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 = neoplasm (Clic here for more details about this article) |
2/85. 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 = 0.07226061309132keywords = cancer (Clic here for more details about this article) |
3/85. An extremely large solitary primary paraganglioma of the lung: report of a case.We present herein the case of a 38-year-old woman found to have an extremely large solitary primary paraganglioma of the lung. The patient presented with chest pain on exertion and a mass was discovered in the left lower lobe of the lung by chest x-rays and computed tomography (CT). As no other neoplasms were detected elsewhere, a left lower lobectomy was performed. The patient has remained well without any evidence of recurrence for 5 years since her operation. The tumor, measuring 13 x 12 x 7 cm, was composed of ovoid cells (Zellballen), which were positive for Fontana-Masson and Grimelius stains, and sustentacular cells. Immunohistochemically, the ovoid cells were positive for neuron-specific enolase, S-100, CAM5.2, Leu7, and chromogranin a, and negative for carcinoembryonic antigen and epithelial membrane antigen. The sustentacular cells were positive for S-100 protein and CAM5.2, and negative for glial fibrillary acid protein. Therefore, the tumor was diagnosed as a paraganglioma. The tumor from our patient is the largest of the 17 solitary primary pulmonary paragangliomas reported thus far in the English-language literature.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/85. Metastatic paraganglioma and paraneoplastic-induced anemia in an adolescent: treatment with hepatic arterial chemoembolization.Mediastinal paragangliomas are rare neoplasms in children. anemia, as a paraneoplastic syndrome, has been described in adults with metastatic paraganglioma. The management of paraneoplastic anemia from metastatic paraganglioma has been problematic, with no reports in the literature describing successful treatment. This article describes a 17-year-old Jehovah's Witness with a mediastinal paraganglioma, hepatic metastases, and severe anemia. The patient and his family refused blood products and the anemia was refractory to erythropoietin and elemental iron therapy. Serial chemoembolization of the hepatic lesions resulted in resolution of the anemia, allowing subsequent debulking of the mediastinal paraganglioma.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/85. Sudden death in a child due to an intrathoracic paraganglioma.A 12-year-old boy under treatment for asthma was found dead in his home. The autopsy revealed a large posterior mediastinal mass that completely compressed the upper lobe of the right lung and the associated airways. This mass extended from the right costovertebral sulcus into the thoracic spinal canal through the spinal foramen and compressed the spinal cord. It was located in the epidural space and was adherent to a nerve root. The histologic and immunocytochemical features were that of a paraganglioma. Although neurogenic tumors are the most common posterior mediastinal masses in the pediatric population, paragangliomas are rare, and spinal involvement has not been described in children. In addition, sudden death has not been reported in association with any of the 13 cases of posterior mediastinal paraganglioma described in the literature as involving the spine. This case illustrates an unusual cause of sudden death in a pediatric patient due to a benign neoplasm.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/85. paraganglioma of the cauda equina. Case report with 33-month recurrence free follow-up and review of the literature.paraganglioma of the cauda equina is an unusual tumor and do not have the secretory properties of the same tumors arising outside the nervous system. In none of the few cases reported in literature a preoperative diagnosis was possible, and the surgical findings raised questions in the differential diagnosis with ependymomas. A rare case of paraganglioma of the cauda equina studied both pre- and postoperatively by MRI, and treated with subtotal excision combined with radiotherapy is described. Results and recurrence rates of the cases reported in literature are reviewed. Though MRI imaging has proven to be more sensitive than other radiological procedures, we stress the difficulties of preoperative diagnosis of paragangliomas in this site. The correct diagnosis of the paraganglioma of the cauda equina still relies on immunochemistry and electron microscopy. Total excision is often very difficult owing the tendency of these neoplasms to infiltrate cauda's roots. A 33-month recurrence free follow-up of our patient confirms that successful treatment is achieved by subtotal resection combined with radiotherapy.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/85. A left atrial paraganglioma patient presenting with compressive dysphagia.Paragangliomas are rare neoplasms of neural crest origin arising in the chromaffin (pheochromocytoma) and chemoreceptor (chemodectoma) tissues. Only a few cases of paragangliomas have been reported in the heart. Most of the cardiac paragangliomas are located in, or adjacent to, the left atrium. The biological activity indicative of catecholamine production has rarely been shown in cardiac paragangliomas. patients with cardiac paragangliomas may present with hypertension, or with various obstructive or compressive symptoms, depending on the location of the tumour. A left atrial paraganglioma compressing the esophagus causing dysphagia is presented. A 37-year-old female patient underwent work-up for dysphagia to solids. Transesophageal echocardiography showed the presence of a large mass in the dilated left atrium. The tumour was removed completely intact and was a benign noncatecholamine-secreting paraganglioma. The patient's dysphagia was relieved. The clinical perspectives of cardiac paraganglioma and cardiac dysphagia are discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/85. 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 = 1keywords = neoplasm (Clic here for more details about this article) |
9/85. paraganglioma of the bladder: report of a case.Paragangliomas are uncommon tumours that comprise less than 0.1% of all primary bladder neoplasms. Approximately half of the patients present the clinical triad of hypertension, haematuria and attacks associated with micturition. About 10% of vesical paragangliomas behave malignantly. This may be manifested as local invasion or metastatic spread, particularly to lymph nodes and lung. The best therapy remains total excision and partial cystectomy is considered adequate treatment for most cases. We report a case of paraganglioma of the bladder with extramural appearance in a 39-year-old man. Clinical symptoms were hypertension, dysuria and a well-defined hypogastric mass. One year after complete removal of the tumour, the patient is asymptomatic and normotensive, with normal catecholamine and vanillylmandelic acid (VMA) levels.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/85. 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 = neoplasm (Clic here for more details about this article) |
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