Sarcomas

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Key points

  • Osteosarcoma and Ewing sarcoma are the 2 most common malignant bone tumors of pediatrics, with peak incidence during the adolescent years.

  • Rhabdomyosarcoma is the most common soft-tissue sarcoma of childhood, with a bimodal age distribution of 2 to 6 years and 15 to 19 years of age.

  • Sarcomas are best treated with multimodality treatment that often includes chemotherapy, surgery, and/or radiation therapy.

  • Children with localized sarcoma fare well with multimodality treatment, whereas those with

Osteosarcoma

OS is a malignancy that derives its origin from mesenchymal cells. The pathologic hallmark is the production of malignant osteoid by pleomorphic malignant cells within a connective tissue matrix. OS can be divided into several subsets defined by location (appendicular vs axial, central vs surface), histologic grade (low, intermediate, or high), and the predominant matrix type.1 Most OS tumors are conventional OS, a high-grade OS made up of 3 major subtypes based on the predominant mix of

Epidemiology

OS is the most common malignant bone tumor in pediatrics, with an incidence of approximately 4.5 cases per 1 million children in the developed world.17 EWS is the second most common malignant bone tumor in pediatrics, with an incidence of nearly 3 cases per 1 million children, a slight predilection for males over females, and a markedly lower incidence in individuals of African descent.17, 18 Both OS and EWS peak in the second decade of life, but whereas OS is very rare in young children, EWS

Clinical features

Both OS and EWS, when they occur as primary bone tumors, most commonly present with locoregional pain and swelling, which often last for weeks to months before presentation. In the appendicular skeleton, the pain can be followed by a palpable mass. In the case of OS, fevers, weight loss, and other systemic complaints are uncommon. In EWS, where there may be bone marrow involvement in advanced disease, systemic symptoms may be present. Because these symptoms can be nonspecific and occur most

Diagnostic workup

Although the initial evaluation of a child or adolescent with a suspected bone or soft-tissue tumor often starts with radiography in the primary care setting, the importance of referral to a pediatric tertiary care center must be emphasized. A multidisciplinary team including pediatric general and orthopedic surgery, oncology, radiology, and pathology is critical for accurate diagnosis, staging, and initial therapy (Figs. 6 and 7).

Plain radiographs are often helpful for discriminating OS or EWS

Staging

For both EWS and OS, conventional TNM (Tumor, Lymph Node, Metastases) staging is not used. Patients with OS and EWS rarely metastasize to the lymph nodes. Instead, tumors are considered either localized or metastatic. In OS, although patients are generally considered to have either localized or metastatic disease, the Enneking staging system exists, which incorporates tumor grade in addition to the presence or absence of metastatic disease to account for those rare low-grade OS tumors such as

Osteosarcoma and Ewing Sarcoma

The most important adverse prognostic factor in OS and EWS is the presence or absence of metastatic disease. Patients with localized disease at diagnosis have a 5-year overall survival of about 75% compared with patients with metastatic disease at diagnosis, who have a 5-year overall survival of approximately 20% to 25%.26, 28 Since complete surgical resection of macroscopic disease is essential for cure in OS, surgical resectability is also an important prognostic factor; therefore, tumors

Management goals

The care of a child or adolescent with a malignant bone or soft-tissue sarcoma requires a multidisciplinary approach to achieve successful multimodality care. The overarching goals are to achieve local disease control with wide surgical resection and/or radiation therapy and distant disease control, typically through the use of systemic chemotherapy. Surgical approaches, the use of radiation therapy, and specific chemotherapy regimens are unique to the sarcoma subtypes, as outlined next.

Summary

The outcome for patients with localized sarcoma has improved dramatically with the addition of systemic, combination chemotherapy in conjunction with local control using surgery and/or radiation. Improvements in surgical technique and the ability to salvage functional limbs have led to greater use of surgery for local control with satisfactory function. However, patients with metastatic and recurrent disease continue to have dismal outcomes despite the use of intensive chemotherapy, surgery,

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