Abstract
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.
Highlights
Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is a very rare malignant tumor of mesenchymal origin
The entity of CCSLTGT was first described by Zambrano et al in 2003, when they reported 6 patients with a disease they called “osteoclast-rich tumor of the gastrointestinal tract”, with features resembling those of soft-tissue clear cell sarcoma (CCS) [1]
Since it is a malignant tumor originating from the interstitium of the wall of the gastrointestinal tract, it must be differentiated from gastrointestinal stromal tumors (GIST), synovial sarcoma, and malignant peripheral nerve sheath tumors [3]
Summary
Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is a very rare malignant tumor of mesenchymal origin. CCSLTGT has characteristic spindle-shaped tumor cells that proliferate in sheets, and osteoclast-like multinucleated giant cells are seen [1, 2]. The patient underwent a double-balloon small intestine endoscopy, which revealed a submucosal tumor accompanied by an ulcer (Fig. 2). We conferred the diagnosis of a primary malignant tumor of the small intestine with lymph node metastasis. Fluorescence in situ hybridization (FISH) using an EWSR1 probe revealed a split signal in the tumor cells: seemingly an EWSR1 gene fusion (Fig. 5). Taken together, these results yielded a diagnosis of CCSLTGT.
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