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Outcome of flat bone sarcomas (other than Ewing's) in children and adolescents: a study of 25 cases.

ABSTRACT: We analysed the clinical features and outcome of young patients with non-Ewing's flat bone sarcoma treated during the era of contemporary chemotherapy. The characteristics and outcome of 25 patients (15 males and 10 females) with primary or radiation-related flat bone sarcoma treated in the Pediatrics Department at the Institut Gustave Roussy from 1981 to 1999 were reviewed. In all, 20 patients had osteosarcoma, four chondrosarcoma and one malignant fibrous histiocytoma. The age at diagnosis ranged from 2 to 23 years (median, 15 years). Nine tumours were located in the craniofacial bones, 11 in the pelvis and five in flat bones at other sites. Four patients had metastatic disease at diagnosis. Radiation-associated flat bone osteosarcoma was diagnosed in 10 out of 25 cases. The projected overall survival and event-free survival (EFS) rates at 5 years were 45.1 and 34.3% for all the 25 patients. The EFS rate of patients with second bone sarcoma was similar to that of patients with de novo flat bone sarcoma (P=0.1). The aim of treatment was curative for 24 patients, 23 of whom were treated with intensive chemotherapy regimens and 19 with surgery. Significant adverse prognostic factors on survival included incomplete surgical resection (P=0.001) and use of regimens without pre- and postoperative chemotherapy (P=0.007). Nine of the 25 patients were treated with pre- and postoperative chemotherapy and complete surgical resection. Among them, eight are alive with no disease. Radical surgical resection is the overriding prognostic factor for flat bone sarcomas in young patients. Nevertheless, our results suggest a more favourable outcome since the advent of intensive chemotherapy.

SUBMITTER: Minard-Colin V

PROVIDER: S-EPMC2409588 | biostudies-other | 2004 Feb

REPOSITORIES: biostudies-other

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Project description:CIC-DUX4 gene fusion, resulting from either a t(4;19) or t(10;19) translocation, is the most common genetic abnormality detected in EWSR1-negative small blue round cell tumors. Following their discovery it was debated if these tumors should be classified as variants of Ewing sarcoma (ie, atypical Ewing sarcoma) or as a stand-alone pathologic entity. As such the WHO classification temporarily grouped the CIC-rearranged tumors under undifferentiated sarcomas with round cell phenotype, until further clinical evidence was available. However, most studies reported so far include small series with limited follow-up information, which preclude a more definitive assessment. The present work investigates the clinicopathologic features of a large cohort of sarcomas with CIC gene rearrangement, to define their clinical presentation, morphologic spectrum, and outcome. Our study further examines the overall survival of the CIC-positive cohort compared with a control group of EWSR1-rearranged Ewing sarcoma matched for age and stage. The study cohort included 115 patients, with a mean age of 32 years and a slight male predominance. Most tumors occurred in the soft tissue (86%), predominantly deep-seated and equally divided among trunk and extremity, followed by visceral locations (12%) and rarely in the bone (3%). Microscopically, most tumors showed round to ovoid cytomorphology but half of the cases showed also focal areas of spindling and epithelioid/rhabdoid phenotype, with frequent myxoid stromal changes. Variable CD99 reactivity was seen in 84% cases, with a diffuse pattern only in 23% of cases, whereas nuclear WT1 was seen in 92%. A CIC-DUX4 fusion was detected in 57% of cases, with either DUX4 on 4q35 (35%) or on 10q26 in 25 (22%) cases. No FOXO4 gene rearrangements were present in 39 cases tested. Clinical follow-up was available in 57 patients, with a 5-year survival of 43%, which was significantly lower than the 77% 5-year survival in the control Ewing sarcoma group (P=0.002). Our findings show that CIC-DUX4 sarcomas occur most commonly in young adults within the somatic soft tissues, having a wide spectrum of morphology including round, epithelioid and spindle cells, and associated with an aggressive clinical course, with an inferior overall survival compared with Ewing sarcoma. The results support the classification of CIC-rearranged tumors as an independent molecular and clinical subset of small blue round cell tumors distinct from Ewing sarcoma.

Project description:The objective of the study was to provide current estimates of the prevalence and trends of overweight and obesity (OW+OB) in Mexican children and adolescents.Body mass index objectively measured was analyzed for 37?147 children and adolescents aged 0-19 years obtained in 2012 as part of the National Health and Nutrition Survey (ENSANUT-2012), a nationally representative sample of the Mexican population. In addition, data from previous National Nutrition Surveys obtained in 1988, 1999 and 2006 were compared with analyze trends over a 24-year period (1988-2012) for children <5 years of age and adolescents and over a 13-year period (1999-2012) for school-age children. World Health Organization Child Growth Standard was used to define OW+OB.In 2012, 33.5% of children <5 years of age (both sexes) were at risk of overweight or were overweight (OW); 32% and 36.9% of girls and boys 5-11 years of age were OW+OB, respectively, and 35.8% and 34.1% of female and male adolescents were OW+OB, respectively. Statistically significant trends were documented for all age groups during the study period. Overall change in the combined prevalence in preschool children was 6.3±1.0 percentage points (pp; P<0.001; 0.26 pp per year) in the last 24 years, showing the highest increase between 1988 and 1999, whereas for school-age girls (from 1999 to 2012) and adolescent females (from 1988 to 2012), OW+OB increased across all periods at a declining trend, with an overall change of 0.5 and 1.0 pp per year, respectively. Changes in the prevalence of OW+OB were highest among children and adolescents in the lowest quintile of the household living condition index.Prevalence of OW+OB among children and adolescents increased significantly during the last 13-24 years. The rate of increase has declined in the last 6 years in all age groups. Changes in prevalence of OW+OB presented here suggest that, in Mexico, the burden of obesity is shifting toward the groups with lower socioeconomic level.

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Outcome of flat bone sarcomas (other than Ewing's) in children and adolescents: a study of 25 cases.

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