Abstract
Primary malignant bone tumors are uncommon and heterogeneous malignancies. This document is a guideline developed by the Spanish Group for Research on Sarcoma with the participation of different specialists involved in the diagnosis and treatment of bone sarcomas. The aim is to provide practical recommendations with the intention of helping in the clinical decision-making process. The diagnosis and treatment of bone tumors requires a multidisciplinary approach, involving as a minimum pathologists, radiologists, surgeons, and radiation and medical oncologists. Early referral to a specialist center could improve patients’ survival. The multidisciplinary management of osteosarcoma, chondrosarcoma, chordoma, giant cell tumor of bone and other rare bone tumors is reviewed in this guideline. Ewing’s sarcoma will be the focus of a separate guideline because of its specific biological, clinical and therapeutic features. Each statement has been accompanied by the level of evidence and grade of recommendation on the basis of the available data. Surgical excision is the mainstay of treatment of a localized bone tumor, with various techniques available depending on the histologic type, grade and location of the tumor. Chemotherapy plays an important role in some chemosensitive subtypes (such as high-grade osteosarcoma). In other subtypes, historically considered chemoresistant (such as chordoma or giant cell tumor of bone), new targeted therapies have emerged recently, with a very significant efficacy in the case of denosumab. Radiation therapy is usually necessary in the treatment of chordoma and sometimes of other bone tumors.
Highlights
Primary bone tumors are an uncommon and heterogeneous group of malignancies
Centralized referral should ideally be done from the moment a diagnosis of a bone sarcoma is suspected (III, A) [1]. These guidelines have been developed by a multidisciplinary panel of specialists in the different fields involved in the diagnosis and treatment of bone tumors
Assessment of percentage of tumor necrosis in tumors treated with neoadjuvant chemotherapy is essential to check the effect of treatment and constitutes one of the most relevant prognostic factors (III, A)
Summary
Primary bone tumors are an uncommon and heterogeneous group of malignancies. Osteosarcoma is the most frequent with. Cancer Chemother Pharmacol (2017) 80:1113–1131 an estimated incidence average of 0.2–0.3 cases per 100,000 per year in Europe It occurs mainly in adolescents and young adults, but is seen in older patients, usually related to prior radiotherapy or Paget’s disease. Chondrosarcoma is the most frequent bone sarcoma in adults, with an incidence average of 0.2 cases per 100,000 per year. It often has an aggressive local behavior but rarely metastasizing, in some cases more aggressive forms can be seen. There is a great deal of evidence demonstrating that early recognition and referral to a specialist center that provides a multidisciplinary diagnosis and therapeutic approach, and manages a high number of cases annually, could improve outcomes in patients with bone tumors. Centralized referral should ideally be done from the moment a diagnosis of a bone sarcoma is suspected (III, A) [1]
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