Cases reported "Carotid Body Tumor"

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1/57. Removal of catecholamine-secreting chemodectoma. The use of neuroleptanaesthesia, adrenergic blockade and sodium nitroprusside.

    A case of catecholamine-secreting chemodectoma of the neck in a 47-year-old male who also had temporal lobe epilepsy is described. Details of presentation, diagnosis and successful treatment are given. He was fully alpha blocked with phenoxybenzamine and given neuroleptic drugs but his blood pressure rose to dangerous levels when the tumour was handled and did not respond to intravenous phentolamine. sodium nitroprusside was successfully used to achieve blood pressure control.
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2/57. Diagnostic and therapeutic approaches to carotid body tumours: report of three cases and review of the literature.

    BACKGROUND: Carotid body tumour is a rare neoplasm of the carotid body. Three cases of carotid body tumour presenting as a painless progressive mass in the neck region are reported here. A review of the relevant literature regarding carotid body tumours is also presented. methods: Angiographic features were diagnostic of carotid body tumour and complete surgical excision was done. RESULTS: There was no mortality and minimum morbidity. There were no malignant tumours. All three patients belong to the high-altitude area of Himachal Pradesh. CONCLUSIONS: A high degree of clinical suspicion of upper posterior triangle neck masses and an accurate diagnostic work-up are needed for operative planning.
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3/57. Management of incidentally discovered cervical paragangliomas: report of two cases and review of current issues.

    Paragangliomas of the head and neck are uncommon neoplasms arising from the extra-adrenal paraganglia and include carotid body and glomus vagale tumors. These lesions may be discovered incidentally by imaging studies performed to evaluate carotid atherosclerotic occlusive disease. Incidental paragangliomas of the head and neck may be smaller than those discovered due to symptoms. Although surgical resection remains the definitive treatment for head and neck paragangliomas, important issues of management arise when such lesions are discovered. Two recent cases are reported. epidemiology, pathophysiology, diagnostic evaluation, and issues of management of head and neck paragangliomas are discussed.
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4/57. 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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5/57. Catecholamine-secreting carotid body tumor and intracranial aneurysm: coincidence?

    BACKGROUND: An extremely rare case of intracranial aneurysm associated with catecholamine-secreting carotid body tumor is presented. CASE DESCRIPTION: A 64-year-old woman suffering from hypertensive attacks was admitted first to the otolaryngology Department with a neck swelling. Right common carotid angiography revealed a hypervascular mass at the carotid bifurcation. On the same angiogram a middle cerebral artery aneurysm was discovered incidentally and the patient was referred to the Neurosurgical Department. Because of her history the tumor was considered to be endocrinologically active and the patient underwent alpha- and beta-blockade to protect intraoperative cardiovascular instability. Despite all precautions, during the operation hypertensive crises developed and the aneurysm was clipped with difficulty. CONCLUSION: Perioperative management designed to avoid complications in treating this rare association is discussed. Although this is the first reported case of an intracranial aneurysm associated with a functional carotid body tumor, a possible etiopathogenesis of the relationship between the aneurysm and hypertensive attacks due to an acute catecholamine-discharging tumor is presented.
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6/57. Diagnostic challenges in the fine-needle aspiration diagnosis of carotid body paragangliomas: report of two cases.

    Two cases of carotid body paragangliomas sampled by fine-needle aspiration (FNA) cytology prior to other medical diagnostic studies are presented. In the first case, the presence of an ipsilateral ulcerative lesion of the nasopharynx along with pronounced atypia of the specimen posed a challenge to the correct cytologic interpretation, which was initially sidetracked in favor of a metastatic epithelial lesion. In the second case, a tumor mass of unusually large size and extension which included the pharynx, coupled with a large amount of profusely hemorrhagic aspirate, presented a diagnostic problem, which was overcome by processing part of the specimen as a cell block, which by its histologic and immunochemical features provided a definitive pathologic diagnosis. In handling these two clinically complex cases of carotid paraganglioma, two learning principles became clear on how to reach a correct FNA diagnosis in such lesions: 1) The anatomic location of the lateral neck mass with its prolonged history, along with a hemorrhagic FNA specimen exhibiting at least some cytologic features reminiscent of endocrine neoplasm, are among the factors that help in arriving at a suggestive diagnosis of paraganglioma, when other clinical features tend to sidetrack from interpretation of the cytologic changes. 2) In the practice of FNA cytology, if the possibility of paraganglioma arises, processing part of the specimen as a cell block with accompanying histology and immunohistochemistry can provide a definitive diagnosis of such lesion.
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7/57. Cervical sympathetic chain schwannomas masquerading as carotid body tumors.

    Cervical sympathetic chain (CSC) schwannoma is a rare neurogenic tumor that can mimic a carotid body tumor (CBT). Two male patients aged 33 and 49 years old were referred for a cervical mass thought to be a CBT. Both patients were found to have an asymptomatic, pulsatile, nontender mass located at the level of the angle of the mandible. Both patients were neurologically asymptomatic and cranial nerves were normal. Both patients underwent neck exploration and resection of the mass involving the CSC. Postoperatively, a Horner's syndrome was present. Histopathology confirmed both tumors to be schwannomas of the CSC. At follow-up, both patients are asymptomatic with no recurrence. The main imaging criterion to differentiate a CBT from a CSC schwannoma is the lack of hypervascularity of the latter. Malignant transformation is extremely rare. Tumor excision gives excellent results with no local recurrence. Horner's syndrome is an expected postoperative complication.
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8/57. Malignant carotid body tumor: a case report.

    Carotid body tumors (CBTs) have an unpredictable history with no correlation between histology and clinical behavior. Of reported cases since 1891, local and distant metastases appear in approximately 10% of cases and remain the hallmark of malignancy. Currently, there are not enough data to support a single treatment regimen for malignant CBTs. The reported case demonstrates some unanswered issues with regard to malignant CBTs to include lymph node dissection, the need for carotid resection, and the role of radiation therapy. A 46-year-old pathologist underwent a resection of a Shamblin I CBT, to include jugular lymph node sampling, without complication. There was lymph node involvement, and tumor cells were found on the margins of the pathologic specimen. Subsequent carotid resection with reversed interposition saphenous vein graft and modified neck dissection were performed again without complication. Follow-up at 4 years has been uneventful. diagnosis of CBTs with the use of magnetic resonance angiography, magnetic resonance imaging, color flow duplex scanning, and the role of arteriography are reviewed. The current treatment options are discussed with reference to primary lymph node sampling, carotid resection, and neck dissection in malignant cases. This case demonstrates that the unpredictable nature of CBTs and their malignant potential warrant aggressive initial local treatment to include jugular lymph node sampling and complete tumor resection.
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9/57. Schwannoma with angiosarcoma. Report of a case and comparison with other types of nerve tumors with angiosarcoma.

    BACKGROUND: Schwannoma with angiosarcomatous change is a rare tumor, the clinical characteristics of which have not been analyzed. methods: A patient with schwannoma with angiosarcoma arising in the midneck and clinically mimicking a carotid body paraganglioma is described with a literature review of all previously reported cases and a comparison of their clinical features with those of schwannoma with conventional malignant transformation and cases of neurofibroma and malignant peripheral nerve sheath tumor (MPNST) with angiosarcoma. RESULTS: There are four reported cases, including the present case. Schwannoma with angiosarcoma affects older adults, mainly men. Three tumors arose from the vagus nerve in the neck. Three of the four angiosarcomas were epithelioid in type. Treatment in all cases was surgical resection followed by radiation and chemotherapy in one case and by radiation alone in another. One patient died with residual local angiosarcoma 5 months after the diagnosis. The remaining three patients were alive and disease free at 27 months, 43 months, and 90 months, with distant metastasis (after 15 months) reported only in the patient described in this case report. CONCLUSIONS: Schwannoma with angiosarcoma should be included in the differential diagnosis of presumed carotid body paragangliomas. Like angiosarcoma alone and schwannoma with conventional malignant transformation, but unlike cases of neurofibroma and MPNST with angiosarcoma, the patients are older adults, and there is a male prevalence. Schwannoma with angiosarcoma is capable of local spread with a fatal outcome and of distant metastasis, but follow-up strongly suggests that these patients have a better prognosis than patients with neurofibroma or MPNST with angiosarcoma. Recommended treatment is attempted complete surgical resection followed by radiation therapy and chemotherapy, if it can be tolerated by the patient.
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10/57. Malignant carotid body tumors: report of three cases.

    The aim of the current report was to describe 3 cases of malignant carotid body tumor (CBT) and to review the literature. My clinical records of 87 CBTs in 81 patients (6 bilateral) were reviewed, 79 of which were operated on. Three malignant cases were found. The first was in a 40-year-old man who presented with pulmonary metastases 6 years after resection of a CBT. He was treated with chemotherapy and interferon, but died with disseminated disease 2 years later. The second case was in a 56-year-old woman who had a 5-cm, fixed, hard mass in the upper aspect of the neck and a paralysis of the left vocal cord. This lesion was completely resected, and a shunt and reconstruction with a saphenous vein graft were performed. pathology revealed a malignant chemodectoma with invasion to 2 of the 5 lymph nodes removed. radiotherapy (50 Gy) was given after the operation. She is well and free of disease 68 months after the resection. The third case was in a 61-year-old woman who presented with an 8-cm nontender, hard, immobile mass in the left upper neck that displaced the left wall of the oropharynx toward the midline. A carotid arteriogram showed a CBT. On computed tomography, the tumor extended to the infratemporal fossa with no bone involvement. The lesion was embolized with a 40% reduction in vascularity. At surgical exploration, the tumor involved the sternocleidomastoid muscle and the lymph nodes at levels II and III, and the internal carotid artery could not be dissected free at the skull base, so only a partial resection was performed. This patient was lost to follow-up. These 3 cases are in agreement with the literature. Locoregional control is usually obtained with complete primary tumor resection and lymphadenectomy and eventual radiotherapy. Surgery with radiotherapy seems to be effective for isolated metastases. Current multidisciplinary treatments have been unsuccessful in controlling disseminated disease.
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