Chemodectomas (nonchromaffin paragangliomas) of the glomus jugulare, glomus vagale and carotid body.
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In summary, MR imaging characteristics of a case of paraganglioma of the facial nerve are reported. The relationship of paragangliomas and the chromaffin system have been discussed. There are many reports of cases of synchronous paragangliomas and pheochromocytomas. These reports, along with simultaneous involvement in familial MEN syndromes, and the common embrylogic origin (neural crest) and similar histopathologic relationships between paragangliomas and pheochromocytoma, all support the fact that they are part of the chromaffin system.
Glomus jugulare tumors have the ability to synthesize, store, and secrete biogenic amines. Although the majority of these tumors remain endocrinologically silent, on rare occasions they present either as a pheochromocytoma or with a carcinoid syndrome. We report a 20-year-old male with two intracranial tumors: an intrasellar neoplasm and a glomus jugulare tumor. Catecholamine catabolites in the urine were not increased, and blood pressure was always normal. The pituitary tumor was an adenoma, immunostaining positive for prolactin. The second patient, a 29-year-old hypertensive male, with a glomus jugulare tumor, had increased vanillylmandelic-acid excretion. In both cases, the paraganglioma tumor cells contained numerous dense-core vesicles (125 to 380 nm in diameter) in electron microscopy, and showed intense fluorescence by the sucrose-potassium phosphate-glyoxylic acid method. Using high-performance liquid chromatography and microspectrofluorometry we were able to establish the presence of large amounts of dopamine in the cytoplasm of the tumor cells.
OBJECTIVES: Meningiomas involving the jugular foramen are rare lesions, with approximately 34 cases reported in the English literature. Clinically, these tumors mimic the more common glomus jugulare tumor. After surgical resection, meningiomas have worse cranial nerve outcomes and higher recurrence rates than glomus tumors. There is controversy regarding the selection of surgical approach, particularly with regard to management of the facial nerve. A reliable means of accurate preoperative diagnosis would help surgical planning and patient counseling. STUDY DESIGN: We present a series of six consecutive large jugular foramen meningiomas resected by a single surgeon from 1996 to 2002. METHODS: Retrospective case series (chart review). RESULTS: The preoperative diagnosis was correct in nearly all (6/7) cases on the basis of the characteristic imaging findings seen with a combination of temporal bone dedicated computed tomography and high-resolution enhanced magnetic resonance imaging. Five of six patients had lateral facial nerve rerouting (infratemporal fossa Fisch type A), and three patients also had posterior rerouting (transcochlear approach with division of greater superficial petrosal nerve). Total tumor removal was accomplished in 83%. One patient suffered recurrence over a mean follow-up period of 2.5 years. New postoperative vocal cord palsies occurred in 50% of patients, and all required vocal cord medialization. At 1 year, 50% of patients had normal or near normal (House-Brackmann I or II) facial function. Postoperative cerebrospinal fluid leaks occurred in two patients, and both eventually required ventriculoperitoneal shunts. CONCLUSIONS: Meningiomas involving the jugular foramen are surgically challenging tumors that pose unique diagnostic issues. The majority of these lesions can be completely resected. A relatively high postoperative complication rate, which can be controlled with appropriate intervention, is seen with these surgeries.
Since 1954, 39 cases of glomus jugulare or vagale tumor have been treated by the authors with radiotherapy. The initial results with orthovoltage (280 kV) therapy were unpredictable, but 30 patients with 32 tumors treated with megavoltage radiotherapy (Cobalt-60 [60Co] or linear accelerator) have shown either no evidence of recurrence or complications, with one exception, or died of causes unrelated to their glomus tumor. Megavoltage radiotherapy is recommended for primary treatment of all glomus tumors that present with evidence of bone invasion or nerve involvement.
Two cases of metastatic carcinoma of the temporal bone, that simulated glomus tumours on thorough preoperative evaluation are described. Although rare, metastatic spread to this area is recognized, but presentation in this way is unique.
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Previous reports of endocrine activity of glomus jugulare tumors have been of pheochromocytomas but not carcinoid apudomas. This paper is to alert the otolaryngologist to the presence of carcinoid apudomas in glomus jugulare tumors. A patient with a long history of explosive diarrhea, facial flushing, and violent headaches, the symptom of carcinoid syndrome, was cured after removal of his large glomus jugulare tumor. The diagnosis of carcinoid syndrome is made with a good history and confirmed with a 24 hour urine collection for 5 Hydroxy indole acetic acid (5 Hiaa). a series of patients treated at the Johns Hopkins Hospital for glomus jugulare tumors is reviewed for the presence of endocrine activity and the literature on endocrine active glomus jugulare tumors is presented. Preoperative evaluation for the presence of both pheochromocytoma and carcinoid apudoma is recommended for all patients with glomus jugulare tumors.
The endaural approach has its in excision surgery of certain forms of jugular glomus tumor. --In the pure, tympanic form, a minimal or extended endaural approach is perfectly suitable. -- In tympano-mastoid forms, without involvement or with minimal involvement of the jugular foramen, a prolonged endaural approach may be used for wider operations involving the whole middle ear and mastoid around a bony aqueduct or an exposed VII. --Nevertheless, in the presence of tympano-jugular or tympano-masto-jugular forms, with marked involvement of jugular foramen, the greatest caution is necessary. It would appear that the endaural route may still be suggested but only for certain tumors, with the express reserve that angiographic studies defining its site and extent are favourable. By virtue of more and more highly developed techniques of exploration (scanner with contrast, films in subtraction) which may be used for measurement and precise localization of the tumor, indications for the type of approach may now be defined with even more precision. --It should be noted that if there is the slightest doubt, the slightest reserve, or the slightest fear that an endaural approach might be inadequate to deal with the situation, it should be definitely avoided with preference for a cervico-retro-auricular or cervicopre and retro-auricular approach, or radiotherapy only, or even abstenstion from operation, with observation. It is only if such rules of good sense and caution are respected that minimal, extended, prolonged endaural approaches will retain a worthy place in the surgical treatment of jugular glomus tumors.
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Glomus jugulare tumors may be bilateral or more commonly associated with a glomus tumor in another location. These tumors can also have a familial distribution which appears to be autosomal dominant. In this paper, two brothers are presented with bilateral glomus jugulare tumors. Such occurrence appears to be a unique familial manifestation.
We present two clinical cases of jugular glomus in which different kinds of treatment have been adopted. In literature we can find a review of the utility and morbidity of the different methods of exploration as well as the results obtained by the different authors according to the kind of treatment. Nevertheless we don't come to definite conclusions due to the fact that the classifications and curing criteria very in the different series.
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Glomus jugulare tumours with metastases are rare as the diagnosis of metastases in this condition can be difficult. In the past this diagnosis has been made on histology of the metastatic lesions which were identified on clinical grounds. This is one of the reasons why there have been only 20 reported cases in the literature up to 1990 (Johnstone et al., 1990). This case report examines the role of meta-iodobenzylguanidine (MIBG) scintigraphy in the diagnosis of metastases. MIBG scintigraphy is an injected radionucleotide isotope scanning technique which is cheap, non-invasive, sensitive as well as being a specific investigation for identifying sites of ectopic neuroendocrine tissue. It may also have a role in the treatment of these tumours.
Neurinomas or schwannomas of the jugular foramen are rare tumors whose differentiation from tumors of the jugular body may be difficult because they share common symptoms. Since schwannomas at this site are less aggressive than tumors of the jugular body and certain elements of their treatment differ, it is important to make a correct diagnosis promptly. A well-documented case of schwannoma of the jugular foramen is reported and its differential diagnosis is discussed in the light of the literature.