1/97. Thoracic involvement with pheochromocytoma: a review.Pulmonary manifestations of pheochromocytoma are infrequent and are not well documented. A medline search in the English language revealed no cases of endobronchial involvement from a pheochromocytoma. We report a case of endobronchial metastases in a 37-year-old woman known to have a recurrent extra-adrenal pheochromocytoma. She presented with symptoms of wheezing and a nonproductive cough for 8 months and was being treated for asthma. A flexible bronchoscopy with endobronchial biopsy established the diagnosis. The patient underwent a Nd-YAG laser photoresection (LPR) to ablate the tumor, which was followed by placement of a Wallstent (Pfizer Medical technology Group; Rutherford, NJ). She remains well 18 months later, having required multiple palliative LPRs. To our knowledge, this is the first reported case of endobronchial pheochromocytoma. The pulmonary manifestations of this rare disease and their management are reviewed.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
2/97. 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 = 0.11024475097656keywords = gland (Clic here for more details about this article) |
3/97. 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 = 0.11024475097656keywords = gland (Clic here for more details about this article) |
4/97. 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 = 6.7097995055454keywords = adrenal gland, adrenal, gland (Clic here for more details about this article) |
5/97. Sustentacular cells in sporadic paraganglioma-like medullary thyroid carcinoma: report of a case with diagnostic and histogenetic considerations.Sustentacular cells (SCs) are glial supporting cells of the fetal, adult normal, and neoplastic extra and adrenal human chromaffin cell lineage. SCs have also been identified in some cases of medullary thyroid carcinoma (MTC), raising both diagnostic and histogenetic problems. We report a rare case of a sporadic paraganglioma-like variant of MTC showing numerous S-100 protein and glial fibrillar acid protein (GFAP) positive SCs encircling neoplastic cells in a nesting (Zellballen) pattern similar to that observed in paragangliomas or pheochromocytomas. Although stromal amyloid deposits were only focally detected, diagnosis was immunohistochemically confirmed by immunoreactivity of the neoplastic cells for cytokeratin, CEA, calcitonin, chromogranin a, neuron-specific-enolase, and synapthopysin. As for the histogenesis of SCs, if we assume that MTC is a neural crest-derived tumor, it is likely that these cells reflect the ability of the common precursor cell to differentiate towards a sustentacular-type glial cell lineage in addition to the typical neuroendocrine one. This viewpoint is supported by the evidence that rare cases of MTC may contain neoplastic or supporting cells showing a multidirectional differentiation (usually neuroendocrine and melanocytic) that recapitulates the different cell lineages arising from the developing neural crests.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
6/97. 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 = 1.1102447509766keywords = adrenal, gland (Clic here for more details about this article) |
7/97. paraganglioma of the pancreas: literature review and case report.Extraadrenal paragangliomas are very rare tumors arising from cells derived from the neural crest. These tumors are encountered only as case reports, and as a result, little is known of their natural history. We present a case of pancreatic paraganglioma and review all previously reported cases.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
8/97. Extraadrenal retroperitoneal paraganglioma causing malignant hypertension and permanent visual loss in an eleven-year-old girl.We report a secretory paraganglioma in an eleven-year-old Trinidadian girl of East Indian descent who presented with malignant hypertension, proteinuria and modest elevation of urinary vanillylmandelic acid levels. The extra-adrenal mass was surgically removed without complications. The patient has been normotensive without evidence of recurrence of disease but with persistent visual sequel two years later. This case reinforces the observation that extraadrenal retroperitoneal paragangliomas can be functionally very active and early computed tomography or other imaging procedure is necessary for rapid noninvasive evaluation, timely surgery and prevention of permanent morbidity.- - - - - - - - - - ranking = 6keywords = adrenal (Clic here for more details about this article) |
9/97. Successful treatment with paclitaxel of a patient with metastatic extra-adrenal pheochromocytoma (paraganglioma). A case report and review of the literature.This case report describes the history of a patient with an aggressive course of a metastatic extra-adrenal pheochromocytoma (paraganglioma) who received different combination chemotherapy regimens with no or short-lasting clinical benefit. However, during treatment with single-agent paclitaxel, there was a significant clinical improvement, a partial biochemical response and a minor roentgenologic response, which was sustained for 1 year. In this report we present this case and also review the literature on the chemotherapy used for this rare disease over the past 15 years. To enable the activity of paclitaxel against this neoplasm to be determined, more patients need to be treated.- - - - - - - - - - ranking = 5keywords = adrenal (Clic here for more details about this article) |
10/97. paraganglioma of the tongue.Paragangliomas are neuroendocrine tumors arising from extra-adrenal autonomic ganglia. We present what is to our knowledge the first immunohistochemically documented case of a paraganglioma of the tongue, a 2.5-cm benign tumor growing in the position of the foramen cecum. The patient was an elderly woman who presented with throat irritation. The histologic profile and ultrastructural appearance of this lesion were classic for paraganglioma, and cells stained strongly for neurosecretory granules by immunohistochemistry. Carcinoid, ectopic thyroid neoplasm, and other tumors that mimic paraganglioma were ruled out on immunohistochemical grounds. To our knowledge, paraganglia have not been previously documented in the tongue; this tumor may have arisen from a branch of cranial nerve VII or IX, or from an embryologic remnant of the thyroid or thyroid capsule.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
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