Clear cell sarcoma originating in the anterior mediastinum

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• 作者：Katsunori Masaki (1)
  Katsuhiko Naoki (1)
  Kenzo Soejima (1)
  Koichi Sayama (1)
  Nobufumi Kamiishi (1)
  Kyuto Tanaka (1)
  Kota Ishioka (1)
  Aya Sasaki (2)
  Yuichiro Hayashi (2)
  Takeru Funakoshi (4)
  Michiro Susa (3)
  Hideo Morioka (3)
  Sadatomo Tasaka (1)
  Koichiro Asano (1)
  Tomoko Betsuyaku (1)
  
• 关键词：Clear cell sarcoma ; Mediastinal tumor ; EWS gene rearrangement ; Malignant melanoma
• 刊名：International Cancer Conference Journal
• 出版年：2013
• 出版时间：October 2013
• 年：2013
• 卷：2
• 期：4
• 页码：211-214
• 全文大小：
• 作者单位：Katsunori Masaki (1)
  Katsuhiko Naoki (1)
  Kenzo Soejima (1)
  Koichi Sayama (1)
  Nobufumi Kamiishi (1)
  Kyuto Tanaka (1)
  Kota Ishioka (1)
  Aya Sasaki (2)
  Yuichiro Hayashi (2)
  Takeru Funakoshi (4)
  Michiro Susa (3)
  Hideo Morioka (3)
  Sadatomo Tasaka (1)
  Koichiro Asano (1)
  Tomoko Betsuyaku (1)
  
  1. Division of Pulmonary Medicine, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan
  2. Department of Pathology, Keio University School of Medicine, Tokyo, Japan
  4. Department of Dermatology, Keio University School of Medicine, Tokyo, Japan
  3. Department of Orthopedic Surgery, Keio University School of Medicine, Tokyo, Japan
  
• ISSN：2192-3183

文摘

A 34-year-old man with intermittent chest pain had a 60-mm mass in the anterior mediastinum. A CT-guided needle biopsy revealed that the tumor was composed of atypical small cells and was positive for HMB-45 without cytoplasmic melanin pigmentation. These results suggested a diagnosis of malignant melanoma (MM) or clear cell sarcoma (CCS). EWS gene rearrangement was detected by fluorescence in situ hybridization, resulting in the definite diagnosis of CCS. Sarcomas originating in the mediastinum account for only 1?% of all sarcoma cases. CCS is a rare soft tissue sarcoma, which usually occurs in the tendons and aponeuroses and is often misdiagnosed as MM because of similar immunophenotypes. There is only one reported case of a primary CCS of the mediastinum. There is no standard treatment for CCS, but for metastatic MM, dacarbazine, ipilimumab, and BRAF-inhibitors can be treatments of choice. Thus, differential diagnoses are important to determine treatment strategies. Meticulous observation of skin lesions and pathological and molecular diagnoses, including EWS gene rearrangement, is recommended in cases with mediastinal tumors suspected of MM or CCS.