Cases reported "Carotid Body Tumor"

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1/9. 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.
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2/9. carotid body tumor associated with differentiated thyroid carcinoma.

    We report a case of a carotid body tumor associated with a primary differentiated thyroid carcinoma. A 44-year-old woman presented with a 10-year history of an asymptomatic mass in her neck. physical examination revealed a pulsatile submandibular mass in her right neck as well as multiple nodules in the thyroid. magnetic resonance imaging, computed tomography and, in particular, angiography were diagnostic of the carotid body tumor. Slight changes in serum thyroglobulin levels and thyroid scintigraphy led us to suspect thyroid carcinoma. Embolization of the arteries feeding the carotid body tumor was performed, and was followed by tumor resection 24 h later. At surgery, histopathology confirmed the presence of follicular and papillary carcinomas of the thyroid, resulting in concurrent resection of the gland. There were no residual cranial nerve deficits. The patient subsequently received radiotherapy. diagnosis and surgical management are discussed, together with pathogenetic factors.
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3/9. Prediction of malignant behavior of pheochromocytomas and paragangliomas using immunohistochemical techniques.

    Pheochromocytomas and paragangliomas arise from the adrenal glands and extraadrenal paraganglia, respectively. Malignant behavior of these tumors is uncommon and is, in part, dependent on their sites of origin, such as extraadrenal location. Morphologic criteria for malignancy of pheochromocytoma and paragangliomas have not been clearly defined. In this study, to clarify the histologic features that distinguish the benign from malignant pheochromocytomas and paragangliomas, we examined metastatic and nonmetastatic tumors using immunohistochemical techniques. A total of eight cases, five pheochromocytomas from the adrenal glands (four benign and one malignant tumor) and three paragangliomas with invasion or metastasis, were studied. The markers used in this study were chromogranin a, synaptophysin, NCAM (CD56), SNAP25, neuron-specific enolase, S-100 protein, and MIB-1. Our results suggest that MIB-1 immunostaining is a useful adjunct marker to predict malignant behavior in these tumors.
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4/9. Malignant paraganglioma with vertebral metastasis: case report.

    A paraganglioma is a rare tumor, composed of chromaffin cells, groups of cells associated to the autonomous system. When the tumor occurs in the adrenal gland, it is called pheochromocitoma. The malignant paraganglioma is a very rare presentation; it is diagnosed by local recurrence after total resection of the primary mass, or findings of distant metastases. We present a case report of a 29-year-old woman with cervico-brachial pain. In 1995 she underwent a carotid body tumor resection. magnetic resonance imaging (MRI), plain x-rays and computerized tomography scan revealed multiple lesions in C5, T5 and T12. She underwent a surgical procedure to correct the cervical lesion. The histological and immunohistochemical assays revealed a malignant paraganglioma. She received adjuvant radiotherapy, showing clinical improvement after treatment, presenting no symptoms after one year. The therapeutic approach is based on the total resection of the tumor. The treatment of distant metastases can be made with adjuvant measures such as conventional radiotherapy, I 131-MIBG, or chemotherapy, especially in malignant pheochromocitomas.
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5/9. hypotension in a woman with a metastatic dopamine-secreting carotid body tumor.

    OBJECTIVE: To describe a woman with metastatic carotid body tumor in whom hypotension occurred in the setting of exceedingly high plasma dopamine levels. methods: We present a case report and review the literature on the topic of dopamine-secreting paraganglioma or pheochromocytoma. RESULTS: A previously healthy 40-year-old Asian woman noted difficulty with swallowing and hoarseness. No neck mass was visible, and she had no symptoms of catecholamine excess and no family history of endocrine disorders or malignant disease. Indirect laryngoscopy revealed a paralyzed left vocal cord and a nonulcerating mass in the left parapharyngeal space. An initial needle biopsy was interpreted as undifferentiated carcinoma. After a second biopsy, this mass was diagnosed as a neuroendocrine tumor, consistent with paraganglioma. The patient underwent surgical resection and radiation therapy (total dose, 40 Gy), after which she remained asymptomatic for 11 years. Then loss of weight, fatigue, nausea, and hypotensive episodes (blood pressures as low as 70/35 mm Hg) prompted whole-body imaging with bone scans, computed tomography, and magnetic resonance imaging, which disclosed several lesions in the liver, lungs, and spine, suggestive of metastatic disease. The adrenal glands were unremarkable. A metaiodobenzylguanidine scan with use of (131)I was negative. liver biopsy of a hypodense lesion revealed a neuroendocrine tumor by histologic and immunohistochemical studies. Because of the patient's history, malignant paraganglioma was diagnosed. The tumor secreted predominantly dopamine at extraordinary levels (plasma concentration 27,942 pg/mL; normal, <30). The patient died before further treatment could be initiated. CONCLUSION: Carotid body tumors usually do not secrete catecholamines but frequently metastasize. During progression, these neuroendocrine tumors may become able to produce and secrete selected catecholamines such as dopamine. dopamine can lower the blood pressure rather than causing hypertension, even though hypertension is one of the main symptoms of a pheochromocytoma.
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6/9. Accessory thyroid gland at carotid bifurcation presenting as a carotid body tumor: case report and review of the literature.

    patients with carotid body tumors referred to vascular surgeons usually undergo magnetic resonance imaging (MRI) as part of the workup. We present a case report of a 39-year-old woman with a presumed carotid body tumor, as was expected from clinical and MRI findings. At surgery, the ectopic thyroid tissue was suspected, and resection was performed. Histologic examination showed normal thyroid tissue with no sign of malignancy. Postoperative thyroid analysis showed a normally located, properly functioning thyroid gland. Ectopic thyroid glands are generally found in the midline, as a result of abnormal median migration. Their presence lateral to the midline with a proper functioning thyroid gland in its normal position is extremely rare. Although several submandibular thyroid glands have been reported, a close relation with the carotid arteries was described only once. When MRI scans of a presumed carotid body tumor show tumor characteristics that are not fully specific for a carotid body tumor, the possibility of ectopic thyroid tissue should be entertained, which can be the patient's only properly functioning thyroid tissue. In such cases, additional assessment, including thyroid tests, should be considered before surgery.
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7/9. Unusual presentation of tuberculosis of the head and neck region. Report of three cases.

    Three cases are reported in which the diagnosis of tuberculosis has only been made following surgery for suspected salivary gland tumor, sebaceous cyst and carotid body tumour. Surgery could have been avoided if tuberculosis had been considered in the initial differential diagnosis.
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8/9. Multiple chemodectomas. carotid body tumor masked by salivary gland uptake on I-123 MIBG scintigraphy.

    Chemodectomas resemble pheochromocytomas in that they are derived from neural crest tissue. Their distribution in the body may be quite different, however, as chemodectomas are more frequently found in the aortic and carotid bodies, while pheochromocytomas are most common in the adrenal medulla. The authors present a case of a patient with a known history of chemodectoma, imaged with I-123 MIBG to rule out recurrent disease. Images of the chest revealed a thoracic tumor representing recurrent periaortic tumor, but an additional carotid body tumor (which was later demonstrated by angiography) was masked by salivary gland uptake. In patients with chemodectomas, SPECT imaging of the neck may be necessary to distinguish normal salivary gland uptake from tumoral uptake of MIBG. In general, knowledge of the characteristics of the individual neural crest derived tumor is mandatory for maximal effectiveness of I-123 MIBG scintigraphy.
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9/9. FDG PET imaging of paragangliomas of the neck: comparison with MIBG SPET.

    Two patients with cervical paragangliomas underwent positron emission tomography (PET) with 2-[18F]-fluoro-2-deoxy-D-glucose (FDG). There was marked tumor uptake and retention of FDG. Adjacent salivary gland accumulation of FDG was minimal, though quite prominent with meta-iodobenzylguanidine. FDG PET offers another potentially useful approach to functional imaging of these uncommon tumors, independent of the presence of specific amine uptake mechanisms or cell surface receptors required by other scintigraphic techniques.
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