Moreno JC, Uson Junior PLS. Clear cell sarcoma of the pancreas, an unusual cancer with unusual metastatic site or unusual primary site? World J Clin Cases 2024; 12(25): 5653-5656 [PMID: 39247732 DOI: 10.12998/wjcc.v12.i25.5653]
Corresponding Author of This Article
Pedro Luiz Serrano Uson Junior, MD, Academic Editor, Academic Research, Assistant Professor, Teacher, Center for Personalized Medicine, Hospital Israelita Albert Einstein, Avenida Albert Einstein 627, São Paulo 05652-900, Brazil. pedro.serrano@einstein.br
Research Domain of This Article
Oncology
Article-Type of This Article
Editorial
Open-Access Policy of This Article
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World J Clin Cases. Sep 6, 2024; 12(25): 5653-5656 Published online Sep 6, 2024. doi: 10.12998/wjcc.v12.i25.5653
Clear cell sarcoma of the pancreas, an unusual cancer with unusual metastatic site or unusual primary site?
Jhenyffer Carvalho Moreno, Pedro Luiz Serrano Uson Junior
Jhenyffer Carvalho Moreno, Universidade 9 de Julho, Sao Paulo 01509120, Brazil
Pedro Luiz Serrano Uson Junior, Center for Personalized Medicine, Hospital Israelita Albert Einstein, São Paulo 05652900, Brazil
Pedro Luiz Serrano Uson Junior, Department of Oncology, Hospital do Coração, Sao Paulo 04004030, Brazil
Author contributions: Moreno JC and Uson Junior PLS wrote the article, reviewed and approved the final manuscript.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Pedro Luiz Serrano Uson Junior, MD, Academic Editor, Academic Research, Assistant Professor, Teacher, Center for Personalized Medicine, Hospital Israelita Albert Einstein, Avenida Albert Einstein 627, São Paulo 05652-900, Brazil. pedro.serrano@einstein.br
Received: March 20, 2024 Revised: April 29, 2024 Accepted: May 17, 2024 Published online: September 6, 2024 Processing time: 118 Days and 11.7 Hours
Abstract
Clear cell sarcoma (CCS) is a type of malignant tumor that can arise from tendons and aponeuroses. This malignant proliferation of cells with melanocytic lineage normally occurs in young patients, and it is normally identified in extremities. However, different sites including gastrointestinal organs are also described. Due difficulties in the molecular and histopathology evaluation, the diagnosis is often confused with malignant melanoma. Most cases are treated with surgical resection, but overall, the prognosis is poor. In this editorial, we will discuss a very interesting case of CCS identified in the pancreas. We will discuss the literature and controversies in the management of this type of cancer. Furthermore, we will address molecular strategies to be incorporated in those cases to better understand the primary location of the tumor. Finally, future perspectives of the field and new strategies of treatment will be described.
Core Tip: Clear cell sarcoma is a rare type of malignant tumor that can arise from tendons and aponeuroses. However, different primary sites including gastrointestinal organs are also described. Next generating sequencing evaluation can help identify molecular biomarkers that can help with the diagnosis and treatment. Although surgical resection is the mainstream treatment for most patients, the recurrence of the disease is common and normally is refractory to standard treatments including chemotherapy.