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Original Article
Clinical Features and Treatment Outcome of Soft Tissue Clear Cell Sarcoma
Clin Pediatr Hematol Oncol 2017;24:114-20.
Published online October 31, 2017
© 2017 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

Hong Yul An, M.D.1,2, Seung Min Baek, M.D.1,2, Bo Kyung Kim, M.D.1,2, Jung Yoon Choi, M.D.1,2, Kyung Taek Hong, M.D.1,2, Che Ry Hong, M.D.1,2, Tae Hyun Choi, M.D., Ph.D.3, Han-Soo Kim, M.D., Ph.D.4, Tae Min Kim, M.D., Ph.D.5, Hyoung Jin Kang, M.D., Ph.D.1,2 and Hee Young Shin, M.D., Ph.D.1,2

1Department of Pediatrics, 2Cancer Research Institute, 3Department of Plastic and Reconstructive Surgery, 4Department of Orthopedic Surgery, 5Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
Correspondence to: Hyoung Jin Kang
Division of Hematology/Oncology, Department of Pediatrics and Cancer Research Institute, Seoul National University College Of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03722, Korea
Tel: +82-2-2072-3304 Fax: +82-2-743-3455 E-mail: kanghj@snu.ac.kr
Received September 25, 2017; Revised October 7, 2017; Accepted October 9, 2017.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
Keywords: Sarcoma clear cell, Pediatrics, Korea, Retrospective studies
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April 2018, 25 (1)
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  • Hyoung Jin Kang 

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