1/93. Olfactory esthesioneuroblastoma.Esthesioneuroblastomas are malignant tumours, usually of slow, invasive growth and low metastatic rate. Skeletal destruction must be assumed to be common, but is often demonstrable only by tomographic sections. Clinically these tumours do not differ from others of the same site, so that the diagnosis has to be based upon the histological appearances. In the light microscope the presence of neurofibrils is considered a specific differential diagnostic factor against other small-cell malignant tumours in this region. There seems to be no basis for a morphological classification into previously described sub-groups, neither according to histogenetic, light, nor ultra-microscopic findings. The general degree of differentiation and the number of mitoses appear to be the main factors of prognostic significance. Combined irradiation and surgical excision is considered the best treatment.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
2/93. Malignant peripheral neuroectodermal tumor (MPNET) of the kidney.BACKGROUND: Malignant peripheral neuroectodermal tumors (MPNETs) are primitive neuroblastic tumors that arise, unlike neuroblastomas, outside the autonomic nervous system. A renal origin has been described in very few cases. CASE REPORT: We report the case of a young male patient with a large MPNET of the right kidney, studied with ultrasound and computed tomography before surgical resection. The main radiologic features, the microscopic appearance and the typical immunohistochemical findings, are described and discussed.- - - - - - - - - - ranking = 0.2keywords = neuroblastoma (Clic here for more details about this article) |
3/93. Radiographic features of olfactory neuroblastoma.Olfactory neuroblastomas are malignant, slowly growing neurogenic tumors originating from the olfactory mucosa of the nasal cavity. Fourteen patients with this tumor were evaluated and showed variable extension into adjacent structures such as the ethmoid and sphenoid sinuses, orbit, and anterior cranial cavity.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
4/93. 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 = neuroblastoma (Clic here for more details about this article) |
5/93. Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location.A peripheral primitive neuroectodermal tumor arising from the abdominopelvic cavity is reported in a 24-year-old young male without any previous remarkable pathology. He was admitted to our hospital with complaints of urinary symptoms (acute urinary retention) and mild intestinal occlusion that had been present for three months. physical examination and CT scan revealed a pelvic mass occupying the entire pelvic cavity. The diagnostic workup included a CT-guided biopsy which defined the tumor as a sarcomatous type. Radical surgery was performed including tumor resection, pelvic exenteration (bladder and prostate gland) and urinary and fecal diversion. Adjuvant chemotherapy (VAIA) was delivered once the histology was confirmed. We reviewed the available literature focusing on the varied nomenclature of this tumor (peripheral neuroepithelioma, Askin's tumor, Ewing's extraosseous tumor, peripheral adult neuroblastoma, peripheral primitive extracranial neuroectodermal tumor (PPNET), the clinical features, the role of diagnostic imaging techniques, pathologic assessment and controversial therapeutic management. In addition, the prognosis and survival of this rare condition were analyzed.- - - - - - - - - - ranking = 0.2keywords = neuroblastoma (Clic here for more details about this article) |
6/93. Peripheral primitive neuroectodermal tumor with neuronal and glial differentiation: report of a case arising in suprarenal region.It is well known that embryonal neuroectodermal tumors of the central nervous system (CNS) not infrequently display varying amount of neoplastic cells acquiring glial differentiation. In contrast, glial differentiation rarely occurs in primitive neuroectodermal tumors outside the CNS being documented in less than ten cases. The author presents herein a case of peripheral primitive neuroectodermal tumor with prominent glial differentiation identified by the presence of glial fibrillary acidic protein (GFAP) arising in the right suprarenal region of a 32-year-old man, histologically indistinguishable from an ordinary neuroblastoma.- - - - - - - - - - ranking = 0.2keywords = neuroblastoma (Clic here for more details about this article) |
7/93. Olfactory neuroblastoma.A case of olfactory neuroblastoma, type 1 (olfactory neurocytoma), in a 44-year-old man is described. The tumour grew extensively in the right nasal cavity with involvement of the maxillary sinus and ethmoidal region, but was radically removed at operation. The prognosis and treatment are discussed in the light of earlier literature.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
8/93. neuroblastoma-like schwannoma: a case report and review of the literature.We report a case of schwannoma (neurilemmoma) predominantly composed of small cells arranged in rosettes around central collagenous cores and discuss the differential diagnosis of this unusual variant. Schwannoma with giant rosettes, previously designated as neuroblastoma-like schwannoma by Goldblum et al., must be differentiated from neuroblastoma, peripheral neuroectodermal tumors, and malignant change in a schwannoma.- - - - - - - - - - ranking = 0.4keywords = neuroblastoma (Clic here for more details about this article) |
9/93. Sinonasal primitive neuroectodermal tumor arising in a long-term survivor of heritable unilateral retinoblastoma.BACKGROUND. patients who survive retinoblastoma (RB) are at risk for having second nonocular tumors, usually osteosarcomas, which often are fatal. Such patients almost always have bilateral RB. methods. This article reports a woman who, at the age of 1 year had been cured of a unilateral RB by radiation therapy and enucleation. Eighteen years later, she had a sinonasal small cell tumor that rapidly recurred and proved fatal 2 months after surgical debulking. The tumor was studied by immunohistochemistry and electron microscopic (EM) examination. RESULTS. It showed diffuse neuron-specific enolase staining, focal weak staining for chromogranin, synaptophysin, and Leu-7 monoclonal antibodies in paraffin-embedded, B5-fixed tissue (Great lakes Diagnostics, Troy, MI). EM study showed an undifferentiated primitive neuroectodermal tumor with many polyribosomes, simple cell junctions, few microtubules, and rare dense core granules. CONCLUSIONS. The combined immunohistochemical, ultrastructural, and clinical features of the tumor were interpreted as a sinonasal primitive neuroectodermal tumor with early neuronal differentiation. The tumor was pathologically indistinguishable from poorly differentiated olfactory neuroblastoma (ONB) and Ewing sarcoma.- - - - - - - - - - ranking = 0.2keywords = neuroblastoma (Clic here for more details about this article) |
10/93. radiation-induced bilateral cystic frontal lobe necroses demonstrating a fluid-blood level--case report.A 41-year-old male developed radiation-induced bilateral cystic frontal lobe necroses after irradiation for an olfactory neuroblastoma. Computed tomography (CT) and magnetic resonance (MR) imaging revealed the lesions, one containing a fluid-blood level on CT scans and niveau formation on MR images. It was proved to be a coagulated hematoma within the cyst at surgery. Such a fluid-blood level in a radiation-induced cyst has never been reported, although hemorrhage frequently accompanies delayed radiation necrosis. Positron emission tomography with multiple tracers may be useful in differentiating cerebral radiation necrosis from tumor recurrence, because of absence of abnormal tracer accumulation.- - - - - - - - - - ranking = 0.2keywords = neuroblastoma (Clic here for more details about this article) |
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