Cases reported "Neuroblastoma"

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11/62. 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 = 1
keywords = sarcoma
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12/62. Desmoplastic cerebral astrocytomas of infancy: a histopathologic, immunohistochemical, ultrastructural, and molecular genetic study.

    The desmoplastic cerebral astrocytoma of infancy (DCAI) is a rare tumor that presents as a large hemispheric mass in infants. Despite an ominous histologic picture that may resemble a sarcoma, the tumor is astrocytic and has a good prognosis. We present two cases of DCAI, with histopathologic, immunohistochemical, ultrastructural, and molecular genetic data, and draw the following conclusions: (1) the diagnosis of DCAI requires a high index of suspicion and immunohistochemical or ultrastructural proof of astrocytic differentiation; (2) the data argue against nosologically equating these tumors with the desmoplastic infantile ganglioglioma, pleomorphic xanthoastrocytoma, or gliofibroma; (3) the components of the extensive tumor basal lamina may be elaborated by the tumor cells themselves and may contribute in an autocrine fashion to the slow growth of these lesions; and (4) if the lack of allelic loss on chromosomes 17p (including the p53 tumor suppressor gene locus) and 10 seen in our cases is found in other cases of DCAI, this may further distinguish the DCAI from other astrocytomas.
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ranking = 0.2
keywords = sarcoma
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13/62. Primitive neuroectodermal tumor of the gallbladder.

    Ewing sarcoma/primitive neuroectodermal tumor (ES/ PNET) most commonly occurs in the soft tissues of the extremities and the paravertebral areas. To our knowledge, no case of PNET has been reported in the gallbladder to date. We report a case of PNET of the gallbladder occurring in a 53-year-old woman. Microscopically, the monotonous small round cells with a formation of Homer-Wright rosettes showed intense membranous positive immunoreactivity for MIC2/CD99, as well as for neuron-specific enolase and synaptophysin. Other small round cell tumors, such as lymphoma, rhabdomyosarcoma, or melanoma, were excluded in light of negative immunoreactivity for leukocyte common antigen, desmin, and S100. The diagnosis of PNET was rendered based on the characteristic morphology and immunohistochemical findings, despite an absence of chromosomal translocations, such as t(11; 22)(q24;q12) and t(21;22)(q22;q12).
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ranking = 0.4
keywords = sarcoma
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14/62. Elevated catecholamine metabolites in patients with costello syndrome.

    costello syndrome is a rare congenital anomaly syndrome with a predisposition to specific tumors, including neuroblastoma, rhabdomyosarcoma, and transitional cell carcinoma of the bladder. The increased risk for solid tumors led to the proposal of a tumor screening protocol. A screening test for neuroblastoma consists of measuring catecholamine metabolites in urine, an assay that may also be used for diagnostic confirmation of a suspected catecholamine secreting tumor. We report eight patients with costello syndrome with elevated catecholamine metabolites, vanillylmandelic acid (VMA) and/or homovanillic acid (HVA), in urine. Each patient had additional laboratory and/or imaging studies. None of the patients was found to have a neuroblastoma or another catecholamine secreting tumor. In two cases, the assays were performed because the patients were symptomatic with diaphoresis and hypertension, respectively, and in the other six cases the assays were performed in order to screen for neuroblastoma. The pathophysiology for the catecholamine metabolite abnormality in these patients with costello syndrome remains unclear. However, it appears that in this patient group an elevation above the normal limit, defined as 2 standard deviations (SD) above the mean for age, is more likely to be a variant, rather than a sign of a neuroblastoma. Thus, it may be prudent not to use this assay as a screening test, and to take the frequently elevated results into consideration when interpreting diagnostic assays.
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ranking = 0.2
keywords = sarcoma
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15/62. Neonatal cancer in denmark 1943-1985.

    In order to describe the incidence, distribution, and prognosis of neonatal cancer, 76 cases (44 boys, 32 girls) of cancer in the first 28 days of life were identified in the Danish Cancer Registry within the period 1943-1985. All cases were reevaluated and the International classification of disease for Oncology (ICD-O) code was applied. The incidence is calculated to 2.38 (1.88-2.98) cases per 100,000 births. The most common tumor was neuroblastoma (26%) followed by leukemias (16%), soft-tissue sarcomas (14%), and brain tumors (11%). The 5-year survival rate was 25% (15.1-39.0) with the highest survival rate among soft-tissue sarcomas (55%) and neuroblastomas (25%) and the lowest among leukemias and brain tumors (0). Eighteen percent of the cases were incidental findings. Two cases of secondary malignancy were identified.
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ranking = 0.4
keywords = sarcoma
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16/62. Fine-needle aspiration of adult small-round-cell tumors studied with flow cytometry.

    Immunophenotypic study is critical for the diagnosis of adult small-round-cell tumors (SRCTs). We describe three patients with Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) and one patient with neuroblastoma in which flow cytometry immunophenotyping (FCI) on the fine-needle aspirate (FNA) and bone marrow aspirate (BMA) demonstrated an abnormal population of cells that were CD45(-) and CD16/CD56( ). Four patients with mean age of 30 years, three male and one female, clinically suspicious for a lymphoma or SRCT are described. FNA, BMA, and biopsy specimens were obtained for routine cytologic and histologic evaluation. Fresh tissue was studied by FCI. In all cases, the cytology smears showed small cells with round nuclei, slightly irregular nuclear membranes, fine chromatin, and scant cytoplasm. FCI showed CD16/56( ) and CD45(-) neoplastic cells in all cases. In one case, 76% of these cells were CD99( ). The diagnoses of ES/PNET were confirmed by immunohistochemical, ultrastructural, and cytogenetic studies. ES/PNET in FNA and BMA can be efficiently and rapidly diagnosed by combining cytologic examination with FCI using a panel including CD45, CD16/56, and CD99.
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ranking = 0.2
keywords = sarcoma
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17/62. Establishment of a new cell line, VA-N-BR, from a primitive neuroblastoma tumor of the abdomen.

    An established cell line was derived from a neuroblastoma originating in the abdomen of a six year old male. This patient had increased urinary homovanillic acid on two occasions, and the tumor had a unique pattern of local peritoneal and hepatic dissemination but not distant spread. The cells were initially highly refractile round cells forming firmly adherent cell clumps which later formed irregularly shaped bodies with mono- or bipolar short processes attached to the plastic surface of the culture flask. Chromosomal tetraploidy and marker chromosomes distinguished these cells from Ewings sarcoma, neuroepithelioma and the common disseminating neuroblastoma. Immunohistochemical staining positive for neurofilament, chromogranin, desmin and vimentin suggested it to be relatively undifferentiated. When heterotransplanted to nude mice, these cells expressed only desmin and vimentin reactivity. This neuroblastoma cell line was established and designated as VA-N-BR. It appears to be different from the more common partially differentiated neuroblastoma of childhood.
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ranking = 0.2
keywords = sarcoma
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18/62. desmin positivity in primitive neuroectodermal tumors of childhood.

    In this report, we describe two rosette-forming primitive neuroectodermal tumors that were found to contain desmin by both immunohistochemistry and Western blotting. Electron microscopy on both cases was consistent with primitive neuroectodermal tumors and revealed that the tumor cells contained cytoplasmic bundles of intermediate filaments. In both cases, studies for MyoD1 protein using immunohistochemistry and Western blotting were negative. Thus, the detection of desmin in a pediatric neoplasm does not absolutely exclude the diagnosis of primitive neuroectodermal tumor and should not be considered as prima facie evidence that a small-cell tumor is a rhabdomyosarcoma.
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ranking = 0.2
keywords = sarcoma
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19/62. radiation-induced osteosarcoma and papillary carcinoma of the thyroid.

    PURPOSE: This article describes a patient with dual radiation-induced malignancies after treatment of neuroblastoma. MATERIALS AND methods: A 12-year-old boy with a history of neuroblastoma was treated with chemotherapy, I-131 MIBG, and radiotherapy at age 4. He was disease-free for 8 years, but then developed left shoulder pain resulting from osteosarcoma. A thyroid malignancy was discovered during the evaluation. RESULTS: The patient was treated with thyroidectomy and then chemotherapy according to a Children's Cancer Group Study protocol. CONCLUSION: radiation-induced sarcoma should be suspected whenever a patient who has received radiation several years previously presents with pain or swelling in the irradiated area.
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ranking = 1.2
keywords = sarcoma
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20/62. Randomization, informed consent and physicians' communication skills in pediatric oncology: a delicate balance.

    parents asked to consent to a child's randomization in a pediatric cancer clinical trial are often unprepared to grasp the implications of this scientifically crucial but seemingly unfair process. physicians must adopt specific communication skills to engage families in open dialogue from the outset in order to elicit truly shared informed consent. Starting from the case of a family with an only child affected by disseminated neuroblastoma, we wish to comment on the problems surfacing in the informed consent process for treatment and research in pediatric oncology that implicate an understanding of bioethical issues and psychological principles. Although the outcome of childhood cancer has improved dramatically over the last 30 years, with overall survival rates now exceeding 70%, there are regretfully still types and stages of cancer carrying a very high risk of death that urgently require new clinical strategies. The response to this need has been the design of experimental protocols that often entail randomized controlled trials (RCT). A large number of these trials concern stage IV neuroblastoma, acute leukemia, rhabdomyosarcoma, and other types of childhood cancers presenting great heterogeneity both in terms of localization and responsiveness to therapy. Most trials for disease relapses also include one or more randomizations. The scientific motivation justifying an RCT is the need to compare and evaluate an innovative protocol (or part thereof) with reference treatment modalities. Nevertheless, the process brings to bear the ethical dilemma of having to weigh the needs of the single afflicted child against the benefit which may ensue for a much larger patient community.
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ranking = 0.2
keywords = sarcoma
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