1/42. A new pattern of multiple endocrine adenomatosis: chemodectoma, bronchial carcinoid, GH-producing pituitary adenoma, and hyperplasia of the parathyroid glands, and antral and duodenal gastrin cells.A female patient was found to have a chemodectoma, a GH-producing pituitary tumour and a bronchial carcinoid combined with hyperplasia of the parathyroids and of antral and duodenal gastrin cells. This combination of endocrine tumours and hyperplasias does not fit with the two multiple endocrine adenomatosis syndromes recognized at present. The case stresses the importance of scanning the patient for other endocrine tumours, once one has been diagnosed.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/42. Cushing's syndrome associated with a chemodectoma and a carcinoid tumour.We present a case of Cushing's syndrome where 111In-octreotide scanning provided evidence for the presence of two neuroendocrine tumours. Uptake in the right neck corresponded to a chemodectoma, but there was no change in the clinical condition or fall in ACTH levels following surgical resection. Uptake in the left chest was assumed to relate to a bronchial carcinoid, but a tumour could not initially be localized on magnetic resonance imaging (MRI), spiral CT scanning or on selective venous sampling. A 1 cm bronchial carcinoid tumour was identified post-mortem which immunostained for ACTH. This case demonstrates that 111ln-octreotide scanning is a useful technique for identifying the source of ectopic ACTH production in difficult cases of Cushing's syndrome. Reliance should not be placed solely on standard imaging techniques to localize the tumour prior to surgery. Although rare, the possibility of a non-ACTH secreting neuroendocrine tumour should also be considered in patients with ectopic ACTH syndrome, who have positive 111In-octreotide scans.- - - - - - - - - - ranking = 0.28571428571429keywords = endocrine (Clic here for more details about this article) |
3/42. paraganglioma of the thyroid: two cases that clarify and expand the clinical spectrum.BACKGROUND: Paragangliomas (PGs) can, on rare occasions, arise within the thyroid parenchyma presumably from displaced laryngeal paraganglia. On the basis of a limited number of reported cases, thyroid PGs invariably affect women, they are always benign, and they are usually mistaken for some other more common thyroid lesion. methods: We describe the histopathologic features, immunohistochemical findings, and clinical characteristics of two thyroid PGs. RESULTS: One tumor was incidentally discovered in a 55-year-old man during evaluation of a carotid bruit. The other tumor aggressively invaded the trachea and esophagus of a 52-year-old woman with a presumed long-standing nodular goiter. In both cases, the initial pathologic evaluation suggested medullary thyroid carcinoma. Both patients are alive without recurrent disease after surgical resection. CONCLUSIONS: These cases emphasize the need to consider PG in the differential diagnosis of neuroendocrine thyroid tumors, even in those tumors involving men or behaving in a locally aggressive fashion. Failure to do so carries grave implications regarding patient prognosis and management.- - - - - - - - - - ranking = 0.14285714285714keywords = endocrine (Clic here for more details about this article) |
4/42. Surgical resection of jugulare foramen tumors by juxtacondylar approach without facial nerve transposition.PRESENTATION: Jugulare foramen tumors (JFT) remain a difficult challenge especially in the forms extending extradurally and invading the petrous bone. In the standard technique, facial nerve function is placed at risks because of its transposition. We report on 31 extradural (N = 11) or intra-extra dural (N = 20) JFT resected surgically using the juxtacondylar approach alone or in combination with the infratemporal approach and without facial nerve transposition. RESULTS: The juxtacondylar approach permits the opening of the JF on its posteroinferior aspect and thus reduces the extent of petrous bone drilling. In tumors strictly located in the JF (N = 11), no petrous bone drilling is necessary and the facial nerve is never exposed. In tumors extending into the petrous bone (N = 20), the facial nerve was never transposed and moreover was kept in its bony canal in 15 cases. In only 5 cases, was the fallopian canal opened as the tumor was invading its bony wall. Similarly hearing function, when pre-operatively intact was always preserved and a better preservation of the lower cranial nerves could be achieved. Whatever the tumoral extent along the petrosal carotid artery, a radical resection could be realized in 30 cases. CONCLUSION: Therefore, we consider the juxtacondylar approach a useful adjunct to increase the possibilities of resection of JFT; it allows a better preservation of the neurovascular structures, especially the facial nerve which is kept in place and moreover kept in its bony canal when it is not invaded by the tumor.- - - - - - - - - - ranking = 0.019880505888231keywords = bone (Clic here for more details about this article) |
5/42. A case of Cushing's syndrome in ACTH-secreting mediastinal paraganglioma.Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.- - - - - - - - - - ranking = 0.14285714285714keywords = endocrine (Clic here for more details about this article) |
6/42. Malignant carotid body tumors: report of three cases.The aim of the current report was to describe 3 cases of malignant carotid body tumor (CBT) and to review the literature. My clinical records of 87 CBTs in 81 patients (6 bilateral) were reviewed, 79 of which were operated on. Three malignant cases were found. The first was in a 40-year-old man who presented with pulmonary metastases 6 years after resection of a CBT. He was treated with chemotherapy and interferon, but died with disseminated disease 2 years later. The second case was in a 56-year-old woman who had a 5-cm, fixed, hard mass in the upper aspect of the neck and a paralysis of the left vocal cord. This lesion was completely resected, and a shunt and reconstruction with a saphenous vein graft were performed. pathology revealed a malignant chemodectoma with invasion to 2 of the 5 lymph nodes removed. radiotherapy (50 Gy) was given after the operation. She is well and free of disease 68 months after the resection. The third case was in a 61-year-old woman who presented with an 8-cm nontender, hard, immobile mass in the left upper neck that displaced the left wall of the oropharynx toward the midline. A carotid arteriogram showed a CBT. On computed tomography, the tumor extended to the infratemporal fossa with no bone involvement. The lesion was embolized with a 40% reduction in vascularity. At surgical exploration, the tumor involved the sternocleidomastoid muscle and the lymph nodes at levels II and III, and the internal carotid artery could not be dissected free at the skull base, so only a partial resection was performed. This patient was lost to follow-up. These 3 cases are in agreement with the literature. Locoregional control is usually obtained with complete primary tumor resection and lymphadenectomy and eventual radiotherapy. Surgery with radiotherapy seems to be effective for isolated metastases. Current multidisciplinary treatments have been unsuccessful in controlling disseminated disease.- - - - - - - - - - ranking = 0.0049701264720578keywords = bone (Clic here for more details about this article) |
7/42. A diagnostic and therapeutic approach to paragangliomas of the larynx.BACKGROUND: Differentiating paragangliomas from moderately differentiated neuroendocrine carcinoma in the larynx is a difficult management problem. As the biological behavior of these 2 entities is different, we developed an algorithm for the preoperative diagnosis and treatment of this disease. DESIGN: The sample case from which the algorithm was developed consisted of a 69-year-old man who was transferred to us after tracheostomy and an attempt at biopsy for airway obstruction secondary to a vascular mass. biopsy resulted in substantial bleeding. Flexible laryngoscopy showed a vascular mass of the supraglottis. A computed tomographic scan showed 2 vascular masses at the carotid bifurcation and in the larynx. An arteriogram confirmed synchronous vascular tumors. RESULTS: The arteriogram showed the superior thyroid artery to be the major feeder vessel to this mass, a situation commonly seen in paragangliomas but not other neuroendocrine tumors. The presence of synchronous lesions and a vascular mass based on the superior thyroid artery helped differentiate paraganglioma from the other neuroendocrine tumors. As the biological behavior of paragangliomas is relatively benign, we performed a conservative supraglottic laryngectomy and excision of the carotid body tumor. Histologic diagnosis and immunohistochemical analysis confirmed the diagnosis of paraganglioma. CONCLUSIONS: The vascular nature of neuroendocrine tumors prevents preoperative pathological diagnosis. Radiologic features demonstrating a vascular mass with a dominant feeder vessel by the superior or inferior thyroid artery may help in the clinical diagnosis of paragangliomas of the larynx. Since paragangliomas are rarely malignant, a conservative surgical procedure should suffice.- - - - - - - - - - ranking = 0.57142857142857keywords = endocrine (Clic here for more details about this article) |
8/42. paraganglioma manifesting as shock: a case report.paraganglioma is a rare neuroendocrine tumor in children that rarely manifests as shock. We describe the case of a 12-year-old girl with paraganglioma who developed impaired cardiac function, pulmonary edema, and shock at the time of admission. Her blood pressure stabilized after intravenous normal saline rescue and dopamine treatment. However, hypertension was noted thereafter. After a series of examinations, paraganglioma was diagnosed and excision of the tumor was performed. After surgery, blood pressure stabilized and her cardiac function had fully recovered at 4 months' follow-up.- - - - - - - - - - ranking = 0.14285714285714keywords = endocrine (Clic here for more details about this article) |
9/42. neuropeptide y secretion from a malignant extraadrenal retroperitoneal paraganglioma.A patient with a malignant extraadrenal retroperitoneal paraganglioma had elevated levels of immunoreactive neuropeptide y (NPY) in the peripheral blood (5988 pg/ml; normal, 123 /- 30 pg/ml [mean /- standard error of the mean]). A 6-month course of chemotherapy allowed surgical removal of the previously unresectable primary tumor. Postoperatively, the plasma NPY level initially fell to 1089 pg/ml; continued chemotherapy caused an additional decrease to 440 pg/ml. Four months after surgery, the plasma NPY level increased to 940 mg/ml, coincident with hepatic metastases. This case is the first report of a NPY-secreting clinically nonfunctional malignant extraadrenal paraganglioma. Determination of circulating NPY levels may be useful in the diagnosis and follow-up of patients with neuroendocrine tumors.- - - - - - - - - - ranking = 0.14285714285714keywords = endocrine (Clic here for more details about this article) |
10/42. Neuroendocrine differentiation in hyalinizing trabecular tumor of the thyroid.The presence of neuroendocrine cells is putative in thyroid hyalinizing trabecular tumor (HTT), although this entity requires a differential diagnosis from paraganglioma or medullary carcinoma of the thyroid, due to similarity of the growth pattern of the cells. Here, we present a case of HTT with a mixture of endocrine cells stained positive for somatostatin, chromogranin a and Grimelius' silver impregnation. The histology and results of other immunostainings were consistent with the features typical for HTT. In the world literature to date, only two cases of HTT with endocrine cells, including the current case, have been reported. Nevertheless, the cases may indicate the diversified differentiation of cells in HTT and may, in part, account for the resemblance of HTT to paraganglioma and medullary carcinoma.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
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