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      Metastatic clear cell sarcoma of the pancreas: An overview

      editorial
      Author(s):
      Publication date (Electronic):
      Journal: World Journal of Clinical Cases
      Publisher: Baishideng Publishing Group Inc
      Keywords: Clear cell sarcoma, Pancreas, Metastasis, Follow-up, Case report

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          Abstract

          Clear cell sarcoma (CCS) is a rare soft-tissue sarcoma that accounts for less than 1% of all cases and was originally reported in 1965. The incidence of CCS is estimated to be approximately 0.014/100000 depending on the surveillance, epidemiology and end results databases. CCS is a highly invasive type that mainly metastasizes to the lungs, followed by the bones and brain; however, pancreatic metastasis is relatively rare. It has a high probability of recurrence or metastasis and has a poor prognosis with a high mortality rate. Finally, even after recovery, it is fundamental to keep regular postoperative follow-up for CCS patients.

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          Most cited references31

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          CLEAR-CELL SARCOMA OF TENDONS AND APONEUROSES. AN ANALYSIS OF 21 CASES.

          Katherine M Enzinger (1965)
          Article 0 comments Cited 72 times – based on 0 reviews     Review now
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            Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients.

            Hiroyuki Tsuchiya, Akira Kawai, Hideo Morioka … (2007)
            Clear cell sarcoma (CCS) of tendons and aponeuroses (malignant melanoma of soft parts) is a rare melanocytic soft tissue sarcoma. The objective of this study was to determine the clinical features, prognostic factors, and optimal treatment policy for patients with this rare disease. Seventy-five consecutive patients with histologically confirmed CCS who received treatment between 1980 and 2004 were analyzed retrospectively. There were 41 men and 34 women, and the median age was 36 years. Sixty-five tumors were located in the extremities, and 10 tumors were located in the trunk. The median tumor size was 4 cm. Seventy-one patients underwent surgical excision, and 56 patients received chemotherapy. Sixteen patients developed local recurrences, and 52 patients developed metastasis. The overall patient survival rates was 47% at 5 years and 36% at 10 years. Univariate analysis showed that sex (P = .018), tumor size (P = .001), tumor depth (P = .002), TNM classification (P = .001), and surgical margin (P = .042) were significant prognostic factors. Among the 52 patients who presented with localized disease, sex (P = .023), tumor size (P = .002), tumor depth (P = .011), TNM classification (P = .004), and chemotherapy (P = .032) were identified as significant prognostic factors. Multivariate analysis showed that tumor size remained an independent prognostic factor in both groups. The current results supported the contention that early diagnosis and initial wide excision are essential for a favorable outcome of CCS. The role of chemotherapy for CCS should be investigated further. (c) 2006 American Cancer Society.
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              Clear Cell Sarcoma (Malignant Melanoma) of Soft Parts: A Clinicopathologic Study of 52 Cases

              O. Hocar, A. Le Cesne, S. Berissi … (2012)
              Clear cell sarcomas are aggressive, rare soft tissue tumors and their classification among melanoma or sarcoma is still undetermined due to their clinical, pathologic, and molecular properties found in both types of tumors. This is a retrospective study of 52 patients with CCS seen between April 1979 and April 2005 in two institutions. The EWS-ATF-1 fusion transcript was studied in 31 patients and an activating mutation of the BRAF or NRAS gene was researched in 22 patients. 30 men and 22 women, with a mean age of 33 were studied. Forty-three tumors (82.69%) were located in the extremities, specially the foot (19 tumors). Median initial tumor size was 4.8 cm (1 to 15 cm). Necrosis involving more than 50% of the tumor cells was found in 14 cases (26.92%). High mitotic rate (>10) was found in 25 cases (48.07%). The EWS/ATF-1 translocation was found in 28 (53.84%) of 31 patients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%). Among the tumor-associated parameters, only tumor size (>4 cm) emerged as a significant prognostic factor. Forty-nine patients had a localized disease at diagnosis (94.23%) and underwent surgical resection immediately (90%) or after neoadjuvant chemotherapy (CT) (10%). Various CT regimens were used in 37 patients (71.15%) with no significant efficacy. The 5- and 10-year OS rates were 59% and 41%, respectively. Tumor size was the only emerging prognosis factor in our series. Complete surgical resection remains the optimal treatment for this aggressive chemoresistant tumor.
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                Author and article information

                Contributors
                Rachid Ait Addi
                Journal
                Journal ID (nlm-ta): World J Clin Cases
                Journal ID (publisher-id): WJCC
                Title: World Journal of Clinical Cases
                Publisher: Baishideng Publishing Group Inc
                ISSN (Electronic): 2307-8960
                Publication date (Print): 16 October 2024
                Publication date (Electronic): 16 October 2024
                Volume: 12
                Issue: 29
                Pages: 6262-6265
                Affiliations
                Department of Biology, Independent Senior Researcher, Marrakesh 41020, Morocco. dr.rachid.aitaddi@ 123456gmail.com
                Author notes

                Author contributions: Ait Addi R contributed to study selection, data extraction, data synthesis, quality assessment, drafting and revision of the manuscript.

                Corresponding author: Rachid Ait Addi, Doctor, PhD, Academic Editor, Associate Professor, Lecturer, Research Associate, Research Scientist, Senior Editor, Senior Researcher, Surgeon, Department of Biology, Independent Senior Researcher, 1368 Rte Essaouira, N 03, Marrakesh 40000, Morocco. dr.rachid.aitaddi@ 123456gmail.com

                Article
                Other ID: jWJCC.v12.i29.pg6262 Publisher-manuscript ID: 94716
                DOI: 10.12998/wjcc.v12.i29.6262
                PMC ID: 11372525
                PubMed ID: 39417060
                SO-VID: 8651e7da-9080-417e-bdf8-1c271b8536fd
                Copyright © ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
                License:

                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.

                History
                Date received : 23 March 2024
                Date revision received : 2 July 2024
                Date accepted : 10 July 2024
                Categories
                Subject: Editorial

                Keywords: clear cell sarcoma,pancreas,metastasis,follow-up,case report
                Data availability:
                Keywords: clear cell sarcoma, pancreas, metastasis, follow-up, case report

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