1/18. 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 = 1keywords = size (Clic here for more details about this article) |
2/18. 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.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
3/18. 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.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
4/18. PET scan assessment of chemotherapy response in metastatic paraganglioma.Paragangliomas are indolent tumors that arise from the chief cells of the paraganglia in the head and neck, mediastinum, and retroperitoneal regions. Less than 10% of paragangliomas metastasize. Paragangliomas are known to regress slowly and usually partially after radiation therapy, which has been attributed to the development of fibrosis within the abundant vascular elements of the tumor. Positron emission tomography (PET) scanning was used to monitor a 33-year-old woman with recurrent paraganglioma of the carotid body with lung and bone metastases before and after chemotherapy with cyclophosphamide, doxorubicin (Adriamycin), and dacarbazine. The patient derived clinical benefit from chemotherapy, with marked improvement of her systemic and respiratory symptoms, improvement of cancer-related anemia, and normalization of chromogranin a levels. A response was demonstrated on PET scan with decreased [18F] fluoro-2-deoxy-d-glucose uptake after chemotherapy, but no significant changes were detected on serial computed tomography (CT) scans. The patient has remained free of disease progression 24 months after chemotherapy completion. It is suggested that metabolic imaging with PET scans is superior to anatomical imaging with CT scans for the monitoring of patients with paragangliomas.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
5/18. Large symptomatic carotid body tumor resection aided by preoperative embolization and mandibular subluxation.Carotid body tumors (CBT) are rare and usually benign neoplasms (60%-90%), originating from the mesoderm and neural ectoderm. In view of the extensive and unrelenting growth of unresected CBT, encasing vital neurovascular structures, and the significant incidence of malignancy (> or = 10%), surgical excision is the standard treatment of choice. Despite progress in CBT imaging and surgical technique, cranial nerve deficit, stroke, and death continue to affect 10% to 40% of patients undergoing curative surgical resection, particularly in large tumors proximal to the skull base. In such cases, CBT shrinkage by preoperative embolization, improved surgical access utilizing mandibular subluxation, and electroencephalographic monitoring combined with meticulous surgical technique may enable curative tumor resection, without prohibitive morbidity. In light of associated disability, preoperative acknowledgment of the ever-present substantial risk of cranial nerve injury cannot be overemphasized. We report on a patient with a large symptomatic CBT treated surgically with the aid of mandibular subluxation and preoperative embolization.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
6/18. Familial paraganglioma.Paragangliomas are unusual tumors that are sometimes familial. We treated a family who exhibited multiple head and neck paragangliomas (HNPGs) and pheochromocytomas. The purpose was to determine the clinical characteristics of paragangliomas with familial history and to define a better standardized proceeding in the management of these tumors. patients diagnosed with head and neck paragangliomas and identified retrospectively through clinical otolaryngology practices were given a medical and family history questionnaire. We studied a family who exhibited familial paragangliomas. This relationship was examined by reviewing the medical records of family members with verified tumors, carrying out neck computed tomography or magnetic resonance imaging on their relatives to look for tumors that had been unrecognized in the past. All patients underwent a complete head and neck examination. The initial evaluation usually included CT and/or MRI. Computed tomography and magnetic resonance imaging contributed additional information about tumor extension. angiography was performed in every patient with carotid body tumor, with one undergoing therapeutic embolization to reduce the tumor size. Eleven tumors were identified in four patients with a familial history. Familial disease was initially determined by pedigree analysis. Four patients with a median age of 31 years (range: 25-42) underwent surgery. Median follow-up was 5 years (range 2-14); carotid angiography provided essential mainstays for the definite diagnosis. All patients underwent successful surgical resection of the tumor after the appropriate preoperative preparation. There were no perioperative deaths or hemiplegia. Three patients had bilaterality carotid body paragangliomas. One patient had three paragangliomas, and two patients had bilateral carotid body paragangliomas associated with pheochromocytoma. Clinically functioning tumors and malignant tumors were not identified, and none of the patients died after surgery. During follow-up, none of the patients developed recurrence or metastatic disease. The carotid body paraganglioma (CBPG) and glomus vagale manifested as asymptomatic neck masses. The clinical pheochromocytomas typically present with uncontrolled hypertension. In conclusion, paragangliomas are rare, with multicentricity being more common in patients with a familial history. In patients with familial paragangliomas, high-resolution computed tomography and magnetic resonance imaging are recommended for early screening and contributed additional information about the tumor extension and definitive treatment. Early surgery is recommended to minimize major risks.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
7/18. Increased urinary dopamine excretion in association with bilateral carotid body tumours-- clinical, biochemical and genetic findings.This report describes a rare case of a patient with increased urinary dopamine excretion in association with bilateral carotid body tumours. Excretion of adrenaline, noradrenaline, metadrenaline, normetadrenaline and 4-hydroxy-3-methoxymandelic acid (HMMA) were within the reference ranges, and an (123)I-meta-iodobenzylguanidine (MIBG) scan showed uptake in the neck masses, with no other abnormal uptake anywhere else in the body. The patient is being managed conservatively as the tumours are not amenable to resection on account of their size and vascularity. There are only four previous case reports of dopamine-secreting tumours of the carotid body described in the literature, all of whom were women. The tumours were unilateral in three cases and bilateral in the fourth case. Familial cases of carotid body tumours have a higher prevalence of bilateral tumours than non-familial cases. Recent reports in the literature have suggested that a significant number of patients with extra-adrenal catecholamine-secreting paragangliomas have a genetic mutation in one of the identified susceptibility genes for catecholamine-secreting tumours, despite having no other affected family members, and a mutation has been found in the succinate dehydrogenase gene for this patient.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
8/18. The current status of chemodectomas.An analysis of the current status of chemodectomas including the report of two additional cases is presented. One patient with a rare malignant intrathoracic tumor failed to survive palliative pneumonectomy. To our knowledge, this represents the seventeenth histologically proved case. The other patient had the more common carotid body tumor which was managed satisfactorily by subadventitial carotid excision. Comments are made regarding the clinical recognition and treatment of chemodectomas in general. Historical, anatomic, physiologic, and pathologic features are emphasized.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
9/18. diagnosis of a carotid body chemodectoma with dynamic radionuclide perfusion scanning.The first case of a carotid body chemodectoma that was diagnosed using dynamic radionuclide perfusion angiography and subsequently excised is presented. The value of this technic in the investigation of pulsatile tumors is emphasized.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
10/18. carotid body tumor and hyperparathyroidism. A case report and review of the literature.carotid body tumor is an uncommon tumor of the head and neck. The coexistence of this entity with primary hyperparathyroidism is even more unusual, with only four cases having been previously recorded. This report describes a patient with a left inferior parathyroid adenoma and a right carotid body tumor. A common neuroectodermal origin is proposed as an explanation for the simultaneous occurrence of these tumors. The fact that this may represent yet another variable expression of the multiple endocrine neoplasia syndromes emphasizes the importance of careful endocrine screening in patients with carotid body tumors.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
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