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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed1991

Immunohistochemical analysis of Ewing's sarcoma cell surface antigen p30/32MIC2.

Monoclonal antibody (MAb) HBA71, which was raised against Ewing's sarcoma cells, recognizes a cell-surface glycoprotein, p30/32MIC2, that is encoded by the MIC2 gene in the pseudoautosomal region of human chromosomes X and Y. This immunohistochemical study evaluates the specificity and sensitivity of MAb HBA71 for tumor diagnosis. Frozen and paraffin-embedded tissues of more than 300 tumors of diverse histologic type, including more than 100 small round cell tumors of childhood and adolescence, were tested with this MAb by the avidin-biotin immunoperoxidase procedure. The authors found HBA71 immunoreactivity in 61 of 63 Ewing's sarcomas studied and 9 of 11 primitive neuroectodermal tumors and peripheral neuroepitheliomas. HBA71-negative tumors included neuroblastomas (0 of 24), melanomas (0 of 13), an esthesioneuroblastoma, small cell osteosarcomas (0 of 2), a malignant ectomesenchymoma, desmoplastic SRCT (0 of 5), and medulloblastomas (0 of 5). Heterogeneous expression of HBA71 immunostaining was found in some embryonal rhabdomyosarcomas (3 of 14) and astrocytomas (4 of 7), and in a few neuroendocrine tumors (4 of 26), carcinomas (3 of 94), and lymphomas (6 of 30). Because Ewing's sarcomas are consistently HBA71 positive, the authors searched for antigen-positive normal cells that may represent precursors for these tumors; however, no obvious candidate for the elusive cell of origin for Ewing's sarcoma was identified in the normal fetal tissues tested. Their findings indicate that HBA71 is a highly restricted cell-surface antigen of Ewing's sarcomas and primitive neuroectodermal tumors, and immunohistochemistry employing this antibody may be of value in the differential diagnosis of selected small round cell tumors in childhood and adolescence.

12E7 Antigen↗

PubMed1994

Peripheral neuroepithelioma presenting as a spinal cord tumour.

A case of spinal cord tumour in a 41-year-old man is presented. The initial diagnosis was of metastatic small cell tumour. One year postoperatively he presented with a left cerebellopontine angle tumour which resolved with radiotherapy. Three years after initial presentation he remains well with no evidence of a primary tumour. Histological review shows the tumour to be a peripheral neuroepithelioma, which has not previously been reported to affect the spinal cord. The differential diagnosis of small cell spine tumours is discussed.

Adult↗

PubMed1987

Treatment of peripheral neuroepithelioma in children and young adults.

Seventeen patients with peripheral neuroepithelioma were treated with an intensive chemotherapy regimen of vincristine, Adriamycin (Adria Laboratories, Columbus, OH), and cyclophosphamide (VADRIAC) in combination with radiation therapy. Fifteen patients with stage III (seven) or stage IV (eight) at presentation were treated on a more intensive regimen including total body irradiation (TBI) (8 Gy). Two patients with stage I (one) or II (one) disease received a less intensive chemotherapy regimen of VADRIAC. Therapy was completed within 6 to 7 months in all patients. The disease arose in the chest wall in 12 patients, pelvis in three patients, and extremity in two patients. Sixteen of the 17 (94%) patients achieved a complete remission. With a median follow-up of 18 months, ten patients remain in complete remission with an actuarial survival of 68% and an actuarial relapse-free survival of 56% at 12 months. On the basis of our initial experience with this tumor, we believe that peripheral neuroepithelioma is a chemoresponsive and radioresponsive tumor.

Adolescent↗

PubMed1993

Peripheral neuroepithelioma of the soft tissues. A retrospective analysis of fifteen pediatric patients.

PURPOSE: The purpose of this study was to determine the clinical outcome for pediatric patients with peripheral neuroepithelioma treated with combined modality therapy and followed long enough to account for late relapses. PATIENTS AND METHODS: Fifteen patients, ages 3 3/12 to 19 10/12 years, with peripheral neuroepithelioma (median follow-up 91 months) were diagnosed at The Children's Hospital, Denver, Colorado over the period 1980-1989. All of these malignancies originated in the soft tissues. A critical review of these cases was performed with particular consideration given to the site and stage of the tumor and to the radiographic findings at presentation. Thirteen patients had bulk (> 5 cm in the greatest dimension) or metastatic disease. Four patients had primary tumors involving the chest wall. All patients received chemotherapy, which included at least doxorubicin, vincristine, and cyclophosphamide. Definitive surgical resections were performed on 13 of 15 patients. RESULTS: Five patients relapsed. Three were late relapses 24-44 months after diagnosis. Three of the five patients who relapsed had chest wall primaries. There were three deaths in this series due to peripheral neuroepithelioma and one due to sepsis. The overall survival was 68.5%, and the recurrence-free, survival 55.2%. Two patients with pulmonary relapses were treated with surgery and intensive chemotherapy and remain free of disease > 51 months following recurrence. CONCLUSIONS: Combined treatment modalities appear to be important for optimal outcome. This series represents the first report of favorable outcome of peripheral neuroepithelioma using a series with follow-up that is long enough to account for late relapses.

Adolescent↗

PubMed1981

Soft tissue sarcomas of childhood: the differential diagnostic dilemma of the small blue cell.

In its histologic features, embryonal rhabdomyosarcoma (RMS), the prototype of malignant soft tissue tumors in childhood, summarizes the problems associated with the diagnosis of this entire group of neoplasms. Many of the tumors that do not fulfill the criteria for RMS have been designated "sarcomas of uncertain histogenesis." The introduction of the concept of a soft tissue equivalent of Ewing's sarcoma may have eased the semantic anxiety without improving our conceptual understanding. It is thought that the embryonal RMS, Ewing's sarcoma, and other are derived from a primitive mesenchymal cell. Another separate category of "small blue cell tumors" are those which presumably originate from the primitive neuroepithelium. Some of the diagnostic terms applied to this category are "neuroepithelioma," "medulloepithelioma," and "peripheral neuroblastoma." Because most of these tumors are hormonally inactive and electron microscopy is not performed, the diagnosis is infrequently considered or proved. The recently described small cell tumor of thoracopulmonary origin is likely a malignant neuroepithelial neoplasm. Hematopoietic tumors, such as non-Hodgkin's malignant lymphomas, granulocytic sarcoma, and malignant histiocytosis, may appear in the soft tissues as the initial manifestation of these system diseases. A final group of malignant soft tissue tumors are the fibrohistiocytic ones with a biphasic pattern of small round cells and spindle cells. It now has become increasingly difficult for the pathologist to satisfy his clinical colleagues with the diagnosis of "undifferentiated malignant tumor" in a child.

Bone Neoplasms↗

PubMed1986

Primary intraorbital extraocular primitive neuroectodermal (neuroepithelial) tumour.

A case is reported of primary primitive neuroectodermal (neuroepithelial) tumour occurring in the right orbit of a 52-year-old man. The intraorbital extraocular location is unique for this kind of neoplasm. The malignant tumour was differentiated into primitive neuroepithelial, ependymal, and oligodendroglial cells. The neuroglia was identified by localisation of cytoplasmic glial fibrillary acidic protein. It is suggested that this primary intraorbital, extraocular, primitive neuroectodermal tumour with neuroglial differentiation is akin to the primitive neuroectodermal tumours of the neuraxis, including the cerebellar medulloblastomas, and to some peripheral nerve tumours known as malignant neuroepitheliomas, malignant ependymomas, and neuroblastomas. The ectomesenchymal remnant of the neural crest or ectopic neuroepithelium or both may have been the origin of the present tumour. Differentiation of the neuroectodermal component of the neural crest or heterotopic neuroepithelium or both would create a primitive neuroectodermal tumour with diverse neuroglial elements.

Humans↗

PubMed1988

Primary peripheral neuroepithelioma of the orbit with intracranial extension.

A 7-year-old girl with clinical signs limited to moderate unilateral proptosis of 2 weeks duration and ipsilateral disc edema was found to have a contiguous orbital and subfrontal intracranial tumor best characterized as a peripheral neuroepithelioma by recent studies. Previously this tumor would have been called an extraosseous Ewing's sarcoma. The tumor had a significant lobular component on either side of the orbital roof. The patient is still alive 24 months posttreatment with multimodal excisional surgery, radiation, and chemotherapy.

Child, Preschool↗

PubMed1987

Translocation t(11;22) in esthesioneuroblastoma.

Esthesioneuroblastoma is an exceedingly rare malignant neuroectodermal tumor of olfactory epithelium origin. We have performed cytogenetic studies on a tissue culture line established from a metastatic lesion in one such patient and observed that, among several chromosomal abnormalities, the cells contained a reciprocal translocation, t(11;22)(q24;q12), indistinguishable from the one that has been reported in Ewing's sarcoma, Askin's tumor, and peripheral neuroepithelioma. The uniqueness of this marker suggests that these tumors may be derived from the same type of stem cell, with varying histopathologic and clinical manifestations.

Adult↗

PubMed1987

[Malignant peripheral neuroepithelial tumors in childhood].

The different therapy modalities and course of disease of 42 patients with a malignant peripheral neuroepithelial tumor are retrospectively analyzed. Therapy was completed in 31 children, 25 of whom had a primary localized tumor and 6 a disseminated neuroepithelioma. 17 of the children with a localized illness survive disease free in contrast to no survivor in the group with a disseminated tumor. The effective chemotherapy combining vincristine, adriamycin, ifosfamide and actinomycin D must be complemented by an efficacious local control because malignant peripheral neuroepithelioma tend to recur locally. The prospective analysis of newly diagnosed patients and a standardized therapy regimen will show if malignant peripheral neuroepithelioma represents a distinct tumor entity, different from a Ewings-Sarcoma.

Adolescent↗

PubMed1991

Trisomy 8 in primary esthesioneuroblastoma.

Esthesioneuroblastoma is a rare malignancy believed to be derived from neuroectodermal stem cells within the olfactory epithelium. We have obtained the karyotype of a primary esthesioneuroblastoma following brief (7-day) in vitro culture, and have determined that the only observable cytogenetic anomaly is the presence of an additional chromosome 8. Previously, the karyotypes of two cell lines established from metastatic esthesioneuroblastomas have been reported to contain the equivalent of three copies of chromosome 8, in addition to other chromosomal aberrations, including the reciprocal translocation, t(11;22)(q24;q12). Examination of the cytogenetic literature suggests that an extra copy of chromosome 8 is a common occurrence in undifferentiated small round cell tumors frequently observed to carry the t(11;22), including esthesioneuroblastoma, Ewing's sarcoma, peripheral neuroepithelioma, Askin's tumor, and rhabdomyosarcoma. These data, combined with our report of a small round cell tumor with the karyotype 47,XY, +8, indicate that trisomy 8 may be a common phenomenon in these tumors, and may also provide some sort of selective advantage to these tumor types.

Chromosome Aberrations↗

PubMed1995

[A peripheral neuroectodermal tumor of the vulva].

A case of malignant peripheral neuroectodermal tumor occurring in the vulva of a 29-year-old woman is reported. It is a rare malignant tumor with aggressive biological behavior belonging to a wide group, the so-called small round-cell tumors. The patient is well and free of disease 8 months after surgery, chemotherapy and radiation therapy.

Adult↗

PubMed1988

Two new cases of primary peripheral neuroepithelioma of soft tissue with translocation t(11;22)(q24;q12).

A direct cytogenetic analysis was performed on tumor samples obtained from two patients with clinical and histopathologic diagnosis of primary peripheral neuroepithelioma. Both tumors presented the translocation t(11;22)(q24;q12). These results confirm those previously obtained by other authors and suggest a common histogenetic origin for this tumor with Ewing's sarcoma and Askin's tumor, in which the same translocation has been described.

Child↗

PubMed1986

Cytogenetic characterization of selected small round cell tumors of childhood.

Small, round, blue-cell tumors (SRCT), including rhabdomyosarcoma, Ewing's sarcoma of bone and soft tissue, mesenchymal chondrosarcoma, small cell osteosarcoma, hemangiopericytoma, neuroblastoma, peripheral neurectodermal tumor (peripheral neuroepithelioma of bone and soft tissue), and the malignant small cell tumor of the thoracopulmonary region described by Askin (Askin's tumor), are often difficult to distinguish by light microscopy. We have evaluated the cytogenetics of these tumors by studying 24 tumor explants in short-term culture and 22 tumor cell lines. In Ewing's sarcoma (a tumor of unknown histogenesis), and in peripheral neuroepithelioma and Askin's tumor (tumors with evidence of neural origin), we have observed an indistinguishable t(11;22) translocation.

Adolescent↗

PubMed1990

t(11;22) in three cases of peripheral neuroepithelioma.

Short-term cultures of three cases of peripheral neuroepitheliomas were studied using G-banding technique. A t(11;22)(q24;q12) was recognized in all three tumors. The results strengthen the hypothesis of a common histogenesis for neuroepithelioma, Ewing's sarcoma, and the Askin tumor.

Adolescent↗

PubMed1987

Differential protooncogene expression characterizes histopathologically indistinguishable tumors of the peripheral nervous system.

We have found highly predictable patterns of protooncogene expression in cell lines and tumor tissue of neuroblastoma (NB), a tumor of the peripheral nervous system (PNS). These patterns make it possible to recognize two different genetically definable subgroups among histopathologically indistinguishable tumors. Additionally, we have identified a difference in neurotransmitter biosynthetic enzyme activity in these two subgroups of NB. The patterns of protooncogene expression and neurotransmitter biosynthetic enzymes suggests that these tumors arise in different cells of the PNS.

Cell Line↗

PubMed1995

Malignant neuroepithelioma of the colon.

A rare case of malignant peripheral neuroepithelioma originating from the right colon is presented. The patient underwent right hemicolectomy followed by combination chemotherapy and there has been no evidence of tumour recurrence or metastases during three years of follow up. Emphasis is given to the extremely unusual location of this tumour and the favorable clinical outcome.

Adult↗

PubMed1989

Malignant neuroepithelioma (peripheral neuroblastoma). A case report.

Malignant neuroepithelioma is a rare neoplasm arising within the peripheral nervous system. It usually occurs in the lower extremities and may involve patients in any age group. In children younger than the age of five years, the tumor must be differentiated from a metastatic neuroblastoma; in adolescents and adults the tumor must be distinguished from other malignant round-cell tumors. The poor prognosis and the need for aggressive, combined surgical and chemotherapeutic modalities in treating this tumor necessitate a prompt and accurate diagnosis.

Adult↗

PubMed1990

Peripheral neuroectodermal tumour (neuroepithelioma) of the thoracopulmonary region: light and electron microscopic cytology.

We describe a case of peripheral neuroectodermal tumour of the thoracopulmonary region diagnosed by fine needle aspiration cytology. Light microscope examination revealed numerous small tumour cells arranged in large irregular aggregates occasionally delimiting empty vascular-type spaces or forming rosette-like structures. Cytologically the tumour cells showed a marked degree of nuclear anaplasia, scanty cytoplasm and long thin cytoplasmic processes. Electron microscopy revealed cells with characteristics of neuroectodermal differentiation.

Humans↗