1/64. paraganglioma in the frontal skull base--case report.A 56-year-old female presented with a paraganglioma in the left anterior cranial fossa who manifesting as persistent headache. Computed tomography and magnetic resonance imaging showed a solid, enhanced tumor with a cystic component located medially. The tumor was attached to the left frontal base and the sphenoid ridge. angiography demonstrated a hypervascular tumor fed mainly by the left middle meningeal artery at the left sphenoid ridge. The preoperative diagnosis was meningioma of the left frontal base. The tumor was totally resected via a left frontotemporal craniotomy. Histological examination revealed the characteristic cellular arrangement of paraganglioma generally designated as the "Zellbaren pattern" on light microscopy. Only 10 patients with supratentorial paraganglioma have been reported, seven located in the parasellar area. The origin of the present tumor may have been the paraganglionic cells which strayed along the middle meningeal artery at differentiation.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
2/64. 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 = 0.1113167771682keywords = chest (Clic here for more details about this article) |
3/64. 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 = 0.22263355433639keywords = chest (Clic here for more details about this article) |
4/64. 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 = 0.069183760577706keywords = upper (Clic here for more details about this article) |
5/64. Calvarial metastasis of a paraganglioma. Case report and review of the literature.BACKGROUND: Metastasis of a paraganglioma (PRG) to the calvarium is very rare. In this paper, the case of a 25-year-old male with metastasis of a PRG to the frontoparietal bone is described. CASE DESCRIPTION: The patient presented with bulging on the left side of the head, headache, and weight loss. magnetic resonance imaging (MRI) revealed a mass lesion in the left frontoparietal region that had destroyed both the external and internal table of the bone, extending under the skin and above the dura mater. After a frontoparietal craniotomy the tumor was removed totally. Histopathological examination revealed the "Zellballen," which are pathognomonic for a PRG. Systemic examination and radiological investigation revealed no primary tumor source. CONCLUSION: Metastasis of a PRG to the calvarium is possible; radical removal of the tumor will provide a cure.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
6/64. A case of hemorrhagic gallbladder paraganglioma causing acute cholecystitis.gallbladder paraganglioma is a very rare tumor and so far only a few cases have been reported. Most of these were asymptomatic and were found incidentally during operation. Recently, we experienced a gallbladder paraganglioma that gave rise to hemorrhage, which in turn caused acute cholecystitis. Our case involved a 45 year-old female patient complaining of an intermittent right upper abdominal pain. After a preoperative evaluation, cholecystectomy and lymphadenectomy were performed under the impression of gallbladder cancer with acute cholecystitis. Postoperative pathologic examination revealed a hemorrhagic gallbladder paraganglioma accompanied by acute cholecystitis. Immunohistochemical staining of the chief cells for neuron specific enolase, chromogranin and synaptophysin were positive. Sustentacular cells also stained positively for S100 protein.- - - - - - - - - - ranking = 0.76080448042032keywords = abdominal pain, upper (Clic here for more details about this article) |
7/64. Nasopharyngeal gangliocytic paraganglioma.Gangliocytic paraganglioma (GP) is a rare neoplasm described almost exclusively in the gastrointestinal tract, especially the periampullary region. However, several examples have been reported at various sites, including the stomach, jejunum, and appendix. Herein we report a case of GP involving the nasopharynx. To our knowledge, this is the first report of GP at this site. A 44-year-old woman presented with headaches and symptoms of fullness and pressure related to mass effect. An initial endoscopic biopsy was followed by surgical excision of the nasopharyngeal mass. The triphasic tumor fulfilled the morphologic and immunohistochemical criteria for GP. The histogenesis of GP is uncertain, and the current belief is that it arises from the embryonic ventral pancreas. This concept is based largely on the location of most cases, which is along the embryologic migration route of the ventral pancreas, as well as the expression of pancreatic polypeptide by the tumor. The nasopharyngeal location of our case clearly refutes the pancreatic origin of GP. We propose that the tumor probably arises from totipotential adult stem cells, which in the right microenvironment differentiate along nonnative cell lineages.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
8/64. A case of adrenocortical tumor coexisted with paragangliomas.We report a case of adrenocortical tumor that coexisted with paragangliomas. A 35-year-old woman was admitted to the hospital because of left upper abdominal pain. A palm-sized mass was palpated at left upper quadrant. Hormonal studies revealed the features of pheochromocytoma. An emergency operation was performed because hemorrhage of the tumor was suspected. A 10 cm diameter ruptured mass was found in the left adrenal area and other tumors were also noted adjacent to inferior vena cava (IVC). The pathologic report revealed that the adrenal mass was an adrenocortical tumor with hemorrhagic necrosis and that the tumors adjacent to IVC were paragangliomas. This was the first case of adrenocortical tumor with paragangliomas in our medline search result, hence we report the case with a review of the literature.- - - - - - - - - - ranking = 0.82998824099802keywords = abdominal pain, upper (Clic here for more details about this article) |
9/64. paraganglioma of the pituitary fossa: diagnosis and management.paraganglioma of the sellar area is extremely rare with only six cases having been reported in the literature. Surgical removal of these tumors is difficult, and the transsphenoidal approach usually results in limited resection. Most authors who published reports on this tumor recommended radiation therapy after partial removal of the tumor. However, considering the benign nature of these tumors, the risk of radiation-induced endocrine insufficiency and optic neuropathy and the lack of proven effectiveness of radiotherapy, its value remains controversial. We describe a 48-year-old woman with parasellar paraganglioma who presented with headaches, visual loss and oligomenorrhea. magnetic resonance imaging (MRI) showed an invasive tumor in the sellar and parasellar areas which extended to both cavernous sinuses and compressed the optic chiasm and the left internal carotid artery. Surgery by the transsphenoidal approach enabled only limited biopsy of the tumor. The patient was reoperated by an extended pterional approach which resulted in a subtotal removal of the tumor and adequate decompression of the adjacent structures. She received no adjuvant treatment during the 8-year postsurgical follow-up and remained in good health. A repeated MRI showed no change in the size of the residual tumor. Contrary to the therapeutic recommendations described in previous reports, we favor postoperative adjuvant therapy only if the symptoms or signs of cranial nerve compression persist following maximal tumor removal, or if there is evidence of subsequent growth of residual tumor.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
10/64. Mesenteric paraganglioma: a case report and review of the literature.Approximately 5% to 10% of paragangliomas occur in extra-adrenal sites, which can extend from the upper cervical region to the pelvis, parallel to the autonomic nervous system. This distribution corresponds to the embryologic development of the paraganglia from neural crest cells. Rarely, extra-adrenal paragangliomas can also occur aberrantly outside this distribution. We report such a case of extra-adrenal paraganglioma occurring in the anterior mesentery in a 76-year-old man. Two case reports exist in the literature describing extra-adrenal paragangliomas in the posterior mesentery. Normal paraganglionic tissue has been described at the roots of the superior and inferior mesenteric arteries, theoretically explaining the origin of the posterior mesenteric paragangliomas. Our case can best be attributed to the ventral migration of paraganglionic tissue through these vessels to reach the anterior mesentery, where they could potentially give rise to paragangliomas in this site.- - - - - - - - - - ranking = 0.069183760577706keywords = upper (Clic here for more details about this article) |
| Next -> |