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      Classic Type of Epithelioid Sarcoma of the Distal Upper Extremity: Clinical and Oncological Characteristics


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          The classic type of epithelioid sarcoma (ES) is a rare, aggressive soft tissue neoplasm that most commonly affects the distal upper extremities of young patients. This study aimed to assess clinical features and provide a long-term report of the oncological outcome.


          We retrospectively analyzed our clinical database for patients with ES of the distal upper extremities.


          Twenty-three patients with ES of the distal upper extremity were treated surgically between January 1990 and August 2018. ES affected most commonly the palmar side of young patients. The most common site affected by a sarcoma was the wrist in 47.8% of cases, followed by metacarpals and fingers with 34.8% and 17.4%, respectively. Most of the patients were treated according to the protocols of interdisciplinary tumor boards with multimodal therapy. A local recurrence was observed in 7 patients (30.4%). The 5 - and 10-year recurrence-free survival was 80.4% (95% confidence interval [CI]: 68.6-76.8) and 60.9% (95% CI: 53.5-68.3), respectively. The 5- and 10-years disease-specific survival was 89.9% (95% CI: 87-92.8) and 61.9% (95% CI: 56.5-67.3), respectively. Five patients (21.7%) had metastasis in regional lymph nodes.


          The classic type of ES represents a group of high-grade sarcomas, which affect the dominantly distal upper extremity. Specific clinical, diagnostic, and oncological characteristics make it difficult to diagnose and therapy. Wide tumor resection as a part of multimodal therapy remains a more viable and common treatment option for patients with ES on distal extremities. High rates of lymph node metastasis are typical for ES.

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          Most cited references32

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          Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma.

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            Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology

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              Prognosis of lymph node metastasis in soft tissue sarcoma.

              We defined the tumor characteristics and prognosis of patients with regional lymph node metastasis (RLNM) from soft tissue sarcoma. All patients with RLNM from soft tissue sarcoma were identified from the Royal Marsden Hospital Sarcoma Unit prospective database from January 1990. A total of 73 (3.4%) of 2127 patients had RLNM. Fifty-seven patients (78.1%) had RLNM as the first site of spread, and 16 patients (21.9%) presented with RLNM and distant metastasis synchronously. The most common histologies were rhabdomyosarcoma, epithelioid sarcoma, and angiosarcoma. There were 9 T1 and 36 T2 tumors, and tumor size was not available in 28 patients. There were 6 grade 1, 16 grade 2, and 51 grade 3 tumors. Forty-two patients presented with RLNM along with the primary tumor. Of the remaining patients, the median time to development of RLNM from diagnosis of the primary tumor was 13.5 months (95% confidence interval [CI], 1-100 months). The 1-year survival for patients with isolated RLNM was 77.49% (95% CI, 62.99%-86.88%), compared with 36.27% (95% CI, 13.32%-60.04%) for patients who presented with RLNM and distant metastasis (P =.005). The 1-year survival for metachronous and synchronous RLNM was 94.44% (95% CI, 66.64%-99.20%) and 67.54% (95% CI, 47.89%-81.12%), respectively (P =.05). Lymph node metastasis is rare. Patients who present with isolated RLNM have an improved survival compared with patients who present with regional and distant metastasis at diagnosis. Synchronous RLNM with the primary tumor have a poorer outcome than metachronous RLNM in the absence of distant metastasis.

                Author and article information

                Hand (N Y)
                Hand (N Y)
                Hand (New York, N.Y.)
                SAGE Publications (Sage CA: Los Angeles, CA )
                20 February 2022
                September 2023
                20 February 2022
                : 18
                : 6
                : 1037-1043
                [1 ]Department of Trauma, Hand and Reconstructive Surgery/Division Plastic and Reconstructive Surgery, University Hospital Essen, Germany
                [2 ]Department of Tumor Orthopedics and Sarcoma Surgery, University Hospital Essen, Germany
                [3 ]Department of Plastic and Reconstructive Surgery, St. Rochus-Hospital, Castrop-Rauxel, Germany
                [4 ]Ruhr-University Bochum, Germany
                Author notes
                [*]Farhad Farzaliyev, Department of Trauma, Hand and Reconstructive Surgery/Department of Tumor Orthopedics and Sarcoma Surgery, Division Plastic and Reconstructive Surgery, University Hospital Essen, Hufelandstr.55, Essen 45147, Germany. Email: farhad.farzaliyev@ 123456uk-essen.de
                Author information
                © The Author(s) 2022

                This article is distributed under the terms of the Creative Commons Attribution 4.0 License ( https://creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

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                tumor,diagnosis,surgery,specialty,wrist,anatomy,hand,outcomes,research & health outcomes
                tumor, diagnosis, surgery, specialty, wrist, anatomy, hand, outcomes, research & health outcomes


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