UK Musculoskeletal Oncology: Something for All Ages Lars Wagner, MD Pediatric Hematology/Oncology University of Kentucky
Pediatric-Type Sarcomas of Bone and Soft Tissue The incidence of sarcoma continues to increase with age There is a distinctly different group of tumors that are seen almost exclusively in in children, adolescents, and young adults Tend to be high-grade tumors, often with chromosomal translocations Have frequent metastases, including to bone, nodes, and marrow Fortunately, they tend to be more chemoresponsive than adult sarcomas Bone: Osteosarcoma, Ewing sarcoma Soft tissue: Rhabdomyosarcoma, Synovial sarcoma
Sarcomas in Children and Adolescents Collectively make up 15% of all pediatric cancers, of which roughly half are soft tissue and half arise in the bone Rhabdomyosarcoma is the most common sarcoma in the first decade of life, and makes nearly 50% of all soft tissue sarcomas in pediatrics The other half are collectively called non-rhabdomyosarcoma soft tissue sarcoma Osteosarcoma and Ewing sarcoma peak in the second decade, and can be seen in young adults as well Importantly, the age distribution of these pediatric-type sarcomas does not arbitrarily cap off at 18 or 21 years
Relative Incidence of Soft Tissue Sarcoma By Age Albtritton, Hematol Oncol Clin N Am 2005
Histologic Subtype of Soft Tissue Sarcoma Varies By Age Albtritton, Hematol Oncol Clin N Am 2005
Sarcomas of Young Adults Roughly 3500 individuals per year between ages 15 and 29 are diagnosed with sarcoma Due to its aggressive nature, sarcoma disproportionately contributes to mortality compared to other cancers in this age group such as Hodgkin s disease and testicular cancer Because increasing age is a negative prognostic factor, these patients do worse than most patients cared for by pediatric oncologists, but still better than patients cared for by adult oncologists Care for these patients is complicated, and dispersed among a variety of medical settings
Prognosis for Young Adults with Sarcoma Prognosis worsens with age: 5-yr OS for localized RMS in young kids is > 75%, but in adults is <40% Synovial sarcoma risk of death increases 0.07 times for every year increase in age Survival worse with increasing age for both Ewing and osteosarcoma This worse prognosis likely reflect combination of: Biological differences in the tumors Tolerance of therapy Delays in diagnosis Physicians less familiar with the disease Lack of participation in, and availability of, clinical trials
Young Adults are Under-Represented in Clinical Trials for Soft Tissue Sarcoma Albtritton, Hematol Oncol Clin N Am 2005
Ongoing Sarcoma Research at UK Ongoing NCI-sponsored national Phase II clinical trial investigating a novel drug combination (cixutumumab + temsirolimus) for treatment of relapsed sarcoma (Wagner, PI) Proposed limited-institution trial investigating the addition of targeted agents to standard chemotherapy for newly-diagnosed osteosarcoma (Badgett, PI) Ongoing investigations to assess: Impact of early lymphocyte response after chemotherapy in osteosarcoma Role of sentinel node biopsy with SPECT/CT localization in pediatric sarcoma Methods to identify minimal residual disease in the bone marrow or blood in Ewing sarcoma patients Impact of thin-cut CT scans on number of lung nodules identified at surgery in pediatric sarcoma patients Role of bone scan vs. PET scan in osteosarcoma patients
Care for Adolescents and Young Adults with Sarcoma at UK It takes a village: Critical mass of specialists now assembled Orthopedic oncology Medical/pediatric oncology Radiation oncology Pathology Radiology (Diagnostic and Interventional) Social work Growing number of clinical trials and research opportunities Commitment to crossing artificial boundaries of age and care settings to provide high-quality, comprehensive care for these complex patients that suits their individual needs