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11/54. Atypical pleomorphic extraosseous ewing tumor/peripheral primitive neuroectodermal tumor with unusual phenotypic/genotypic profile.

    A pleomorphic undifferentiated tumor primarily located in the retroperitoneum with a phenotype compatible with an extraosseous Ewing tumor/peripheral primitive neuroectodermal tumor (ET/pPNET) pattern and unusual molecular features is described. Immunohistochemically, HBA-71 (CD99/mic2) and several neural markers were intensively expressed together with scattered cells expressing carcinoembryonic antigen (CEA). Short-term culture showed biphasic neuroblastic and epithelioid cell populations, with the latter expressing germ cell markers (CEA, alpha-fetoprotein, and the beta-subunit of chorionic gonadotrophin). Conventional cytogenetics displayed several chromosomic rearrangements, especially a complex translocation t(17,2,22,13) (q21::q11-->q33::q12-->q13::q14). These structural abnormalities were confirmed using fluorescence in situ hybridization analysis. Molecular studies revealed EWS-FEV fusion transcripts (exon 7 of the EWS gene and exon 2 of the FEV gene). In addition, a new p53 mutation not previously reported in ET/pPNET involving exon 5 codon 138: GCC to GAC (Ala/Asp) was detected.In our case, we emphasize the presence of atypical features not only from the phenotypic point of view but also at the genetic level as well as the value of detecting such markers in the differential diagnosis with other abdominal pleomorphic tumors.
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ranking = 1
keywords = ewing
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12/54. Extraosseous Ewing's sarcoma of the kidney.

    Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (EES/PNET) of the kidney is an uncommon entity and usually arises from the retroperitoneum and paraspinal region. We report a case of renal EES/PNET and discuss its clinical features, histological findings and management.
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ranking = 0.27234185928208
keywords = sarcoma
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13/54. Primitive neuroectodermal tumour of the mandible.

    Primitive neuroectodermal tumors (PNETs) are relatively rare tumors. Tumors that once would have been diagnosed as Ewing's sarcoma are now often designated as peripheral neuroepithelioma or synonymously PNET. Herein we report the first case of PNET of the mandible in a 6-year-old female who was admitted to the hospital with a two-months history of painless, progressively enlarging lower jaw mass.
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ranking = 0.054468371856416
keywords = sarcoma
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14/54. Schwannoma with rhabdomyoblastic differentiation: a unique variant of malignant triton tumor.

    A 54-year-old woman presented with intractable perianal, bilateral buttock, and radiating thigh/calf pain. An MRI scan showed an intradural, contrast-enhancing, ovoid mass in the cauda equina region at L1-L2. At laminectomy, the ovoid mass arose from a nerve root and, intact, was gross totally resected. Histologically, the dominant pattern was that of schwannoma. One year thereafter, the symptoms recurred. An MRI scan demonstrated an irregular, heterogeneously enhancing tumor recurrence. A repeat laminectomy disclosed a large fleshy tumor involving multiple nerve roots. The lesion was subtotally resected and showed pluridirectional differentiation toward embryonal rhabdomyosarcoma, primitive neuroectodermal tumor, and rare malignant epithelial cells. review of the original tumor disclosed only foci of embryonal rhabdomyosarcoma and primitive neuroectodermal tumor. Based upon available data regarding divergent differentiation in peripheral nerve sheath tumors, this is a unique, previously undescribed tumor demonstrating rhabdomyosarcomatous, primitive neuroectodermal tumor and scant epithelial differentiation in a schwannoma. In essence, it is a variant of malignant Triton tumor because of its origin in a tumor consisting of well-differentiated schwann cells. It supports the contention that the Schwann cell is the source of a variety of heterologous elements in nerve sheath tumors.
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ranking = 0.16340511556925
keywords = sarcoma
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15/54. Primary cutaneous pre-B lymphoblastic lymphoma immunohistologically mimics Ewing's sarcoma/primitive neuroectodermal tumor.

    Precursor B-cell lymphoblastic lymphomas (B-LBLs) are rare and most often involve the skin in the head and neck region. Histologically, cutaneous B-LBLs may be confused with other small round-cell neoplasms. Moreover, half of B-LBL patients are negative for CD45 (leucocyte common antigen, LCA), a widely used marker for the diagnosis of lymphoma, and a significant portion express CD99, a marker for Ewing's sarcoma (ES) or primitive neuroectodermal tumor (PNET). Therefore, an extranodal B-LBL may be misinterpreted as PNET or ES. Here, we report on 2 boys, aged 10 and 5 years, with primary cutaneous B-LBL of the scalp. PNET was initially misdiagnosed because the tumor cells were negative for CD45 but strongly positive for CD99. Advanced stage of acute lymphoblastic leukemia (ALL) developed later and both patients died during the course of treatment for ALL. In retrospective analyses, tumor cells in the initial biopsy specimens of both patients were found to be reactive to terminal deoxynucleotidyl transferase (TdT), CD43 and CD10. Thus, the diagnosis of B-LBL was confirmed. These cases illustrate the possibility that primary cutaneous B-LBL may mimic ES or PNET immunophenotypically, and that correct diagnosis in doubtful cases may be facilitated by analysis using a complete panel of antibodies, particularly including TdT and CD43.
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ranking = 0.27234185928208
keywords = sarcoma
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16/54. Evidence of neural differentiation in a case of post-therapy primitive neuroectodermal tumor/Ewing sarcoma of bone.

    Neural differentiation with the appearance of ganglion-like cells has been reported in untreated primitive neuroectodermal tumor/Ewing sarcoma (PNET/EWS) at peculiar sites, such as the cauda equina, and following treatment. The case is presented here of a 17-year-old girl with a tumor in the iliac bone. An open biopsy was diagnosed as PNET/EWS of the bone. The tumor had the typical morphology of this tumor type and showed diffuse membranous immunoreactivity for CD99, intense immunoreactivity for synaptophysin, and focal immunoreactivity for neuron-specific enolase and S-100 protein. Occasional reactivity for vimentin was evident, while no immunoreactivity for NB84a, Hu, chromogranins A and B, neurofilaments, cytokeratins, and desmin was present. The patient underwent chemotherapy and radiotherapy, followed by right internal hemipelvectomy. The post-treatment residual viable tumor showed a morphologic appearance resembling a neuroblastoma, with immunoreactivity for NB84a, Hu, synaptophysin, and chromogranins A and B, but not for CD99. RT-PCR performed on tumor tissue before and after therapy showed the presence of the EWS-FLI1 fusion transcript, type I in both samples. This case of PNET/EWS is unique in the sense of showing the typical fusion transcript associated with this tumor both in the morphologically typical pretherapy tumor and in the sample from the post-therapy specimen showing neuroblastoma-like features.
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ranking = 0.27234185928208
keywords = sarcoma
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17/54. Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature.

    STUDY DESIGN: Report of four cases and a review of the literature. OBJECTIVES: To study the clinical features and prognosis of primitive neuroectodermal tumor or extraskeletal Ewing sarcoma arising around the spinal column. SUMMARY OF BACKGROUND DATA: Primitive neuroectodermal tumor or extraskeletal Ewing sarcoma that originates around the spinal column is very rare, and its prognosis is very poor. methods: Four patients were diagnosed and underwent treatment. RESULTS: Although all the patients received high-dose chemotherapy with or without radiotherapy after surgery, three patients died of the disease. Only one patient who received en bloc resection of the tumor combined with multiagent chemotherapy followed by high-dose chemotherapy with peripheral blood stem cell transplantation remains alive and continues to be disease free. CONCLUSION: The prognosis of the patients with primitive neuroectodermal tumor or extraskeletal Ewing sarcoma around the spinal column is very poor. Multiagent chemotherapy combined with en bloc resection and radiation therapy is the preferred treatment for patients with primitive neuroectodermal tumor or extraskeletal Ewing sarcoma around the spinal column.
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ranking = 0.43574697485133
keywords = sarcoma
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18/54. Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature.

    BACKGROUND: Peripheral primitive neuroectodermal tumor (pPNET) is aggressive and rare, comprising 1% of soft tissue sarcomas. Involvement of the reproductive tract is unusual. CASE: A 35-year-old woman had a pelvic mass and omental cake. Frozen-section examination at laparotomy revealed small round cell tumor confirmed as pPNET. Chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide with mesna yielded complete response. The patient's mother died of a similar tumor at age 52 years, and the patient's husband had adult Ewing sarcoma, constituting an unusual cluster of related tumors. CONCLUSIONS: Genetic recombination resulting in a chimeric transcript of the Ewing sarcoma gene and the Friend leukemia virus integration site is characteristic of these tumors. Surgical resection and multiagent chemotherapy may enhance survival.
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ranking = 0.16340511556925
keywords = sarcoma
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19/54. Primitive neuroectodermal tumor of the vagina.

    OBJECTIVES: Primitive neuroectodermal tumor is grouped together with Ewing sarcoma of the bone, but its presentation includes a wider age range and more central locations than its osseous counterpart. Presentation in vagina is exceedingly rare. CASE: A 34-year-old woman with personal history of kidney transplant presented with a painless vaginal mass. After surgical excision, pathology results demonstrated a primitive neuroectodermal tumor. Chemotherapy was given, followed by external beam and vaginal intracavitary brachytherapy. After 20 months postexcision, there is no clinical nor radiographic evidence of recurrence. CONCLUSIONS: This is the second case of primitive neuroectodermal tumor located in the vagina reported in the literature.
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ranking = 0.054468371856416
keywords = sarcoma
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20/54. Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management.

    Primitive neuroectodermal tumors are in the Ewing's sarcoma family of tumors and are composed of small round cells. Because of their rare occurrence, optimal therapy is challenging, particularly if they occur in the head and neck. diagnosis is based on history, immunostaining with at least 2 neural markers, ultrastructural examination, and evidence of an abnormal t(11;22)(q24;q12) translocation as the hallmark for the Ewing's sarcoma family. The prognosis in general is poor because of overt metastasis at the time of diagnosis. Of 27 reported patients with primitive neuroectodermal tumors of the head and neck, 23 were less than 20 years of age. Most patients presented with a tumor in the nasal cavity, paranasal sinuses, or neck. Symptoms developed rapidly (3.6 months, on average), and a lethal outcome occurred in 9 patients. This highly malignant tumor requires an aggressive combination of radical resection, chemotherapy, and radiotherapy. A close follow-up with regular radiographic examination for at least 5 years is mandatory.
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ranking = 0.10893674371283
keywords = sarcoma
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