1/8. costello syndrome: a cancer predisposing syndrome?costello syndrome is a specific MCA/MR syndrome mainly characterized by dysmorphic facial features, peculiar biphasic growth pattern, motor and mental retardation, ectodermal anomalies involving skin and nails, and age dependent development of nasal and perianal papillomata. heart malformations and/or hypertrophic cardiomyopathy are frequently observed. We report a 4-year-old girl with costello syndrome who developed an intrathoracic ganglioneuroblastoma. In previous reports two patients with ectodermal tumours have been described, a ganglioneuroblastoma of the adrenal gland and an epithelioma. This third report suggests that neural crest neoplasia may be a significant risk factor for children with costello syndrome.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/8. Sacrococcygeal ganglioneuroma.Ganglioneuromas are benign slow-growing masses that can be treated with complete surgical extirpation without any adjuvant therapy. Such lesions involving the sacrococcygeal region are exceedingly rare. The authors present the case of a 70-year-old woman with a sacrococcygeal ganglioneuroma treated by total en bloc resection. This patient also had a previous coccygeal fracture. To the authors' knowledge, there are no other reports of ganglioneuroma in association with a history of trauma.- - - - - - - - - - ranking = 3keywords = ganglion (Clic here for more details about this article) |
3/8. ganglioneuroma : primary tumor or maturation of a suspected neuroblastoma?ganglioneuroma is a benign neurogenic tumor. These tumors are originating from neuroepithelium along sympathetic ganglia. Main localization is the mediastinum in children older than 10 years. An association with malignant neuroblastoma is rarely observed and it still remains a topic under current discussion. We describe the clinical course of a 17 year-old female patient with a large presacral mass causing amenorrhoea and weight loss. Eleven years before presentation, an incidental urine test showed an elevation of vanillylmandelic acid (VMA) and homovanillic acid (HMA) and a neuroblastoma was suspected. However, further investigations showed no tumor and the test results turned out to be normal within 1 year. Now, a malignant neurogenic tumor was again suspected, but a CT-guided biopsy revealed a benign tumor. The mass was originating from the left sacral nerve roots. A tumor resection via a dorsal approach was performed. Final histology showed a differentiated ganglioneuroma. This is, to our knowledge, the first report describing a patient with elevated VMA/HMA and suspected neuroblastoma who developed a ganglioneuroma 11 years later. The association of ganglioneuroma and neuroblastoma and the abnormal urine tests pointing toward a neuroblastoma 11 years ago remains unclear and the possible answers are discussed in our report.- - - - - - - - - - ranking = 1.5keywords = ganglion (Clic here for more details about this article) |
4/8. Quantitative study of radioiodinated metaiodobenzylguanidine uptake in children with neuroblastoma: correlation with tumor histopathology.Six children with neuroblastoma and one with ganglioneuroma received [125I] metaiodobenzylguanidine (MIBG) before major surgery. Uptake of [125I]MIBG in the excised tissues was measured by scintillation counting, and the material was submitted for histopathology. The ranges of uptake of [125I]MIBG, expressed as percent of the injected dose per gram of tissue, were as follows: for neuroblastoma 0.0013-0.071, for ganglioneuroma 0.0017-0.0028, and for non-neoplastic control tissues 0.0002-0.011. The quantitative uptake of [125I]MIBG by neuroblastoma varied between different patients and between different parts of individual tumors. The more undifferentiated tumors took up more [125I]MIBG and may be more likely to respond to targeted radiotherapy with MIBG.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
5/8. Melanotic schwannoma arising in the sympathetic ganglion.A case of melanotic schwannoma, the 28th reported in the literature and the second to arise in the sympathetic ganglion, was reported in a 39-year-old man who remains free of recurrence or metastasis since removal of the tumor. In common with earlier benign melanotic schwannomas, its ultrastructural study revealed well-developed contiguous external lamina as opposed to absent or only minimally developed external lamina seen in malignant melanotic schwannomas. This emphasizes the use of electron microscopy in the diagnosis of this tumor. The literature yields only nine melanotic tumors (including one malignant melanotic schwannoma) arising in the sympathetic nervous system; all nine tumors had a malignant clinical course. Therefore, the present case represents the first "benign" tumor among ten melanotic tumors, and the second among 28 melanotic schwannomas to arise in the sympathetic nervous system.- - - - - - - - - - ranking = 2.5keywords = ganglion (Clic here for more details about this article) |
6/8. Neuroblastoma IV-S in a patient with bilateral microphthalmia.Neuroblastoma, a tumor of post-ganglionic sympathetic neurons, may be associated with a variety of genetic defects and congenital malformations (1). We report a case of neuroblastoma (NB) stage IV-S (2) in an infant with bilateral microphthalmia and other ocular malformations.- - - - - - - - - - ranking = 0.5keywords = ganglion (Clic here for more details about this article) |
7/8. Amplification of N-myc gene and increase of urinary VMA and HVA in patients with neuroblastic tumors.Neuroblastic tumor cases in our departments were evaluated in terms of the stage of the tumor, N-myc amplification, urinary vanillylamndelic acid (VMA)/homovanillic acid (HVA) and survival rate. Two asymptomatic cases, diagnosed when under a year old by mass screening, had no amplifications of N-myc but showed more than one value of urinary VMA/HVA ratio. The patients are now doing well eight years after complete excision of the neuroblastoma which had originated in the sympathetic ganglion. On the other hand, two other symptomatic cases, operated on at the ages of 3 and 5 years, showed remarkable amplifications with less than one value of urinary VMA/HVA, and died from the tumor soon after partial resection of the neuroblastoma and ganglioneuroblastoma which had originated in the adrenal gland and the sympathetic ganglion, respectively. The present monograph reports our cases and discusses prognostic factors.- - - - - - - - - - ranking = 1.5keywords = ganglion (Clic here for more details about this article) |
8/8. Primary malignant schwannoma involving simultaneously the right Gasserian ganglion and the distal part of the right mandibular nerve. Case report.A 66-year-old man presented to our Department with trigeminal neuralgia of the third division of the right trigeminal nerve. He also complained of some difficulty in mastication because of a tumour mass in the right lower jaw. A brain CT scan, x-rays of the lower jaw and viscerocranium CT scan revealed a malignant schwannoma of the right Gasserian ganglion involving simultaneously the distal end of the right mandibular nerve. The patient was operated on and has undergone a course of external irradiation. Seven years later, he is living a normal life. The value of the preoperative diagnosis of this extremely rare pathology is discussed because it seems that this tumour is radiosensitive.- - - - - - - - - - ranking = 2.5keywords = ganglion (Clic here for more details about this article) |