PDF(Pubmed)
Abstract：
Ewing sarcoma is one of the small round blue cell tumors of childhood that typically affects bone. Recently, a subgroup of undifferentiated round-cell sarcomas has been genetically identified as BCOR (B-cell Line 6 Corepressor)-altered sarcomas (BAS). We present a case of a six-year-old male child who presented with a chief complaint of shortness of breath and tachypnea and was found to have a large mediastinal mass concerning sarcoma. Preliminary biopsy results were positive for small round blue cells, possibly Ewing sarcoma. After six cycles of chemotherapy, with subsequent shrinkage of mediastinal mass, the patient was able to undergo wedge resection and excision of the mass with en bloc resection of the fifth and sixth rib, preserving his right lung. Final tissue pathology was positive for BAS. There have been only four reported cases of BAS of the chest wall and zero reported cases of primary tumor presentation of the lung, making this a rare presentation of the disease.
摘要：
尤因肉瘤是儿童时期的小圆形蓝色细胞肿瘤之一，通常会影响骨骼。最近,未分化的圆形细胞肉瘤的一个亚组已被遗传鉴定为BCOR（B细胞系6辅抑制因子）改变的肉瘤（BAS）。我们介绍了一例6岁的男性儿童，该儿童以呼吸急促和呼吸急促为主要主诉，并被发现有与肉瘤有关的纵隔肿块。初步活检结果为小圆形蓝色细胞阳性，可能是尤因肉瘤.经过六个周期的化疗，纵隔肿块随后收缩，患者能够进行楔形切除和切除肿块，同时整块切除第五和第六肋骨，保护他的右肺.最终组织病理为BAS阳性。仅有4例报告的胸壁BAS病例和零例报告的肺原发肿瘤表现病例，使这成为这种疾病的罕见表现。
