分子特征和临床结果在121例-经验从三级转诊中心在南印度

A. Prabhu, Kiruthiga K G, R. Pai, L. Mathew, S. Backianathan
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尤文氏肉瘤是儿童和青少年骨骼中第二常见的肉瘤。关于印度尤文氏肉瘤患者的临床特征、形态学、易位和随访的公开资料很少。本研究的目的是分析Ewing肉瘤的临床病理特征,并比较易位情况、bor免疫组织化学、治疗和生存。2008-2015年在韦洛尔基督教医学院病理系诊断出406例尤文氏肉瘤。本研究纳入135例通过RT-PCR进行常见易位分子检测的患者。患者确诊时的平均年龄为22岁。肿瘤最常见的部位是附肢骨骼(31.4%),19%的肿瘤累及实体器官。21.5%的患者存在远处转移。易位63例(46.7%),EWS-FLI1 I型(87%),EWS-FLI1 II型(11%),1例EWS-ERG易位。30例患者中有7例为“BCOR基因改变肉瘤”。接受6个化疗周期的患者平均生存期较好。平均随访13.72个月,3年无事件生存率为93.8%。肿瘤存活率<10%是预测生存的唯一重要组织学参数。诊断年龄≤15岁,性别为女性,体型≤12cm,骨外部位,化疗和易位阳性预测生存率提高。本研究分析了印度尤因肉瘤患者的突变/易位类型,并将影响生存的临床和病理因素联系起来。
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Molecular profile & clinical outcome in 121 cases - experience from a tertiary referral centre in South India
Ewing sarcoma is the second most common sarcoma involving the bones in children and adolescents. Published data on the clinical features, morphology, translocation and follow-up of patients with Ewing sarcoma from India, is sparse. Objectives of this study were to analyse the clinico-pathologic features of Ewing sarcoma and compare with translocation status, BCoR immunohistochemistry, treatment and survival. 406 cases of Ewing sarcoma were diagnosed in the Department of Pathology, Christian Medical College, Vellore during the period 2008-2015. 135 patients underwent molecular testing for common translocations by RT-PCR and were included in this study.Mean age of patients at diagnosis was 22 years. Most common location of tumour was appendicular skeleton (31.4%) and 19% had solid organ involvement. Distant metastasis was present in 21.5% patients. Translocation was seen in 63 (46.7%) cases, EWS-FLI1 type I (87%), EWS-FLI1 type II(11%) and one patient had EWS-ERG translocation. Seven out of 30 patients were classified as “Sarcoma with BCOR genetic alteration”. Patients who underwent 6 cycles of chemotherapy had a better mean survival. Mean follow-up was 13.72 months and 3 year event free survival of patients was 93.8%.Percentage of viable tumour <10% was the only significant histologic parameter predicting survival. Age at diagnosis ≤15yrs, female sex, size ≤12cm, extra-osseous site, chemotherapy and translocation positivity predicted an improved survival. This study has analysed the type of mutations/translocations seen in a subset of Indian patients with Ewing sarcoma and correlated the clinical and pathological factors affecting survival.
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