Sarcoma 

About: Sarcoma is an academic journal published by Hindawi Publishing Corporation. The journal publishes majorly in the area(s): Sarcoma & Soft tissue sarcoma. It has an ISSN identifier of 1357-714X. It is also open access. Over the lifetime, 727 publications have been published receiving 17128 citations. The journal is also known as: connective and soft tissue neoplasm & tumor of soft tissue and skeleton.

Guidelines for the Management of Soft Tissue Sarcomas

Abstract: These guidelines were drawn up following a consensus meeting of UK sarcoma specialists convened under the auspices of the British Sarcoma Group and are intended to provide a framework for the multidisciplinary care of patients with soft tissue sarcomas The guidelines published by the European Society of Medical Oncology (ESMO) and the National Comprehensive Cancer Network (NCCN) were used as the basis for discussion and adapted according to UK clinical practice and local requirements Note was also taken of the National Institute for Health and Clinical Excellence (NICE) improving outcomes guidance (IOG) for people with sarcoma and existing technology appraisals The guidelines are not intended to challenge NICE guidance but discrepancies may exist where current guidance does not reflect an international standard of care owing to the ever-evolving nature of cancer treatment It is acknowledged that these guidelines will require updating on a regular basis An appendix lists the key recommendations which are summarised below Any patient with a suspected soft tissue sarcoma should be referred to a diagnostic centre and managed by a specialist sarcoma multidisciplinary team Surgical excision followed by post operative radiotherapy is the standard management of high grade limb sarcomas although occasionally amputation remains the only option Pre-operative treatment with chemotherapy or radiotherapy should be considered for patients with borderline resectable tumours Isolated limb perfusion may permit limb salvage in some cases where amputation is the only other option Adjuvant chemotherapy is not routinely recommended but may be considered in certain specific situations Regular follow up is recommended to assess local control and the development of metastatic disease Single agent doxorubicin is the standard first line therapy for metastatic disease Ifosfamide is an alternative if anthracyclines are contraindicated Combination therapy may be considered in individual patients Second line agents include ifosfamide, dacarbazine, trabectedin and the combination of gemcitabine + docetaxel Surgical resection of local recurrence and pulmonary metastases should be considered in individual patients There is specific guidance on the management of retroperitoneal and uterine sarcomas

A meta-analysis of osteosarcoma outcomes in the modern medical era.

Abstract: Four decades ago, specialized chemotherapy regimens turned osteosarcoma, once considered a uniformly fatal disease, into a disease in which a majority of patients survive Though significant survival gains were made from the 1960s to the 1980s, further outcome improvements appear to have plateaued This study aims to comprehensively review all significant, published data regarding osteosarcoma and outcome in the modern medical era in order to gauge treatment progress Our results indicate that published survival improved dramatically from 1960s to 1980s and then leveled, or in some measures decreased Recurrence rates decreased in the 1970s and then leveled In contrast, published limb salvage rates have increased significantly every recent decade until the present Though significant gains have been made in the past, no improvement in published osteosarcoma survival has been seen since 1980, highlighting the importance of a new strategy in the systemic management of this still very lethal condition

The Molecular Pathogenesis of Osteosarcoma: A Review

Abstract: Osteosarcoma is the most common primary malignancy of bone. It arises in bone during periods of rapid growth and primarily affects adolescents and young adults. The 5-year survival rate for osteosarcoma is 60%-70%, with no significant improvements in prognosis since the advent of multiagent chemotherapy. Diagnosis, staging, and surgical management of osteosarcoma remain focused on our anatomical understanding of the disease. As our knowledge of the molecular pathogenesis of osteosarcoma expands, potential therapeutic targets are being identified. A comprehensive understanding of these mechanisms is essential if we are to improve the prognosis of patients with osteosarcoma through tumour-targeted therapies. This paper will outline the pathogenic mechanisms of osteosarcoma oncogenesis and progression and will discuss some of the more frontline translational studies performed to date in search of novel, safer, and more targeted drugs for disease management.

The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS-I Through IRS-IV Studies as Background for the Current IRS-V Treatment Protocols.

Abstract: Purpose. To enumerate lessons from studying 4292 patients with rhabdomyosarcoma (RMS) in the Intergroup Rhabdomyosarcoma Study Group (IRSG, 1972± 1997). Patients. Untreated patients < 21 years of age at diagnosis received systemic chemotherapy, with or without irradiation (XRT) and/or surgical removal of the tumor. Methods. Pathologic materials and treatment were reviewed to ascertain compliance and to confirm response and relapse status. Results. Survival at 5 years increased from 55 to 71% over the period. Important lessons include the fact that extent of disease at diagnosis affects prognosis. Re-excising an incompletely removed tumor is worthwhile if acceptable form and function can be preserved. The eye, vagina, and bladder can usually be saved. XRT is not necessary for children with localized, completely excised embryonal RMS. Hyperfractionated XRT has thus far not produced superior local control rates compared with conventional, once-daily XRT. Patients with non-metastatic cranial parameningeal sarcoma can usually be cured with localized XRT and systemic chemotherapy, without whole-brain XRT and intrathecal drugs. Adding doxorubicin, cisplatin, etoposide, and ifosfamide has not significantly improved survival of patients with gross residual or metastatic disease beyond that achieved with VAC (vincristine, actinomycin D, cyclophosphamide) and XRT. Most patients with alveolar RMS have a tumor-specific translocation. Mature rhabdomyoblasts after treatment of patients with bladder rhabdomyosarcoma are not necessarily malignant, provided that the tumor has shrunk and malignant cells have disappeared. Discussion. Current IRSG-V protocols, summarized herein, incorporate recommendations for risk-based management. Two new agents, topotecan and irinotecan, are under investigation for patients who have an intermediate or high risk of recurrence.

Using Epidemiology and Genomics to Understand Osteosarcoma Etiology

Abstract: Osteosarcoma is a primary bone malignancy that typically occurs during adolescence but also has a second incidence peak in the elderly It occurs most commonly in the long bones, although there is variability in location between age groups The etiology of osteosarcoma is not well understood; it occurs at increased rates in individuals with Paget disease of bone, after therapeutic radiation, and in certain cancer predisposition syndromes It also occurs more commonly in taller individuals, but a strong environmental component to osteosarcoma risk has not been identified Several studies suggest that osteosarcoma may be associated with single nucleotide polymorphisms in genes important in growth and tumor suppression but the studies are limited by sample size Herein, we review the epidemiology of osteosarcoma as well as its known and suspected risk factors in an effort to gain insight into its etiology