosis and treatment, which they considered more appropriate.

Adult↗

Extra-adrenal pheochromocytoma (paraganglioma) in a cat.

Extra-adrenal pheochromocytoma (paraganglioma) arising from periadrenal tissue was diagnosed in an 18-year-old spayed domestic shorthair cat. The tumor was palpable on physical examination, but not apparent on plain radiographs. The cat developed temporary cardiac arrhythmia while the mass was being handled during excision, suggesting that the tumor was functional. The tumor was characterized histologically by nests and sheets of neoplastic cells separated by thin, vascular stroma. The cells had abundant eosinophilic granular cytoplasm and prominent nuclei. Diffuse, dark, intracytoplasmic granules were seen in sections stained with Grimelius stain. Ultrastructurally, the cells contained round and oblong, membrane-limited, dense core neurosecretory-type granules. Serotonin was detected in the cytoplasm of the neoplastic cells by use of immunocytochemical analysis.

Adrenal Gland Neoplasms↗

Spinal metastases of carotid paraganglioma. One case and review of the literature.

Paragangliomas of the carotid body are uncommon tumours usually regarded as benign or locally malignant. Metastasis occurs in 5 to 25% of the cases, involving mainly the liver, lung and bones. A case of carotid body tumour without histological evidence of malignancy but with local invasion and, subsequently, multiple spinal metastases is reported. Since the histological prognosis of the initial tumour is impossible to make in most cases, the authors underline the importance of signs of local invasion which is predictive of metastasis and requires regular monitoring with radionuclide bone scanning and MRI.

Adult↗

[Oncocytic paraganglioma of the carotid body].

One case of benign oncocytic paraganglioma which had originated from the non-chromaffin cells of the carotid body in a woman, 66 years of age, is described in this paper. The microscopic pattern of the tumor consisted mainly of large, round, eosinophilic cells arranged in solid nests and sheets. Pale rather than eosinophilic cells of polygonal shape were predominant in the peripheral areas. Ultrastructural analysis revealed the presence of three cell types: oncocytes, cells with electron-dense granules, 150 to 200 nm in size, and transitional forms between these two. Possible development of oncocytes from APUD cells is discussed.

Aged↗

Surgical treatment of carotid paragangliomas presenting unusual technical difficulties. The value of preoperative embolization.

Although the application of reconstructive vascular surgical procedures to the treatment of carotid paragangliomas has made their resection the method of choice and has produced excellent cure rates, it has not obviated some of the technical problems presented by excessively vascular, adherent, or bulky lesions. Our experience with preoperative trans-catheter embolization for the reduction of the vascularity in six cases of this group of lesions is presented and the conclusion is made that preoperative embolization greatly reduced operative technical difficulties.

Adult↗

Malignant paraganglioma (pheochromocytoma) of the urinary bladder: report of a case and review of the literature.

A rare case of malignant paraganglioma of the urinary bladder with metastasis to a lymph node in a 12-year-old girl is reported, and eight other previously recorded cases are reviewed. Headache, fainting, and hypertension initiated by voiding were the most prominent clinical symptoms. Hematuria was present in three cases and aided in prompt diagnosis. Cystography, pelvic angiography, and cystoscopy were most diagnostic. Segmental cystectomy when feasible, was the preferred surgical treatment. The prognosis was slightly better than for other forms of extraadrenal pheochromocytomas.

Adolescent↗

[Carotid paragangliomas].

Three cases of carotid body paragangliomas are reported. The authors underline the critical diagnostic role of carotid arteriogram whenever such disease is suspected. Surgical treatment can lead to good results even in malignant forms. In the latter, treatment should be completed with radiotherapy.

Adult↗

Functional carotid paragangliomas. Biochemical, ultrastructural, and histochemical correlation with clinical symptoms.

To our knowledge, this study is the first to correlate the results of biochemical, electron microscopic (EM), and histochemical analyses of tissues from functional paragangliomas of the upper carotid area with a patient's clinical symptoms. The tumor tissues from our two patients contained epinephrine, norepinephrine, and dopamine. Neurosecretory granules were identified in both tumors by EM and by the Grimelius silver stain. The patient with the most severe symptoms exhibited evidence of higher circulating levels of catecholamines by biochemical, EM, and histochemical analyses.

Adult↗

[Iatrogenic complications of paraganglioma of the jugular foramen].

Twenty-eight cases of tumours of the paraganglioma of the foramen jugulare arising from the jugular foramen were treated from 1982 to 1990. The follow-up and the iatrogenic effect of embolization, surgery and radiotherapy were studied. Routine pre-operative embolisation has changed the therapeutic approach to these tumours although a certain number of mainly neurological complications still occur (32% of the cases with 18% of major accidents) especially when the internal carotid component must be excluded. Current oto-neurosurgical techniques allow wider and more complete exeresis of the tumour but often lead to neurological deficits involving the facial nerve (45%), the mixed nerves (32%) and/or the XII (18%). These deficits occur due to unavoidable sacrifice or peroperative manipulation. Radiotherapy is an essential therapeutic possibility with only minor complications and can be proposed in cases of inoperable or relapsing tumours or after an incomplete surgical exeresis. Our critical analysis led us to propose the present indications for surgery. Surgery is indicated in patients with two or more of the following criteria: age less than 55, unilateral tumour, a tumour considered to be inoperable, preoperative otoneurological manifestations, invasion of the posterior fossa. In the other cases, radiotherapy remains the indicated therapy. It is effective and should be used more often for inoperable tumours of the para-jugular lymph nodes, for relapses or after incomplete surgical exeresis.

Adult↗

Carotid body paraganglioma with coexistent pheochromocytoma in childhood.

A male child aged six years presented with a swelling in neck and hypertension. He was diagnosed to have and operated for Carotid body paraganglioma with Coexistent pheochromocytoma. Hypertension persisted even after excision of both the tumours which could be controlled with anti-hypertensive agents. The case is discussed with review of literature.

Adrenal Gland Neoplasms↗

[An active endocrine glomus tumor (paraganglioma) as a cause of tinnitus and hypertension].

A glomus jugulare tumor arises from the glomus bodies located in the adventitia of the dome of the jugular bulb. A glomus tumor has the same microscopic appearance as a carotid body tumor. The present report includes the extraordinary clinical and morphological features of a catecholamine-secreting glomus tumor (paraganglioma) in a 41-year-old woman. The patient presented with uncontrollable hypertension und pulsatile tinnitus. The diagnostic and therapeutic management is reported, including MIBG scintigraphy, computed tomography, magnetic resonance imaging, embolization, angiography, selective venous sampling studies and control of hypertension. After cardiovascular stabilization and tumor embolization, the tumor was removed surgically, with subsequent resolution of hypertension. While light microscopical analysis showed a classical glomus tumor, ultrastructural analysis revealed particular details of the tumor cells.

Adult↗

[An interdisciplinary therapy concept in treatment of paragangliomas of the skull base--demonstrated with a case].

Surgical resection of paragangliomas in the cervical region is complicated due to the tumour vascularity and is associated with the risk of major intraoperative blood loss. Preoperative angiographic embolization of the tumour supplying arteries by intravascular injection of gelfoam and implantation of microcoils was performed to decrease tumour vascularity. We report the case of a 78 years old female patient suffering from a carotid body tumour. Computed tomography demonstrated spreading of the tumour from the carotid bifurcation up to the base of the skull. Primary surgical resection was aborted because of the bleeding tendency of the tumour. Tumour embolization was required in order to achieve the therapeutic aim of a complete extirpation of tumourous tissue by preserving the vascular system of the internal carotid artery. The reported case is completed by a review of literature.

Aged↗

[Interruption of the lateral sinus in surgery of paraganglioma and acoustic neurinoma. Apropos of 2 complications].

The sigmoid sinus, one of the main structures of the venous cerebral circulation, may be interrupted by various pathological or surgical processes. Usually, interrupted circulation is compensated by collateral or contralateral venous circulation. No neurological deficit is observed. We report two neurological complications: cerebellar infarction and intracranial hypertension, secondary to lateral sinus interruption during removal of a paraganglioma and an acoustic neuroma. Attention is drawn on the importance of preoperative evaluation of the venous cerebral circulation to prevent such complications.

Cerebral Angiography↗