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11/92. 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 = 1 keywords = neoplasm (Clic here for more details about this article) | 12/92. sciatica due to malignant nerve sheath tumour of sciatic nerve in the thigh. Malignant peripheral nerve sheath tumour (MPNST) is a rare malignant neoplasm arising from the supportive non-neural component of the peripheral nerves. An unusual case of pain and weakness of the foot and calf muscles due to a giant MPNST of the sciatic nerve in the posterior compartment of the thigh is presented. The patient was already investigated as a case of sciatica due to a lumbar disc disease with a negative magnetic resonance imaging and then unsuccessfully operated elsewhere twice, with a misdiagnosis of tarsal tunnel syndrome. Neurosurgical referral prompted a diagnostic magnetic resonance study of the thigh, revealing the lesion, which was completely excised microsurgically with total relief in the pain and partial improvement in the weakness and sensations in the sole of the foot.
- - - - - - - - - - ranking = 0.5 keywords = neoplasm (Clic here for more details about this article) | 13/92. Primary intrathoracic meningioma: histopathological, immunohistochemical and ultrastructural study of two cases. Meningiomas are common, usually benign slow-growing neoplasms of the central nervous system thought to arise from meningocytes capping arachnoid villi. Primary ectopic meningiomas are exceedingly rare extracranial and extraspinal tumors of controversial origin; they are usually limited to the head and neck region or to the paravertebral soft tissues. Only one mediastinal ectopic meningioma and few pulmonary ectopic meningiomas have been described in the literature until now. Because of their rarity and their intriguing pathogenesis, we report here a second case of primary mediastinal meningioma and an additional case of primary pulmonary meningioma. Their possible origin and differential diagnosis are discussed.
- - - - - - - - - - ranking = 0.5 keywords = neoplasm (Clic here for more details about this article) | 14/92. Malignant granular cell tumor of the lateral femoral cutaneous nerve: report of a case with cytogenetic analysis. Malignant granular cell tumors (MGCTs) are rare neoplasms of uncertain histogenesis. We report a case of MGCT involving a peripheral nerve with peritoneal and omental dissemination in which cytogenetic findings are available. Our results show that MGCTs share some cytogenetic abnormalities with malignant peripheral nerve sheath tumors (MPNSTs), supporting the hypothesis that they may represent histogenetically related lesions.
- - - - - - - - - - ranking = 0.5 keywords = neoplasm (Clic here for more details about this article) | 15/92. Second malignancies in children with neuroblastoma after combined treatment with 131I-metaiodobenzylguanidine. BACKGROUND: (131)I-metaiodobenzylguanidine ((131)I-MIBG) is selectively taken up by cells of neural crest origin, allowing targeted radiotherapy of tumors such as neuroblastoma (NB) and pheochromocytoma. radiotherapy may provide additional benefits in the treatment of NB, with moderate side effects such as hematologic and thyroid toxicity. However, with longer follow-up, other complications might occur. We describe our experience with second cancers occurring in children treated with (131)I-MIBG and chemotherapy. methods: The clinical records of 119 consecutive NB cases treated with (131)I-MIBG at a single institution between 1984 and 2001 were reviewed for the occurrence of a second malignant neoplasm (SMN). RESULTS: Overall, five cases of SMN occurred in the study patients. In particular, two cases of myeloid leukemia, one of angiomatous fibrous histiocytoma, one of malignant schwannoma, and one case of rhabdomyosarcoma were detected. The schwannoma and the rhabdomyosarcoma developed within the residual neuroblastic mass after first-line therapy. CONCLUSIONS: Should (131)I-MIBG treatment become more broadly employed in the therapeutic strategy for neuroblastoma, the risk of second cancer will have to be taken into consideration. The organization of an international registry of subjects treated with (131)I-MIBG might better define the frequency and features of second malignancies following this radiometabolic approach.
- - - - - - - - - - ranking = 0.5 keywords = neoplasm (Clic here for more details about this article) | 16/92. Multiple endocrine adenomas in a patient with the Maffucci syndrome. A patient with multiple cutaneous hemangiomas and skeletal dyschondroplasia (the Maffucci syndrome) was found to have a pituitary chromophobe adenoma, a parathyroid adenoma and two other neoplasms. The presence of two endocrine tumors suggested the syndrome of multiple endocrine adenomatosis, and raised the issue of an etiologic relationship between this disease and the Maffucci syndrome. Dyschondroplasia, however, has no known influence on the secretion of parathyroid hormone or any of the pituitary hormones. The Maffucci syndrome is associated with a high incidence of malignancy, but it involves primarily mesodermal derivatives whereas multiple endocrine adenomatosis affects tissues of ectodermal origin. The association of the two in our patient is probably fortuitous.
- - - - - - - - - - ranking = 0.5 keywords = neoplasm (Clic here for more details about this article) | Central neurocytoma represents a rare neoplasm of the central nervous system with advanced neurocytic and sometimes focal lipomatous differentiation, a low proliferative potential and a favorable prognosis depending on the efficiency of surgical resection. This entity has been described as an intraventricular tumor near the foramen Monroi. Here, we report a case of a 21-year-old male with peripheral neurocytoma. Using computed tomography, a tumor of unknown origin was located behind the bladder. After complete surgical resection of the tumor, histologically small uniform cells, zones of fibrillarity and neuropil-like islands were seen. immunohistochemistry revealed positivity for the neuronal markers synaptophysin, neuron-specific enolase and neurofilaments. vimentin, pan-keratin, desmin, chromogranin, CD-99 and glial fibrillary acidic protein were immuno-negative. A low proliferation rate (1-2%) was found. Several case reports described extraventricular central neurocytomas. A sole publication documented a peripheral neurocytoma arising within a mature cystic teratoma of the ovary. To our knowledge, this is the second reported case of a neurocytoma outside the central nervous system, indicating that this entity may also occur infrequently in peripheral tissues.
- - - - - - - - - - ranking = 0.5 keywords = neoplasm (Clic here for more details about this article) | Glomus tumours are the most common primary neoplasms of the middle ear, typically benign and slowly progressive. Pulsatile tinnitus and ipsilateral hearing loss are the most common symptoms at presentation by far; otalgia, aural fullness and otorrhoea being less frequent. A case of primary glomus tympanicum presenting with recurrent epistaxis, previously unreported in the literature, is described and associated imaging presented.
- - - - - - - - - - ranking = 0.5 keywords = neoplasm (Clic here for more details about this article) | 19/92. Granular cells in a cellular neurilemmoma. In this article, we describe a case of a cellular neurilemmoma with focal granular cell elements. The schwann cells showed S100 and Leu-7 immunoreactivity. The granular cells were periodic acid-Schiff-positive, diastase resistant with strong S100 immunoreactivity. Electron microscopy showed electron dense, heterogeneous granules in the granular cells and interdigitating processes and elongated nuclei of the schwann cells. Although many granular cell tumors are believed to be derived from schwann cells, neoplasms with both elements are rarely presented.
- - - - - - - - - - ranking = 0.5 keywords = neoplasm (Clic here for more details about this article) | 20/92. Primitive neuroepithelial tumors with vermiform processes (filiform neuroepithelial tumors). Immunocytochemical and ultrastructural study of 2 cases. Two unique, poorly-differentiated neuroepithelial tumors are described, one in a 35-year-old woman with an anterior mediastinal tumor and one in a 71-year-old woman with a left femoral mass. Immunocytochemical stains demonstrated Neuron specific enolase in both tumors and Chromogranin in one. Electron microscopy showed the cells of both neoplasms to contain abundant, thick, vermiform, organelle-free processes, previously described solely in large cell lymphomas. Rare dense-core granules were present, and very few processes were suggestive of neurites. These observations enlarge the spectrum of poorly differentiated neuroepithelial tumors.
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