1/24. Visualisation of a paraganglioma by technetium-99m-sestamibi scintigraphy.A 68-yr-old woman presented to our observation with multinodular goiter and a contiguous right laterocervical mass. In spite of ultrasound, technetium and iodine scan, CT and fine-needle biopsy, the precise origin of the mass remained uncertain. On additional multi-phase sestamibi scan, the neck region showed an early high uptake rapidly decreasing over time in the laterocervical mass, and a persistent inhomogeneous distribution in the thyroid gland. This behavior suggested that the laterocervical mass could derive from an anatomical structure other than the thyroid. Surgical exploration established the extrathyroid nature of the laterocervical mass and the histological examination confirmed that it was a typical paraganglioma. This finding is in keeping with a recent report of positive sestamibi uptake in a cervical paraganglioma, although our case showed a more rapid kinetic. This tumor should be therefore taken into consideration in the differential interpretation of focal sestamibi uptake.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/24. A new observation of the Carney's triad with long follow-Up period and additional tumors.The etiology of the Carney's triad (gastrointestinal stromal tumors, pulmonary chondromas, and paragangliomas) is unknown, and only 57 cases have been reported since its identification in 1977. We report the clinical course of a female with the complete triad and some additional tumors. Bilateral vagal paragangliomas were treated surgically and with radiotherapy between the ages of 24 and 26 years. Subsequently she underwent surgery for a gastric leiomyosarcoma (27 years), a pleomorphic adenoma of the parotid gland (49 years) and a multifocal breast cancer with axillary spread (50 years). A calcified lesion was also noticed in the left lung, the radiologic diagnosis of which was consistent with chondroma. A mediastinal paraganglioma, detected at 56 years on a control X-ray of the chest, was partially excised at 63 years. At the last control, performed at 66 years, the patient was alive with residual cervical and mediastinal paraganglioma. Her younger brother was affected by Hirschsprung's disease and died at 54 years of rectal cancer. Her daughter is 33 and has been suffering since birth with severe constipation. In conclusion, this is one of the longest followed-up patients with Carney's triad. Her case illustrates the need for early recognition of the setting in order to detect the component tumors at a stage when surgery may be curative, and careful and life-long follow-up, both because the multicentricity of the classic components tends to manifest metachronously and because of the tendency to develop other tumors, some of which may be malignant. Furthermore, the presence of Hirschsprung's disease in the patient's family, coupled with the alleged common origin of two component lesions from derivatives of the neural crest, open new avenues for the understanding of this disorder.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/24. Malignant human renin producing paraganglionoma-localization with 123I-MIBG and treatment with 131I-MIBG.A 22-year-old woman presenting severe hypertension and hypokalaemia is described. Initial evaluation showed a large tumour localized at the position of the left adrenal gland. Subsequent surgery temporarily relieved all signs and symptoms caused by the tumour. The symptoms relapsed after a 2-year disease-free interval. At re-evaluation, the tumour was shown to produce an uncontrolled secretion of renin, thus triggering aldosterone-dependent hypertension. This report describes the diagnosis, treatment and clinical course of this unique patient with a malignant paraganglionoma of adrenal origin.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/24. 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 = gland (Clic here for more details about this article) |
5/24. Intracerebral paraganglioma.Paragangliomas of the CNS are relatively rare. Cases of location in the pineal and pituitary glands, cerebellopontine angle, cauda equina and filum terminale are known. In our neurosurgical unit a 42-year-old male patient with a history of vertigo and a generalized seizure underwent an operation for a fronto-temporal tumour. The histological diagnosis was paraganglioma.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
6/24. paraganglioma and adrenal pheochromocytoma presenting simultaneously in an elderly female. Case report and review of the literature.Paragangliomas are chromaffin tissue tumors arising in an extra-adrenal location. It is quite rare to find a paraganglioma concurrently with a pheochromocytoma. We report a patient who underwent resection of a retroperitoneal mass that was characterized pathologically as a malignant paraganglioma. An incidental finding was a microscopic pheochromocytoma in the ipsilateral adrenal gland.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/24. pheochromocytoma and multifocal functioning paraganglioma in a 9-year-old boy with von hippel-lindau disease.We report the case of a 9-year-old boy presenting with multifocal paraganglioma partially involving the right suprarenal gland, abdominal paraganglia, and the left para-aortal retropleural region. von hippel-lindau disease (VHL) was diagnosed exclusively by VHL-gene analysis. Other clinical signs of VHL were lacking. After a complaint-free period of 7 months the boy developed a metachronous paraganglioma adjacent to the opposite adrenal gland. patients with VHL disease should undergo adrenal-sparing surgery.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
8/24. 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 = gland (Clic here for more details about this article) |
9/24. Thyroid paraganglioma.paraganglioma of the thyroid gland is a rare neoplasm. Only eight cases have previously been reported. The neoplasm was confined to the thyroid in all but two patients; laryngeal involvement was seen in one and tracheal involvement in another. A review of these previously reported cases is presented along with the details of an additional case with laryngeal involvement. Laryngeal and thyroid paragangliomas are compared and contrasted. The anatomic and embryologic basis of these tumors and the diagnosis and treatment are discussed. We suggest changing the nomenclature and reporting of these lesions on the basis of their apparent common origin. Surgery remains the treatment of choice in most paragangliomas, including those involving the larynx and thyroid. Although the number of reported cases is small, there have been no cases of distant metastases or local recurrence after adequate surgical removal.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
10/24. Primary paraganglioma of the thyroid gland.We describe the case of a 46-yr-old euthyroid woman, who was submitted to right lobectomy plus isthmusectomy because of a 30 mm large, rapidly growing thyroid nodule. Two cytological examinations of fine needle aspiration biopsy (FNAB) specimens were not diagnostic. histology showed a neoplasm composed of nests of chief cells, almost completely replacing thyroid parenchyma, infiltrating the capsule and surgical resection margins, and invading perithyroid tissues. Immunohistochemical analysis revealed that the tumor stained positively to chromogranin, synaptophysin, NSE, S-100 protein and tyrosine hydroxylase, whereas no immunoreactivity was detected against cytokeratin, thyroglobulin, TTF-1, calcitonin and CEA. A diagnosis of thyroid paraganglioma (PG) was finally made. No complications developed following operation. Laboratory analysis and imaging study excluded multicentric disease, metastases to neck or extracervical organs, and multiple endocrine neoplasia (men). We report this unusual case, underscore its clinical and immunohistochemical features and discuss differential diagnosis.- - - - - - - - - - ranking = 4keywords = gland (Clic here for more details about this article) |
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