Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:Q06643 (non-Hodgkin's lymphoma)
11,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifteen children ranging in age from 4 to 18 years with leptomeningeal metastases were evaluated for extent of tumor and treated with radiotherapy or chemotherapy. Histologic diagnosis included: acute lymphoblastic leukemia (4); anaplastic astrocytoma (1); ependymoma (2); nonseminomatous germ cell tumor (1); non-Hodgkin's lymphoma (1); pineoblastoma (1); and primitive neuroectodermal tumor not otherwise specified (2). Pretreatment imaging studies demonstrated recurrent intracranial parenchymal disease in nine, spinal disease in four, and abnormal radioisotope ventriculography in six. Systemic disease was seen in four children (3 leukemia; 1 non-Hodgkin's lymphoma). All children were treated with systemic chemotherapy and intraventricular chemotherapy. Nine children received radiotherapy to bulky or symptomatic leptomeningeal disease. Median survival was 6 months (range, 4-18 months). Children with hematologic malignancies had superior outcomes compared to children with solid tumors. Three children with leukemic or lymphomatous meningitis are alive and disease free whereas all children with carcinomatous meningitis died. In conclusion, leptomeningeal metastases in children portends a limited survival, and therapies at this time remain palliative except in children with hematologic malignancies.
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PMID:Pediatric leptomeningeal metastases: outcome following combined therapy. 901 Jul 96

To investigate the cumulative incidence of second malignancy and the competing risk of death due to any other cause in patients who were treated for childhood acute myeloid leukemia (AML), we analyzed the outcomes in a cohort of 501 patients who were treated at St Jude Children's Research Hospital between 1970 and 1996. Five patients developed a second cancer (two carcinomas of the parotid gland, one non-Hodgkin's lymphoma, one supratentorial primitive neuroectodermal tumor, one acute lymphoblastic leukemia) as compared with 0.47 expected in the general population (standardized incidence ratio, 10.64; 95% confidence interval, 3.28 to 22.34). A third neoplasm (meningioma) developed in one patient. At 15 years after the diagnosis of AML, the estimated cumulative incidence of second malignancy was 1.34% +/- 0.61%, whereas the cumulative incidence of death due to any other cause was 72.96% +/- 2.14%. We concluded that although a more than 10-fold increased risk of development of cancer was found in survivors of childhood AML as compared to the general population, the risk of this late complication is small when compared to the much larger risk of death because of the primary leukemia or the early complications of its treatment. Future studies should focus on improving treatments for primary AML while preventing second malignancies.
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PMID:Second malignancy after treatment of childhood acute myeloid leukemia. 1124 97

Secondary, radiation-induced, supratentorial primitive neuroectodermal tumors (PNETs) are extremely rare entities which may present in survivors of childhood cancers after central nervous system radiation. These lesions have been described after a number of pediatric cancers and demonstrate unique treatment problems and an accelerated clinical course compared to primary PNETs. We present a case of a sixteen year old male with a history of non-Hodgkin's lymphoma who presented with a radiation-induced PNET, and describe our treatment for this lesion. These secondary, radiation-induced tumors increase in significance as the survival of childhood malignancy increases in West Virginia.
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PMID:Radiation-Induced Supratentorial Primitive Neuroectodermal Tumor after Treatment for Non-Hodgkin's Lymphoma. 2624 31

Peripheral primitive neuroectodermal tumor (PNET) is a rare histology to be found in primary tumors of the kidney. There are less than a hundred cases reported in the English literature. Most of these have been diagnosed after surgery for a renal neoplasm diagnosed on imaging. PNET has rarely been reported as a second malignancy, and has never been reported as a second malignancy after non-Hodgkin's lymphoma (NHL). Herein, we present our case of a 38-year-old female who developed a second malignancy in the kidney after the treatment for NHL.
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PMID:Renal primitive neuroectodermal tumor as a second malignancy after chemotherapy and radiation for Non-Hodgkin's Lymphoma--treatment-related or just poor old bad luck?: A case report. 2645 16

Ewing sarcoma/primitive neuroectodermal tumor (Ewing/PNET sarcomas or EPS) are a group of round cell tumors. Malignant round cell tumors form a large and diverse group that includes rhabdomyosarcoma, synovial sarcoma, non-Hodgkin's lymphoma, neuroblastoma, hepatoblastoma, Wilm's tumor, desmoplastic small round cell tumor, and other morphologically similar entities. Differential diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (Ewing/PNET sarcomas or EPS) is difficult. In addition to morphology and immunohistochemistry (IHC), differential diagnosis of these tumors is based on molecular analysis of the EWSR1 gene rearrangement using fluorescent in situ hybridization (FISH) technique. We investigated the diagnostic value of combined CD99 immunostaining and EWSR1 t(22q12) alteration using a dual-color, break-apart rearrangement probe in forty-one formalin-fixed paraffin-embedded (FFPE) tissue samples from pediatric and adult patients diagnosed with EPS. IHC was performed in all cases using the CD99 antibody and showed a positivity of 92.7% in the enrolled cases (38/41) followed by FISH analysis where 48.8% of the cases (20/41) were rearranged. Sensitivity and specificity for IHC assays were 88% and 58%, respectively. Notably, FISH had a sensitivity of 100% and a specificity of 87%. In addition, CD99 positivity was found to correlate with EWSR1 rearrangement (p < 0.05). This report shows that FISH has better sensitivity and specificity than IHC in the Moroccan population, and supports its combination with CD99 immunostaining as diagnostic biomarkers for this rare malignant entity."
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PMID:EWSR1 Rearrangement and CD99 Expression as Diagnostic Biomarkers for Ewing/PNET Sarcomas in a Moroccan Population. 3031 19