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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 973 records · Page 54Linked to original sources
PubMed2001

[Primary uterine and vulvar Ewing's sarcoma/peripheral neuroectodermal tumors in children: two unusual locations].

Two genital cases of Ewing's sarcoma/peripheral neuroectodermal tumor in 14 and 15 year old teenagers are reported. They arose in uteri and vulva, as small round cell tumors with some rosette-like formations and intense membranous immunoreactivity for CD99. The EWS/FLI-1 transcript was present in the vulvar tumor. Under chemotherapy, uterine tumor outcome was fatal within 9 months. The vulvar lesion was treated by surgery and chemotherapy, without relapse after 7 months. Likewise to these 2 cases, the literature seems to indicate different prognosis for uterine and vulvar tumors.

12E7 Antigen↗
PubMed2001

Primitive neuroectodermal tumor of the uterus. A case report.

BACKGROUND: Primitive neuroectodermal tumors (PNETs) of the uterus are very rare. Even preoperative curettage specimen morphology does not permit establishing a proper histogenetic diagnosis. CASE: A 16-year-old woman was admitted to the hospital because of metrorrhagia of three months' duration. The curettage specimen was interpreted as poorly differentiated sarcoma. The patient was operated on, and a total abdominal hysterectomy with bilateral salpingo-oophorectomy with omentectomy was performed. Histologically the tumor was composed of uniform, rounded, oval and sometimes spindle shaped cells with a narrow rim of eosinophilic cytoplasm. The cells were positive for neurogenic marker protein gene product, neuron-specific enolase and Ewing's sarcoma-related HBA-71. All cells were uniformly negative for Ber EP4 monoclonal antibody recognizing an epithelium-specific surface antigen. A diagnosis of PNET was rendered. The patient received combined therapy, external radiation to the pelvis and chemotherapy. Four years later she was alive, without signs of recurrent tumor. CONCLUSION: In spite of the generally recognized aggressive behavior of PNET, it can have a long disease-free survival rate when appropriately treated.

Adolescent↗
PubMed2001

Cytodiagnosis of retroperitoneal primitive neuroectodermal tumour (PNET)--a case report.

A rare case of Retroperitoneal Primitive Neuro Ectodermal Tumour (PNET) in thirty years old female is presented. Fine needle aspiration cytology (FNAC) characterised by small round cells with scanty cytoplasm, poorly to well formed rosettes with neurofibrillary background; whereas periodic acid-schiff (PAS) rarely react with Primitive Neuro Ectodermal Tumour (PNET), prompted the diagnosis of PNET at cytology itself. Cytological appearances of PNET have been reviewed and the various differential diagnoses are discussed.

Adult↗
PubMed1975

[Remote prognosis in esthesioneuromas (apropos of 11 cases)].

Research was carried out into a group of 11 patients with tumours of the olfactory placode or aesthesioneuromas observed and treated by the authors (10 of them unpublished cases). The authors recall the histological characteristics which, theoretically, distinguish aesthesioneuroepithelioma from aesthesioneurocytoma and aesthesioneuroblastoma and the risk of mistaken interpretation due to confusion with other types of tumour. The following conclusions emerged from this research : 1) Aesthesioneuroma is indisputably a radiosensitive tumour as has been proved by the long-term cures obtained either by irradiation alone or by irradiation after incomplete surgery. 2) However it is exceptional for diagnosis to be made merely by intranasal biopsy. In all doubtful cases, surgical investigation is necessary. This requires a broad para-latero nasal approach route which is the only one affording accurate anatomical localisation of the lesions and complete ablation of the tumour including, where necessary, removal of the lamina cribrosa.

Adolescent↗
PubMed2002

[Askin tumor: two cases].

Askin tumor is uncommon, usually observed in young subjects. We report two cases of Askin tumor observed in patients aged 15 and 27 years who were hospitalized for a painful tumefaction of the chest wall with pleural involvement in one patient. We reviewed progress in patient management which has resulted from better understanding of the natural history of Askin tumor and earlier diagnosis due to advances in imaging and immunohistochemistry techniques as well as cytogenetics. A number of points remain controversial.

Adolescent↗
PubMedDate not supplied

MAP2 and nestin co-expression in dysembryoplastic neuroepithelial tumors.

BACKGROUND: The ontogeny and maturity of neurons and oligodendroglia-like cells (OLC) found in dysembryoplastic neuroepithelial tumors (DNT) remains controversial. A developmental origin has been proposed based on the close association to cortical dysplasia and the benign microscopic and clinical course. Our goal was to characterize the expression of nestin, a neuroepithelial precursor/stem cell antigen in DNT, along with other pathological and clinical features of this entity. METHODS: The clinical and operative features of 13 surgical specimens meeting the histological criteria for DNT were reviewed. Nestin, microtubule-associated protein 2 (MAP2), neurofilament (NF) and glial fibrillary acidic protein (GFAP) were examined by immunohistochemistry and confocal scanning laser microscopy. RESULTS: Select neuronal cells in all cases demonstrated strong MAP2 immunoreactivity. Nestin-positive cells of neuronal morphology were found in 6 cases. OLC demonstrated frequent selective staining for MAP2, GFAP and nestin. Confocal microscopy demonstrated numerous examples of cells co-expressing nestin and MAP2. CONCLUSIONS: Our study suggests that OLCs represent a united population of immature neuronal (nestin + MAP2) and glial (GFAP) phenotypes. Larger, morphologically recognizable neurons also showed occasional co-expression of nestin and MAP2, suggesting a degree of dysmaturity in common with their OLC counterparts. The apparent mixed lineage of OLCs lends support to theories suggesting that DNTs arise from pluripotent neuroepithelial cells.

Adolescent↗
PubMed1976

[Esthesioblastoma of the nose].

Morphological investigations of biopsy materials in 8 cases of esthesioblastomas of the nasal cavity were carried out. The three morphological variants of the tumour are described: esthesioneuroepithelioma, esthesioneurocytoma, and esthesioneuroblastoma. Rare occurrence of esthesioblastomas, peculiar features of their histological structure and difficulties of diagnosis in histological verification are emphasized. Lymphosarcoma is recommended to be differentiated from nondifferentiated cancer and sympathoblastoma. An important differentially-diagnostic procedure is considered to be a positive reaction to argentation (for identifying neurofibrils).

Adolescent↗
PubMed2003

Glucosylceramide synthase and its functional interaction with RTN-1C regulate chemotherapeutic-induced apoptosis in neuroepithelioma cells.

Glucosylceramide synthase (GCS), the key enzyme in the biosynthesis of glycosphingolipids, has been implicated in many biological phenomena, including multidrug resistance. GCS inhibition, by both antisense and the specific inhibitor (D-threo)-1-phenyl-2-decanoylamino-3-morpholino-1-propanol (PDMP), results in a drastic decrease of apoptosis induced by the p53-independent chemotherapeutic agent N-(4-hydroxyphenyl)retinamide in neuroepithelioma cells. By using the yeast two-hybrid system, we have identified a member of the reticulon (RTN) family (RTN-1C) as the major GCS-protein partner. Interestingly, RTN-1C not only interacts with GCS at Golgi/ER interface but also modulates its catalytic activity in situ. In fact, overexpression of RTN-1C sensitizes CHP-100 cells to fenretinide-induced apoptosis. These findings demonstrate a novel p53-independent pathway of apoptosis regulated by Golgi/endoplasmic reticulum protein interactions, which is relevant for cancer combined therapy.

Amino Acid Sequence↗