1/6. 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 = 1keywords = behaviour (Clic here for more details about this article) |
2/6. Metaiodobenzylguanidine total-body scintigraphy required for revealing occult neuroblastoma in opsoclonus-myoclonus syndrome.A girl aged 13 months presented with clinical features of subacute progressive ataxia leading to abasia, astasia, loss of unsupported sitting and apraxia. In addition, an opsoclonus, myoclonia and introvert behaviour developed. MRI of the brain, EEG, extensive tests of blood, urine and CSF showed no abnormalities. Based on clinical symptoms only, the diagnosis of opsoclonus-myoclonus syndrome (OMS) could be made. Under the suspicion of a neuroblastoma, further investigations were performed: a lateral and antero-posterior X-ray examination of the chest showed no tumour; neither did ultrasound of the abdomen. Concentrations of catecholamines and their metabolites in 24 h urine were normal and none of five tested anti-neuronal antibodies were found. However, a total-body scintigraphy with [I(123)] metaiodobenzylguanidine (MIBG) revealed a paravertebral hot spot on the left side compatible with a neural crest tumour. A MRI scan of the abdomen confirmed the supraphrenic lesion. [I(123)]MIBG uptake was sufficient for [I(131)]MIBG therapy. The response of the tumour to this therapy was favourable. The neurological symptoms of the patient slightly improved under steroid treatment. CONCLUSION: opsoclonus-myoclonus syndrome is a serious disease in infants, sometimes associated with occult neuroblastoma for which a full oncological work-up, including metaiodobenzylguanidine total-body scintigraphy is required.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
3/6. Long term survival with early childhood intracerebral tumours.Three young adults are described who presented during early childhood with a seizure disorder due to an underlying intracerebral tumour. The tumours were excised incompletely 14-19 years later. The histological findings were those of a temporal lobe benign capillary haemangioblastoma (Case 1), parietal lobe subependymoma (Case 2), and parietal lobe ganglioglioma (Case 3). After a mean period of follow-up of 22 years (range 18-26), only mild residual physical disabilities exist in each patient. These three cases illustrate (1) the need promptly to investigate children who present with focal seizures or whose EEG shows definite focal abnormalities, (2) the relevant investigations should include cranial CT or MRI in such cases and (3) that certain supratentorial tumours have a favourable outcome due to their benign biological behaviour rather than their location.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
4/6. Neuroblastoma in adults.Two cases of neuroblastoma are described in patients who were both more than 25 years old when their symptoms first appeared. The behaviour of this tumour appears less aggressive in adults than in children, but the cases presented illustrate its resistance to chemotherapy and radiotherapy, and surgery is suggested as the first line of treatment in adults, even if the growth cannot be removed completely.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
5/6. ganglioglioma of the spinal cord. A case with a long survival.A case of ganglioglioma or neuroastrocytoma of the spinal cord in a 78-year-old man is reported. diagnosis was based on the histological identification of the neoplastic cells and on the study of the architecture of the tumour. The presence of cellular anaplasia, sometimes of marked degree, and of small nests of infiltration suggested an initial malignant behaviour regarding both cellular types. A survey of the five reported cases of spinal ganglioglioma is presented.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |
6/6. Maturing neuroblastoma and ganglioneuroblastoma: a study of four cases with long survival.A retrospective study of four tumours of the peripheral nervous system originally diagnosed as neuroblastomas or ganglioneuroblastomas has been made. The patients have all survived since the original diagnosis. Evidence of residual tumour was seen only in one patient, and was associated with evidence of increasing histological differentiation over a period of time. Maturation of primitive cell types is known to alter the prognosis of neuroblastic tumours. It is suggested first, that even minimal evidence of maturation indicates a better prognosis. Second, that evidence of maturation may be uneven, small areas occurring in otherwise totally undifferentiated tumours, or, immature areas remaining even in apparently benign fully mature tumours; if these areas show maturation the prognosis remains good and the tumour should not be confused with a composite ganglioneuroblastoma. Third, that maturing tumours may behave in a malignant fashion, invade and metastasise, but but with maturation of the primary tumour and its metastases the ultimate prognosis may be better than the initial behaviour suggests.- - - - - - - - - - ranking = 0.5keywords = behaviour (Clic here for more details about this article) |