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Soft-tissue sarcoma in adults: An update on the current state of histiotype-specific management in an era of personalized medicine.

Literature Information

DOI10.3322/caac.21605
PMID32275330
JournalCA: a cancer journal for clinicians
Impact Factor232.4
JCR QuartileQ1
Publication Year2020
Times Cited270
Keywordsadvanced soft-tissue sarcoma, extremity sarcoma, retroperitoneal sarcoma, soft-tissue sarcoma
Literature TypeJournal Article, Research Support, Non-U.S. Gov't, Review
ISSN0007-9235
Pages200-229
Issue70(3)
AuthorsAdriana C Gamboa, Alessandro Gronchi, Kenneth Cardona

TL;DR

This study highlights the rarity and complexity of soft-tissue sarcomas (STS), which represent a small fraction of adult cancers and exhibit diverse behaviors and outcomes, underscoring the necessity for specialized, multidisciplinary care. It reviews recent advancements in histiotype-specific management strategies for extremity/truncal and retroperitoneal STS, focusing on tailored surgical, radiation, and chemotherapy approaches to improve patient outcomes.

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advanced soft-tissue sarcoma · extremity sarcoma · retroperitoneal sarcoma · soft-tissue sarcoma

Abstract

Soft-tissue sarcomas (STS) are rare tumors that account for 1% of all adult malignancies, with over 100 different histologic subtypes occurring predominately in the trunk, extremity, and retroperitoneum. This low incidence is further complicated by their variable presentation, behavior, and long-term outcomes, which emphasize the importance of centralized care in specialized centers with a multidisciplinary team approach. In the last decade, there has been an effort to improve the quality of care for patients with STS based on anatomic site and histology, and multiple ongoing clinical trials are focusing on tailoring therapy to histologic subtype. This report summarizes the latest evidence guiding the histiotype-specific management of extremity/truncal and retroperitoneal STS with regard to surgery, radiation, and chemotherapy.

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Primary Questions Addressed

  1. What are the specific challenges in managing different histologic subtypes of soft-tissue sarcomas in adults?
  2. How does the multidisciplinary team approach enhance treatment outcomes for patients with soft-tissue sarcomas?
  3. What recent advancements in personalized medicine have been made for the treatment of extremity and retroperitoneal soft-tissue sarcomas?
  4. How do clinical trials influence the development of histiotype-specific therapies for soft-tissue sarcomas?
  5. In what ways can centralized care in specialized centers improve the prognosis for adults diagnosed with soft-tissue sarcomas?

Key Findings

Key Insights

  1. Research Background and Purpose: Soft-tissue sarcomas (STS) represent a rare and diverse group of tumors, constituting approximately 1% of all adult cancers. They encompass over 100 distinct histologic subtypes, primarily localizing in the trunk, extremities, and retroperitoneum. The variability in their clinical presentation, behavior, and prognosis complicates treatment strategies. This highlights the necessity for specialized care within dedicated centers employing a multidisciplinary approach. The purpose of this research update is to provide a comprehensive overview of current histiotype-specific management strategies for adult STS, particularly focusing on advancements in personalized medicine over the past decade.

  2. Main Methods and Findings: The report synthesizes recent evidence concerning the management of STS, specifically for extremity/truncal and retroperitoneal subtypes. It discusses the roles of surgical intervention, radiation therapy, and chemotherapy within the context of histologic classification. Key findings indicate a growing emphasis on individualized treatment protocols based on the tumor's specific histologic characteristics and anatomical location. Ongoing clinical trials are instrumental in refining these strategies, aiming to optimize therapeutic outcomes through tailored approaches that consider the unique biology of each STS subtype.

  3. Core Conclusions: The study underscores the importance of histology in guiding treatment decisions for STS. There is a clear movement towards personalized medicine, wherein therapies are aligned with the histiotype of the sarcoma, enhancing the potential for improved patient outcomes. The findings advocate for centralized, multidisciplinary care to ensure that patients receive the most effective and appropriate interventions. Furthermore, the ongoing clinical trials and emerging evidence are crucial for evolving treatment paradigms and improving survival rates for STS patients.

  4. Research Significance and Impact: This research holds significant implications for the management of soft-tissue sarcomas, a field that has historically suffered from a lack of standardized treatment protocols due to the rarity and complexity of these tumors. By emphasizing histiotype-specific management and personalized treatment approaches, this work advances the understanding of STS and potentially transforms clinical practice. The integration of multidisciplinary teams and ongoing clinical trials is pivotal for developing innovative therapies, thereby improving both survival and quality of life for patients afflicted with these challenging malignancies. This update serves as a critical resource for clinicians, researchers, and policymakers in shaping future strategies in sarcoma care.

Literatures Citing This Work

  1. Value of peri-operative chemotherapy in patients with CINSARC high-risk localized grade 1 or 2 soft tissue sarcoma: study protocol of the target selection phase III CHIC-STS trial. - Thomas Filleron;Sophie Le Guellec;Christine Chevreau;Bastien Cabarrou;Tom Lesluyes;Sabrina Lodin;Angélique Massoubre;Muriel Mounier;Muriel Poublanc;Frédéric Chibon;Thibaud Valentin - BMC cancer (2020)
  2. Unraveling the Heterogeneity of Sarcoma Survivors' Health-Related Quality of Life Regarding Primary Sarcoma Location: Results from the SURVSARC Study. - Ilse van Eck;Dide den Hollander;Ingrid M E Desar;Vicky L M N Soomers;Michiel A J van de Sande;Jacco J de Haan;Cornelis Verhoef;Ingeborg J H Vriens;Johannes J Bonenkamp;Winette T A van der Graaf;Winan J van Houdt;Olga Husson - Cancers (2020)
  3. Incorporating the Patient Voice in Sarcoma Research: How Can We Assess Health-Related Quality of Life in This Heterogeneous Group of Patients? A Study Protocol. - Dide den Hollander;Marco Fiore;Javier Martin-Broto;Bernd Kasper;Antonio Casado Herraez;Dagmara Kulis;Ioanna Nixon;Samantha C Sodergren;Martin Eichler;Winan J van Houdt;Ingrid M E Desar;Isabelle Ray-Coquard;Claire Piccinin;Hanna Kosela-Paterczyk;Aisha Miah;Leopold Hentschel;Susanne Singer;Roger Wilson;Winette T A van der Graaf;Olga Husson - Cancers (2020)
  4. Recent advances in the understanding and management of liposarcoma. - Candace L Haddox;Richard F Riedel - Faculty reviews (2021)
  5. Clinical Characteristics and Prognostic Factors of Testicular Sarcoma: A Population-Based Study. - Xingyuan Wang;Zeyu Chen;Shi Qiu;Dehong Cao;Kun Jin;Jin Li;Bo Chen;Haoran Lei;Yin Huang;Yige Bao;Lu Yang;Liangren Liu;Qiang Wei - Frontiers in oncology (2021)
  6. Oncolytic virotherapy promotes radiosensitivity in soft tissue sarcoma by suppressing anti-apoptotic MCL1 expression. - Toshinori Omori;Hiroshi Tazawa;Yasuaki Yamakawa;Shuhei Osaki;Joe Hasei;Kazuhisa Sugiu;Tadashi Komatsubara;Tomohiro Fujiwara;Aki Yoshida;Toshiyuki Kunisada;Yasuo Urata;Shunsuke Kagawa;Toshifumi Ozaki;Toshiyoshi Fujiwara - PloS one (2021)
  7. Fourteen-Day Gemcitabine-Docetaxel Chemotherapy Is Effective and Safer Compared to 21-Day Regimen in Patients with Advanced Soft Tissue and Bone Sarcoma. - Minggui Pan;Maily K Trieu;Manpreet Sidhu;Jeanette Yu;Tiffany Seto;Kristen Ganjoo - Cancers (2021)
  8. A Nomogram Model to Predict Prognosis of Patients With Genitourinary Sarcoma. - Linde Li;Jiayu Liang;Turun Song;Saifu Yin;Jun Zeng;Qiang Zhong;Xiaobing Feng;Zihao Jia;Yu Fan;Xianding Wang;Tao Lin - Frontiers in oncology (2021)
  9. A study of three-dimensional reconstruction and printing models in two cases of soft tissue sarcoma of the thigh. - Linqian Li;Ke Zhang;Renfeng Wang;Yun Liu;Min Zhang;Wenshan Gao;Bao Ren;Xiaozhe Zhou;Shujie Cheng;Jinghua Li - International journal of computer assisted radiology and surgery (2021)
  10. Construction, validation and, visualization of a web-based nomogram for predicting the overall survival and cancer-specific survival of leiomyosarcoma patients with lung metastasis. - Zhehong Li;Junqiang Wei;Xintian Gan;Mingze Song;Yafang Zhang;Haiying Cao;Yu Jin;Jilong Yang - Journal of thoracic disease (2021)

... (260 more literatures)


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