Multimodality Management of Sarcomas

Dear Colleagues,

Due to their rarity and heterogeneity, with a wide variety of histological subtypes, biological behaviors and tumor localization, the optimal management of sarcomas remains challenging. The treatment of sarcomas has to be individualized and consists, usually, of a combination of modalities. Currently, sarcomas are managed in a multimodality manner.

Initial appropriate imaging is of outmost importance to demonstrate the exact localization and extension of the tumor and its relation to adjacent structures, in order to facilitate the performance of a diagnostic core needle biopsy and the planning of a surgical procedure when systemic disease is excluded. A preoperative biopsy is of outmost importance to differentiate between benign and malignant tumors and to define the sarcoma subtype. The pathological examination of those rare and heterogenous sarcoma tumors is very demanding and expertise is required. The treatment plan of each sarcoma is personalized according to its imaging and pathology.

Until recently, most extremity soft tissue sarcomas were locally treated with amputation; nowadays, the majority of patients receive a limb-salvage treatment with a wide excision of the tumor and radiotherapy. Current evidence shows that preoperative radiotherapy may be preferred over the typical postoperative radiotherapy, whereas in low-risk patients radiotherapy may even be omitted. In selected cases, preoperative systemic chemotherapy or isolated limb perfusion may be indicated in an attempt to preserve the extremity. For retroperitoneal sarcomas, recently, a more aggressive surgical approach has been advocated for, whereas the exact role of preoperative radiotherapy or chemotherapy to facilitate resection and to reduce the high risk of local recurrence has still to be defined. The systemic therapy of soft tissue sarcomas, either as an adjuvant treatment or for metastatic disease, has evolved from the classical anthracycline-based regime, to a more individualized histology-driven chemotherapy, whereas novel drugs have been developed. Bone sarcomas are usually also managed in a multimodality manner, depending on the histological type. Although localized chondrosarcomas are initially treated by surgical excision and/or radiotherapy, Ewing sarcomas are traditionally treated with systemic chemotherapy, followed by a local treatment, including surgery and/or radiotherapy, and the continuation of systemic chemotherapy. Similarly, osteosarcomas are usually treated with a combination of chemotherapy and surgery, with or without radiotherapy and the continuation of chemotherapy.

As this illustrates, the management of sarcomas is challenging and multimodal, including radiology and pathology for initial diagnosis and, subsequently, surgery, and through the use of chemotherapy and/or radiotherapy for definite treatment in order to achieve optimal oncological and functional outcomes. The various aspects of this multimodality management and their current roles will be discussed in this Special Issue.

Prof. Dr. Jean-Yves Blay
Prof. Dr. Eelco de Bree
Prof. Dr. Frank Traub
Guest Editors

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• sarcomas
• multimodal
• pathology
• radiology
• surgery
• chemotherapy
• radiotherapy

Title: Neoadjuvant treatment of locally advanced soft tissue sarcoma of the extremities to avoid amputation. Which are valid treatment options?
Author: de Bree
Highlights: Locally advanced soft tissue sarcomas of the extremities may require amputative surgery. The aim of neoadjuvant treatment is to minimize the need for amputation or surgery that leads to major functional impairment, with acceptable toxicity and without having a negative impact on survival. Various neoadjuvant treatment strategies have been proposed to reduce the risk of amputation and limb dysfunction.