Soft-tissue sarcoma
Prem Puri in Newborn Surgery, 2017
Soft-tissue sarcoma is a rare tumor in childhood and infancy, and its management is made complex by the lack of consistency between histology and clinical behavior. Management must be tailored to the individual patient and on the basis of consensus discussion at an MDT Tumour Board. It is important to differentiate rhabdomyosarcoma from congenital infantile fibrosarcoma. In general, the aim is curative excision, but where this is not possible or considered mutilating (functionally or anatomically), then neoadjuvant chemotherapy with vincristine and actinomycin D (avoiding cyclophosphamide) is offered. Residual tumor is excised, but complete clinical and radiological remission (CIFS) can be observed without surgery. Microscopic CIFS after surgery may be observed, but RMS must be treated further. Very young patients may also be closely observed, since as many as 35% of IFS may undergo spontaneous resolution. The rarity of this tumor and the complexity of management indicate the need for multicenter prospective database and collaboration.
Overview of Carbon-Ion Radiotherapy in Japan
Manjit Dosanjh, Jacques Bernier in Advances in Particle Therapy, 2018
Overall local control (LC) at 5 and 10 years was 82.4% and 80.0%, respectively, and overall survival (OS) at 5 and 10 years was 90.2% and 68.1%, respectively. The results at 10 years after CIRT were particularly superior to PBT or photon therapy. Sacral chordoma, which comprises about 50% of chordoma cases, is very difficult to control for a large tumour. PBT has been mostly performed following surgical resection in the United States, whereas CIRT has been used definitively as a sole treatment in Japan. Between 1996 and 2013, a total of 188 patients with unresectable sacral chordoma were treated at NIRS, delivering 67.2–73.6 Gray (RBE) in 16 fractions. The five-year LC, OS and disease-free survival rates were 77.2%, 81.1% and 50.3%, respectively, which is favourably compared with LC of 35%–50% after resection [12]. Osteosarcomas arising from the pelvis and vertebra are rare, representing 6.3% of all osteosarcoma patients. They are usually difficult to operate on and are resistant to X-rays. For this tumour, CIRT combined with chemotherapy offered superior results. The five-year LC and OS rates were 62% and 33%, respectively, which were better than the results after combined surgical resection and PBT [13]. Soft-tissue sarcomas develop from the soft tissues like fat, muscle, nerves, fibrous tissues, blood vessels, or deep skin tissues. They can be found in any part of the body, and most of them develop in the arms or legs. They also develop in the trunk, head and neck and retroperitoneum, which are the major candidates for CIRT. The five-year LC and five-year OS for unresectable retroperitoneal tumours (n = 24) was 69% and 50%, respectively [14]. Considering that most patients were not eligible for surgical resection and had high-grade sarcomas, these results are very promising.
Integrative hyperthermia treatments for different types of cancer
Clifford L. K. Pang, Kaiman Lee in Hyperthermia in Oncology, 2015
However, with the progress of chemotherapy in recent years, some researchers have begun applying resection of tumor segment or total femur resection, with artificial prosthetic for replacement. Limb salvage has become a major operation. Conduct preoperative standardized chemotherapy for 6–8 weeks, and then implement tumor resection. Resection margin is required to be radical or extensive. Artificial joint replacement is commonly used for bone defects. If limb salvage is not suitable or in the case that there is no condition for limb salvage, amputation should be decisively implemented, but postoperative chemotherapy has to be applied. Chondrosarcoma treatment focuses on surgery, and the efficacy depends on the breadth of tumor resection and malignancy of histological grade. Ewing’s sarcoma is very sensitive to radiation and chemotherapy. In current treatment protocols, the most advocated is neoadjuvant chemotherapy combined with extensive or radical resection of tumor. The therapeutic principle of treatment for bone malignant fibrous histiocytoma is similar to that of osteosarcoma. Surgical treatment is an important means for limited soft tissue sarcomas. For ideal local excision, tumors of all positions must be removed together with the surrounding normal tissue and we often have to give up some of the normal organizational structure to ensure complete resection. Surgical resection should include the site of biopsy and the nearby skin and partial muscles. For muscle tumor, the affected muscle should be excised completely. Only when lymph node involvement is shown clinically can lymph node dissection be carried out. For soft tissue tumors that cannot be completely excised, debulking surgery can be applied, and postoperative integrative treatments are combined to improve quality of life and prolong survival time. Amputation is applicable for advanced patients with a giant tumor accompanied by massive ulcer hemorrhage that cannot be stopped; patients accompanied by severe infections such as sepsis, tetanus, and so on that endanger their life; patients with a tumor that grows rapidly and causes severe pain and is difficult to control with medication; or patients who cannot be saved by using other methods due to pathological fractures, loss of activity, and other serious conditions. The most important treatment for malignant melanoma is surgical removal of skin lesions. For malignant melanoma in situ, the recommended resection margin from the lesion or biopsy scar is 0.5–1 cm. For lesions with a thickness less than 1 mm, a margin of 1 cm can also be accepted. For lesions with a thickness of 1 to 2 mm, a margin of 2 cm is strived for.
Olaratumab for the treatment of advanced soft tissue sarcoma
Published in Expert Review of Anticancer Therapy, 2017
Scott H. Okuno, Avudaiappan Maran, Steven I. Robinson
Introduction: Olaratumab, a human monoclonal antibody against platelet derived growth factor receptor alpha (PDGFR- α), is the first drug that in combination with doxorubicin for the treatment of patients with advanced/metastatic soft tissue sarcoma (STS) that has showed an improved overall survival compared to doxorubicin alone. These initial results are exciting and have the potential to change the landscape of treatment for patients with STS. Areas covered: This article reviews the development of olaratumab for oncology use by reviewing articles in PubMed for ‘platelet derived growth factor’ and ‘receptor’ and ‘soft tissue sarcoma’. We provide an overview of the published studies to date for olaratumab and specifically the use in soft tissue sarcoma. Expert commentary: Olaratumab is a well-tolerated drug that, when combined with doxorubicin, has shown an improved overall survival compared to doxorubicin alone and the phase III confirmatory study is eagerly awaited.
Clinical efficacy of eribulin mesylate for the treatment of metastatic soft tissue sarcoma
Published in Expert Opinion on Pharmacotherapy, 2017
Sheik Emambux, Antoine Italiano
Introduction: Metastatic soft tissue sarcoma, a devastating disease, has a median overall survival of only 12–18 months. Treatment options remain scarce. However, eribulin mesylate, a first-in-class halichondrin B-based microtubule dynamics inhibitor, has recently been approved for the management of patients with advanced liposarcoma. Areas covered: Based on a review of the literature between 2005 and 2017, we present a summary of eribulin mesylate’s mechanism of action and the studies showing its clinical efficacy in locally advanced or metastatic sarcomas. Expert commentary: Future development includes the definition of a biomarker signature related to patient outcome with eribulin. Further investigation via controlled clinical trials is needed to identify combination regimens that can optimize the efficacy of eribulin while providing an acceptable safety profile in sarcoma patients.
Emerging therapies for adult soft tissue sarcoma
Published in Expert Review of Anticancer Therapy, 2014
Stefano Radaelli, Sivia Stacchiotti, Paolo G Casali, Alessandro Gronchi
Soft tissue sarcoma (STS) are a broad group of rare tumors. Cornerstone of treatment is surgery. Complementary radiotherapy is recommended in high-risk STS arising from extremities. Doxorubicine ± ifosfamide based cytotoxic chemotherapy, explored in few randomized trials, showed a certain degree of activity, playing an established role only in unresectable disease. Since peculiar chemosensitivity towards alternative drugs was described for different metastatic subtypes in second or further lines, the modern concept of ‘histology-driven chemotherapy’ has been accepted and employed: gemicitabine ± dacarbazine, trabectedin and taxanes used respectively in patients with leiomyosarcoma, solitary fibrous tumor, myxoid/round cell liposarcoma, angiosarcoma. Recent discoveries about molecular pathways involved in STS tumorogenesis led to develop molecular targeted agents such as imatinib used in advanced dermatofibrosarcoma protuberans (DFSP) or metastatic DFSP-related fibrosarcoma, pazopanib, approved as second line regimen in advanced non-adipocitic STS and recently sunitinib in solitary fibrous tumors, alveolar soft part sarcoma and extraskeletal myxoid chondrosarcoma.
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