1/12. Cortical blindness: an unusual complication after removal of a ganglioneuroma of the neck.A 5-year-old girl was operated upon after a huge mass had been found on the left side of her neck. Preoperatively the anatomy was studied using MRI, including 3D-reconstruction of the tumor. The left vertebral artery was seen to cross the tumor, and during the operation the vessel was preserved. Postoperatively it thrombosed and a permanent cortical blindness developed. One year later MRI angiography of the intracerebral vessels was performed and a pre-existing anomaly of the circle of willis was demonstrated, which had contributed to the development of the cortical blindness.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/12. ganglioneuroma presenting as an asymptomatic huge posterior mediastinal and retroperitoneal tumor.ganglioneuroma is a rare, differentiated, benign and slow-growing tumor that commonly arises from sympathetic ganglion cells. Most of them are asymptomatic and found incidentally. We here report a quite rare case of silent huge ganglioneuroma growing in both posterior mediastinum and retroperitoneum occurring in a 3.5-year-old girl. The patient was relatively well before and incidentally found to have a huge chest mass by chest x-ray film at an episode of respiratory tract infection. Computed tomography showed a huge tumor extending from bilateral posterior mediastinum to the level of the adrenal gland in the retroperitoneum. Initially, neuroblastoma was highly suspected and 24-hour urine vanillyl mandelic acid was slightly elevated. Cytology by bone marrow aspiration revealed no tumor nests or clumps. biopsy and pathology proved it as ganglioneuroma (GN). Due to too extensive involvement of the tumor and compression of the vital vessels, surgical removal became difficult. The family of the patient refused surgery due to there being no significant symptoms. Because of the potential for growth of unresectable GN and because the component of neuroblasts could not be completely excluded, the patient was still in dangerous status. The only thing we can do is to keep the family alert and continue regular follow-up.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
3/12. Massive spontaneous hemothorax associated with Von Recklinghausen's disease.A 30-year-old woman with Von Recklinghausen's disease was admitted to our hospital because of sudden onset of dyspnea and syncope. A chest roentgenogram showed a massive right pleural effusion and insertion of an intercostal tube drained 1,600 cc of blood. A computed tomographic chest scan with contrast revealed a hyperdense mass in the right paravertebral area. At thoracotomy, retained clotted hemothorax and continued bleeding from tumor vessels was noted. The apex of the right hemithorax and the tumor location was packed. The pathologic diagnosis was ganglioneuroma and follow-up of the patient for 2 years after re-thoracotomy and removal of the packs revealed no complication and morbidity. We report this case to emphasize the importance of early recognition and prompt surgical intervention in spontaneous hemothorax associated with Von Recklinghausen's disease.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
4/12. Neurogenic tumours of the parapharyngeal space in the paediatric age group.Two cases of neurogenic tumours of parapharyngeal space in children of 7 years old are presented. Difficulties during the diagnosis, investigations and management are discussed along with a review of the available literature. CAT scan with sialography, carotid angiography during surgery, angle mandibulectomy and liberal incision to expose the great vessels for better control of bleeding are stressed to minimise the peri-operative complication and to assist in complete removal of the tumours.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
5/12. Pseudodrusen of the optic disc. papilledema simulating buried drusen of the optic nerve head.The distinction between true papilledema and pseudopapilledema rests on characteristics of the optic disc when examined ophthalmoscopically. Buried disc drusen frequently simulate papilledema and often result in misdirected diagnostic maneuvers in search of a cause for presumed intracranial hypertension. When an elevated optic disc exhibits an irregular, "lumpy, bumpy" border, a diagnosis of buried drusen of the optic nerve is usually made. We report a case with papilledema secondary to increased intracranial pressure in which the margins of the swollen optic disc presented this lumpy, bumpy border characteristic of buried drusen. The lumpy character of the disc border disappeared with resolution of the papilledema, and ultrasonography demonstrated the absence of any buried drusen. Other characteristics of papilledema, including extension of the disc swelling into the peripapillary nerve fiber layer, telangiectasia of the superficial vessels of the optic disc, and obscuration of the retinal vessels as they crossed the margins of the optic disc, provided strong evidence of true papilledema and remain the most reliable findings allowing a distinction between true papilledema and pseudopapilledema.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/12. Hypothalamic gangliocytoma. Selective appearance of neurofibrillary changes, granulovacuolar degeneration, and argentophilic bodies.A hamartomatous gangliocytoma was observed in the hypothalamus of a 54-year-old woman. The ganglion cells were atypical, highly pleomorphic and often multinucleated, and they possessed neurofibrillary changes, granulovacuolar degeneration, and argentophilic bodies. The neuronal changes were highly selective and were not found in other parts of the brain. The tumor was also characterized by the presence of angiomatous blood vessels having such degenerative changes as fibrosis and thrombosis. The angiomatous blood vessels were found only in the lesion. The ultrastructural features of the neurofibrillary changes were similar to those observed in Alzheimer's disease. Vascular alterations have been suggested to be a possible contributor to the morphogenesis of neurofibrillary changes. In this case the exact etiology of these neuronal changes remains unclear, however, the possibility of a regional vascular role is considered with respect to their morphogenesis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/12. radiation-induced leiomyosarcoma of the great vessels presenting as superior vena cava syndrome.A patient with a pleomorphic intravascular leiomyosarcoma of the great vessels of the neck and mediastinum presented clinically with a superior vena cava syndrome. A latent period of 29 years elapsed between receiving orthovoltage radiation to the neck and right side of chest to treat recurrent ganglioneuroblastoma, and the appearance of a leiomyosarcoma and subsequent recurrences. The patient underwent partial resection of the tumor, received adjunct chemotherapy, and was shown to be free of disease by clinical tests and by magnetic resonance imaging (MRI) 17 months after completion of chemotherapy. The criteria for the diagnosis of radiation-induced sarcomas are reviewed in relation to the present case. The critical role of magnetic resonance imaging in both the diagnosis and continued follow-up of the patient is described. This would appear to be the first reported case of radiation-induced intravascular leiomyosarcoma of the great vessels of the neck and mediastinum presenting as a superior vena cava syndrome.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
8/12. The sonographic evaluation of the great vessels' interspace in the pediatric retroperitoneum.The great vessels' interspace, in the pediatric retroperitoneum, deserves special attention during abdominal sonographic examination. By rotating the child into the right anterior oblique position, the full length of this interspace is demonstrated. Normally, it has a uniform appearance. When invaded by disease, the sonographic pathology can be identified and differentiated from the surrounding structures. The studies were gathered from experience with 1658 retroperitoneal sonographic examinations. Fourteen children were found with disease involving the great vessels' interspace: 4 patients with lymphoma, 3 patients with sympathetic ganglioneuroblastoma, 2 patients with retroperitoneal rhabdomyosarcoma, and one case each, respectively, of adrenal neuroblastoma, Wilms' tumour, clear cell carcinoma of the kidney, retroperitoneal teratoma and toxocara canis.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
9/12. Primary pulmonary ganglioneuroblastoma in an adult: maturation, involution and the immune response.The features of a primary pulmonary ganglioneuroblastoma occurring in an adult are presented. The tumour showed evidence of both maturation and involution. Maturation appeared to be occurring in a centrifugal manner, a rim of mature ganglioneuromatous tissue enclosing the primitive neuroblastoma. necrosis of the neuroblastomatous element was widespread and associated with deposition in the walls of numerous small vessels of an amorphous eosinophilic amyloid-like material. Accumulation of this material had led to occlusion of some vessels with resultant necrosis of related tumour. A collarette of lymphocytes surrounded the tumour, and lymphocytic aggregates were prominent at the interface between neuroblastoma and ganglioneuroma. Despite widespread vascular invasion, the patient remains well and apparently tumour-free, 2 1/2 years post-resection. The appearances may represent a combined cellular and humoral host response, and a possible relationship of this response to tumour maturation is suggested. The potential role of immunostimulation in the treatment of neuroblastoma is discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/12. Bilateral pheochromocytoma-ganglioneuroma of the adrenal in type 1 neurofibromatosis.A 61-year-old woman with cafe-au-lait pigmentation and severe cutaneous neurofibromatosis type I was noted to have persistent hypertension after coronary artery bypass grafts. Clinical investigation revealed bilateral adrenal medullary tumors. The patient did not have a duodenal lesion or gastrointestinal symptoms. Histologic examination showed both tumors to be composed of typical pheochromocytoma with large areas of ganglioneuroma (compound or composite pheochromocytomas). The neuromatous foci contained areas of cystic degeneration and thick-walled vessels. The ganglion cells and neuromatous areas were negative for chromogranin, glial fibrillary acidic protein, synaptophysin and vasoactive intestinal peptide. The typical pheochromocytomatous areas were strongly immunopositive for chromogranin and synaptophysin. Bilateral classic pheochromocytomas are rare in type 1 neurofibromatosis, and we believe that bilateral composite pheochromocytomas are an extension of this association.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
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