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Primitive Neuroectodermal Tumor (primitive + neuroectodermal_tumor)
Selected AbstractsPrimitive neuroectodermal tumor of the adrenal glandINTERNATIONAL JOURNAL OF UROLOGY, Issue 5 2006SHUICHI KOMATSU Abstract, We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery. [source] New malignancies following childhood cancer in the United States, 1973,2002INTERNATIONAL JOURNAL OF CANCER, Issue 10 2007Peter D. Inskip Abstract The objectives of our study were to quantify risks for developing new malignancies among childhood cancer survivors, identify links between particular types of first and subsequent cancer, and evaluate the possible role of treatment. A cohort of 25,965 2-month survivors of childhood cancer diagnosed in the U.S. during 1973,2002 was identified and followed through SEER cancer registries. Observed-to-expected ratios (O/E) were calculated, and Poisson regression was used to compare risks among treatment groups. Childhood cancer survivors were at nearly 6-fold risk of developing a new cancer relative to the general population (O/E = 5.9, 95% CI: 5.4,6.5). Most common were subsequent primary cancers of the female breast, central nervous system, bone, thyroid gland and soft tissue, as well as cutaneous melanoma and acute non-lymphocytic leukemia (ANLL). The greatest risks of subsequent cancers occurred among patients diagnosed previously with Hodgkin lymphoma (HL), Ewing sarcoma, primitive neuroectodermal tumor, or retinoblastoma. Risk of subsequent solid cancers was higher among persons whose initial treatment for childhood cancer included radiotherapy, whereas the excess of subsequent ANLL was strongly related to chemotherapy. The O/E for subsequent ANLL increased with increasing calendar year of initial cancer diagnosis among survivors of cancers other than HL, most likely due to increasing use of leukemogenic drugs for solid cancers and non-Hodgkin lymphoma. Childhood cancer survivors are at markedly increased risk of developing a variety of new cancers relative to the general population, but the magnitude of excess risk and specific types of second cancer vary widely by type of first cancer. © 2007 Wiley-Liss, Inc. [source] Primitive neuroectodermal tumor of the adrenal glandINTERNATIONAL JOURNAL OF UROLOGY, Issue 5 2006SHUICHI KOMATSU Abstract, We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery. [source] Pinealoblastoma with prominent retinoblastic differentiation: An unusual case in an adultNEUROPATHOLOGY, Issue 4 2010Nandita Ghosal We present an extremely rare case of pinealoblastoma with retinoblastic differentiation in a 32-year-old woman who presented with a history of intermittent headache of 2 years duration and diminution of vision for 2 months which eventually lead to total loss of vision. The fundus examination showed bilateral secondary optic atrophy. She did not have any previous history of retinoblastoma. The family history was non-contributory. Paraffin section of the tumor showed a primitive neuroectodermal tumor with numerous Flexner-Wintersteiner rosettes and the tumor cells were strongly positive for synaptophysin and negative for GFAP, S-100 protein and epithelial membrane antigen. This is the first case in the literature of a sporadic case of pinealoblastoma with prominent retinoblastic differentiation as evidenced histomorphologically by the presence of numerous Flexner-Wintersteiner rosettes in an adult female. [source] Tumor thrombus in a child with primitive neuroectodermal tumorPEDIATRIC BLOOD & CANCER, Issue 6 2002Ömer Görgün MD No abstract is available for this article. [source] High-dose chemotherapy with autologous stem cell rescue for children with recurrent malignant brain tumorsCANCER, Issue 6 2008Chie-Schin Shih MD Abstract BACKGROUND. High-dose chemotherapy (HDCT) with autologous stem cell rescue (ASCR) has been reported to be effective in treating children with recurrent central nervous system (CNS) malignancies. METHODS. To evaluate the efficacy and toxicities of HDCT and ASCR, the medical records of 27 children with recurrent CNS malignancies who received such therapy at St. Jude Children's Research Hospital between 1989 and 2004 were reviewed. RESULTS. The median age at diagnosis was 4.5 years (range, 0.4,16.6 years) and that at ASCR was 6.7 years (range, 1.1,18.5 years). Diagnoses included medulloblastoma (13 patients), primitive neuroectodermal tumor (3 patients), pineoblastoma (2 patients), atypical teratoid rhabdoid tumor (2 patients), ependymoma (3 patients), anaplastic astrocytoma (2 patients), and glioblastoma multiforme (2 patients). The 5-year overall and progression-free survival (PFS) rates were 28.2% and 18.5%, respectively. The 5-year PFS rate for patients aged <3 years at diagnosis (57.1%) was significantly better than older patients (5.0%) (P = .019). Among the 6 long-term survivors (5 with M0 disease and 1 with M3 disease at diagnosis), 5 received both radiotherapy and HDCT as part of their salvage regimen; 4 were aged <3 years at diagnosis and had received chemotherapy only as part of frontline therapy. Two patients died of transplant-related toxicities; 44% experienced grade 3 or 4 transplant-related toxicities (toxicities were graded according to the National Cancer Institute Common Toxicity Criteria). CONCLUSIONS. HDCT with ASCR is not an effective salvage strategy for older children with recurrent CNS malignancies. The significantly better outcome in the younger cohort was most likely related to the use of radiotherapy as part of the salvage strategy. Cancer 2008. © 2008 American Cancer Society. [source] Recent advances in the molecular pathology of soft tissue sarcoma: Implications for diagnosis, patient prognosis, and molecular target therapy in the futureCANCER SCIENCE, Issue 2 2009Yoshinao Oda In the present paper, recent advances in the molecular pathology of soft tissue sarcomas (STS) and the implications for their prognostic value are reviewed, and the potential targets of molecular therapy are discussed. According to the molecular genetic aspect, STS are divided into two groups: chromosome translocation-associated sarcomas and sarcomas without specific translocation. In the former group, specific fusion transcripts, such as SS18,SSX, EWS,FLI1, and PAX3,FKHR, could be detected in synovial sarcoma, Ewing's sarcoma and primitive neuroectodermal tumor, and alveolar rhabdomyosarcoma, respectively. The direct or indirect interactions between these fusion transcripts and cell cycle regulators have been elucidated by several investigators. Therefore, these fusion transcripts are promising candidates as molecular targets. As evaluated in carcinomas, alterations of several tumor-suppressor genes and adhesion molecules and overexpression of growth factors and their receptors have been extensively assessed in STS. In mixed-type STS, epidermal growth factor receptor overexpression was associated with decreased overall survival, suggesting the beneficial role of epidermal growth factor receptor inhibitors in STS. In malignant rhabdoid tumor and epithelioid sarcoma, frequent alteration of the SMARCB1/INI1tumor-suppressor gene and the loss of its protein have been demonstrated, indicating that this molecule could be an effective target of these sarcomas. In sarcomas with epithelioid differentiation, such as synovial sarcoma and epithelioid sarcoma, overexpression of dysadherin, which downregulates E-cadherin expression, was a poor prognostic factor. In conclusion, further studies are necessary to search for effective and specific molecules for the inhibition of tumor growth in each type of STS, especially in sarcomas without specific translocation. (Cancer Sci 2009; 100: 200,208) [source] Maternal medication use and the risk of brain tumors in the offspring: The SEARCH international case-control studyINTERNATIONAL JOURNAL OF CANCER, Issue 5 2006Amanda H. Cardy Abstract N -nitroso compounds (NOC) have been associated with carcinogenesis in a wide range of species, including humans. There is strong experimental data showing that nitrosamides (R1NNO·COR2), a type of NOC, are potent neuro-carcinogens when administered transplacentally. Some medications are a concentrated source of amides or amines, which in the presence of nitrites under normal acidic conditions of the stomach can form NOC. Therefore, these compounds, when ingested by women during pregnancy, may be important risk factors for tumors of the central nervous system in the offspring. The aim of the present study was to test the association between maternal use of medications that contain nitrosatable amines or amides and risk of primary childhood brain tumors (CBT). A case-control study was conducted, which included 1,218 cases and 2,223 population controls, recruited from 9 centers across North America, Europe and Australia. Analysis was conducted for all participants combined, by tumor type (astroglial, primitive neuroectodermal tumors and other glioma), and by age at diagnosis (,5 years; >5 years). There were no significant associations between maternal intake of medication containing nitrosatable amines or amides and CBT, for all participants combined and after stratification by age at diagnosis and histological subtype. This is the largest case-control study of CBT and maternal medications to date. Our data provide little support for an association between maternal use of medications that may form NOC and subsequent development of CBT in the offspring. © 2005 Wiley-Liss, Inc. [source] Immunoreactivity of CD99 in invasive malignant melanomaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 10 2006Anne E. Wilkerson Background:, CD99, also known as p30/32, is a glycoprotein product of the MIC2 gene. It was originally utilized in immunohistochemistry as a unique marker for Ewing sarcoma, other primitive neuroectodermal tumors, and subsequently in other tumors. Its expression in malignant melanoma (MM) has not been well documented, with just two isolated cases of MM recently reported. Recent studies have documented CD99 expression in a significant percentage of atypical fibroxanthomas (AFX), posing potential diagnostic problems in differentiating these two entities. As mistaking MM for AFX based on immunohistochemical staining pattern has significant consequences, we sought to determine the percentage of invasive MM in our archives that have this staining pattern. Methods:, Seventy-eight cases of invasive melanoma were retrieved from our files. Each case was stained with mouse anti-human CD99 and evaluated for membranous expression. Results:, Our evaluation revealed that 47 of 78 MM cases (60%) stain positive for CD99. Conclusion:, This study is the first to demonstrate, in a large series, the prevalence of CD99 expression in primary cutaneous melanoma. Additionally, this introduces in the histologic differential diagnosis of CD99 expressing dermal spindle cell lesions. [source] CD99 Immunoreactivity in Metastatic Malignant MelanomaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005AE Wilkerson CD99, also known as p30/32, is a glycoprotein product of the MIC2 gene, which is located on the short arm of both chromosome X and Y. This transmembrane protein was originally utilized in immunohistochemistry as a unique marker for Ewing sarcoma, other primitive neuroectodermal tumors, and more recently in a wide variety of tumors. It's expression in malignant melanoma (MM) has not been well documented. A recent study at our institution demonstrated membranous staining in approximately 61% of primary MM. As CD99 is expressed by hematopoeitic cells, it has been proposed as a mechanism for lymphocytes to gain access to the vasculature.1 This study is designed to determine if CD99 expression in melanoma cells has a similar role using cases of metastatic MM from our archives. Our evaluation shows that 13 of 28 cases (46.4%) demonstrated membranous CD99 staining. A case of this magnitude has not been previously reported. Reference: 1. Shenkel AR, Mamdouh Z, Chen X, Liebman RM, Muller WA. CD99 plays a major role in the migration of monocytes through endothelial junctions. Nature Immunol 2002;3:143,150. [source] | ||||||||||