Clear Cell Sarcoma-Like Tumor of the Gastrointestinal Tract

Clinical presentation and imaging characteristics of clear cell sarcoma-like tumour of the gastrointestinal tract with liver metastasis: a case description

“…Our patient underwent radical resection and quickly experienced recurrence without any further therapy. This is consistent with previous case studies that report increased possibility of recurrence and distant metastasis in patients who were diagnosed with metastasis at the first presentation (3,8,10). The patient was diagnosed with recrudescent lesions in both the stomach and the liver due to the distinguishing imaging characteristics that were observed at the initial visit.…”

“…These tumors often grow large before detection and are accompanied by hemorrhage, necrosis, or cystic changes. CCSLTGT grows slowly but is highly aggressive and prone to metastasis, leading to a poor prognosis and a low survival rate (1)(2)(3)(7)(8)(9)(10)(11). Due to the lack of specific clinical manifestations and the limitations of gastrointestinal endoscopy, diagnosis is difficult.…”

“…We made several notable observations by reviewing the pathological features and the few available CT images of CCSLTGT provided in the literature (7,11,12). The available case reports of intestinal CCSLTGT that vaguely describe imaging characteristics typically describe masses protruding from the cavity (8,9), which is different from CCSLTGT of the stomach. In our case, no lesions protruded from the cavity or broke through the serosa.…”

“…A surgical biopsy of the wall of the gastric antrum showed that the tumor was composed of epithelioid cells arranged in sheets and nests with local ulceration. Immunohistochemistry showed strong, positive expression of the focal nerve or neuroectodermal markers vimentin (+) and CD56 (+) and diffuse S100 (Figure 2), coupled with a complete absence of melanocytic markers (HMB45 and Melan-A) (7,8). The pathology of the tumor and the liver metastasis were consistent with CCSLTGT.…”

Clinical, pathological, and genetic profile of clear cell sarcoma-like tumour of jejunum: report of a rare aggressive tumour of small bowel

“…CCSLGT is mostly seen in young adults in the late 20 s and early 30 s. Most common site of involvement is the small bowel; however, they are reported to arise from colon and the stomach, as well [3]. They present with non-specific complaints like anaemia, fatigue, fever, and abdominal pain [4]. They are aggressive tumours which are metastatic at presentation or develop recurrence after surgical excision [5,6].…”