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Yanar F, Öner G, Yüksel S, Salduz A, Azamat İF, Özçınar B, Bilgic B, Günöz Cömert R, Kozanoğlu E, Kurtoğlu M. Femoral artery aneurysm developed on intimal sarcoma: Case report. Turk J Surg 2022; 38:306-311. [PMID: 36846070 PMCID: PMC9948668 DOI: 10.47717/turkjsurg.2022.4381] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/11/2020] [Accepted: 05/05/2020] [Indexed: 12/24/2022]
Abstract
Intimal angiosarcoma is a rare vascular malignancy, and diagnosis is very difficult due to nonspecific symptoms. There are controversial points regarding the diagnosis, treatment and follow-up of intimal angiosarcomas. The purpose of this case report was to evaluate the diagnosis and treatment process of a patient diagnosed with femoral artery intimal angiosarcoma. Furthermore, in line with previous studies, it was aimed to illuminate controversial points. A 33-year-old male patient, who had been operated on due to ruptured femoral artery aneurysm, was diagnosed with intimal angiosarcoma with the pathology result. Recurrence was observed during clinical follow-up, and the patient was treated with chemotherapy and radiotherapy. Since there was no response to treatment, the patient underwent aggressive surgery including the surrounding tissues. No recurrence or metastasis was observed in the patient's 10th month follow-up. Although intimal angiosarcoma is rare, it should be considered in differential diagnosis when femoral artery aneurysm is detected. The most important step in treatment is aggressive surgery, but adding chemo-radiotherapy to the treatment should be considered.
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Affiliation(s)
- Fatih Yanar
- Department of General Surgery, İstanbul University Faculty of Medicine, İstanbul, Türkiye
| | - Gizem Öner
- Multidisciplinary Oncologic Centre (MOCA), Antwerpen University Hospital, Antwerpen, Belgium
| | - Sercan Yüksel
- Department of General Surgery, İstanbul University Faculty of Medicine, İstanbul, Türkiye
| | - Ahmet Salduz
- Department of Orthopaedic Surgery and Traumatology, İstanbul University Faculty of Medicine, İstanbul, Türkiye
| | - İbrahim Fethi Azamat
- Department of General Surgery, İstanbul University Faculty of Medicine, İstanbul, Türkiye
| | - Beyza Özçınar
- Department of General Surgery, İstanbul University Faculty of Medicine, İstanbul, Türkiye
| | - Bilge Bilgic
- Department of Pathology, İstanbul University Faculty of Medicine, İstanbul, Türkiye
| | - Rana Günöz Cömert
- Department of Radiology, İstanbul University Faculty of Medicine, İstanbul, Türkiye
| | - Erol Kozanoğlu
- Department of Plastic and Reconstructive Surgery, İstanbul University Faculty of Medicine, İstanbul, Türkiye
| | - Mehmet Kurtoğlu
- Department of General Surgery, İstanbul University Faculty of Medicine, İstanbul, Türkiye
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2
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Abstract
➤ Soft-tissue sarcomas (STS) in adults comprise a heterogeneous group of tumors of mesenchymal origin that share similar biological patterns of local tumor growth and metastatic dissemination. ➤ The judicious use of imaging studies, biopsy techniques, and pathological evaluations is essential for accurate diagnosis and for planning treatment strategy. ➤ Wide local resection and radiation therapy form the cornerstone of management of high-grade STS. The role of adjuvant radiation therapy is questionable in the management of small (≤5 cm) superficial lesions that can be resected with negative margins. Chemotherapy given to patients who have nonmetastatic, high-grade STS results in varying benefit in terms of local tumor control and overall survival. ➤ Coordinated care by a multidisciplinary team of trained surgeons, medical oncologists, radiologists, radiation oncologists, and pathologists has been documented to improve local tumor control and the overall survival of patients with STS. ➤ Although considerable progress in tumor diagnostics and targeted therapies has been made over the past 2 decades, recommendations and controversies relating to tumor imaging and margins of resection have not changed.
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Affiliation(s)
- Jacob Bickels
- Unit of Orthopedic Oncology, Orthopedic Division, Hillel-Yaffe Medical Center, Hadera, Israel, affiliated with the Rappaport Faculty of Medicine, The Technion, Haifa, Israel
| | - Martin M Malawer
- Department of Orthopedic Surgery, Georgetown University School of Medicine, Washington, DC
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3
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Neoadjuvant Radiotherapy-Related Wound Morbidity in Soft Tissue Sarcoma: Perspectives for Radioprotective Agents. Cancers (Basel) 2020; 12:cancers12082258. [PMID: 32806601 PMCID: PMC7465163 DOI: 10.3390/cancers12082258] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2020] [Revised: 07/10/2020] [Accepted: 08/10/2020] [Indexed: 12/18/2022] Open
Abstract
Historically, patients with localized soft tissue sarcomas (STS) of the extremities would undergo limb amputation. It was subsequently determined that the addition of radiation therapy (RT) delivered prior to (neoadjuvant) or after (adjuvant) a limb-sparing surgical resection yielded equivalent survival outcomes to amputation in appropriate patients. Generally, neoadjuvant radiation offers decreased volume and dose of high-intensity radiation to normal tissue and increased chance of achieving negative surgical margins-but also increases wound healing complications when compared to adjuvant radiotherapy. This review elaborates on the current neoadjuvant/adjuvant RT approaches, wound healing complications in STS, and the potential application of novel radioprotective agents to minimize radiation-induced normal tissue toxicity.
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Gómez J, Tsagozis P. Multidisciplinary treatment of soft tissue sarcomas: An update. World J Clin Oncol 2020; 11:180-189. [PMID: 32355640 PMCID: PMC7186235 DOI: 10.5306/wjco.v11.i4.180] [Citation(s) in RCA: 20] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2019] [Revised: 03/13/2020] [Accepted: 03/22/2020] [Indexed: 02/06/2023] Open
Abstract
Standard treatment for soft tissue sarcoma, based on complete surgical resection with or without adjuvant radiotherapy and chemotherapy, has not substantially changed during the last several decades. Nevertheless, recent advances have contributed to considerable improvement in the management of these patients; for example, new magnetic resonance imaging sequences such as diffusion-weighted imaging and magnetic resonance imaging radiomics can better assess tumor extension and even estimate its grade. Detection of circulating genetic material (liquid biopsy) and next-generation sequencing are powerful techniques for genetic analysis, which will increase our understanding of the underlying molecular mechanisms and may reveal potential therapeutic targets. The role of chemotherapy in non-metastatic disease is still controversial, and there is a need to identify patients who really benefit from this treatment. Novel chemotherapeutic regimens have entered clinical praxis and can change the outcome of patients with metastatic disease. Advances in radiotherapy have helped decrease local adverse effects and sustain good local control of the disease. The following report provides an updated view of the diagnosis, treatment, and future perspectives on the management of patients with soft tissue sarcomas.
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Affiliation(s)
- Jorge Gómez
- Department of Orthopedic Surgery, Clínica Universidad de Navarra, University of Navarra, Pamplona 31008, Spain
| | - Panagiotis Tsagozis
- Department of Molecular Medicine and Surgery, Karolinska Institute, Stockholm 17176, Sweden
- Muskuloskeletal Tumour Service, Karolinska University Hospital, Stockholm 17176, Sweden
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Villano AM, Zeymo A, Chan KS, Unger KR, Shara N, Al-Refaie WB. Variations in Retroperitoneal Soft Tissue Sarcoma Outcomes by Hospital Type: A National Cancer Database Analysis. JCO Oncol Pract 2020; 16:e991-e1003. [PMID: 32267809 DOI: 10.1200/jop.19.00460] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023] Open
Abstract
PURPOSE Surgical resection remains the cornerstone of retroperitoneal soft tissue sarcoma (RPS) treatment. Patient- and sarcoma-related factors are well known to influence survival outcomes. The effect of hospital-related factors on long-term survival, however, are not well understood. We sought to assess the relative contribution of hospital-level factors to mortality after surgical treatment of RPS. METHODS AND MATERIALS The 2004-2015 National Cancer Database was used to identify 10,113 patients who underwent surgical treatment of RPS. Patient-, sarcoma-, hospital-, and treatment-level factors were compared by increasing survival times. Stepwise multivariable Cox regression was performed that controlled for covariates to measure the relative contributions of these factors on overall survival (OS). Effect modification analyses ascertained how hospital type modulates the volume relationship with respect to RPS mortality. RESULTS Factors predictive of worsening OS were older age, nonprivate insurance, low income, presence of comorbidities, tumor histology, high grade or stage, and R2 resection (for all, P < .05). Increasing hospital surgical volume predicted decreasing risk of death across all survival times. However, analysis by hospital type demonstrated that compared with academic centers, the risk of death at community centers increased significantly as surgical volume increased (hazard ratio, 1.26; 95% CI, 1.03 to 1.53). CONCLUSION Hospital factors affect mortality after surgical treatment of RPS. Specifically, hospital type alters the surgical volume-outcome relationship for RPS mortality such that community centers perform worse with increasing volumes. Recommendations that higher surgical volume improves outcomes cannot be applied universally and must be re-examined in other complex surgical cancers.
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Affiliation(s)
- Anthony M Villano
- MedStar-Georgetown Surgical Outcomes Research Center, Washington, DC.,Department of Surgery, MedStar-Georgetown University Hospital, Washington, DC
| | - Alexander Zeymo
- MedStar-Georgetown Surgical Outcomes Research Center, Washington, DC.,MedStar Health Research Institute, Hyattsville, MD
| | - Kitty S Chan
- MedStar-Georgetown Surgical Outcomes Research Center, Washington, DC.,MedStar Health Research Institute, Hyattsville, MD.,Department of Biostatistics, Bioinformatics and Biomathematics, Georgetown University, Washington, DC
| | - Keith R Unger
- Department of Radiation Oncology, MedStar-Georgetown University Hospital, Washington, DC
| | - Nawar Shara
- MedStar Health Research Institute, Hyattsville, MD.,Georgetown-Howard Universities Center for Clinical and Translational Science, Washington, DC
| | - Waddah B Al-Refaie
- MedStar-Georgetown Surgical Outcomes Research Center, Washington, DC.,Department of Surgery, MedStar-Georgetown University Hospital, Washington, DC.,MedStar Health Research Institute, Hyattsville, MD
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Interdisciplinary approach allows minimally invasive, nerve-sparing removal of retroperitoneal peripheral nerve sheath tumors. Langenbecks Arch Surg 2020; 405:199-205. [PMID: 31925504 PMCID: PMC7239799 DOI: 10.1007/s00423-019-01851-5] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2019] [Accepted: 12/22/2019] [Indexed: 12/21/2022]
Abstract
Purpose En bloc resection of retroperitoneal peripheral nerve sheath tumors (PNST) is advocated by a variety of surgical disciplines. Yet, microsurgical, nerve-sparing tumor resection might be better suited to improve symptoms and maintain neurological function, especially in cases where patients present with preoperative neurological deficits. However, neurosurgeons, versed in nerve-sparing techniques to remove PNST, are generally unfamiliar with the visceral approaches to retroperitoneal PNST. Methods We retrospectively evaluate a series of 16 patients suffering from retroperitoneal PNST. Patients were treated by a unique interdisciplinary approach, combining the visceral surgeon’s skills to navigate the complex anatomy of the retroperitoneal space and the neurosurgeon’s familiarity with microsurgical, nerve-sparing tumor removal. Specifically, we assess whether our interdisciplinary approach is suited to improve preoperative symptoms and maintain neurological function and study whether oncological outcome, surgical morbidity, and operative times are comparable to those reported for “classical” retroperitoneal PNST resection. In addition, we study two cases of suspected PNST that were diagnosed as malignant peripheral nerve sheath tumors (MPNST) after surgery. Results Total macroscopic tumor resection was achieved in 14/16 PNST patients. Mean intraoperative blood loss was 680.6 ml (95% CI, 194.3–1167.0 ml) and mean operative time was 162.5 min (95% CI, 121.6–203.4 min). We did not record any major postoperative surgical or neurological complications. A total of 8/11 patients with preoperative pain symptoms reported long-lasting improvement of their symptoms. In terms of oncological outcome, all patients that had been subjected to total tumor removal and for whom follow-up was available, were tumor-free after a mean follow-up of 761.9 days (95% CI, 97.6–1426.0 days). One of the two MPNST patients, who presented with tumor progress 15 months after initial surgery, was subjected to radical re-resection. Conclusions Interdisciplinary, nerve-sparing removal of retroperitoneal PNST is well suited to improve preoperative symptoms and maintain neurological function, while achieving an oncological outcome and a surgical morbidity similar to previously reported results for radical retroperitoneal PNST resection. Radical re-resection was feasible in a patient with post hoc MPNST diagnosis.
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Hale R, Sandakly S, Shipley J, Walters Z. Epigenetic Targets in Synovial Sarcoma: A Mini-Review. Front Oncol 2019; 9:1078. [PMID: 31681608 PMCID: PMC6813544 DOI: 10.3389/fonc.2019.01078] [Citation(s) in RCA: 29] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2019] [Accepted: 09/30/2019] [Indexed: 01/25/2023] Open
Abstract
Synovial Sarcomas (SS) are a type of Soft Tissue Sarcoma (STS) and represent 8-10% of all STS cases. Although SS can arise at any age, it typically affects younger individuals aged 15-35 and is therefore part of both pediatric and adult clinical practices. SS occurs primarily in the limbs, often near joints, but can present anywhere. It is characterized by the recurrent pathognomonic chromosomal translocation t(X;18)(p11.2;q11.2) that most frequently fuses SSX1 or SSX2 genes with SS18. This leads to the expression of the SS18-SSX fusion protein, which causes disturbances in several interacting multiprotein complexes such as the SWItch/Sucrose Non-Fermentable (SWI/SNF) complex, also known as the BAF complex and the Polycomb Repressive Complex 1 and 2 (PRC1 and PRC2). Furthermore, this promotes widespread epigenetic rewiring, leading to aberrant gene expression that drives the pathogenesis of SS. Good prognoses are characterized predominantly by small tumor size and young patient age. Whereas, high tumor grade and an increased genomic complexity of the tumor constitute poor prognostic factors. The current therapeutic strategy relies on chemotherapy and radiotherapy, the latter of which can lead to chronic side effects for pediatric patients. We will focus on the known roles of SWI/SNF, PRC1, and PRC2 as the main effectors of the SS18-SSX-mediated genome modifications and we present existing biological rationale for potential therapeutic targets and treatment strategies.
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Affiliation(s)
- Ryland Hale
- Translational Epigenomics Team, Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
| | - Sami Sandakly
- Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom
| | - Janet Shipley
- Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom
| | - Zoë Walters
- Translational Epigenomics Team, Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
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Witt RG, Baldini EH, Raut CP. Screening populations at high risk for soft tissue sarcoma and surveillance following soft tissue sarcoma resection. J Surg Oncol 2019; 120:882-890. [PMID: 31432526 DOI: 10.1002/jso.25676] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/21/2019] [Accepted: 08/07/2019] [Indexed: 11/12/2022]
Abstract
Soft tissue sarcomas (STS) are a rare and diverse group of tumors that affect both adult and pediatric populations. This review discusses current screening recommendations for populations at increased risk for STS, including those with genetic predispositions. We also review surveillance guidelines for those at risk for recurrence following curative-intent surgery.
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Affiliation(s)
- Russell G Witt
- Department of Surgery, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts
| | - Elizabeth H Baldini
- Department of Radiation Oncology, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts
| | - Chandrajit P Raut
- Department of Surgery, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts
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Baldi GG, Orbach D, Bertulli R, Magni C, Sironi G, Casanova M, Ferrari A. Standard treatment and emerging drugs for managing synovial sarcoma: adult's and pediatric oncologist perspective. Expert Opin Emerg Drugs 2019; 24:43-53. [PMID: 30841761 DOI: 10.1080/14728214.2019.1591367] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
INTRODUCTION in this review we discuss the standard of care for both pediatric and adult synovial sarcoma (SS), the prognostic differences between them, and the treatments available for localized and advanced diseases. We also overview the biology and the recent drugs under consideration in clinical trials on SS. Areas covered: we focus on new targeted therapies being investigated for advanced SS, especially anti-angiogenic drugs, and immunotherapy. We review all the published data and ongoing trials dedicated to SS or to soft tissue sarcoma in general, paying particular attention to the results obtained in SS patients. Expert opinion: we expect new treatment strategies to become available for SS in the near future. The ongoing and published trials on targeted therapies and immunotherapy mainly concern adult patients, but the somatic biology of pediatric SS has some similarities as in adult disease. A stronger cooperation between adult and pediatric oncologists in recent years has led to a more shared effort to find new treatment strategies for advanced SS patients, regardless of their age.
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Affiliation(s)
- Giacomo G Baldi
- a "Sandro Pitigliani" Medical Oncology Department , Hospital of Prato , Prato , Italy
| | - Daniel Orbach
- b SIREDO Oncology Center , PSL University, Institut Curie , Paris , France
| | - Rossella Bertulli
- c Medical Oncology Unit 2, Medical Oncology Department , Fondazione IRCCS Istituto Nazionale dei Tumori , Milan , Italy
| | - Chiara Magni
- d Pediatric Oncology Unit , Fondazione IRCCS Istituto Nazionale dei Tumori , Milan , Italy
| | - Giovanna Sironi
- d Pediatric Oncology Unit , Fondazione IRCCS Istituto Nazionale dei Tumori , Milan , Italy
| | - Michela Casanova
- d Pediatric Oncology Unit , Fondazione IRCCS Istituto Nazionale dei Tumori , Milan , Italy
| | - Andrea Ferrari
- d Pediatric Oncology Unit , Fondazione IRCCS Istituto Nazionale dei Tumori , Milan , Italy
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Sari SY, Yazici G, Gultekin M, Hurmuz P, Gurkaynak M, Ozyigit G. Sarcoma. Radiat Oncol 2019. [DOI: 10.1007/978-3-319-97145-2_8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
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Traub F, Griffin AM, Wunder JS, Ferguson PC. Influence of unplanned excisions on the outcomes of patients with stage III extremity soft-tissue sarcoma. Cancer 2018; 124:3868-3875. [DOI: 10.1002/cncr.31648] [Citation(s) in RCA: 43] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2018] [Revised: 05/07/2018] [Accepted: 05/24/2018] [Indexed: 12/24/2022]
Affiliation(s)
- Frank Traub
- University of Toronto Musculoskeletal Oncology Unit; Mount Sinai Hospital; Toronto Ontario Canada
- Department of Orthopaedic Surgery, Faculty of Medicine; Eberhard Karls University of Tübingen; Tübingen Germany
| | - Anthony M. Griffin
- University of Toronto Musculoskeletal Oncology Unit; Mount Sinai Hospital; Toronto Ontario Canada
| | - Jay S. Wunder
- University of Toronto Musculoskeletal Oncology Unit; Mount Sinai Hospital; Toronto Ontario Canada
- Division of Orthopaedic Surgery, Department of Surgery; University of Toronto; Toronto Ontario Canada
- Sarcoma Site Group, Princess Margaret Cancer Center; Toronto Ontario Canada
| | - Peter C. Ferguson
- University of Toronto Musculoskeletal Oncology Unit; Mount Sinai Hospital; Toronto Ontario Canada
- Division of Orthopaedic Surgery, Department of Surgery; University of Toronto; Toronto Ontario Canada
- Sarcoma Site Group, Princess Margaret Cancer Center; Toronto Ontario Canada
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Pennati A, Riggio E, Marano G, Biganzoli E. Autologous fat grafting after sarcoma surgery: Evaluation of oncological safety. J Plast Reconstr Aesthet Surg 2018; 71:1723-1729. [PMID: 30224167 DOI: 10.1016/j.bjps.2018.07.028] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2018] [Accepted: 07/27/2018] [Indexed: 12/25/2022]
Abstract
BACKGROUND The regenerative effectiveness of lipoaspirate procedures relies on the presence of mesenchymal stem cells, but the stromal microenvironment and hormonal secretions of the adipose tissue may be involved in cancer growth. Only few oncological outcome studies of fat grafting at the surgical site of malignant neoplasms of mesenchymal origin are available; none of these studies examined a series of sarcoma cases. OBJECTIVES We analyzed outcome in terms of local or distant spread and overall survival to investigate the oncological safety of fat grafting in patients with sarcoma. PATIENTS AND METHODS Sixty consecutive patients who had undergone 143 fat grafting procedures after surgical resection of bone and soft tissue sarcomas of the head, trunk, and limbs with clear resection margins were enrolled from 2004 to 2015 in our tertiary care center. A multidisciplinary sarcoma team administered adjuvant therapies. Patients were recurrence free at fat grafting. RESULTS The overall median follow-up was 7.5 years. At follow-up after fat grafting (2.4 years), one patient had distant metastasis and two had local relapse. Kaplan-Meier analysis showed disease-free survival rate of 95.4% (CI: 89.1-100.0) at 24 months. The risk of local recurrence (LR) within 24 months was 4.6% (CI: 0.0-20.9). The probability of not having LR after fat grafting was ≥ 89.1%. CONCLUSION We found no evidence of an increased cancer risk after fat grafting procedures in patients with sarcoma, but a stimulatory role of fat cannot be excluded for bone sarcomas based on the cases reported here, and further studies are therefore needed.
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Affiliation(s)
- Angela Pennati
- Unit of Plastic and Reconstructive Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milano, Italy
| | - Egidio Riggio
- Unit of Plastic and Reconstructive Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milano, Italy.
| | - Giuseppe Marano
- Laboratory of Medical Statistics, Biometry and Bioinformatics 'G. A. Maccacaro', Department of Clinical Science and Community Health, University of Milan, Campus Cascina Rosa, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Vanzetti 5, 20133 Milano, Italy
| | - Elia Biganzoli
- Laboratory of Medical Statistics, Biometry and Bioinformatics 'G. A. Maccacaro', Department of Clinical Science and Community Health, University of Milan, Campus Cascina Rosa, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Vanzetti 5, 20133 Milano, Italy; Unit of Medical Statistics, Biometry and Bioinformatics, Campus Cascina Rosa, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Vanzetti 5, 20133 Milano, Italy
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Fiore M, Ford S, Callegaro D, Sangalli C, Colombo C, Radaelli S, Frezza AM, Renne SL, Casali PG, Gronchi A. Adequate Local Control in High-Risk Soft Tissue Sarcoma of the Extremity Treated with Surgery Alone at a Reference Centre: Should Radiotherapy Still be a Standard? Ann Surg Oncol 2018; 25:1536-1543. [PMID: 29470819 DOI: 10.1245/s10434-018-6393-x] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2017] [Indexed: 09/06/2023]
Abstract
BACKGROUND Established practice for the management of soft tissue sarcoma (STS) of the extremity and trunk wall combines perioperative radiotherapy (RT) with limb-preserving surgery. OBJECTIVE The aim of this study was to explore whether high-quality surgery at high-volume centers may offer equivalent local control in selected cases, when RT needs to be avoided. METHODS All consecutive adult cases of primary, high-risk STSs treated in a high-volume reference center over a 12-year timeframe were included, and, on retrospective analysis, were divided into two groups. Group A received RT with surgery, and Group B received surgery alone. The primary endpoint was local recurrence-free survival (LRFS). RESULTS Overall, 390 patients were included (318 in Group A and 72 in Group B), with a median follow-up of 53 months. The main reasons for avoiding RT were patient choice and technical considerations (vascular bypass or flap reconstruction). No difference in R0 resection was seen between the groups (79% vs. 70%; p = 0.18), but Group A had more G3 tumors (80.5% vs. 68%; p = 0.021). No difference in 5-year LRFS was evident (84% vs. 81%; p = 0.16). CONCLUSIONS LRFS did not differ between patients with high-risk STSs receiving perioperative RT and those treated with surgery alone. The study was retrospective and omission of RT was largely uncontrolled with inherent bias. Nonetheless, data suggest that in experienced centers, the omission of RT did not diminish local disease outcome. Future studies on a selective approach to RT administration are awaited.
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Affiliation(s)
- Marco Fiore
- Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
| | - Samuel Ford
- Department of Surgery, Queen Elizabeth Hospital, Birmingham, UK
| | - Dario Callegaro
- Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Claudia Sangalli
- Department of Radiation Therapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Chiara Colombo
- Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Stefano Radaelli
- Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Anna Maria Frezza
- Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Salvatore L Renne
- Department of Pathology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Paolo G Casali
- Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Alessandro Gronchi
- Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
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Surgery alone is sufficient therapy for children and adolescents with low-risk synovial sarcoma: A joint analysis from the European paediatric soft tissue sarcoma Study Group and the Children's Oncology Group. Eur J Cancer 2017; 78:1-6. [PMID: 28391003 DOI: 10.1016/j.ejca.2017.03.003] [Citation(s) in RCA: 49] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2016] [Revised: 01/07/2017] [Accepted: 03/09/2017] [Indexed: 12/31/2022]
Abstract
BACKGROUND Multimodal risk-adapted treatment is used in paediatric protocols for synovial sarcoma (SS). Retrospective analyses suggest that low-risk SS patients can be safely treated with surgery alone, but no prospective studies have confirmed the safety of this approach. This analysis pooled data from the two prospective clinical trials to assess outcomes in SS patients treated with a surgery-only approach and to identify predictors of treatment failure. METHODS Patients with localised SS enrolled on the European paediatric Soft tissue sarcoma Study Group (EpSSG) NRSTS2005 and on the Children Oncology Group (COG) ARST0332 trials, treated with surgery alone were eligible for this analysis. Patients must have undergone initial complete resection with histologically free margins, with a grade 2 tumour of any size or a grade 3 tumour ≤5 cm. RESULTS Sixty patients under 21 years of age were eligible for the analysis; 36 enrolled in the COG (from 2007 to 2012) and 24 in the EpSSG study (from 2005 to 2012). The 3-year event-free survival was 90% (median follow-up 5.2 years, range 1.9-9.1). All eight events were local tumour recurrence, whereas no metastatic recurrences were seen. All patients with recurrence were effectively salvaged, resulting in 100% overall survival. CONCLUSION This joint prospective analysis showed that patients with adequately resected ≤5 cm SS, regardless of grade, can be safely treated with a surgery-only approach. Avoiding the use of adjuvant chemotherapy and radiotherapy in this low-risk patient population may decrease both short- and long-term morbidity and mortality.
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López-Gómez J, Flores-Vázquez ER, Salazar-Álvarez MA, Adame RY, Garcia-Ortega DY, Cuellar-Hübbe M. Treatment of intimal sarcoma of peripheral veins. Int J Surg Case Rep 2017; 31:93-98. [PMID: 28129609 PMCID: PMC5266490 DOI: 10.1016/j.ijscr.2017.01.018] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/14/2016] [Revised: 01/09/2017] [Accepted: 01/09/2017] [Indexed: 11/30/2022] Open
Abstract
Intimal sarcoma is an extremely rare group. Intimal sarcoma of large veins even rarer information and case reports are limited. Unique case in the medical literature and the best current evidence in the biological behavior and the response of the multiple options of treatment in this type of tumors. Introduction Intimal sarcoma is an extremely rare group of undifferentiated pleomorphic sarcoma arising from the intimal layer of vessels accounting for only 1% of all sarcomas, intimal sarcoma of large veins are even less common. Cases presentation We present two cases of intima sarcoma, one originated form the basilar vein and the other from the cephalic vein, the first one was treated with surgery and postoperative chemotherapy followed by Radiotherapy (RT), the second case was treated with isolated limb perfusion followed by marginal resection and RT. Both patients progressed to the lungs in a short time, the first case was treated with metastasectomy of the lung and is without evidence of disease 7 months after surgery; the second case treated with isolated limb perfusion has stable disease. Discussion Intimal sarcoma are very aggressive tumors, with a high metastatic potential, the two patients progressed to lung in a short time (2 months) after local treatment. Both cases exhibit good response to chemotherapy and metastasectomy with a disease – free period of 7 months. Conclusion We propose that given the aggressive behavior of these tumors, they should be treated with chemoradiotherapy postoperative, either by systemic chemotherapy or isolated limb perfusion for the limp sparing surgery in this histology.
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Affiliation(s)
- Javier López-Gómez
- Department of Oncologic Surgery, National Cancer Institute, Mexico City, Av. San Fernando No. 22, Col. Sección XVI, C.P. 14080, Mexico.
| | - Erwin R Flores-Vázquez
- Department of Oncologic Surgery, National Cancer Institute, Mexico City, Av. San Fernando No. 22, Col. Sección XVI, C.P. 14080, Mexico
| | - Ma Alejandra Salazar-Álvarez
- Department of Oncologic Surgery, National Cancer Institute, Mexico City, Av. San Fernando No. 22, Col. Sección XVI, C.P. 14080, Mexico
| | - Rodrigo Y Adame
- Department of Oncologic Surgery, National Cancer Institute, Mexico City, Av. San Fernando No. 22, Col. Sección XVI, C.P. 14080, Mexico
| | - Dorian Y Garcia-Ortega
- Department of Skin and Soft Tissue Tumors, National Cancer Institute, Mexico City, Av. San Fernando No. 22, Col. Sección XVI, C.P. 14080, Mexico
| | - Mario Cuellar-Hübbe
- Department of Skin and Soft Tissue Tumors, National Cancer Institute, Mexico City, Av. San Fernando No. 22, Col. Sección XVI, C.P. 14080, Mexico
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Abstract
External beam radiation therapy is essential in the management of a wide spectrum of musculoskeletal conditions, both benign and malignant, including bony and soft-tissue sarcomas, metastatic tumors, pigmented villonodular synovitis, and heterotopic ossification. Radiation therapy, in combination with surgery, helps reduce the functional loss from cancer resections. Although the field of radiation therapy is firmly rooted in physics and radiation biology, its indications and delivery methods are rapidly evolving. External beam radiation therapy mainly comes in the form of four sources of radiotherapy: protons, photons, electrons, and neutrons. Each type of energy has a unique role in treating various pathologies; however, these energy types also have their own distinctive limitations and morbidities.
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17
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The role of 18F-FDG PET/CT in diagnosis and staging of musculoskeletal soft tissue sarcomas. Clin Transl Imaging 2015. [DOI: 10.1007/s40336-015-0108-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023]
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18
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Pan E, Goldberg SI, Chen YL, Giraud C, Hornick JL, Nielsen GP, Hornicek FJ, Raut CP, DeLaney TF, Baldini EH. Role of post-operative radiation boost for soft tissue sarcomas with positive margins following pre-operative radiation and surgery. J Surg Oncol 2014; 110:817-22. [DOI: 10.1002/jso.23741] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2014] [Accepted: 07/10/2014] [Indexed: 11/08/2022]
Affiliation(s)
- Elizabeth Pan
- Department of Radiation Oncology; Boston Massachusetts
- Massachusetts General Hospital; Boston Massachusetts
- University of Southern California Keck School of Medicine; Los Angeles California
| | - Saveli I. Goldberg
- Massachusetts General Hospital; Boston Massachusetts
- Department of Biostatistics and Biomathematics; Boston Massachusetts
| | - Yen-Lin Chen
- Department of Radiation Oncology; Boston Massachusetts
- Massachusetts General Hospital; Boston Massachusetts
| | - Christine Giraud
- Department of Radiation Oncology; Boston Massachusetts
- Massachusetts General Hospital; Boston Massachusetts
| | - Jason L. Hornick
- Department of Pathology; Boston Massachusetts
- Brigham and Women's Hospital and Dana-Farber Cancer Institute; Boston Massachusetts
| | - Gunnlaugur P. Nielsen
- Massachusetts General Hospital; Boston Massachusetts
- Department of Pathology; Boston Massachusetts
| | - Francis J. Hornicek
- Massachusetts General Hospital; Boston Massachusetts
- Department of Surgery; Boston Massachusetts
| | - Chandrajit P. Raut
- Brigham and Women's Hospital and Dana-Farber Cancer Institute; Boston Massachusetts
- Department of Surgery; Boston Massachusetts
| | - Thomas F. DeLaney
- Department of Radiation Oncology; Boston Massachusetts
- Massachusetts General Hospital; Boston Massachusetts
| | - Elizabeth H. Baldini
- Department of Radiation Oncology; Boston Massachusetts
- Brigham and Women's Hospital and Dana-Farber Cancer Institute; Boston Massachusetts
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19
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Kneisl JS, Coleman MM, Raut CP. Outcomes in the management of adult soft tissue sarcomas. J Surg Oncol 2014; 110:527-38. [PMID: 24965077 DOI: 10.1002/jso.23685] [Citation(s) in RCA: 37] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2014] [Accepted: 05/02/2014] [Indexed: 12/14/2022]
Abstract
Adult soft tissue sarcomas (STSs) are heterogeneous neoplasms that account for 11,410 new diagnoses and 4,390 deaths per year. This article summarizes recent NCCN guidelines for diagnosis and management of STSs of the extremities and retroperitoneum, as well as gastrointestinal stromal tumors (GIST). AJCC staging and recently reported NCDB data regarding outcomes are reviewed. Currently accepted STS prognostic variables are presented, as are future directions regarding the utility of molecular prognosticators and nomograms.
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Affiliation(s)
- Jeffrey S Kneisl
- Department of Orthopaedic Surgery, Levine Cancer Institute, Carolinas Healthcare System, Charlotte, North Carolina
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20
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Baldini EH, Raut C. Radiation Therapy for Extremity Soft Tissue Sarcoma: In the Absence of a Clear Survival Benefit, Why Do We Give It? Ann Surg Oncol 2014; 21:2463-5. [DOI: 10.1245/s10434-014-3735-1] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/05/2014] [Indexed: 12/28/2022]
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21
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Soyfer V, Corn BW, Kollender Y, Issakov J, Dadia S, Flusser G, Bickels J, Meller I, Merimsky O. Hypofractionated adjuvant radiation therapy of soft-tissue sarcoma achieves excellent results in elderly patients. Br J Radiol 2013; 86:20130258. [PMID: 23709514 DOI: 10.1259/bjr.20130258] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
OBJECTIVE Adjuvant radiation therapy (RT) is an essential part of combined limb-sparing treatment of soft-tissue sarcoma (STS). Elderly or medically unfit patients often have difficulty in completing 6-7 weeks of standard fractionated daily treatment. Our aim was to evaluate the efficacy of a hypofractionated adjuvant approach with RT for STS in elderly and debilitated patients. METHODS 21 elderly patients were treated with a short course of adjuvant RT (39-48 Gy, 3 Gy per fraction) for STS. The medical records of the patients were retrospectively reviewed for local or distant recurrence and side effects of RT. RESULTS At a mean 26 months of follow-up, three local recurrences (14%) were detected. Eight patients (38%) had lung metastases during the observed period. Three of them died from metastatic disease. The hypofractionated radiation was well tolerated with minimum long-term side effects. CONCLUSION Hypofractionated adjuvant radiation appears to be an effective treatment in terms of local control in elderly and debilitated patients. ADVANCES IN KNOWLEDGE The results of this study might provide an alternative to commonly used standard fractionation of radiotherapy in sarcoma patients.
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Affiliation(s)
- V Soyfer
- Department of Oncology, Tel Aviv Sourasky Medical Center, Ashdod, Israel
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22
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Fedors NH, Demos TC, Lomasney LM, Mehta V, Horvath LE. Radiologic case study: your diagnosis? Synovial sarcoma. Orthopedics 2010; 33:861. [PMID: 21117558 DOI: 10.3928/01477447-20101021-35] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Affiliation(s)
- Nathan H Fedors
- Department of Radiology, Loyola University Medical Center, Maywood, Illinois, USA
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23
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Wang L, Tan MH. The Natural History and Prognosticative Factors of Adult Extremity Soft Tissue Sarcomas: An Asian Perspective. ANNALS OF THE ACADEMY OF MEDICINE, SINGAPORE 2010. [DOI: 10.47102/annals-acadmedsg.v39n10p771] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/11/2023]
Abstract
Introduction: We describe the natural history of Asian adult soft tissue sarcomas (STSs) in the extremities and predict prognosticative factors for local recurrence, metastasis and tumour-related death. Materials and Methods: Between January 1999 and May 2009, 67 adult patients with fi rst presentation STSs of extremity sites underwent surgical treatment at a single institution. The associations between patient demographics and pathological features with local recurrence, metastasis and mortality were studied using univariate and multivariate analysis. Results: The mean age of our patients was 52.4 years with most presentations occurring in the thigh. Majority of Asian STSs were high grade (61.3%) and large tumours with 81.0% being >5 cm. Stages Ia, Ib, IIa, IIb, IIc, III and IV accounted for 6.6%, 6.6%, 26.2%, 11.5%, 3.3%, 42.6% and 3.3% of presentations, respectively. Patients were followed-up for a mean period of 45.9 months. On univariate analysis, high tumour grade and advanced stage (IIc to IV) were predictive of local recurrence and metastasis. Deep lesions were more likely to recur but not metastasise or cause death. Age, sex, size, and margin positivity were not predictive for all end-points. On multivariate testing, only pathological high grade was associated adversely with local recurrence [odds ratio (OR) = 10.0, 95% CI, 1.2 to 84.9, P = 0.035], metastasis (OR = 12.7, 95% CI, 2.46 to 65.2, P = 0.002) and mortality (OR = 16.2, 95% CI, 1.95 to 135.0, P = 0.010). Conclusions: Asian adult extremity soft tissue sarcomas present late and are most commonly found in the thigh. High pathological grade is a consistent independent predictor for local failure, distant spread and tumour-related death. Our results reaffirm the current thinking that tumour biology is of primary importance in determining patient outcomes.
Key words: Extremities, Prognostic factors, Natural history, Outcomes
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24
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Krasin MJ, Davidoff AM, Xiong X, Wu S, Hua CH, Navid F, Rodriguez-Galindo C, Rao BN, Hoth KA, Neel MD, Merchant TE, Kun LE, Spunt SL. Preliminary results from a prospective study using limited margin radiotherapy in pediatric and young adult patients with high-grade nonrhabdomyosarcoma soft-tissue sarcoma. Int J Radiat Oncol Biol Phys 2010; 76:874-8. [PMID: 19625137 PMCID: PMC2823850 DOI: 10.1016/j.ijrobp.2009.02.074] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2008] [Revised: 02/13/2009] [Accepted: 02/27/2009] [Indexed: 11/19/2022]
Abstract
PURPOSE To demonstrate the safety and efficacy of limited margin radiotherapy in the local control of pediatric and young adult patients with high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). METHODS AND MATERIALS Pediatric patients with high-grade NRSTS requiring radiation were treated on an institutional review board approved prospective institutional study of conformal/intensity-modulated/interstitial brachytherapy using a 2-cm anatomically constrained margin. RESULTS A total of 32 patients (median age, 15.3 years; range, 2-22 years) received adjuvant (27 patients) or definitive (5 patients) irradiation. With a median follow-up of 32 months, the 3-year cumulative incidence of local failure was 3.7% for patients undergoing irradiation after surgical resection. Four patients experienced local failure; the mean dose to the volume of recurrence was >or=97% of the prescribed dose. CONCLUSIONS Delivery of limited margin radiotherapy using external beam or brachytherapy provides a high rate of local tumor control without marginal failure. Further follow-up is required to determine whether normal tissue effects are minimized using this approach.
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Affiliation(s)
- Matthew J Krasin
- Department of Radiological Sciences, St Jude Children's Research Hospital, Memphis, TN 38105, USA.
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25
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Lachenmayer A, Yang Q, Eisenberger CF, Boelke E, Poremba C, Heinecke A, Ohmann C, Knoefel WT, Peiper M. Superficial soft tissue sarcomas of the extremities and trunk. World J Surg 2009; 33:1641-9. [PMID: 19430830 DOI: 10.1007/s00268-009-0051-1] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
BACKGROUND Superficial soft tissue sarcomas (sSTS) are an important and frequent subtype of soft tissue sarcoma (STS). A wider knowledge of this tumor type may lead to better strategies in tumor therapy. METHODS An institutional review was performed on all patients with primary sSTS of the extremities and trunk operated on between 1990 and 2003. RESULTS The medical records of 108 patients with sSTS were analyzed. The local recurrence rate was 11% after a median of 25 (mean 42) months. Metastases occurred in 21 patients (19%), and 79 patients lived without evidence of disease after a mean follow-up of 112 +/- 42 months. Mean survival time was 89 months at a cumulative 5-year survival rate of 85%. R0 resection significantly enhanced cumulative survival (p = .001), as did patient age < 60 years (p = .002), tumor grading G1 and G2 compared to G3 (p = .004), absence of positive lymph nodes (p = .018), and no occurrence of metastases (p = .001). Tumor size < or = 5 cm reduced the local recurrence rate significantly (p = .044). Significant multivariate risk factors for metastases were age > or = 60 years (p = .016) and tumor grade G3 (p = .021). CONCLUSIONS Patients with sSTS who are > or = 60 years of age or who have G3 tumors have a high risk of distant metastases. Patients with T2 tumors have an elevated risk for local recurrence. Certainly all patients with sSTS should be in a tight after-care program to allow early diagnosis of local recurrence or distant metastases. Age < 60 years, tumor grade G1/2, no positive regional lymph nodes (N0), and a R0 resection are significant prognostic factors for survival.
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Affiliation(s)
- Anja Lachenmayer
- Department of General, Visceral, and Pediatric Surgery, University Hospital Duesseldorf, Moorenstrasse 5, 40225, Duesseldorf, Germany.
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