1/44. Paragangliomas of the spinal canal.We report the clinical MRI and histopathological features of five consecutive cases of spinal paraganglioma. Three intradural tumours were found in the typical location (two at the L4, one at the S2 level); one intradural extramedullary tumour arose at an unusual level, from the ventral C2 root, and one extradural tumour growing along the L5 nerve root sheath had an aggressive growth pattern with early, local paraspinal recurrence and, eventually, intradural metastatic spread. This type of growth pattern has not been described previously. Paragangliomas of the spinal canal are more common than previously thought and can be located anywhere along the spine, although the lumbosacral level is the most common. Their appearance on MRI can not distinguish them from other tumours in the spinal canal. Even though paragangliomas in general are benign and slowly growing their growth pattern can vary and be more aggressive, to the point of metastatic spread.- - - - - - - - - - ranking = 1keywords = spinal canal, spinal, canal (Clic here for more details about this article) |
2/44. Sudden death in a child due to an intrathoracic paraganglioma.A 12-year-old boy under treatment for asthma was found dead in his home. The autopsy revealed a large posterior mediastinal mass that completely compressed the upper lobe of the right lung and the associated airways. This mass extended from the right costovertebral sulcus into the thoracic spinal canal through the spinal foramen and compressed the spinal cord. It was located in the epidural space and was adherent to a nerve root. The histologic and immunocytochemical features were that of a paraganglioma. Although neurogenic tumors are the most common posterior mediastinal masses in the pediatric population, paragangliomas are rare, and spinal involvement has not been described in children. In addition, sudden death has not been reported in association with any of the 13 cases of posterior mediastinal paraganglioma described in the literature as involving the spine. This case illustrates an unusual cause of sudden death in a pediatric patient due to a benign neoplasm.- - - - - - - - - - ranking = 0.1922077735819keywords = spinal canal, spinal, canal (Clic here for more details about this article) |
3/44. paraganglioma of the external auditory canal: an unusual case.Primary paraganglioma of the external auditory canal is a rare otological finding. To date, only three cases have been reported in the world-wide literature. Such a tumour is now described in a 42-year-old female. The clinical and histopathological features of the case are discussed. The role of immunocytochemistry in defining diagnosis, from a large list of differentials, is illustrated. We present the first magnetic resonance images of this rare tumour and highlight the benefit of intra-operative frozen section in limiting surgery to canal excision.- - - - - - - - - - ranking = 0.04074963347128keywords = canal (Clic here for more details about this article) |
4/44. Surgical resection of jugulare foramen tumors by juxtacondylar approach without facial nerve transposition.PRESENTATION: Jugulare foramen tumors (JFT) remain a difficult challenge especially in the forms extending extradurally and invading the petrous bone. In the standard technique, facial nerve function is placed at risks because of its transposition. We report on 31 extradural (N = 11) or intra-extra dural (N = 20) JFT resected surgically using the juxtacondylar approach alone or in combination with the infratemporal approach and without facial nerve transposition. RESULTS: The juxtacondylar approach permits the opening of the JF on its posteroinferior aspect and thus reduces the extent of petrous bone drilling. In tumors strictly located in the JF (N = 11), no petrous bone drilling is necessary and the facial nerve is never exposed. In tumors extending into the petrous bone (N = 20), the facial nerve was never transposed and moreover was kept in its bony canal in 15 cases. In only 5 cases, was the fallopian canal opened as the tumor was invading its bony wall. Similarly hearing function, when pre-operatively intact was always preserved and a better preservation of the lower cranial nerves could be achieved. Whatever the tumoral extent along the petrosal carotid artery, a radical resection could be realized in 30 cases. CONCLUSION: Therefore, we consider the juxtacondylar approach a useful adjunct to increase the possibilities of resection of JFT; it allows a better preservation of the neurovascular structures, especially the facial nerve which is kept in place and moreover kept in its bony canal when it is not invaded by the tumor.- - - - - - - - - - ranking = 0.02037481673564keywords = canal (Clic here for more details about this article) |
5/44. Metastatic paraganglioma causing spinal cord compression.We describe two cases of metastatic retroperitoneal paraganglioma associated with extradural spinal cord compression. Both occurred in young men; one being metastatic at presentation, the other becoming metastatic 19 years after attempted surgical resection. Despite a long, relatively stable natural history after diagnosis (10 and 19 years, respectively) both had an acceleration of their disease once extradural disease was detected. These cases illustrate the potentially aggressive nature of this disease, the need for long-term follow-up and the effectiveness of a variety of therapies for palliation, and also raise the possibility of "prophylactic" treatment to prevent spinal cord compression.- - - - - - - - - - ranking = 0.05746749055928keywords = spinal (Clic here for more details about this article) |
6/44. Oncocytic paraganglioma of the cauda equina in a child. Case report and review of the literature.The authors report a case of oncocytic paraganglioma of the cauda equina in a 12-year-old girl who presented with lower back and leg pain on the right side of 6 months' duration. magnetic resonance imaging revealed an ellipsoidal, intradural, extramedullary mass causing cord compression at the level of L1. Total laminectomy was performed on T12 and L1, and the tumor was excised completely without difficulty despite adherence of the tumor to the spinal cord. Postoperatively, the leg pain and motor weakness were much improved. The use of electron microscopy, and the immunohistochemical demonstration of synaptophysin in this tumor, allowed a confident diagnosis of an oncocytic paraganglioma to be made. To the authors' knowledge, this patient represents the first definite case of an oncocytic paraganglioma of the cauda equina in a child.- - - - - - - - - - ranking = 0.0095779150932133keywords = spinal (Clic here for more details about this article) |
7/44. Thoracic paraganglioma presenting with spinal cord compression and metastases.We report a case of a 41-year-old man with a primary thoracic spine paraganglioma presenting with spinal cord compression and distant metastasis. Paragangliomas arising within the spinal canal are rare and when they occur are usually found in the intradural, extramedullary compartment and in the lumbosacral region. Thoracic paragangliomas are very rare, with only five prior cases reported in the literature and with distant metastasis seen in only one of these. In this case, the tumor was entirely extradural and produced extensive sclerosis of the surrounding vertebrae. The clinical, radiographic, and pathologic features of this case are detailed and the relevant literature is reviewed.- - - - - - - - - - ranking = 0.21136360376833keywords = spinal canal, spinal, canal (Clic here for more details about this article) |
8/44. 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.- - - - - - - - - - ranking = 0.025947435764973keywords = spinal, canal (Clic here for more details about this article) |
9/44. Multiple familial facial glomus: case report and review of the literature.Facial paraganglioma is an extremely rare tumor that originates from abnormal paraganglionic tissue situated in the intrapetrous facial canal. A review of the English-language literature shows that only 8 cases of facial nerve paraganglioma have been published. In each case the facial glomus presented itself sporadically, completely independent of any other form of paraganglioma. This study reports an intrapetrous facial glomus that occurred in a case of multiple paragangliomas with a hereditary pattern. To our knowledge, this is the first report of such a combination.- - - - - - - - - - ranking = 0.0067916055785467keywords = canal (Clic here for more details about this article) |
10/44. Gangliocytic paraganglioma arising from mature cystic teratoma of the ovary.BACKGROUND: Gangliocytic paraganglioma is a rare neoplasm involving the small intestine, stomach, and spinal cord. Ovarian gangliocytic paraganglioma has not been reported in the medical literature. CASE: A 55-year-old caucasian woman underwent exploratory laparotomy, total abdominal hysterectomy, and bilateral salpingo-oophorectomy for evaluation of a persistent right adnexal mass. Microscopic examination of the right ovary revealed a mature cystic teratoma with a mural nodule consistent with gangliocytic paraganglioma. As there was no evidence of significant pleomorphism or neoplastic infiltration, surgical staging was not performed. CONCLUSION: Gangliocytic paraganglioma may arise from ovarian cystic teratoma. Although most cases of gangliocytic paragangliomas are benign, surgical staging and retroperitoneal lymphadenectomy may be required if histopathology is suggestive of invasive disease or if enlarged lymph nodes are noted.- - - - - - - - - - ranking = 0.0095779150932133keywords = spinal (Clic here for more details about this article) |
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