11/34. A thyroid nodule revealing a paraganglioma in a patient with a new germline mutation in the succinate dehydrogenase B gene.A 32-year-old asymptomatic female was diagnosed with an isolated thyroid nodule of 2.5 cm diameter. Fine needle aspiration suggested a medullary thyroid carcinoma. Consequently, a total thyroidectomy was performed. The nodule stained positive for chromogranin a, neurone-specific enolase and synaptophysin, but not for calcitonin. Finally, pathological analysis showed a thyroid paraganglioma. Although the tumour appeared to be sporadic in a patient with no personal or familial history of paraganglioma and/or pheochromocytoma, we have identified a new mutation (392delC) of the succinate dehydrogenase-B (SDHB) gene in the genomic dna extracted from the leukocytes of the patient. That mutation induced a shift in the reading frame of the gene creating a premature stop codon (P131fsX135) which was predicted to result in a truncated SDHB protein of 135 amino acids.This report highlights the difficulties of this unexpected diagnosis of hereditary thyroid paraganglioma. It also discusses the clinical involvements in terms of familial screening and the necessary follow-up of the patient.- - - - - - - - - - ranking = 1keywords = thyroid nodule, thyroid, nodule (Clic here for more details about this article) |
12/34. 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 = 0.049423666203296keywords = thyroid (Clic here for more details about this article) |
13/34. 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 = 0.28889400721767keywords = thyroid nodule, thyroid, nodule (Clic here for more details about this article) |
14/34. The importance of the interpretation of urine catecholamines is essential for the diagnosis and management of patient with dopamine-secreting paraganglioma.Phaeochromocytoma or paraganglioma that exclusively secretes dopamine is very rare. This case illustrates its atypical presentation and the importance of interpretative reporting for urine catecholamines leading to the diagnosis and subsequent management of a patient with this condition. We report a 71-year-old Chinese woman with a large dopamine-secreting paraganglioma. She presented with low back pain for six months. On examination, a right abdominal mass was palpable incidentally. Her blood pressure was normal throughout. Serial 24-h urine collections for catecholamines showed enormous elevation of urine dopamine excretion to 80.7 micromol/day (normotensive:<2.6 m mol/day). However, the daily excretions of urine adrenaline and noradrenaline, as well as their metabolites were within their respective reference intervals. Good communication between chemical pathologists and physicians prompted the arrangement of the whole body 131I-meta-iodobenzylguanidine (MIBG) scintigraphy, which showed a large signal in the right upper quadrant of the abdomen corresponding to a large extra-adrenal tumour detected by both ultrasonography and computerized tomography (CT) of the abdomen. Histological section of the tumour tissue revealed paraganglioma, which stained positive for chromogranin and neuron-specific enolase. After four months, the patient presented with chest symptoms and CT of the thorax revealed multiple nodules. lung metastases were suspected. However, follow-up urine catechola- mine and dopamine excretions were again within their respective normotensive reference intervals. A second MIBG scintigraphy was performed, but no specific uptake at either the thorax or the abdomen could be demonstrated. Fine-needle aspiration cytology using the thoracoscopic technique was performed and immunochemical staining of the biopsy specimen showed the presence of non- small-cell carcinoma of the lung.- - - - - - - - - - ranking = 0.00034295974130014keywords = nodule (Clic here for more details about this article) |
15/34. Surgical approach to submucosal lesions of the supraglottic larynx: the supero-lateral thyrotomy.The surgical approach to the hypopharynx by lateral pharyngotomy as described by Trotter has found widespread use in management of supraglottic carcinoma. A similar but more conservative approach may be employed for removal of cysts and benign or well-encapsulated neoplasms of the epiglottis and supraglottic space. We call this approach a supero-lateral thyrotomy, to differentiate it from the classic lateral pharyngotomy. Surgery consists of subperichondrial resection of the superior half of the ipsilateral thyroid cartilage with preservation of internal lining and superior laryngeal nerve. The lesion may then be enucleated or resected, and the defect, if any exists, closed with overlying mucosa and the flap of preserved perichondrium. The technique has been employed in cases of paraganglioma, haemangiopericytoma and saccular cysts.- - - - - - - - - - ranking = 0.012355916550824keywords = thyroid (Clic here for more details about this article) |
16/34. Management of delayed intracranial and intraspinal metastases of intradural spinal paragangliomas.The preferential site of extra-adrenal paragangliomas is the head and neck region. However intradural spinal paragangliomas are commonly described and are considered as benign entities. We report the case of a paraganglioma of the cauda equina followed after complete surgical removal by intracranial and intraspinal cerebrospinal fluid metastases. Seven years after the first operation, a cystic cerebellar lesion was successfully treated by surgery. During a long follow-up, four locations in the posterior fossa, a lumbar recurrence and metastatic nodules at T6 and S1-S2 level were also discovered. radiotherapy stopped the lesions' growth and allowed improvement of the neurological status. Through a review and analysis of the literature, we discuss the management of this unusual evolution.- - - - - - - - - - ranking = 0.00034295974130014keywords = nodule (Clic here for more details about this article) |
17/34. paraganglioma-like medullary thyroid carcinoma: a rare entity.OBJECTIVE: The wide variety of rare histologic variants of medullary thyroid carcinoma (MTC) may make the differential diagnosis difficult. Pathologic examination of the resected specimen will not always be able to confirm the diagnosis, leaving the surgeon with an uncertainty as to what type of resection is best for the patient. The following report describes the case of a 58-year-old man with the rare diagnosis of paraganglioma-like MTC. methods: The patient presented with markedly elevated calcitonin (CT) and carcinoembryonic antigen (CEA) levels. A 1-cm tumor was detected in the right lobe of the thyroid. Based on the clinical diagnosis of MTC, we performed a total thyroidectomy with lymphadenectomy of the central and right lateral compartments. paraffin sections of the resected specimen were stained with hematoxylin and eosin (H&E) and immunohistochemically characterized using antibodies to CT, CEA, chromogranin a, thyroglobulin, synaptophysin, sustentacular cells (SCs), low- and high-molecular cytokeratins (CK 5/6, 7, 18, 20), epidermal growth factor-receptor (EGFR), thyroid transcription factor-1 (TTF-1), bcl-2, Melan A, C-kit, neuron-specific enolase (NSE), and galectin-3. The patient's blood and tumor tissue were examined for mutations in the RET-protooncogene. RESULTS: H&E staining of both frozen and permanent sections was unable to differentiate benign from malignant tissue. Typical morphologic characteristics for MTC were completely absent. Only the additional finding of positivity for synaptophysin and numerous SC cells visible in-between neoplastic cells made the diagnosis of paraganglioma-like MTC possible. Sequencing of the RET proto-oncogene revealed no mutations. CONCLUSIONS: There are subgroups of MTC that present clinically similar to classic MTC, but in which missing typical morphologic characteristics make histopathology diagnosis difficult. In these cases, diagnosis, operative decisions, and follow-up strategies should be based on preoperative biochemical markers, imaging findings, and clinical parameters in accordance to the guidelines for classic MTC.- - - - - - - - - - ranking = 0.098847332406593keywords = thyroid (Clic here for more details about this article) |
18/34. Cervical paraganglioma mimicking a parathyroid adenoma on Tc-99m sestamibi scintigraphy.A 32-year-old man with a clinical and biochemical profile suggestive of primary hyperparathyroidism presented with a soft tissue mass on the left side of the neck. Multiphasic planar and SPECT Tc-99m sestamibi (MIBI) scintigraphy showed focally increased tracer concentration in the mass. Subsequent histopathology and immunostaining identified the mass as a parathyroid hormone-secreting cervical paraganglioma, indicating that this tumor should be considered in the differential interpretation of MIBI concentration in the neck.- - - - - - - - - - ranking = 0.074135499304944keywords = thyroid (Clic here for more details about this article) |
19/34. paraganglioma of the vulva.A case of vulvar paraganglioma is reported. Following presentation with vulvar pain, a 1-cm nodule was excised from the labium minus of a 58-year-old woman. Histologically, the tumor was composed of nests of round eosinophilic cells with moderately pleomorphic nuclei, beneath an intact squamous epithelium. Ultrastructural studies indicated two cell types within the neoplasm: chief cells with numerous small neurosecretory granules and peripheral slender sustentacular cells. A reticulin stain confirmed the "zellballen" nature of the neoplasm, and the neoplastic cells showed moderate argyrophilia on a Grimelius stain. The immunoperoxidase stains for chromogranin and neuron-specific enolase were strongly positive in the neoplastic chief cells. Immunostaining using anti-S-100 antibody confirmed the finding of sustentacular cells by identifying many slender cellular processes among the chief cells. These light and electron microscopic findings are diagnostic of paraganglioma, an entity not previously reported in the vulva to our knowledge.- - - - - - - - - - ranking = 0.00034295974130014keywords = nodule (Clic here for more details about this article) |
20/34. Coexistence of bilateral pheochromocytoma and pancreatic islet cell tumor: report of a case and review of the literature.A 14-year-old Japanese male with a previously undescribed combination of bilateral pheochromocytoma and an islet cell tumor of the pancreas is presented. The combination of endocrine neoplasms in this patient overlaps multiple endocrine neoplasia (MEN) Type 1 and Type 2. A total of 14 reported cases of MEN overlapping Type 1 and Type 2 is reviewed. Of the 14, 7 patients with acromegaly developed a paraganglioma(s), 2 patients with Sipple syndrome had a pituitary adenoma, and in the other 5 patients, an intestinal carcinoid or a pancreatic islet-cell tumor occurred in association with either a thyroid medullary carcinoma or a paraganglioma(s). We believe that the occurrence of MEN overlapping Type 1 and Type 2 is more than a fortuitous association, and can be explained on the basis of the neuroectodermal origin.- - - - - - - - - - ranking = 0.012355916550824keywords = thyroid (Clic here for more details about this article) |
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