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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 199 records · Page 11Linked to original sources
PubMed1997

[Mediastinal neuroectodermal tumor. Apropos of a case].

Peripheral malignant neuroectodermal tumors are rare and aggressive small-cell tumors seen predominantly in children and young adults. Mediastinal location is very uncommon. A mediastinal primitive neuroectodermal tumor was diagnosed in a 27-year-old man. Despite treatment combining surgery, radiotherapy and chemotherapy, disease recurred locally along with lungs, spinal and epidural metastases, leading to death. CT and MRI examinations show heterogeneous masses with necrosis, hemorrhage and intense enhancement after Gadolinium injection. The CT and MRI findings are not specific and the diagnosis is based on pathologic, immunohistochemical and electron microscopic features. Although uncommon, peripheral neuroectodermal tumor should be considered in the differential diagnosis of posterior mediastinal masses in children and young adults.

Adult↗
PubMed1982

Congenital primitive neuroectodermal tumor (neuroepithelioma) of the chest wall.

Primitive neuroectodermal tumor (neuroepithelioma) is a relatively common central nervous system tumor in children. Those arising from a peripheral nerve are extremely rare in childhood. There is only one reported case in 6-year-old where the tumor arose from the sciatic nerve. A case of neuroectodermal tumor of the chest wall, arising from the intercostal nerve, in a newborn is presented. The tumor metastasized to the brain. Prominent Homer-Wright rosettes, with central eosinophilic fibrillar substance similar to that seen in neuroepithelioma of the central nervous system, were present in the primary tumor and brain metastases. Ultrastructure, as revealed by transmission electron microscopy, is also described.

Brain Neoplasms↗
PubMed1986

Evidence for neural origin and PAS-positive variants of the malignant small cell tumor of thoracopulmonary region ("Askin tumor").

The differential diagnoses of childhood and adolescent tumors composed of small round cells include a distinctive clinicopathological entity called malignant small cell tumor (MSCT) of the thoracopulmonary region in childhood. In the present study, 15 such tumors that fulfilled the criteria by Askin et al. were examined for features of possible neural differentiation by light and electron microscopy (EM). With hematoxylin-eosin stain (H&E) the tumors were made up of small undifferentiated cells; rosette formation was noticed in four cases. By immunohistochemistry all 15 tumors were positive for neuron/specific enolase (NSE), which is a specific marker for neural elements and their tumors including neuroblastomas. Ten of 15 MSCT had positive PAS staining. Ultrastructurally dense core (neurosecretory) granules and cell processes indicative of neuronal differentiation could be recognized in 10 of 14 tumors. The dense core granules were often atypical. Filamentous cytoskeleton, never observed in Ewing's sarcoma, was often present. Based on the current results, MSCT of the thoracopulmonary region can be considered a peripheral neuroectodermal tumor with the possible origin in intercostal nerves. MSCTs are generally misdiagnosed as Ewing's sarcoma due to their primitive appearance in H&E sections and their periodic acid-Schiff positivity. NSE immunostaining, preferably augmented by electron microscopy, is necessary for their correct diagnosis.

Adolescent↗
PubMed1994

Multiple primitive neuroectodermal tumors.

Primitive neuroectodermal tumors are rare, highly aggressive neoplasms that affect both sexes and occur in all age groups. They are a large group of neoplasms with neuroepithelial differentiation, including cutaneous neuroblastomas. Histopathologically they are characterized by a rather uniform population of small, dark cells, with or without Homer Wright rosettes. Immunohistochemically they stain for one or more of the reputed neural markers. Ultrastructurally they show interdigitating processes containing neurosecretory granules, intermediate filaments, and microtubules. We describe a patient with unusual multiple peripheral neuroectodermal tumors localized to the skin, with benign biologic behavior. The patient related the appearance of new lesions for several years without evidence of visceral dissemination or systemic complications. The histopathologic, immunohistochemical, and ultrastructural findings support the diagnosis of a peripheral neuroectodermal tumor.

Diagnosis, Differential↗
PubMed1987

Extraskeletal Ewing's sarcoma.

A well documented case of extraskeletal Ewing's sarcoma arising in a finger of a 16-year-old man was studied by light microscopy, immunohistochemistry and electron microscopy. The differential diagnoses of similar "round cell neoplasms" are discussed. Emphasis is placed on the distinction between extraskeletal Ewing's sarcoma and peripheral neuroepithelioma.

Adolescent↗
PubMed1987

Experimental evidence for a neural origin of Ewing's sarcoma of bone.

The histogenesis of Ewing's sarcoma remains unknown. Recent studies have suggested a relationship to an unusual form of childhood neural tumor, often termed peripheral neuroepithelioma or primitive neuroectodermal tumor. Five Ewing's sarcoma tumor cell lines were studied for evidence of a neural phenotype. Under normal culture conditions, no morphologic evidence of neural differentiation was detected. Treatment with retinoic acid, an agent known to induce marked neural differentiation in neuroblastoma, had no demonstrable effect. Treatment with either cyclic AMP or TPA, in contrast, induced pronounced morphologic evidence of neural differentiation. Cells developed elongate processes with varicosities by phase-contrast microscopy; filaments, microtubules, and uraniffin-positive dense core granules were present by electron microscopy. Three neural markers (NSE, NFTP, and cholinesterase) were absent or barely detectable in untreated cells, but became abundant after treatment. These results provide convincing evidence for a neural histogenesis of Ewing's sarcoma. They also suggest a close relationship between Ewing's sarcoma and peripheral neural tumors, including the chest wall tumor described by Askin, but only a distant relationship to neuroblastoma.

Adolescent↗
PubMed1993

Cytogenetics of Askin's tumour. Case report and review of the literature.

The eleventh cytogenetically analyzed Askin's tumour, diagnosed in a two-year-old girl, is reported. Chromosomal analysis revealed a pseudodiploid karyotype of tumour cells with translocations of t(11;22)(q24;q12) and der(4)t(2;4)(q24;q35). The observed t(11;22)(q24;q12) is not only a unique characteristic of all cytogenetically analyzed Askin's tumours but it also occurs in 92-100% of peripheral neuroepithelioma and of Ewing's sarcoma, irrespective of its osseous or extraosseous localization. This genetical similarity further supports a nosological concept according to which Askin's tumour, Ewing's sarcoma and peripheral neuroepithelioma represent phenotypic variations of the same tumour, namely the peripheral primitive neuroectodermal tumour.

Child, Preschool↗