Parasuraman S, Rao B N, Bodner S, Cain A, Pratt C B, Merchant T E, Pappo A S
Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.
Pediatr Hematol Oncol. 1999 Nov-Dec;16(6):539-44. doi: 10.1080/088800199276831.
Clear cell sarcoma is a rare soft tissue neoplasm whose clinical behavior and outcome has not been previously characterized. This study reviewed the clinical characteristics and outcome of all children with clear cell sarcoma of the soft tissues who were treated at St. Jude Children's Research Hospital from March 1962 through August 1998. Of 225 children with nonrhabdomyosarcomatous soft tissue sarcomas, 5 (2.2%) were diagnosed with clear cell sarcoma. Median age at diagnosis was 15 years 3 months. Primary sites included the extremities (n = 3), chest wall (n = 1), and abdomen (n = 1). At diagnosis 3 patients had localized disease. Following surgical resection (n = 3), radiotherapy (n = 2), and chemotherapy (n = 1) all three survive disease-free 10, 11, and 90 months after diagnosis, respectively. The remaining two patients with metastatic disease at diagnosis died 21 days and 9 months after diagnosis. Clear cell sarcoma of the soft tissues is rare in pediatrics. Complete surgical resection with negative margins is the most effective treatment for this disease. Patients with metastatic disease are candidates for multiinstitutional chemotherapy trials.
透明细胞肉瘤是一种罕见的软组织肿瘤，其临床行为和预后此前尚未得到明确描述。本研究回顾了1962年3月至1998年8月在圣裘德儿童研究医院接受治疗的所有软组织透明细胞肉瘤患儿的临床特征和预后。在225例非横纹肌肉瘤性软组织肉瘤患儿中，5例（2.2%）被诊断为透明细胞肉瘤。诊断时的中位年龄为15岁3个月。原发部位包括四肢（n = 3）、胸壁（n = 1）和腹部（n = 1）。诊断时3例患者为局限性疾病。经过手术切除（n = 3）、放疗（n = 2）和化疗（n = 1）后，这3例患者分别在诊断后10个月、11个月和90个月无病存活。其余2例诊断时伴有转移性疾病的患者分别在诊断后21天和9个月死亡。软组织透明细胞肉瘤在儿科中较为罕见。切缘阴性的完整手术切除是治疗该疾病最有效的方法。伴有转移性疾病的患者适合参加多机构化疗试验。
