1/125. Ukrain treatment in a patient with stage IV neuroblastoma. A case report.A 22-month-old boy with stage IV neuroblastoma underwent laparotomy with tumor removal and nephrectomy, followed by treatment with Ukrain. Two months later, a remaining abdominal tumor, a retroperitoneal tumor (approximately 2 x 1 cm), and lung, brain, pelvis, kidney, and distal femur metastases were found. growth of the neuroblastoma around the spinal cord and growth into the spinal canal was also found. Ukrain was administered in 3-week therapy series with 3-week pauses between each series. Some tumors disappeared, others were smaller, and growth stopped in the remaining tumors. Various metastases were no longer detectable.- - - - - - - - - - ranking = 1keywords = spinal cord, spinal, cord (Clic here for more details about this article) |
2/125. Spinal cord vascular injuries following surgery of advanced thoracic neuroblastoma: an unusual catastrophic complication.BACKGROUND: Spinal cord injury is a possible complication associated with removal of thoracic dumbbell neuroblastomas. Our experience with two children whose postsurgical course was complicated by midthoracic spinal cord ischemia is reported there. Permanent paraplegia resulted in both. PROCEDURE AND RESULTS: Preoperative awareness of the origin and distribution of the Adamkiewicz artery (arteria radiculomedullaris magna, ARMM) and of the possible collateral pathways for spinal cord blood supply may be helpful in the planning of operations that involve dissection in the midthoracic posterior mediastinum. Otherwise, a flaccid paraplegia may result. CONCLUSIONS: The syndrome is presumed to be triggered by a spasm, an embolism, or a iatrogenic interruption of the ARMM.- - - - - - - - - - ranking = 1.9709203689214keywords = spinal cord, spinal, cord (Clic here for more details about this article) |
3/125. Neuroblastoma of the urinary bladder, preclinically detected by mass screening.BACKGROUND: Since the introduction of mass screening of infants for neuroblastoma, the incidence of neuroblastoma has increased in japan. The reason for this increased incidence is the possible inclusion of many neuroblastomas that would have regressed spontaneously and would never have been detected clinically. An extremely rare tumor at the dome of the urinary bladder in a 7-month-old infant was detected by the mass screening. methods: A case of neuroblastoma of the urinary bladder is reported with a review of the literature. The data in the japan Children's Cancer Registry are also reviewed to analyze the incidence and the site of origin of neuroblastoma for evaluation of mass screening. RESULTS: A 7-month-old female infant was referred because of a positive urine vanillylmandelic acid screening test. ultrasonography showed a solid mass attached to the urinary bladder. At laparotomy a 35 x 30 x 25-mm egg-shaped tumor was found at the dome of the bladder, and a partial cystectomy was performed. During the operation no changes in blood pressure occurred when the tumor was manipulated. Histologic diagnosis was rosette-fibrillary neuroblastoma originating in the bladder wall, with a favorable Shimada histopathologic classification. N-myc was not amplified, which predicted a favorable prognosis, and no postoperative chemotherapy was given. The patient was free of symptoms and tumor after a follow-up period of 16 months. literature review revealed that this was the second case of neuroblastoma of the urinary bladder ever reported in the world, although several cases of pheochromocytoma originating in the bladder wall had been reported. Both neuroblastoma and pheochromocytoma derive from the neural crest. The sympathogonia from the neural crest, a common stem cell, differentiates into a ganglion cell or into a secretory cell known as a chromaffin cell, able to manufacture catecholamines. The first case in the world that was reported as neuroblastoma of the urinary bladder was in a 4-month-old infant who was noted to have a 4-cm lower abdominal mass on routine physical examination. A ganglioneuroblastoma of the dome of the bladder was excised and the patient was doing well. On reviewing the japan Children's Cancer Registry, the incidence of neuroblastomas in infants has increased as well as the number of stage 1, 2, and 4s (stage 4 special) neuroblastomas since the introduction of mass screening. However, there has been no significant change in the number of stage 3 or 4 diseases diagnosed in older children. According to the japan Children's Cancer Registry, pelvic origin neuroblastoma, which has been noted to have spontaneous regression, was more frequent in the primary tumors detected by mass screening when compared with those presenting clinically. During preparation of this manuscript another case of bladder dome neuroblastoma was detected by urinary vanillylmandelic acid screening of 6-month-old infants for neuroblastoma in japan. CONCLUSION: These extremely rare cases of neuroblastoma of the urinary bladder involved children younger than 1 year of age and were incidentally detected by routine physical examination or mass screening. This raises the question of whether these tumors might have regressed spontaneously had they gone undetected and untreated.- - - - - - - - - - ranking = 0.062431374927282keywords = cord (Clic here for more details about this article) |
4/125. Primary cerebral neuroblastoma. A clinicopathological study of 35 cases.A series of 35 primary cerebral neuroblastoma is reported. These rare tumours occur most often in children in the first half of the first decade. Grossly the tumors are often massive, discrete, lobular, firm and cystic. Histologically three variants, largely determined by the extent and distribution of the fibrous connective tissue stroma, are recognized: (1) a classical variant, which most resembles the peripheral neuroblastoma and is characterized by a high frequency of Homer Wright rosettes and a relatively high frequency of ganglionic differentiation; (2) a desmoplastic variant, which is characterized by an intense connective tissue stroma; and (3) a transitional variant, in which both the classical and the desmoplastic features may be present within the same case, either concurrently or consecutively. Both the desmoplastic and the transitional forms are less likely to exhibit differentiation to mature ganglion cells, but the importance of identifying the primitive cell elements as neuroblasts is emphasized. With rare exceptions, this can be established only by specific silver impregnations on frozen material. Occasionally the direction of growth may be largely leptomeningeal. Seven illustrative clinical histories with pathological correlations are described. The over-all clinical behaviour of these tumours is that of malignant neuroepithelial neoplasms, characterized by a high recurrence rate. recurrence may, however, be a late development, in some cases occurring five or seven years after apparently successful surgical removal. The tumour shows shows a high incidence of metastatic spread, almost 40 per cent of the cases examined at autopsy having disseminated in the cerebrospinal pathways. Exceptionally, extraneural metastases may also develop. However, long post-operative survival occasionally occurs, and the subsequent clinical course is not always predictable in the individual case. The differential diagnosis is briefly discussed. The cellular nature of the tumour and its biological behaviour recall those of the cerebellar medulloblastoma. Post-operative radiation to the entire neuraxis should be considered for these neoplasms.- - - - - - - - - - ranking = 0.17061825285749keywords = spinal (Clic here for more details about this article) |
5/125. Neuroblastoma with spermatic cord metastasis in a child: sonographic findings.We report a case of neuroblastoma in a patient who had no involvement of the spermatic cord at diagnosis but who developed spermatic cord metastasis 2 months later. The metastasis appeared on sonography as a hypoechoic, highly vascular, fusiform, hard, 14x10x7 mm mass located in the right inguinal canal and extending into the scrotum. The diagnosis of spermatic cord metastasis was confirmed by resection and histopathologic examination. We recommend that the scrotum and spermatic cord be evaluated by high-resolution sonography in children with neuroblastoma, both at the time of diagnosis and during follow-up.- - - - - - - - - - ranking = 0.49945099941826keywords = cord (Clic here for more details about this article) |
6/125. Posterior mediastinal capillary hemangioma with extradural extension resembling neuroblastoma.We present two patients with posterior mediastinal capillary hemangiomas that were paraspinal and had intraspinal extension. Computed tomography demonstrated the strikingly hypervascular nature of these tumors, distinguishing them from neuroblastoma.- - - - - - - - - - ranking = 0.34123650571498keywords = spinal (Clic here for more details about this article) |
7/125. Neuroblastoma associated with seizures and arrested development.Two unrelated cases of childhood peripheral neuroblastoma associated with infantile seizures and developmental problems (but without opsoclonus-myoclonus) are presented. The considerable body of evidence supporting the view that the opsoclonus-myoclonus syndrome associated with neuroblastoma has an immunologic basis is reviewed. patients with neuroblastoma and opsoclonus-myoclonus syndrome commonly have subsequent developmental problems and, rarely, may have seizures. The authors postulate that the seizures and developmental problems in their two patients may result from an immunologic mechanism similar to that suggested for the opsoclonus-myoclonus syndrome of neuroblastoma. The only laboratory evidence to support an immunologic mechanism in these two patients was the presence of raised cerebrospinal fluid immunoglobulins in Patient 2. Specific antineuronal antibody tests in Patient 2 were negative. It is therefore possible that the association reported in these two unrelated cases is coincidental. However, reasonably extensive investigations did not uncover an alternative etiology for the presence of the seizures and developmental delay.- - - - - - - - - - ranking = 0.17061825285749keywords = spinal (Clic here for more details about this article) |
8/125. incidence of occult cancer in children presenting with musculoskeletal symptoms: a 10-year survey in a pediatric rheumatology unit.OBJECTIVES: To assess the frequency and types of cancer found in children presenting to our Unit with musculoskeletal symptoms over a 10-year period. methods: The medical records of patients with musculoskeletal symptoms and a final diagnosis of cancer were reviewed. In each case age, gender, presenting symptoms, laboratory data, diagnostic procedures, provisional and final diagnoses, and time between clinical onset and correct diagnosis were reviewed. RESULTS: An underlying neoplasia was found in 10 of 1,254 patients (<1%) complaining of musculoskeletal symptoms. The types of malignancies found included acute lymphocytic leukemia (ALL) (6 cases), lymphoma (2 cases), neuroblastoma (1 case), and Ewing's sarcoma (1 case). The mean time between disease onset and final diagnosis was 3.2 months. The most common presenting feature was monoarthritis, involving the larger joints such as the elbows, knees or ankles. Juvenile idiopathic arthritis (JIA) was the most frequent provisional diagnosis. In the preliminary hematologic evaluation, eight patients had an increased erythrocyte sedimentation rate (ESR) or c-reactive protein (CRP) value. White blood cell (WBC) count was normal in almost all children, with a normal differential count. Lactic dehydrogenase (LDH) was raised in all children. bone marrow aspirates and lymph node or bone biopsies were necessary to reach the final diagnosis. CONCLUSIONS: A malignancy should always be excluded in children with musculoskeletal symptoms, especially when the clinical pattern is not characteristic of a specific rheumatic disease. Routine laboratory tests may be misleading. The simultaneous presence of high LDH or alpha-hydroxybutyric dehydrogenase (alpha-HBDH) levels and raised ESR or CRP, even with normal blood cell counts, should lead to additional investigations. RELEVANCE: All patients presenting with arthritis or other musculoskeletal symptoms should have a thorough clinical examination. Disproportionate pain levels and an atypical pattern of "arthritis," especially in the presence of systemic manifestations, suggest a possible underlying malignancy.- - - - - - - - - - ranking = 0.062431374927282keywords = cord (Clic here for more details about this article) |
9/125. Supratentorial primitive neuroectodermal tumours: diffusion-weighted MRI.We report the clinical and pathological findings of supratentorial primitive neuroectodermal tumours (PNETs). These are rare, poorly differentiated, highly malignant neoplasms occurring primarily in young individuals. They frequently show dissemination to the spinal cord and sometimes also beyond neuraxis. Preoperative radiological diagnosis is difficult, due to the nonspecific CT and MRI characteristics. Our findings indicate that diffusion-weighted imaging (DWI) can be used to show the solid portion of the tumour preoperatively and to monitor postsurgical recovery. We describe the MRI findings in three patients with histologically confirmed supratentorial PNET, focussing on the role of DWI for improving the specificity of radiological diagnosis.- - - - - - - - - - ranking = 0.82938174714251keywords = spinal cord, spinal, cord (Clic here for more details about this article) |
10/125. Preservation of olfaction in anterior skull base surgery.OBJECTIVES/HYPOTHESIS: In selected unilateral tumors and defects of the anterior skull base, the preservation of contralateral olfaction is achievable through a localized subcranial approach without compromising surgical objectives of resection or repair. STUDY DESIGN: Description of a functional adaptation of anterior skull base surgical techniques through a retrospective patient series. methods: Nine patients underwent anterior skull base surgery for unilateral cribriform plate disease including four malignant and two benign tumors, two encephaloceles, and one iatrogenic cribriform injury with cerebrospinal rhinorrhea. All nine patients consented to a localized subcranial approach to the anterior skull base to preserve the contralateral olfactory nerves. In four patients with benign disease a portion of the ipsilateral nerves was additionally conserved. Postoperative olfaction was assessed objectively with a commercially available smell test. Indications, technique, results, and complications are reported and discussed. RESULTS: All patients had eradication of disease with preservation of functional olfaction CONCLUSIONS: Conservation of olfaction is possible in selected cases of anterior skull base surgery when the lesion is unilaterally confined.- - - - - - - - - - ranking = 0.17061825285749keywords = spinal (Clic here for more details about this article) |
| | Next -> |