Managing sarcoma: where have we come from and where are we going?

Sarcomas are a heterogeneous group of neoplasms of mesenchymal origin. Approximately 80% arise from soft tissue and 20% originate from bone. To date more than 100 sarcoma subtypes have been identified and they vary in molecular characteristics, pathology, clinical presentation and response to treatm...

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Main Authors: Jenna S. Bleloch, Reyna D. Ballim, Serah Kimani, Jeannette Parkes, Eugenio Panieri, Tarryn Willmer, Sharon Prince
Format: Article
Language:English
Published: SAGE Publishing 2017-10-01
Series:Therapeutic Advances in Medical Oncology
Online Access:https://doi.org/10.1177/1758834017728927
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author Jenna S. Bleloch
Reyna D. Ballim
Serah Kimani
Jeannette Parkes
Eugenio Panieri
Tarryn Willmer
Sharon Prince
author_facet Jenna S. Bleloch
Reyna D. Ballim
Serah Kimani
Jeannette Parkes
Eugenio Panieri
Tarryn Willmer
Sharon Prince
author_sort Jenna S. Bleloch
collection DOAJ
description Sarcomas are a heterogeneous group of neoplasms of mesenchymal origin. Approximately 80% arise from soft tissue and 20% originate from bone. To date more than 100 sarcoma subtypes have been identified and they vary in molecular characteristics, pathology, clinical presentation and response to treatment. While sarcomas represent <1% of adult cancers, they account for approximately 21% of paediatric malignancies and thus pose some of the greatest risks of mortality and morbidity in children and young adults. Metastases occur in one-third of all patients and approximately 10–20% of sarcomas recur locally. Surgery in combination with preoperative and postoperative therapies is the primary treatment for localized sarcoma tumours and is the most promising curative possibility. Metastasized sarcomas, on the other hand, are treated primarily with single-agent or combination chemotherapy, but this rarely leads to a complete and robust response and often becomes a palliative form of treatment. The heterogeneity of sarcomas results in variable responses to current generalized treatment strategies. In light of this and the lack of curative strategies for metastatic and unresectable sarcomas, there is a need for novel subtype-specific treatment strategies. With the more recent understanding of the molecular mechanisms underlying the pathogenesis of some of these tumours, the treatment of sarcoma subtypes with targeted therapies is a rapidly evolving field. This review discusses the current management of sarcomas as well as promising new therapies that are currently underway in clinical trials.
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spelling doaj.art-f2c92b138bd84104919e684be05a27202022-12-21T23:11:08ZengSAGE PublishingTherapeutic Advances in Medical Oncology1758-83401758-83592017-10-01910.1177/1758834017728927Managing sarcoma: where have we come from and where are we going?Jenna S. BlelochReyna D. BallimSerah KimaniJeannette ParkesEugenio PanieriTarryn WillmerSharon PrinceSarcomas are a heterogeneous group of neoplasms of mesenchymal origin. Approximately 80% arise from soft tissue and 20% originate from bone. To date more than 100 sarcoma subtypes have been identified and they vary in molecular characteristics, pathology, clinical presentation and response to treatment. While sarcomas represent <1% of adult cancers, they account for approximately 21% of paediatric malignancies and thus pose some of the greatest risks of mortality and morbidity in children and young adults. Metastases occur in one-third of all patients and approximately 10–20% of sarcomas recur locally. Surgery in combination with preoperative and postoperative therapies is the primary treatment for localized sarcoma tumours and is the most promising curative possibility. Metastasized sarcomas, on the other hand, are treated primarily with single-agent or combination chemotherapy, but this rarely leads to a complete and robust response and often becomes a palliative form of treatment. The heterogeneity of sarcomas results in variable responses to current generalized treatment strategies. In light of this and the lack of curative strategies for metastatic and unresectable sarcomas, there is a need for novel subtype-specific treatment strategies. With the more recent understanding of the molecular mechanisms underlying the pathogenesis of some of these tumours, the treatment of sarcoma subtypes with targeted therapies is a rapidly evolving field. This review discusses the current management of sarcomas as well as promising new therapies that are currently underway in clinical trials.https://doi.org/10.1177/1758834017728927
spellingShingle Jenna S. Bleloch
Reyna D. Ballim
Serah Kimani
Jeannette Parkes
Eugenio Panieri
Tarryn Willmer
Sharon Prince
Managing sarcoma: where have we come from and where are we going?
Therapeutic Advances in Medical Oncology
title Managing sarcoma: where have we come from and where are we going?
title_full Managing sarcoma: where have we come from and where are we going?
title_fullStr Managing sarcoma: where have we come from and where are we going?
title_full_unstemmed Managing sarcoma: where have we come from and where are we going?
title_short Managing sarcoma: where have we come from and where are we going?
title_sort managing sarcoma where have we come from and where are we going
url https://doi.org/10.1177/1758834017728927
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