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      Complete resection and arterial reconstruction for primary sarcoma arising from superior mesenteric artery

      case-report

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          Abstract

          In the treatment of retroperitoneal sarcoma involving major vessels, complete resection with vascular reconstruction is challenging. We describe the case of a 72-year-old man who presented with 8 months of abdominal pain. Diagnostic workup revealed occlusion of the celiac trunk and the origin of the superior mesenteric artery due to a soft tissue sarcoma. Radical resection of the tumor and vessels was performed. Guided by intraoperative angiography, arterial reconstruction was performed without mesenteric ischemia. All arterial margins were negative. At the 6-year follow-up, the patient was alive with no evidence of recurrence.

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          Guidelines for the Management of Soft Tissue Sarcomas

          Robert Grimer, Ian Judson, David Peake … (2010)
          These guidelines were drawn up following a consensus meeting of UK sarcoma specialists convened under the auspices of the British Sarcoma Group and are intended to provide a framework for the multidisciplinary care of patients with soft tissue sarcomas. The guidelines published by the European Society of Medical Oncology (ESMO) and the National Comprehensive Cancer Network (NCCN) were used as the basis for discussion and adapted according to UK clinical practice and local requirements. Note was also taken of the National Institute for Health and Clinical Excellence (NICE) improving outcomes guidance (IOG) for people with sarcoma and existing technology appraisals. The guidelines are not intended to challenge NICE guidance but discrepancies may exist where current guidance does not reflect an international standard of care owing to the ever-evolving nature of cancer treatment. It is acknowledged that these guidelines will require updating on a regular basis. An appendix lists the key recommendations which are summarised below. Any patient with a suspected soft tissue sarcoma should be referred to a diagnostic centre and managed by a specialist sarcoma multidisciplinary team. Surgical excision followed by post operative radiotherapy is the standard management of high grade limb sarcomas although occasionally amputation remains the only option. Pre-operative treatment with chemotherapy or radiotherapy should be considered for patients with borderline resectable tumours. Isolated limb perfusion may permit limb salvage in some cases where amputation is the only other option. Adjuvant chemotherapy is not routinely recommended but may be considered in certain specific situations. Regular follow up is recommended to assess local control and the development of metastatic disease. Single agent doxorubicin is the standard first line therapy for metastatic disease. Ifosfamide is an alternative if anthracyclines are contraindicated. Combination therapy may be considered in individual patients. Second line agents include ifosfamide, dacarbazine, trabectedin and the combination of gemcitabine + docetaxel. Surgical resection of local recurrence and pulmonary metastases should be considered in individual patients. There is specific guidance on the management of retroperitoneal and uterine sarcomas.
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            Patterns of care and outcomes of patients with METAstatic soft tissue SARComa in a real-life setting: the METASARC observational study

            Marion Savina, Axel Le Cesne, Jean-Yves Blay … (2017)
            Background Well-designed observational studies of individuals with rare tumors are needed to improve patient care, clinical investigations, and the education of healthcare professionals. Methods The patterns of care, outcomes, and prognostic factors of a cohort of 2225 patients with metastatic soft tissue sarcomas who were diagnosed between 1990 and 2013 and documented in the prospectively maintained database of the French Sarcoma Group were analyzed. Results The median number of systemic treatments was 3 (range, 1–6); 27% of the patients did not receive any systemic treatment and 1054 (49%) patients underwent locoregional treatment of the metastasis. Half of the patients who underwent chemotherapy (n = 810) received an off-label drug. Leiomyosarcoma was associated with a significantly better outcome than the other histological subtypes. With the exception of leiomyosarcomas, the benefit of a greater than third-line regimen was very limited, with a median time to next treatment (TNT) and overall survival (OS) ranging between 2.3 and 3.7 months and 5.4 and 8.5 months, respectively. The TNT was highly correlated with OS. Female sex, leiomyosarcoma histology, locoregional treatment of metastases, inclusion in a clinical trial, and treatment with first-line polychemotherapy were significantly associated with improved OS in the multivariate analysis. Conclusions The combination of doxorubicin with a second drug, such as ifosfamide, represents a valid option, particularly when tumor shrinkage is expected to provide clinical benefits. After failure of the second-line therapy, best supportive care should be considered, particularly in patients with non-leiomyosarcoma histology who are not eligible to participate in a clinical trial. Locoregional treatment of metastasis should always be included in the therapeutic strategy when feasible. TNT may represent a useful surrogate endpoint for OS in clinical studies. Electronic supplementary material The online version of this article (doi:10.1186/s12916-017-0831-7) contains supplementary material, which is available to authorized users.
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              Management of primary retroperitoneal sarcoma (RPS) in the adult: a consensus approach from the Trans-Atlantic RPS Working Group.

              (2014)
              Retroperitoneal soft tissue sarcomas (RPS) are rare tumors that include several well-defined histologic subtypes. Although surgery is the mainstay of curative therapy, no universally accepted recommendations concerning the best management have been developed to date. Optimization of the initial approach is critical for maximizing patient outcomes. An RPS Trans-Atlantic Working Group was established in 2013. The primary aim was to evaluate the current evidence critically and to develop a consensus document on the approach to this difficult disease. The outcome applies to primary RPS that is nonvisceral in origin. The evaluation included sarcomas of major veins (inferior vena cava, renal vein, ovarian/testicular vein), undifferentiated pleomorphic sarcoma of the psoas, and ureteric leiomyosarcoma (LMS). It excluded desmoid, lipoma and angiomyolipoma, gastrointestinal stromal tumors, visceral sarcomas such as those arising from the gut or its mesentery, uterine LMS, prostatic sarcoma, paratesticular/spermatic cord sarcoma, Ewing's sarcoma, alveolar/embryonal rhabdomyosarcoma, primitive peripheral neuro-ectodermal tumor, sarcoma arising from teratoma, carcinosarcoma, sarcomatoid carcinoma, clear cell sarcoma, radiation-induced sarcoma, paraganglioma, and malignant pheochromocytoma. Management of RPS was evaluated from diagnosis to follow-up, and a level of evidence was attributed to each statement. This rare and complex malignancy is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, and an individualized management plan should be made based on the statements included in this article. International collaboration is critical for adding to the current knowledge. A prospective registry will be set up.
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                Author and article information

                Contributors
                Yoshikatsu Nomura
                Journal
                Journal ID (nlm-ta): J Vasc Surg Cases Innov Tech
                Journal ID (iso-abbrev): J Vasc Surg Cases Innov Tech
                Title: Journal of Vascular Surgery Cases, Innovations and Techniques
                Publisher: Elsevier
                ISSN (Electronic): 2468-4287
                Publication date PMC-release: 23 December 2021
                Publication date Collection: March 2022
                Publication date (Electronic): 23 December 2021
                Volume: 8
                Issue: 1
                Pages: 70-74
                Affiliations
                [a ]Department of Cardiovascular Surgery, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan
                [b ]Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan
                [c ]Division of Hepato-Biliary-Pancreatic Surgery, Department of Surgery, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan
                Author notes
                [∗ ]Correspondence: Yoshikatsu Nomura, MD, PhD, Department of Cardiovascular Surgery, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-cho, Chuou-ku, Kobe, Hyogo 650-0017, Japan y_katsu1027@ 123456yahoo.co.jp
                Article
                Publisher Item ID: S2468-4287(21)00203-3
                DOI: 10.1016/j.jvscit.2021.11.008
                PMC ID: 8790618
                PubMed ID: 35112036
                SO-VID: 355cb7c1-b186-4669-a581-e56984f46f8b
                Copyright © © 2021 The Author(s)
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 21 September 2021
                Date accepted : 23 November 2021
                Categories
                Subject: Case report

                Keywords: pleomorphic sarcoma,arterial reconstruction,complete resection
                Data availability:
                Keywords: pleomorphic sarcoma, arterial reconstruction, complete resection

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