Clear Cell Sarcoma of the Ileum

Donner, Ludvik R.; Trompler, Raymond A.; Dobin, Sheila M.

doi:10.1097/00000478-199801000-00016

Cited by 59 publications

(1 citation statement)

References 13 publications

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“…However, a similar case was already reported in 1985 under the term malignant neuroendocrine tumor of the jejunum with osteoclast-like giant cells [107]. In 1998, it became apparent that these tumors share the same genetic characteristics [108], with most tumors harboring an EWSR1-CREB1 fusion and less often an EWSR1-ATF1 fusion [109]. Since then, there has been discussion whether these tumors are CCSs or a separate entity as they have morphological and genetic similarities [21,[110][111][112].…”

Section: Clear-cell Sarcoma-like Tumor Of the Gastrointestinal Tract (Osteoclastrich Tumor Of The Gastrointestinal Tract Or Malignant Gasmentioning

confidence: 57%

EWSR1—The Most Common Rearranged Gene in Soft Tissue Lesions, Which Also Occurs in Different Bone Lesions: An Updated Review

et al. 2021

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EWSR1 belongs to the FET family of RNA-binding proteins including also Fused in Sarcoma (FUS), and TATA-box binding protein Associated Factor 15 (TAF15). As consequence of the multifunctional role of EWSR1 leading to a high frequency of transcription of the chromosomal region where the gene is located, EWSR1 is exposed to aberrations such as rearrangements. Consecutive binding to other genes leads to chimeric proteins inducing oncogenesis. The other TET family members are homologous. With the advent of widely used modern molecular techniques during the last decades, it has become obvious that EWSR1 is involved in the development of diverse benign and malignant tumors with mesenchymal, neuroectodermal, and epithelial/myoepithelial features. As oncogenic transformation mediated by EWSR1-fusion proteins leads to such diverse tumor types, there must be a selection on the multipotent stem cell level. In this review, we will focus on the wide variety of soft tissue and bone entities, including benign and malignant lesions, harboring EWSR1 rearrangement. Fusion gene analysis is the diagnostic gold standard in most of these tumors. We present clinicopathologic, immunohistochemical, and molecular features and discuss differential diagnoses.

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Section: Clear-cell Sarcoma-like Tumor Of the Gastrointestinal Tract (Osteoclastrich Tumor Of The Gastrointestinal Tract Or Malignant Gasmentioning

confidence: 57%

EWSR1—The Most Common Rearranged Gene in Soft Tissue Lesions, Which Also Occurs in Different Bone Lesions: An Updated Review

et al. 2021

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Clear cell sarcoma of the stomach

Lagmay

Ranalli

Arcila

et al. 2009

Pediatric Blood & Cancer

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Clear cell sarcoma (CCS) is a high grade soft tissue sarcoma with a distinct molecular profile. Gastrointestinal CCS is very rare and most reported cases are in adults. We describe a 10-year-old female with a 4-month history of anemia who later developed fever, weight loss and abdominal pain. She was subsequently found to have a large infiltrative gastric mass. A diagnosis of CCS was confirmed by molecular and cytogenetic studies. This case illustrates the necessity of a multimodal approach, particularly the use of molecular studies, in the diagnostic evaluation of rare tumors presenting in unusual sites.

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