1/85. Laryngeal paraganglioma in a five-year-old child--the youngest case ever recorded.A paraganglioma is a neuroendocrine neoplasm that originates from the paraganglion cells of the parasympathetic system. The average age of presentation is in the fifth decade. We report a case of laryngeal paraganglioma in a five-year-old child, the youngest case ever recorded. The features of paraganglioma which differentiate it from other tumours are also discussed.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
2/85. 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 = 2.0177464799184keywords = neuroendocrine, carcinoma (Clic here for more details about this article) |
3/85. 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 = 1.0035492959837keywords = neuroendocrine, carcinoma (Clic here for more details about this article) |
4/85. 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 = neuroendocrine (Clic here for more details about this article) |
5/85. Duodenal gangliocytic paraganglioma as a radiological moving defect.BACKGROUND: Gangliocytic paraganglioma of the duodenum is a peculiar neuroendocrine tumor, often revealed as an incidental radiographic finding or as a result of gastrointestinal hemorrhage, due to frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology, consisting of endocrine, ganglion and spindle-shaped schwann cells. methods: A case of a duodenal gangliocytic paraganglioma is reported in a 50-year-old woman presenting with episodes of melena. Enteroclysis revealed a pedunculated tumor observed either in the second or third portion of the duodenum. Upper gastrointestinal endoscopy revealed a pedunculated ulcerated tumor which was subsequently confirmed by computed tomography. The tumor was simply excised through a duodenotomy. RESULTS: The size of the tumor was 4.5 x 3 x 1.5 cm. Immunohistochemical and ultrastructural features of the tumor classified it as a typical duodenal gangliocytic paraganglioma with positive cellular reaction for neuron-specific enolase, chromogranin, somatostatin, gastrin and S100. CONCLUSION: Forty-six months after surgery, the patient is well with no evidence of recurrence. The majority of the reported duodenal gangliocytic paragangliomas were of benign nature. However, the fact that in 4 isolated cases there was lymph node involvement indicates that thorough investigation for lymph node metastases is needed, as well as thorough postoperative follow-up.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
6/85. Functional imaging as an aid to decision-making in metastatic paraganglioma.Malignant paraganglioma is a rare and slow growing tumour of neuroendocrine origin. At the time of diagnosis, the tumour is usually widespread, with limited therapeutic options. A variety of functional imaging studies are available for staging the disease, guiding therapy and monitoring treatment response. These include 123I-MIBG or 131I-MIBG, 111In-pentetreotide or 111In-lanreotide (somatostatin analogues), and 18F-FDG positron emission tomography. Various radionuclides, including 131I and 90Y, can be targeted to the tumour using MIBG or pentetreotide. Such targeted radionuclide therapy may provide valuable long-term palliation in such patients. We present two cases with metastatic paragangliomas who had widespread soft tissue and bone metastases. One patient was treatment naive and the second had received previous chemotherapy. The functional imaging work-up performed and the targeted radionuclide therapies considered in these patients are described. Both patients were treated with 131I-MIBG. Partial tumour response and complete symptomatic and hormonal response was achieved in one patient; in the second patient there was no change.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
7/85. Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms.The authors report a case of gangliocytic paraganglioma of the lung, which has not yet been described in a pulmonary neoplasm. A 75-year-old man underwent right middle and lower lobe lobectomy. A slightly yellowish mass was located at the bifurcation between the lower and middle lobe bronchus, protruding into the truncus intermedius. The neoplastic cells were composed of three cellular elements: uniform endocrine cells in a Zellballen arrangement, large ganglion-like cells within the nests of endocrine cells, and spindle-shaped cells arranged in streams to surround the nests. Each component exhibited the characteristic immunohistochemical properties, which were similar to those of the corresponding neuroendocrine neoplasms: endocrine cells were positive for CAM 5.2, chromogranin a, and synaptophysin, like carcinoid tumor; ganglion-like cells were positive only for neurofilament, like ganglioneuroma; and spindle-shaped cells were positive for neurofilament and S-100 protein, like paraganglioma. These results agreed with those in gangliocytic paraganglioma of the duodenum. Pulmonary gangliocytic paraganglioma is similar to that in the duodenum, and is a hamartomatous proliferation of epithelial endocrine and neuronal cells of the bronchus.- - - - - - - - - - ranking = 5keywords = neuroendocrine (Clic here for more details about this article) |
8/85. Fine needle aspiration cytology of primary pulmonary paraganglioma. A case report.BACKGROUND: Primary pulmonary paragangliomas are rare tumors. To our knowledge, there is no prior report on fine needle aspiration cytology (FNAC) in pulmonary paraganglioma. CASE: A 34-year-old man presented with an incidentally found solitary pulmonary mass. FNAC showed papillarylike clusters of epithelioid cells with round to oval nuclei, evenly dispersed chromatin, micronucleoli and occasional anisonucleosis. These cytologic features were suggestive of a sclerosing hemangioma or bronchioloalveolar carcinoma. A right lower lobectomy revealed a primary pulmonary paraganglioma. CONCLUSION: The possibility of pulmonary paraganglioma should be considered in the differential diagnosis of FNAC showing pseudopapillary clusters of epithelioid cells.- - - - - - - - - - ranking = 0.0035492959836827keywords = carcinoma (Clic here for more details about this article) |
9/85. Periampullary gangliocytic paraganglioma.A33-year-old man presented with features of obstructive jaundice. Enterohepatic retrograde cholangiopancreatography examination revealed a periampullary growth. After pancreatoduodenectomy, histologic examination of the growth revealed three elements: a spindle cell element, large ganglion cells, and smaller paraganglioma-like cells arranged in small nests and groups. A diagnosis of gangliocytic paraganglioma was given. Gangliocytic paraganglioma is a rare benign duodenal tumor, with uncertain histogenesis. It is possible to identify the three components that are essential for its diagnosis on routine hematoxylin and eosin sections without the use of immunohistochemical markers. Because this lesion may present clinically as a periampullary carcinoma, it is important to recognize and diagnose this rare, but benign, entity.- - - - - - - - - - ranking = 0.0035492959836827keywords = carcinoma (Clic here for more details about this article) |
10/85. Preoperative embolization in the management of a mediastinal paraganglioma.Parangliomas are rare and highly vascular tumors of neuroendocrine cell origin which are treated by complete surgical resection. Preoperative embolization to reduce perioperative bleeding complications, although described in paragangliomas of the neck and carotid body, has never before been described in the case of a mediastinal paraganglioma. The following is a presentation of such a case of mediastinal paraganglioma, in which embolization was used successfully before surgical resection.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
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