Osteosarcoma in very young children

Abstract

BACKGROUND: This study was conducted to investigate presentation, treatment, and outcome in very young children with osteosarcoma. METHODS: The authors retrospectively analyzed the data of 2706 consecutive COSS patients with newly diagnosed osteosarcoma and identified 28 (1.0%) patients aged younger than 5 years at diagnosis. Demographic, diagnostic, tumor, treatment-related variables, response, and survival data were analyzed. RESULTS: Of the 28 preschoolers, 27 presented with high-grade central osteosarcoma of an extremity, and 1 had a secondary osteosarcoma of the orbit. This analysis focused on the 27 patients with extremity tumors. The size of the primary was large (!one-third of the involved bone) in 20 of 27 patients. Primary metastases were detected in 4 of 27 children. All patients received multiagent chemotherapy, and 11 of 18 analyzed tumors responded well (>90% necrosis) to neoadjuvant chemotherapy. Limb-sparing surgery was performed in 9 cases, ablative procedures were performed in 15, and, in 3 cases, no local surgery was performed. With a median follow-up of 4 years (6.2 years for survivors), 13 patients were alive. Four patients never achieved a complete remission, and 11 developed recurrences; 14 of these 15 patients died. Five-year overall and event-free survival probabilities were 51% (standard error of the mean [SE], 10%) and 48% (SE, 10%). Better survival was correlated with good response to chemotherapy and later time period of diagnosis. CONCLUSIONS: Osteosarcoma is extremely rare in preschool children. These patients often have large tumors that may require mutilating resections. Prognosis is in the range of that reported for older patients.

Osteoporosis in Children and Young Adults: A Late Effect After Chemotherapy for Bone Sarcoma

“…1A-B The chemotherapeutic treatment guidelines for Ewing's sarcoma and osteosarcoma according to study proven protocols are shown. (A) The graph shows the chemotherapeutic protocols and major chemotherapeutic agents used for treatment of Ewing's sarcoma [3,7,15,31] and (B) osteosarcoma [3,6,15,21]. …”

“…Both tumors affect the long bones of the appendicular skeleton and occur with occult metastases at the time of diagnosis in as much as 90% of cases [3]. Chemotherapy, following standardized study protocols [3,4,6,7,15,21,31], and potential radiotherapy (Ewing's sarcoma) in addition to wide resection have been seen to increase patients' survival [19]. Although the 5-year survival rate for patients with nonmetastasized Ewing's sarcoma has been less than 10% with resection only (with or without radiotherapy) [3], it has reached 50% to 75% with additional chemotherapeutic treatment [3].…”

Enhancement of antitumor immunity by combining anti-cytotoxic T lymphocyte antigen-4 antibodies and cryotreated tumor lysate-pulsed dendritic cells in murine osteosarcoma

“…Remarkable advances in the treatment of osteosarcoma have occurred, including the introduction of adjuvant chemotherapy and appropriate surgical excision (1)(2)(3)(4). However, there have also been advances in the field of immunotherapy for the treatment of osteosarcoma that have received less attention (5,6).…”

In vitro generation of cytotoxic T lymphocyte response using dendritic cell immunotherapy in osteosarcoma

“…This type of bone and soft tissue sarcoma predominantly affects children and young adults (1). Rapid advancements have occurred in the treatment of osteosarcoma, including the introduction of adjuvant chemotherapy, conformal radiotherapy and improved surgical excision (2)(3)(4)(5), and the 5-year survival rate in patients with localized disease is 50-70% (6). However, prognosis remains poor in relapsed disease and in patients that are resistant to the standard treatment modalities (7).…”