Clinician-to-Clinician Update Clinician-to-Clinician Update

Advances in the Treatment of Pediatric Sarcoma

October 2016

Sarcomas, appearing either as bone or soft tissue tumors, account for fewer than 1,500 annual cases in children and adolescents in the United States.1 Soft tissue sarcomas represent 8% of pediatric malignant tumors.2 Malignant bone tumors represent 3% of tumors in children and adolescents, a total that includes osteosarcoma and the Ewing sarcoma family of tumors, the most common among sarcomas.3

Because sarcomas are rare and include more than 100 diagnoses and because symptoms can seem to mimic those of common conditions, sarcomas can be challenging to diagnose. Providers should watch for pediatric sarcoma “red flags.” These include a joint that will not fully extend and a lump or bump that enlarges over time, involves deep tissue, and presents with or without pain. Pain or tenderness that occurs at night, persists while resting, or does not correspond to any known injury can also be symptoms.4

Sarcomas can appear as localized tumors, and surgical procedures, most often combined with chemotherapy, can provide control of small, resectable tumors. Radiation therapy may also be incorporated into treatment plans. Many sarcomas, however, rapidly metastasize to the lungs, liver, bones, subcutaneous tissue, and lymph nodes.5 While patients with metastatic disease face significantly more life-threatening prognoses6, metastatic sarcomas have demonstrated significant sensitivity to chemotherapy.5

— Biopsy of an osteosarcoma showing tumor cells (blue lines) and abnormal bone (pink areas). University of Minnesota Health pediatric specialists will lead a phase I study of a treatment that targets the protein that stimulates growth of osteosarcomas. Photo ©iStock.

For young patients diagnosed with sarcoma, early diagnosis2 and evaluation and treatment by a multidisciplinary team of pediatric cancer specialists experienced in sarcoma care provide the best outcomes. University of Minnesota Masonic Children’s Hospital treats more pediatric sarcoma patients than any other facility in the state. University of Minnesota Health physicians are leaders in research into sarcoma, and the hospital houses a National Cancer Institute-funded Children’s Oncology Group Phase I Program, among only 21 such institutions in the country. As part of this cancer research network, University of Minnesota Masonic Children’s Hospital offers patients access to more clinical trials of new therapies than any other area hospital.

Genomic analysis of specific sarcomas and trials of novel chemotherapy regimens may provide new approaches to treating these rare cancers. A genomic analysis conducted by University of Minnesota researchers has uncovered a protein that stimulates the growth of human osteosarcomas. The researchers using mice models have tested an antibody that targets the protein. University of Minnesota Health pediatric specialist Emily Greengard, MD, will be leading a national Phase I study to evaluate this antibody for treating young patients with sarcoma. In another national phase I study, Dr. Greengard is studying dosages and side effects of a novel combination of chemotherapy and crizotinib for young patients with relapsed or refractory solid tumors or anaplastic large cell lymphoma.


  1. Quartuccio N, Wexler L, Schöder H. Pediatric Sarcomas. Clinical Nuclear Medicine in Pediatrics. 2016:279-300. doi:10.1007/978-3-319-21371-2_14.
  2. Ferrari A, Miceli R, Casanova M, et al. The symptom interval in children and adolescents with soft tissue sarcomas. Cancer. 2010;116(1):177-83. doi:10.1002/cncr.24695.
  3. Jackson TM, Bittman M, Granowetter L. Pediatric Malignant Bone Tumors: A Review and Update on Current Challenges, and Emerging Drug Targets. Current Problems in Pediatric and Adolescent Health Care. 2016;46(7):213-228.doi:10.1016/j.cppeds.2016.04.002.
  4. Rein in Sarcoma Foundation. The Red Flags of Sarcoma Cancers. Brochure.
  5. Amankwah EK, Conley AP, Reed DR. Epidemiology and therapies for metastatic sarcoma. Clin Epidemiol. 2013;5:147-62. doi:10.2147/clep.s28390.
  6. Arndt CA, Rose PS, Folpe AL, Laack NN. Common Musculoskeletal Tumors of Childhood and Adolescence. Mayo Clinic Proceedings. 2012;87(5):475-487. doi:10.1016/j.mayocp.2012.01.015.

When to refer

The pediatric cancer program at the University of Minnesota Masonic Children’s Hospital has extensive experience treating sarcoma. Our pediatric oncology specialists provide collaborative, multidisciplinary care and the latest, evidence-based therapies.

Common symptoms of sarcoma include lumps and bumps that enlarge over time, involve deep tissue, and present with or without pain. Leg or back pain, especially pain or tenderness that occurs at night, persists while resting, or does not correspond to any known injury can also be symptoms. Patients whose symptoms do not resolve under standard treatment and who return with the same complaint more than once should be referred for further evaluation. The first referral should be to an orthopaedic surgeon or pediatric surgeon who should consider ordering scans and may conduct a biopsy.

Upon receiving a patient with a sarcoma diagnosis, our pediatric cancer program’s multidisciplinary team rapidly mobilizes to evaluate the patient. The team draws on the expertise of pediatric oncologists, radiation oncologists, orthopaedic and pediatric surgeons, nurses, physical therapists, nutritionists, pharmacists, child-and-family life specialists, and social workers, among other specialists.

As the site of Minnesota’s only National Cancer Institute-funded Children’s Oncology Group (COG) Phase 1 Program, our hospital participates in a national research and clinical network. We offer patients access to clinical trials of emerging treatments, providing patients access to some of the most advanced and innovative treatments available.

To view current clinical trials available through M Health providers, visit

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