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1/54. Primitive neuroectodermal tumor in sinonasal region.

    An elderly woman having a nasal tumor diagnosed as alveolar rhabdomyosarcoma was referred to our hospital. Histological reexamination of the patient revealed that the tumor was composed of lobules of compactly arranged small round atypical cells. The atypical cells contained large oval to round vesicular nuclei, and scanty cytoplasm showing PAS positive glycogen material. The tumor cells were immunohistochemically positive for NSE, S-100 protein and vimentin, but showed negative reaction for myoglobin, desmin, EMA, keratin, LCA, chromogranin and MIC2. Ultrastructurally, the tumor cells contained a few cluster of glycogen particles and less organellae and filaments, and there were no cytoplasmic processes, neurosecretory granules or neurofilaments. Pathological findings suggested primitive neuroectodermal tumor resembling extraskeletal Ewing's sarcoma, but negative reaction for MIC2 immunohistochemically could not ascertain the diagnosis. Location of the tumor and age of the patient of the present case were unusual.
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ranking = 1
keywords = sarcoma
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2/54. Peripheral primitive neuroectodermal tumors of bone. A review of three cases.

    The recently individualized and still incompletely understood family of peripheral neuroectodermal tumors encompasses several tumor types, of which some have a predilection for bone. Immunocytochemical studies are essential and usually provide the diagnosis. A t(11;22)(q24;q12) translocation is present in over 80% of cases. Ewing's sarcoma is now viewed as an undifferentiated form of peripheral neuroectodermal tumor, and both tumors require management with combination chemotherapy plus radiation therapy and/or surgery. Contradictory data have been reported regarding the comparative prognosis of peripheral neuroectodermal tumor and Ewing's sarcoma, indicating a need for further studies in large numbers of patients. We illustrate these points by three case-reports, two in girls diagnosed with a vertebral primary at five and nine years of age, respectively, and one in a man diagnosed with a pelvic primary at 29 years of age.
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ranking = 1
keywords = sarcoma
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3/54. Cytokeratin immunoreactivity in Ewing's sarcoma: prevalence in 50 cases confirmed by molecular diagnostic studies.

    Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are characterized by the presence of the specific t(11;22)(q24;q12) or variants thereof, producing diagnostic EWS fusion transcripts. Cytokeratin has been reported sporadically to be expressed in some cases of ES/PNET. However, its prevalence has not been assessed systematically in a series of cases with confirmatory molecular or cytogenetic evidence of a diagnostic translocation. We present in detail three index patients in whom strong cytokeratin immunoreactivity was a confounding factor in the diagnosis. To establish further the prevalence of cytokeratin immunoreactivity in a series of well-characterized ES/PNET, we then performed immunohistochemical studies with antibodies CAM5.2 and AE1/AE3 on 50 cases of ES/PNET diagnosed at Memorial Sloan-Kettering Cancer Center in which molecular evidence of a specific ES/PNET-associated translocation were available. Immunoreactivity to cytokeratin was present in 10 cases (20%), in five diffusely and five focally. There was no significant association between cytokeratin expression and the following parameters: patient age, sex, skeletal and extraskeletal primary site, and the type of EWS fusion transcript. Cytokeratin expression, a manifestation of epithelial differentiation, is present in as many as 20% of ES/PNET in either a diffuse or focal pattern.
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ranking = 2.5
keywords = sarcoma
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4/54. Soft tissue Ewing sarcoma--peripheral primitive neuroectodermal tumor with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript.

    This study describes a new case of Ewing sarcoma (ES)-peripheral primitive neuroectodermal tumor (pPNET) with unusual phenotype and fusion gene structure. The tumor located in the inguinal area of a 15-year-old boy showed a highly aggressive behavior with hematogenous metastases after intensive chemotherapy and bone marrow transplant, causing death 28 months after diagnosis. The tumor displayed a clear cell pattern, and several neuroectodermal markers proved positive both in the original tumor and in xenografts. This neuroectodermal character was confirmed by electron microscopy. Moreover, cytogenetically the tumor has an unusual chromosomal rearrangement, t(2;22)(q13;q22,t(3;18)(p21;q23); representing a new EWS-FEV fusion type in which exon 7 of EWS gene is fused with exon 2 of FEV gene. This is the third published study of an ES-pPNET showing EWS-FEV fusion described, but it is the first study of a tumor with the aforementioned fusion points. These findings support the genetic and morphologic heterogeneity existing within the group of ES-pPNET tumors.
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ranking = 2.5
keywords = sarcoma
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5/54. Primary primitive neuroectodermal tumor of the kidney.

    Primitive neuroectodermal tumor (PNET) is a small round cell sarcoma that mainly develops in the central nervous system and soft tissues of childhood; however recently, primary occurrence of this tumor in the kidney has been reported. We experienced one case of PNET primarily arose in the kidney without metastasis. The patient was a 28-year-old man whose chief complaint was abdominal pain, especially on exercise. On computed tomography scan and magnetic resonance imaging, a solid lesion was found in the left kidney, and a left nephrectomy was performed based on the diagnosis of a tumor in the left kidney. The tumor was within the parenchyma of lower end of left kidney protruding into the abdominal cavity. Histologically, diffuse proliferation of primitive small round cells with rosette formation was found. Immunohistochemically, MIC2 gene product, neuron-specific enolase and S-100 protein were positive. No metastasis to the regional lymph nodes was found. From these observations, the tumor was diagnosed as PNET primarily arising in the left kidney. Although chromosome analysis was not performed, EWS-FLI1 chimera gene was identified by reverse transcriptase-polymerase chain reaction on the freshly frozen specimen and fluorescence in situ hybridization on paraffin sections.
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ranking = 0.5
keywords = sarcoma
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6/54. High-dose anthracyclines in the treatment of advanced primitive neuroectodermal tumors in adults--a single institution experience.

    Primitive neuroectodermal tumors (PNET) are rare malignancies of presumed neural crest origin, most often presenting as bone or soft tissue masses in the trunk or axial skeleton, in children and young adults. Treatment of advanced PNET in adults is not clearly defined in the literature. Data concerning dose-intensive chemotherapy regimens for poor-risk patients with those tumors are sparse, due to rarity of PNET in adults, their diverse presentation, the variable treatment procedures applied and the absence of direct comparisons. On the other hand, the role of anthracyclines in the treatment of advanced soft tissue sarcomas is well known and substantial. Six advanced PNET patients were treated at the Institute for Oncology and radiology of serbia, during last five years, with high-doses of doxorubicin or epidoxorubicin combined with cisplatin. The paper reviews each of our patients, discussing how does chemotherapy influence the outcome in these patients, in context of the feasibility of high-doses of anthracyclines in advanced settings. High dose anthracyclines (epidoxorubicin 150 mg/m2) in combination with cisplatin 120 mg/m2 induced a complete response lasting for 63 months in a patient with desmoplastic medulloblastoma of the cerebellum metastatic to bones and bone marrow. The same treatment but with the epidoxorubicin dose of 180 mg/m2 induced a complete response in a patient with olfactory neuroblastoma. Administration of high dose doxorubicin (75 mg/m2) seems feasible in association with irradiation treatment in patients with extraosseal Ewing sarcoma/PNET but the place of high dose chemotherapy within this setting remains to be determined.
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ranking = 1
keywords = sarcoma
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7/54. PNET-like features of synovial sarcoma of the lung: a pitfall in the cytologic diagnosis of soft-tissue tumors.

    Fine-needle aspiration (FNA) cytology of soft-tissue tumors is evolving. As more experience is gained, we are becoming aware of potential pitfalls. We describe 2 cases of synovial sarcoma of the lung, primary and metastatic, in patients who had FNA biopsy performed on a lung mass. The cytologic smears showed extremely cellular groups of malignant small round cells, intersected by small blood vessels, with numerous loose single cells, in a background of macrophages and mature lymphocytes. The tumors displayed monomorphic cells forming rosettes and displaying occasional mitoses. A diagnosis of neuroendocrine tumor/primitive neuroepithelial tumor (PNET) was suspected. Furthermore, this suspicion was supported by immunohistochemical stains, which showed positivity for a neuroendocrine marker, Leu 7 (case 1), and for a neural marker, CD 99 (O 13 or HBA 71) (both cases); and negativity for cytokeratins (case 1). The resection specimen of case 1 had mostly tightly packed small round cells, with occasional rosettes, similar to the FNA biopsy, and focal areas composed of spindle cells, organized in a focal fibrosarcoma-like and hemangiopericytoma-like pattern. A balanced translocation between chromosomes X and 18, demonstrated by both karyotyping and fluorescent in situ hybridization (FISH), enabled us to make a diagnosis of synovial sarcoma, which was histologically classified as poorly differentiated. Case 2 was a metastatic biphasic synovial sarcoma of the arm, with a prominent epithelial component. Synovial sarcoma, when composed mainly of small round cells on cytologic smears, is a great mimicker of neuroendocrine/PNET tumors, with light microscopic and immunohistochemical overlap. awareness of this potential pitfall may aid in preventing a misdiagnosis. Its recognition is of major concern, especially for the poorly differentiated variant, because it is associated with a worse prognosis.
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ranking = 4.5
keywords = sarcoma
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8/54. Primary Ewing's sarcoma/peripheral primitive neuroectodermal tumor at the vertex of the skull with elevated serum carcinoembryonic antigen: case report.

    A primary Ewing's sarcoma arising in the skull is relatively rare. Although a small number of case reports noted elevated carcinoembryonic antigen (CEA) in patients with primary central nervous system (CNS) neoplasms, there is no report of Ewing's sarcoma/peripheral primitive neuroectodermal tumor (PNET) with elevated serum levels of CEA. A 7-year-old boy who had episodes of headache and vomiting had noticed a solid mass in the vertex of the head. Imaging studies revealed a large intra- and extracranial tumor at the vertex of the skull. Hematological examination demonstrated high serum levels of CEA: 91.09 ng/ml. The patient initially underwent an embolization of the bilateral middle meningeal arteries with Gelfoam particles. One week later, the patient was operated on and a subtotal resection of the tumor was performed. On histopathological and molecular genetic examination, the tumor was diagnosed as a Ewing's sarcoma/peripheral PNET. Immunohistochemical study showed strongly positive staining for CEA in the tumor cells. The serum level of CEA was normalized at 0.83 ng/ml after the tumor was removed and the boy underwent radiotherapy and 3 courses of chemotherapy. This is the first reported case of a primary Ewing's sarcoma/peripheral PNET at the vertex of the skull with elevated serum CEA.
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ranking = 4
keywords = sarcoma
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9/54. Malignant teratoma of the thyroid with primitive neuroepithelial and mesenchymal sarcomatous components.

    A 15-year-old black girl had a near total resection of a malignant thyroid teratoma with bilateral nodal involvement and mediastinal extension. A predominant neuroepithelial pattern had ependymal rosettes and mitoses, stained for neuron-specific enolase, neuron-specific B tubulin, and synaptophysin. A malignant spindle cell component stained for smooth-muscle actin, muscle actin, and to a lesser extent S-100. Loose myxoid tissue resembled primitive cartilage. Epithelial membrane antigen and cytokeratin identified epithelial foci. chromogranin a, MIC2, glial fibrillary acidic protein, and thyroid stimulating hormone receptor stains were negative. There was focal anaplasia. dna ploidy by laser scanning cytometry was 1.2. The tumor from the left and right thyroid lobes exhibited trisomy 8, the right also had hyperdiploid cell lines. She was treated with aggressive combination chemotherapy and radiation. Presently there is no residual disease 16 months after diagnosis. Malignant thyroid teratoma is an aggressive tumor, with 15 of 27 reported patients dying 2 weeks to 3 years after diagnosis. survivors have been treated with total or subtotal resection, combination chemotherapy with agents effective in the treatment of germ cell tumors as well as sarcomas, and radiation for either recurrent or residual disease. The heterologous elements, lacking MIC2 staining and t(11;22), support the diagnosis of malignant teratoma rather than a neuroepithelial tumor. trisomy 8 is the first cytogenetic abnormality described in malignant thyroid teratoma. Therapy should be tailored to the management of all transformed histologies.
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ranking = 2.5
keywords = sarcoma
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10/54. Peripheral primitive neuroectodermal tumour (pPNET) of the cerebellopontine angle presenting in adult life.

    We report a case of a peripheral neuroectodermal tumour (pPNET) of the cerebellopontine angle of a 67-year-old woman. The patient's age at presentation was highly unusual. This case highlights the difficulties encountered, both clinically and pathologically, in securing the correct diagnosis of such a rare condition presenting in this relatively inaccessible area. The development of the nomenclature and classification of neuroectodermal tumours is traced. Recent advances in immunohistochemistry and genetic typing have shown the close relationship between pPNET and the previously difficult to classify Ewing's sarcoma and Askin's tumour.
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ranking = 0.5
keywords = sarcoma
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