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A long-term survivor of clear cell sarcoma-like tumor of the gastrointestinal tract with liver metastasis: a case report

  • Okada, Takuhisa1, 2
  • Hirano, Yasumitsu1
  • Ishikawa, Shintaro1
  • Kondo, Hiroka1
  • Ishii, Toshimasa1
  • Yamaguchi, Shigeki1
  • 1 Saitama Medical University International Medical Center, 1397-1, Yamane, Hidaka-City, Saitama-Pref, 350-1298, Japan , Hidaka-City (Japan)
  • 2 Gunma University Graduate School of Medicine, 3-39-22 Showa-Machi, Maebashi-City, Gunma-Pref, 371-8511, Japan , Maebashi-City (Japan)
Published Article
Surgical Case Reports
Springer Berlin Heidelberg
Publication Date
Oct 06, 2020
DOI: 10.1186/s40792-020-01028-z
Springer Nature


BackgroundClear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is extremely rare. It is a mesenchymal neoplasm that usually forms in the small intestine of adolescents and young adults, is prone to local recurrence and metastasis, and has a high mortality rate. We report a patient with CCSLTGT with lymph node- and liver metastases, who continues to survive 6 years after initial surgical resection.Case presentationA 38-year-old woman presented with lightheadedness. Laboratory analysis revealed anemia (hemoglobin, 6.7 g/dL), and enhanced computed tomography (CT) demonstrated a mass in the small intestine, about 6 cm in diameter, with swelling of 2 regional lymph nodes. Double-balloon small intestine endoscopic examination revealed a tumor accompanied by an ulcer; the biopsy findings suggested a primary cancer of the small intestine. She was admitted, and we then performed a laparotomy for partial resection of the small intestine with lymph node dissection. Pathologic examination revealed CCSLTGT with regional lymph node metastases. About 3 years later, follow-up CT revealed a single liver metastasis. Consequently, she underwent a laparoscopic partial liver resection. Histopathologic examination confirmed that the liver metastasis was consistent with CCSLTGT. It has now been 3 years without a recurrence.ConclusionRepeated radical surgical resection with close follow-up may be the only way to achieve long-term survival in patients with CCLSTGT.

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