1/28. 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 = nature (Clic here for more details about this article) |
2/28. 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 = 1keywords = nature (Clic here for more details about this article) |
3/28. Metastatic ovarian neuroblastoma: a case report.Ovarian metastasis of childhood tumors is rare despite their aggressive nature. The childhood tumor that spreads to the ovary most frequently is the neuroblastoma. The clinical features and frequent bilaterality of ovarian metastatic tumors are helpful diagnostic features in many cases, but when the ovarian tumor is the presenting manifestation of the disease, differentiation from various primary ovarian tumors may be difficult. In this paper, a rare case of metastatic ovarian neuroblastoma is reported.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/28. Cerebellar neuroblastoma in an infant.A cerebellar neoplasm in an 8-month-old boy is reported. While this tumour was composed of small cells and had regions resembling desmoplastic medulloblastoma, it showed ultrastructural neuronal characteristics including bundles of microtubules in the cell processes, numerous synaptic vesicles, and occasional abortive or complete synapses. These characteristic features warranted the diagnosis of a neuroblastoma of the cerebellum. The nature of this rare intraparenchymal tumour in infants is also briefly discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/28. Intracranial esthesioneuroblastoma. A light and electron microscopic study.A 31 year-old black woman with unilateral facial dysesthesia was found to have an intracranial parasellar mass that extended into the sphenoid sinus. By light microscopy, the neoplasm appeared as nests of poorly differentiated neuroblasts in a finely fibrillary stroma and was diagnosed as an esthesineuroblastoma. Electron microscopy confirmed the neuroblastic nature of the tumor with demonstration of neurites containing neurofilaments and neurotubules, synapses and dense cored biogenic amine granules in perikarya and processes. This neoplasm was further characterized by the presence of numerous dystrophic axons that were evident only by electron microscopy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/28. rhabdomyosarcoma of the orbit in the newborn.A full-term black boy had a 2- to 3-cm, round, bluish mass on his right lower eye-lid at birth, later diagnosed as rhabdomyosarcoma. It was cystic in nature and extended into the nasal cavity. The tumor was initially classified as neuroblastoma. The child died eitht months later and necropsy report confirmed an original ophthalmologic pathology diagnosis of embryonal rhabdomyosarcoma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/28. Primary cerebral neuroblastoma: a case report and review.Primitive neuroepithelial tumors are the least common among supratentorial tumors in children. They pose great diagnostic difficulty, preoperatively as well as pathologically. Being quite rare, cerebral neuroblastomas are accepted as a distinct pathological entity, which differs from other neuroectodermal tumors, although clinically, radiologically, and morphologically at operation they are indistinguishable. Also differentiation between primary cerebral neuroblastoma and the other primitive neuroectodermal tumors may be difficult on light microscopy and be misleading. A 9-year-old girl with primary cerebral neuroblastoma who was initially misdiagnosed is reported. The other cases from the literature are reviewed and the nature of this rare tumor and its differential diagnosis is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/28. Clinicopathological experience with intraventricular neurocytomas.Intraventricular neurocytoma is a rare clinicopathological entity that has been recently described. We are reporting our experience with four diagnosed cases and the previously reported cases from the available literature are reviewed. These neoplasms occur mainly in young adults, and their histological diagnosis is difficult on light microscope, because they are almost indistinguishable from oligodendrogliomas. Nevertheless, the presence of tumoral cells arranged around nucleus-free fibrillary zones, resembling the large rosettes of pineocytomas and the immunohistochemical demonstration of synaptophysin are useful data for the pathological diagnosis. This diagnosis is easy on electron microscope, because it demonstrates the neuronal nature of tumoral cells. Regarding prognosis, we have found increasing evidence that these tumors are associated with a favourable course after surgery, and at present there is no clear evidence of the usefulness of radiotherapy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/28. Unusual chromaffin cell differentiation of a neuroblastoma after chemotherapy and radiotherapy: report of an autopsy case with immunohistochemical evaluations.Neuroblastomas are derived from neural crest cells that are capable of multilineage differentiation. Ganglionic neuronal differentiation of childhood neuroblastoma is seen with increasing age, leading to more differentiated tumors called ganglioneuroblastomas or ganglioneuromas. Despite the fact that neuroblastomas most often arise from the adrenal medulla, chromaffin-cell differentiation in neuroblastomas is not widely recognized. Tumor cells with a chromaffin-cell nature have only been detected using histochemical techniques in neuroblastoma cell lines or focal areas of certain in vivo tumors. We describe a neuroblastoma that exhibited an unusual differentiation toward chromaffin cells in a patient that had been treated with surgery, intensive chemotherapy, and radiotherapy. Although a biopsy specimen of the retroperitoneal primary tumor was extensively necrotic, possibly because of a previous chemotherapy regimen, surgically resected metastatic tumors of bilateral ovaries were viable and diagnosed as poorly differentiated neuroblastomas according to the International neuroblastoma pathology classification system. However, metastatic tumors of bilateral lungs examined at the time of autopsy exhibited histologic features similar to those of a pheochromocytoma/paraganglioma, and immunohistochemical examinations demonstrated that these tumors were composed of extra-adrenal chromaffin cells. This case confirms that neuroblastomas in childhood can transform into pheochromocytoma/paraganglioma-like tumors under special conditions.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/28. Cerebral neuroblastoma.A cerebral neuroblastoma removed surgically from a female child is presented. Electron microscopy showed numerous neuronal processes with growth cones which are a feature of the developing neurone. In addition there were some rosettes with distinct lumina. The luminal surfaces were covered with a smooth plasma membrane lacking any surface differentiation and the lateral surface of these cells had many cell junctions (terminal bars), reminiscent of a primitive neural tube. These features in a nerve cell tumor help to substantiate it as a neuroblastoma arising from immature rather than differentiated cells. The nature of this rare tumor is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
| | Next -> |