Cancer Research and Treatment

Original Article

Incidence and Survival of Pediatric Soft Tissue Sarcomas: Comparison between Adults and Children: Sun Min Lim, Cheol Joo Yoo, Jung Woo Han, Yong Jin Cho, Soo Hee Kim, Joong Bae Ahn, Sun Young Rha, Sang Joon Shin, Hyun Cheol Chung, Woo Ick Yang, Kyoo-Ho Shin, Jae Kyung Rho, Hyo Song Kim; Cancer Res Treat. 2015;47(1):9-17. Published online August 21, 2014; DOI: https://doi.org/10.4143/crt.2013.157

Purpose
Pediatric-type sarcomas such as rhabdomyosarcoma (RMS), Ewing sarcoma (EWS), primitive neuroectodermal tumor (PNET), and desmoplastic small round-cell tumor (DSRCT) are rare in adults, with limited studies on their prognosis and optimal treatment strategies. We aimed to examine the outcome of children and adult patients with RMS, EWS, PNET, and DSRCT and relevant prognostic factors. Materials and Methods We retrospectively reviewed 220 pediatric-type sarcoma patients at a single institution between 1985 and 2011. Comparisons were made in order to examine differences in demographics, disease characteristics, and survival. Survival analyses were performed using the Kaplan-Meier method with log-rank tests and Cox proportional hazards models. Results A total of 220 consecutive patients were identified at our institute. Median age was 15.6 years (range, 0 to 81 years) and there were 108 children (49%) and 112 adult patients (51%). According to histological classification, 106 patients (48.2%) had RMS, 60 (27.3%) had EWS, 50 (22.7%) had PNET, and 4 (1.8%) had DSRCT. With a median follow-up period of 6.6 years, the estimated median overall survival (OS) of all patients was 75 months (95% confidence interval [CI], 27.2 to 122.8 months) and median event-free survival (EFS) for all patients was 11 months (95% CI, 8.8 to 13.2 months). No significant difference in OS and EFS was observed between adults and children. In multivariate analysis, distant metastasis (hazard ratio [HR], 1.617; 95% CI, 1.022 to 2.557; p=0.040) and no debulking surgery (HR, 1.443; 95% CI, 1.104 to 1.812; p=0.012) showed independent association with worse OS. Conclusion Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.

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Case Report

Multimodal Treatment of Primary Extraskeletal Ewing's Sarcoma of the Chest Wall: Report of 2 Cases: Woo Surng Lee, Yo Han Kim, Hyun Keun Chee, Jae Joon Hwang, Jun Seok Kim, Song Am Lee, Eun Gu Hwang, Yo Han Cho, Gyu Rak Chon; Cancer Res Treat. 2009;41(2):108-112. Published online June 30, 2009; DOI: https://doi.org/10.4143/crt.2009.41.2.108

Abstract PDF PubReader ePub

Extraskeletal Ewing's sarcoma (EES) is a type of Ewing's sarcoma that arises in soft tissue and is now regarded as a member of a family of small round cell neoplasms of bone and soft tissue, including primitive neuroectodermal tumors (PNETs). EES occurs predominantly in adolescents and young adults between the ages of 10 and 30 years. The disease follows an aggressive course with a high recurrence rate. The presence of a distant metastasis is also common. EES arises in the soft tissue of either the trunk or extremities. We recently experienced two cases of EES that occurred in the chest wall. The two patients underwent wide resection and combined radiochemotherapy. There was no evidence of disease 30 and 22 months, respectively, after surgery. Although extremely rare, EES should be considered in the differential diagnosis of chest wall tumors. We report two cases of EES with a brief review of the literature.

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Original Article

Immunohistochemical Study on Expression of the p53 Protein in Medulloblastoma/PNET: Eun Jung Kim, Sang Soo Park, Young Ho Lee, Ahn Hong Choi, Seo Hee Rha, Soon Yong Lee, Hye Kyoung Yoon, Young Tak Lim, Do Yoon Park, Kang Suek Suh; J Korean Cancer Assoc. 1997;29(5):867-873.

Abstract PDF: PURPOSE
The present study explores the expression rate of p53 mutation and the correlation between the expression of p53 protein and prognostic factors in medulloblastoma/ PNET (primitive neuroectodermal tumor).
MATERIALS AND METHODS
We studied retrospectively 24 patients with medulloblastoma/ PNET, who were admitted in Dong-A University Hospital, Pusan National University Hospital and Inje University Pusan Paik Hospital from 1988 to 1995. Detection of p53 mutations was made by immunohistochemical staining of p53 protein on paraffin- embedded tissues. The correlation between the expression of p53 protein and prognostic factors was evaluated by the Spearman correlation analysis.
RESULTS
p53 protein was expressed in 6 of 24 patients (25%). In 20 patients who could be evaluated for metastasis, 16 patients of M0, 1 patient of M1 and 3 patients of M2 were grouped by M stage, and the expression of p53 was detected in 1 of 16 M0 group (6.3%) and 3 of 3 M2 group (100%). p53 expression was significantly related to the M stage of medulloblastoma/PNET (r=0.73, p<0.001). The detection of p53 was not significantly associated with T stage, cellular differentiation and the relapse rate of medulloblastoma/ PNET.
CONCLUSION
The immunohistochemical detection rate of p53 protein in medulloblastoma/ PNET was 25%. The expression of p53 protein was significantly related to the M stage, with higher expression rate in M2 group of medulloblsatoma/PNET.

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