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

Abstract Background Clear 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 presentation A 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. Conclusion Repeated 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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Imprint cytology of clear cell sarcoma-like tumor of the gastrointestinal tract in the small intestine: A case report

Diagnostic Cytopathology ◽

10.1002/dc.23786 ◽

2017 ◽

Vol 45 (12) ◽

pp. 1137-1141 ◽

Cited By ~ 5

Author(s):

Takashi Kato ◽

Shin Ichihara ◽

Hiroko Gotoda ◽

Shunji Muraoka ◽

Terufumi Kubo ◽

...

Keyword(s):

Small Intestine ◽

Case Report ◽

Gastrointestinal Tract ◽

Clear Cell ◽

Clear Cell Sarcoma ◽

Imprint Cytology ◽

Cell Sarcoma

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Malignant Gastrointestinal Neuroectodermal Tumor: a case report and a review of the literature

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqab191.138 ◽

2021 ◽

Vol 156 (Supplement_1) ◽

pp. S66-S67

Author(s):

B Youssef ◽

D Asberry ◽

R Mohamed

Keyword(s):

Case Report ◽

Gastrointestinal Tract ◽

Clear Cell ◽

Muscularis Propria ◽

Clear Cell Sarcoma ◽

Neuroectodermal Tumor ◽

High Rate ◽

Cell Sarcoma ◽

Molecular Features ◽

Eosinophilic Cytoplasm

Abstract Introduction/Objective Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare soft tissue tumor arising in the wall of the gastrointestinal tract. The GNET was first described as an osteoclast rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma, with only few cases reported in the literature. Methods/Case Report We report a case of a 71-year-old man with a past medical history of hypertension, hyperlipidemia, and benign prostatic hyperplasia presented with complaints of dyspnea, dizziness, fatigue, black stools, and a recent syncopal episode. Laboratory testing revealed anemia (HB 5.4 g/dL). Esophagogastroduodenoscopy demonstrated a submucosal gastric mass. An abdominal CT scan confirmed a 7.8 cm mass along the gastric cardia and fundus. Results (if a Case Study enter NA) Biopsy rendered a gastrointestinal neuroectodermal tumor (GNET). Microscopically, the tumor cells were spindle with eosinophilic cytoplasm and arranged in fascicules. They were positive for CD56, CD99, and Fli-1, synaptophysin, and negative for chromogranin, TTF-1, SMA, desmin, CD34, EMA, pan-cytokeratin, and lymphoid markers. Two months later, further imaging confirmed metastasis to the liver and spleen. GNETs typically arise within the muscularis propria of the gastrointestinal tract and often extend into the submucosa and subserosa. Conclusion The most important differential of GNET is the clear cell sarcoma of the gastrointestinal tract (CCS-GI). Both share similar morphological as well as molecular features and show S100 positivity; however, the lack of melanocytic differentiation in GNET distinguishes it from CCS-GI. Both typically show rearrangements of the EWSR1 gene, with t(12;22) (q13;q12) EWSR1-ATF1 or t(2;22)(q34;q12) EWSR1-CREB1 fusions. Pathologists should be aware of GNET diagnostic entity due to its aggressive behavior and high rate of recurrence and mortality even after complete resection.

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Clear cell sarcoma-like tumor of the gastrointestinal tract presenting as a symptomatic metastasis to the humerus

Human Pathology Case Reports ◽

10.1016/j.ehpc.2017.11.002 ◽

2018 ◽

Vol 11 ◽

pp. 73-75

Author(s):

Howard J. Lee ◽

Emily McCracken ◽

Diana M. Cardona ◽

Dan G. Blazer

Keyword(s):

Gastrointestinal Tract ◽

Clear Cell ◽

Clear Cell Sarcoma ◽

Cell Sarcoma

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Clear cell sarcoma of tendons and aponeuroses, and osteoclast-rich tumour of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: a review and update

Journal of Clinical Pathology ◽

10.1136/jcp.2008.057471 ◽

2010 ◽

Vol 63 (5) ◽

pp. 416-423 ◽

Cited By ~ 72

Author(s):

Kemal Kosemehmetoglu ◽

Andrew L Folpe

Keyword(s):

Gastrointestinal Tract ◽

Young Adult ◽

Malignant Melanoma ◽

Soft Tissue ◽

Clear Cell ◽

Clear Cell Sarcoma ◽

Soft Tissue Neoplasm ◽

Cell Sarcoma ◽

Melanocytic Differentiation ◽

Gastrointestinal Neoplasm

Clear cell sarcoma (CCS) is a rare, distinctive soft tissue neoplasm, typically occurring in the distal extremities of young adult patients. Although CCS shows melanocytic differentiation, it is now clear that it is clinicopathologically and genetically distinct from conventional malignant melanoma. The ‘osteoclast-rich tumour of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts’ is an extraordinarily rare gastrointestinal neoplasm that shares some features of CCS, but differs from it in other ways. The historical, histopathological, ultrastructural, immunohistochemical and genetic aspects of these two tumours are reviewed in this article.

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