Clinical Features and Treatment Outcome of Soft Tissue Clear Cell Sarcoma
10.15264/cpho.2017.24.2.114
- Author:
Hong Yul AN
1
;
Seung Min BAEK
;
Bo Kyung KIM
;
Jung Yoon CHOI
;
Kyung Taek HONG
;
Che Ry HONG
;
Tae Hyun CHOI
;
Han Soo KIM
;
Tae Min KIM
;
Hyoung Jin KANG
;
Hee Young SHIN
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. kanghj@snu.ac.kr
- Publication Type:Original Article
- Keywords:
Sarcoma clear cell;
Pediatrics;
Korea;
Retrospective studies
- MeSH:
Diagnosis;
Drug Therapy;
Humans;
Korea;
Lower Extremity;
Neoplasm Metastasis;
Neural Crest;
Pediatrics;
Prognosis;
Radiotherapy;
Retrospective Studies;
Sarcoma, Clear Cell;
Seoul;
Treatment Outcome
- From:Clinical Pediatric Hematology-Oncology
2017;24(2):114-120
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
BACKGROUND: Soft tissue clear cell sarcoma is a rare tumor which originates from neural crest cells. Due to its rarity and lack of established treatment, the prognosis of clear cell sarcoma is poor. Here, we reviewed the clinical data and outcome of patients diagnosed with soft tissue clear cell sarcoma in our institution.METHODS: A retrospective study was conducted on pediatric patients who were treated for pathologically confirmed soft tissue clear cell sarcoma at the Seoul National University Hospital, between January 2000 and July 2017.RESULTS: Six patients (3 boys and 3 girls) were diagnosed with soft tissue clear cell sarcoma at a median age of 14 years 4 months (range 11 years 7 months - 19 years 3 months). The median size of the tumor was 5.6 cm (range, 0.6 cm to 7.9 cm). The most frequent symptom was pain (67%), and the most common primary site was the lower limb (67%). Three patients (50%) presented with metastases at diagnosis. Four patients underwent chemotherapy with various therapeutic combinations. Four patients received surgical resection. Only one patient received local radiotherapy. One patient died of primary refractory disease, three patients relapsed, while the remaining two survive event-free.CONCLUSION: Soft tissue clear cell sarcoma is a rare and highly aggressive tumor, for which there is no established treatment. All surviving patients received surgery, indicating that surgery is a key treatment modality. Further genetic studies of soft tissue clear cell sarcoma are needed to find a better treatment strategy.
