Medical and Pediatric Oncology, Not Adult and Pediatric Oncology

Abstract

There are at least three good reasons to study patients with sarcomas; sarcomas can serve as a model of other more common human cancers, important clinically relevant genetic discoveries have been made disproportionately more often in sarcoma than in other tumors (p53, RB, c-kit, pdgfr, etc), and sarcomas affect patients of all ages from the newborn to the elderly. Despite many newly gained laboratory insights, as with many other cancers, progress in sarcoma is often too slow. Unlike other epithelial cancers, sarcomas are quite uncommon in our population. For every 100,000 Americans, 5,000 will have cancer. Of those 5,000 people, fewer than 30 will have any one of the 70 or more types of sarcomas. Progress is partly slowed in sarcomas because of competition for patient accrual into important clinical trials. Pediatric oncologists have been highly successful in accruing patients to clinical trials. Recently, however, there has been a decline in accrual to pediatric studies, perhaps because of new competition or insufficient funding for clinical trial participation. Pediatric oncologists have made important strides in patients with the Ewing’s family of cancers, osteosarcoma, and rhabdomyosarcoma, achieving cure rates from 65% to 75% in patients without metastasis. Many medical oncologists are in awe of the up to 25% cure rate of these diseases, even in the face of metastatic disease. But what about other soft tissue sarcomas? Pediatric oncologists have become comfortable with the phrase “nonrhabdomyosarcoma soft tissue sarcomas.” Medical oncologists giggle snidely at the phrase. Paul Meyers (pediatric oncologist at Memorial Sloan-Kettering Cancer Center, New York, NY) has said it is not adult and pediatric oncologists, but rather medical and pediatric oncologists. The following two articles in this issue are relevant to this point: “Phase II Trial of Neoadjuvant Vincristine, Ifosfamide, and Doxorubicin With Granulocyte ColonyStimulating Factor Support in Children and Adolescents With Advanced-Stage Nonrhabdomyosarcomatous Soft Tissue Sarcomas: A Pediatric Oncology Group Study” by Pappo et al and “Adult-Type Soft Tissue Sarcomas in Pediatric Age: Experience at the Istituto Nazionale Tumori in Milan” by Ferrari et al. I must confess that I like the sound of the second title much more than the first, but the fact is that both titles are accurate. The histologies studied do not include the rhabdomyosarcomas; rather, the histologies studied more often occur in the adult age group. Both articles describe sizeable patient populations. The Children’s Oncology Group article describes 39 patients in a prospective trial. Patients were accrued over less than a 3-year period; 26 institutions contributed patients to the study, of which 24 enrolled one patient. The Milan study reported retrospectively on a group of 182 patients treated at a single institution over a 25-year period. Both studies agree that this population represents 3% to 4% of pediatric cancers and that these cancers are quite heterogeneous in biology and natural history. Both studies agree that synovial sarcoma is the most common histology among the pediatric nonrhabdo soft tissue sarcomas. The Italian group indicates that “most of the experience gained in the treatment of pediatric NRSTS [nonrhabdomyosarcoma soft tissue sarcomas] either derives from managing soft tissue sarcomas in adults or relies on the principles deriving from the management of RMS [rhabdomyosarcomas].” Evidence for the managing principles being derived from rhabdomyosarcoma includes pretreatment evaluation of these patients with bone scans and bone marrow biopsies. Neither of these diagnostic studies is routinely used by medical oncologists who treat soft tissue sarcoma patients. Similar to medical oncologists, many pediatric oncologists choose to treat patients with anthracyclineand ifosfamide-based chemotherapy regimens. However, sometimes the experiences of medical oncologists are not considered by pediatric oncologists. Both studies included JOURNAL OF CLINICAL ONCOLOGY E D I T O R I A L VOLUME 23 NUMBER 18 JUNE 2