1/32. KTP laser assisted excision of glomus tympanicum.A 39-year-old female with a two-year history of mild hearing loss and discomfort on air flight descent was found to have a pulsatile mass behind an intact tympanic membrane. A suspected diagnosis of glomus tympanicum was confirmed by computed tomography (CT) scan imaging. The lesion filled the mesotympanum and hypotympanum but the jugular bony plate was intact, confirming the tympanic site of the lesion. This very vascular tumour was exposed by a tympanomeatal flap and the KTP laser used to shrink and coagulate the tumour progressively with minimal haemorrhage and blood loss. Complete excision of the lesion was achieved without the need for bony removal, and with minimal blood loss. The use of the KTP laser to coagulate this vascular lesion allowed safe removal of the tumour and avoided the need for extended facial recess or hypotympanotomy surgery.- - - - - - - - - - ranking = 1keywords = glomus (Clic here for more details about this article) |
2/32. Familial paragangliomas: the emerging impact of molecular genetics on evaluation and management.HYPOTHESIS: Advancements in molecular genetics has direct impact on the evaluation and management of patients and family members with familial paragangliomas (FP). BACKGROUND: Familial paragangliomas. in contrast to sporadic cases, are commonly multiple, bilateral, and present at an earlier age. Familial tumors are inherited in an autosomal dominant pattern with genomic imprinting of the paternal allele. Mapping studies have identified regions on chromosome 11q as harboring the genetic defect responsible for paraganglioma formation. methods: A multigenerational family with five affected females with head and neck paragangliomas underwent clinical and genetic evaluation. Genetic mapping was performed with microsatellite markers froin chromosome 11q13 and q23. Nonaffected individuals were screened for carrying the affected haplotype. In addition, by using dna obtained from an amniotic fluid sample. in utero screening of a fetus was performed. RESULTS: The most common complaints were hearing loss and neck masses that usually manifested by age 25. Genetic mapping identified loci 11q13 and q23 as sites likely responsible for tumorogenesis. Three unaffected family members, including a fetus, were identified as carriers of the affected haplotype. The genetic findings were used to design a screening protocol for family members at risk for developing glomus tumors. CONCLUSIONS: Genetic screening of unaffected family members can identify individuals harboring the mutated allele. Identification of family members at risk for developing FP by molecular genetic techniques may lead to early detection of head and neck paragangliomas and may directly impact morbidity from glomus tumors and their treatment.- - - - - - - - - - ranking = 0.4keywords = glomus (Clic here for more details about this article) |
3/32. paraganglioma as a systemic syndrome: pitfalls and strategies.Tumours of the neuroendocrine system in the head and neck region are mostly paragangliomas of the glomus tympanicum or jugulare, or of the carotid body. The majority of these tumours are benign, and the coexistence of multiple paragangliomas seems to be rare. Pre-operative embolization and surgery are regarded as primary therapy for these tumours. The treatment regimen in any patient depends on age, general health, hearing status and the function of the lower cranial nerves. Several presentations are possible in which paragangliomas occur as systemic disease. 1. Paragangliomas may occur bilaterally, or, in rare cases, in multiple areas. Pre-operative bilateral angiography is of utmost importance. In case of multicentricity, it might be necessary to proceed without, or just with, unilateral surgery for preservation of adjacent structures. In surgery of jugular vein paraganglioma, we usually perform a modified transmastoidal and transcervical approach with preservation of middle-ear structures and the ossicles. As an alternative or supplement to surgery, radiotherapy or definitive embolization may be used in the treatment of paragangliomas. 2. Paragangliomas may occur as multiple endocrine neoplasia (men) syndrome combined with medullary thyroid gland carcinoma, and, facultatively, pheochromocytoma. In these cases, endocrinological examination and magnetic resonance imaging (MRI) of the adrenal region, the thorax and the neck are required for an adequate therapeutic strategy. As men may be inherited, family history should be evaluated. 3. Paragangliomas can became malignant and metastasize. Thus, cervical lymph node metastases or distant metastases may occur. We recommend the removal of all ipsilateral lymph nodes and their histological examination.- - - - - - - - - - ranking = 2.2434952500801keywords = jugulare, glomus (Clic here for more details about this article) |
4/32. neck mass caused by an intraluminal jugular paraganglioma.Paragangliomas can develop in a number of head and neck sites, the most common being the carotid body paragangliomas and glomus jugulare tumours. This is a case of a paraganglioma confined entirely within the lumen of the jugular vein.- - - - - - - - - - ranking = 13.467010765117keywords = glomus jugulare, glomus jugulare tumour, jugulare, jugulare tumour, glomus (Clic here for more details about this article) |
5/32. 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 = 8.1739810003204keywords = jugulare (Clic here for more details about this article) |
6/32. 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.- - - - - - - - - - ranking = 0.2keywords = glomus (Clic here for more details about this article) |
7/32. Treatment of progressive metastatic glomus jugulare tumor (paraganglioma) with gemcitabine.Paragangliomas are rare tumors of the paraganglia composed of specialized neural crest cells arising in association with sympathetic ganglia. Here we report a case of progressive, metastatic paraganglioma (glomus jugulare tumor) responsive to single agent gemcitabine. In addition, a brief review of chemotherapy for paraganglioma follows the case presentation.- - - - - - - - - - ranking = 37.748066433458keywords = glomus jugulare, jugulare, glomus (Clic here for more details about this article) |
8/32. A case of primitive floor of the mouth paraganglioma in a child: an embryological theory unifying viscerocranium appended paragangliomas.BACKGROUND: Paragangliomas are unusual tumors in the head and neck originating from the paraganglia or glomus cells of neural crest origin. methods: We describe the first case of a primitive paraganglioma of the floor of the mouth presenting in childhood. RESULTS: Complete surgical removal was performed after embolization of the left lingual artery. There was no evidence of either persistent or recurrent disease 5 years after surgery. The embryologic and anatomic origins of head and neck paragangliomas are reviewed. CONCLUSIONS: An embryologic theory based on the common neural crest origin and migration pathways of both autonomic viscerocranium appended ganglias and paragangliomas is proposed that unifies the topographically heterogeneous group of viscerocranium-appended paragangliomas.- - - - - - - - - - ranking = 0.2keywords = glomus (Clic here for more details about this article) |
9/32. Clinical report on the L95P mutation in a Dutch family with paraganglioma.OBJECTIVE: To describe the new L95P mutation of the paraganglioma 1 gene for glomus tumors in a Dutch paraganglioma 1 family with six affected family members and to report the clinical findings and results of treatment in nine glomus tumors with a maximum follow-up of 34 years. SETTING: Tertiary referral centers. RESULTS: mutation analysis of the SDHD gene of paraganglioma 1 showed the L95P mutation in six affected family members and two nonaffected carriers protected from becoming affected by genomic imprinting. In six affected family members, nine glomus tumors (five glomus caroticum tumors, two glomus vagale tumors, and two glomus jugulare tumors) were traced. The ages at presentation varied from 25 to 61 years. In two of six affected family members with a total of four tumors, all the tumors were traced in the extended family study, using magnetic resonance imaging; at that time these tumors were silent. After radiotherapy in one patient at the age of 34 years, a T4 planocellular carcinoma of the tongue occurred within the previous radiation field 27 years later, when the patient was 61 years old. Volume measurements of three untreated glomus tumors (two glomus vagale tumors, one glomus caroticum tumor) during 25 months showed an increase in two tumors (left glomus caroticum, left glomus vagale tumor) and a decrease in one tumor (right glomus vagale tumor). Surgery to remove two bilateral and one unilateral glomus caroticum tumors was successful. A wait-and-see policy is being applied to two glomus vagale tumors. CONCLUSIONS: In family members of paraganglioma 1 patients, mutation analysis can be used to make an early diagnosis of glomus tumors. radiotherapy may have induced a carcinoma. Modalities of treatment can include a wait-and-see policy. Long-term follow-up studies on the natural course of glomus tumors are needed to improve decisions about treatment modalities.- - - - - - - - - - ranking = 10.549613286692keywords = glomus jugulare, jugulare, glomus (Clic here for more details about this article) |
10/32. 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 = 60.396906293533keywords = glomus jugulare, jugulare, glomus (Clic here for more details about this article) |
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