Cases reported "Paraganglioma"

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1/21. paraganglioma of the cauda equina: MR findings.

    The MR findings of a case with paraganglioma of the cauda equina are presented. T2-weighted images showed the tumor to have a hyposignal rim and serpiginous flow voids, suggesting vessels capping the tumor. Contrast-enhanced MR images highlighted the tumor and tumor vessels more clearly. knowledge of MR findings in this unusual disorder may aid in diagnosis.
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2/21. paraganglioma of the sphenoid sinus appearing as labile hypertension.

    Paragangliomas, also known as glomus tumors, are unusual, generally benign tumors most commonly found in association with major vessels and cranial nerves. Only a small number of these tumors have been described arising in the paranasal sinuses, and none of these has been physiologically active. The following case report describes a metabolically active paraganglioma arising in the sphenoid sinus.
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3/21. Mesenteric paraganglioma: a case report and review of the literature.

    Approximately 5% to 10% of paragangliomas occur in extra-adrenal sites, which can extend from the upper cervical region to the pelvis, parallel to the autonomic nervous system. This distribution corresponds to the embryologic development of the paraganglia from neural crest cells. Rarely, extra-adrenal paragangliomas can also occur aberrantly outside this distribution. We report such a case of extra-adrenal paraganglioma occurring in the anterior mesentery in a 76-year-old man. Two case reports exist in the literature describing extra-adrenal paragangliomas in the posterior mesentery. Normal paraganglionic tissue has been described at the roots of the superior and inferior mesenteric arteries, theoretically explaining the origin of the posterior mesenteric paragangliomas. Our case can best be attributed to the ventral migration of paraganglionic tissue through these vessels to reach the anterior mesentery, where they could potentially give rise to paragangliomas in this site.
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4/21. Laparoscopic resection of a functional paraganglioma in the organ of Zuckerkandl.

    We describe the successful laparoscopic resection of a functional paraganglioma in the organ of Zuckerkandl. A 47-year-old man with hypertension and diabetes mellitus was found to have an abdominal mass beside the aorta. The tumor was diagnosed as a functional paraganglioma by diagnostic imaging and biochemical tests. We then performed a transperitoneal laparoscopic resection for removal. After freeing the left ureter, resecting the inferior mesenteric artery, and dividing the small blood vessels, the tumor was isolated and found to be preserved in its capsule. It was retrieved in a bag through an enlarged incision. The operation time was 450 min and blood loss was 410 ml. The postoperative course was uneventful and there has been no local recurrence or distant metastasis during the 18-month follow-up period. Laparoscopic resection of functional extraadrenal paragangliomas is technically feasible and safe if adequate pre- and intraoperative medical management and a careful, steady surgical technique are used.
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5/21. carotid body tumour.

    A case of carotid body tumour (paraganglioma) which is both unusual and highly vascular, arising from the carotid body is reported. The patient was a 68-year-old female with a right submandibular swelling. The initial pathological diagnosis was obtained from the incisional biopsy. Carotid angiography revealed the feeding vessels arising only from the external carotid artery. The tumour was completely removed and no evidence of recurrence could be found 2-years postoperatively.
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6/21. Complex tumors of the glomus jugulare: criteria, treatment, and outcome.

    OBJECT: Tumors of the glomus jugulare are benign, slow-growing paragangliomas. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there remains a subgroup of complex tumors--multiple, giant, malignant, neuropeptide-secreting lesions, and those treated previously by an intervention with an adverse outcome--that is high risk, presents surgical challenges, and is associated with treatment controversy. In this article the authors report on a series of patients with complex glomus jugulare tumors and focus on treatment decisions, avoidance of complications, surgical refinements, and patient outcomes. methods: In this retrospective study, the patient population was composed of 11 male and 32 female patients (mean age 47 years) with complex tumors of the glomus jugulare who were treated by the senior author within the past 20 years. These include 38 patients with giant tumors, 11 with multiple paragangliomas (seven bilateral and four ipsilateral), two with tumors that hypersecreted catecholamine, and one with a malignant tumor. Six patients had associated lesions: one dural arteriovenous malformation, one carotid artery (CA) aneurysm, two adrenal tumors, and two other cranial tumors. All but one patient presented with neurological deficits. Cranial nerve deficits, particularly those associated with the lower cranial nerves, were the prominent feature. Twenty-eight patients underwent resection in an attempt at total removal, and gross-total resection was achieved in 24 patients. Particularly challenging were cases in which the patient had undergone prior embolization or CA occlusion, after which new feeding vessels from the internal CA and vertebrobasilar artery circulation developed. The surgical technique was tailored to each patient and each tumor. It was modified to preserve facial nerve function, particularly in patients with bilateral tumors. Intrabulbar dissection was performed to increase the likelihood that the lower cranial nerves would be preserved. Each tumor was isolated to improve its resectability and prevent blood loss. No operative mortality occurred. In one patient hemiplegia developed postoperatively due to CA thrombosis, but the patient recovered after an endovascular injection of urokinase. In four patients a cerebrospinal fluid leak was treated through spinal drainage, and in five patients infection developed in the external ear canal. Two of these infections progressed to osteomyelitis of the temporal bone. There were two recurrences, one in a patient with a malignant tumor who eventually died of the disease. CONCLUSIONS: Despite the challenges encountered in treating complex glomus jugulare tumors, resection is indicated and successful. Multiple tumors mandate a treatment plan that addresses the risk of bilateral cranial nerve deficits. The intrabulbar dissection technique can be used with any tumor, as long as the tumor itself has not penetrated the wall of the jugular bulb or infiltrated the cranial nerves. Tumors that hypersecrete catecholamine require perioperative management and malignant tumors carry a poor prognosis.
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7/21. Use of radial forearm free flap with palmaris longus tendon in reconstruction of total maxillectomy with sparing of orbital contents.

    Nasal paragangliomas are extremely rare. The most adequate treatment is total excision. After surgical excision requiring total maxillectomy, there has been no ideal technique for reconstruction. A 47-year-old man was admitted to our clinic because of recurrent epistaxis, which lasted for 2 months. He was also suffering from nasal airway obstruction. The physical examination revealed a mass originating from the medial aspect of the middle turbinate of the right nasal cavity. It invaded the anterior maxillary wall and hard and soft palate. Endoscopic examination showed that the mass pushed the nasal septum to the left side and protruded into the nasopharynx. The mass was fleshy and had a rich capillary network. Conventional paranasal sinus radiographs were normal. Computerized tomography of the skull showed the mass protruding into the nasopharynx. A total maxillectomy was performed. Histopathological evaluation showed neoplastic tissue consisting of round, oval, or slightly elongated cells, altogether of a rather monomorphous appearance, tending to arrange themselves in clusters adjacent to or around capillary blood vessels. The blood vessels were numerous and branched. reticulum staining showed a typical Zellballen arrangement of the neoplastic cells to provide a firmer basis for the diagnosis of paraganglioma. To reconstruct the total maxillectomy defect, a radial forearm free flap with the palmaris longus tendon was elevated to inlay the nasal cavity and the oral cavity and to suspend the ocular globe. The flap was placed into the defect, and the palmaris longus tendon was medially and laterally anchored to the periosteum of the frontal bone to suspend the ocular globe in the orbital cavity. One part of the skin island was used to close the defect of the nasal mucosal cavity, and the other part was used to repair the oral mucosal defect of the palate. Consequently, speech was considered near normal; the patient was able to eat an unrestricted diet and to retain both solid and liquid food inside the oral cavity without drooling, and there was no diplopia or enophthalmos. Six months later, porous polyethylene was inserted and fixed to the zygomatic bone with a miniplate and miniscrews to restore malar contour. No further procedure was believed to be necessary later on. Two years later, a satisfactory and functional esthetic result was obtained, providing an acceptable suspension of the ocular globe and filling of the total maxillectomy defect. We believe that a total maxillectomy is indicated if it is needed in nasal paragangliomas and that microsurgical repair with the composite radial forearm-palmaris longus free flap has several advantages: 1) it can offer en bloc reconstruction of the entire defect after a total maxillectomy in terms of good function and cosmesis; 2) it can repair mucosal defects; and 3) it can anchor and suspend the ocular globe in its original anatomical location, protecting against gravity through the sling effect of the palmaris longus tendon. The composite radial forearm-palmaris longus free flap has not been described previously for suspension of the ocular globe.
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8/21. Nonfunctioning paraganglioma of the aortopulmonary window.

    Aortopulmonary paraganglioma is a rare tumor of the mediastinum. The only effective treatment is complete resection, which may pose a surgical challenge because of its proximity to the heart, great vessels, and trachea, often rendering a complete resection difficult to achieve. We report a case in which the tumor was excised under cardiopulmonary bypass and resulted in massive bleeding only controlled by means of packing the pleural cavity during 48 hours, known as damage control strategy. The patient survived and has been disease-free for 2 years.
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9/21. Mediastinal paraganglioma irrigated by coronary vessels in a patient with an atypical chest pain.

    We present a case of non-functioning anterior mediastinal paraganglioma in a 61-year-old woman. The tumour was accidentally discovered on a coronary angiography performed in an atypical precordial pain and dyspnea. The coronary angiography showed a vascular tumour in the paraaortic localization irrigated from two branches of coronary arteries. diagnosis was confirmed by a thoracic tomographic scan which revealed an anterior mediastinal tumour. Complete tumour resection was done through a sternotomy with extracorporeal circulation without cardiac arrest. The histological examination of the operative specimen was characteristic of paraganglioma.
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10/21. nasopharynx paraganglioma with extension in the clivus.

    paraganglioma is a rare benign tumor arising from the sympathetic nervous system. Here we describe an exceptional case of a paraganglioma located in the nasopharynx with an extension through the clivus up to the dura. Atypically, no contact with any major vessels was found. A radical resection of the mass was performed by an anterior transmaxillary approach through a Le Fort I osteotomy. One year follow up reveals no signs of local or distant recurrence. No cosmetic changes can be observed after the surgery and nasal and masticatory functions are unmodified. We review the clinical presentation, workup of paraganglioma, as well as the surgical approaches to the clivus.
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