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KMID : 0387820170240020114
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Clinical Pediatric Hematology-Oncology
2017 Volume.24 No. 2 p.114 ~ p.120
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Clinical Features and Treatment Outcome of Soft Tissue Clear Cell Sarcoma
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An Hong-Yul
Baek Seung-Min
Kim Bo-Kyung
Choi Jung-Yoon
Hong Kyung-Taek
Hong Che-Ry
Choi Tae-Hyun
Kim Han-Soo
Kim Tae-Min
Kang Hyoung-Jin
Shin Hee-Young
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Abstract
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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.
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KEYWORD
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Sarcoma clear cell, Pediatrics, Korea, Retrospective studies
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