Clinical Aspects of Craniofacial Osteosarcoma in Childhood: A Single Institution Experience

Yoon, Jong Hyung; Park, Jeong Ah; Kim, Eun Kyung; Kang, Hyoung Jin; Shin, Hee Young; Ahn, Hyo Seop

Abstract

PURPOSE: Craniofacial osteosarcoma is a very rare bone malignancy in childhood. The authors analyzed the clinical features, treatment and outcome of craniofacial osteosarcoma in childhood in a single institution.
METHODS
A retrospective review of 6 patients with non-metastatic craniofacial osteosarcomas who were diagnosed at the Department of Pediatrics, Seoul National University Children's Hospital between January 1990 and June 2006 was undertaken.
RESULTS
In six patients who were diagnosed as craniofacial osteosarcoma, the male to female ratio was 5 : 1, and their median age was 13 years 2 months. The involved sites were mandible (3 patients), maxilla, temporal bone and frontal bone. Craniofacial osteosarcomas in three patients were secondary malignancies after the treatment of rhabdomyosarcoma, squamous cell carcinoma and retinoblastoma, respectively, including cranial radiation. Five patients had surgery, and one of them had a positive resection margin. One patient only had incisional biopsy. Histologically, 4 patients showed osteoblastic type, 1 patient showed chondroblastic type, and 1 patient showed mixed type (osteoblastic and chondroblastic). All patients had chemotherapy after operation or biopsy, including two patients with neoadjuvant chemotherapy, but one patient stopped chemotherapy due to the patient's refusal. Four of them had long-term disease-free survival, one died due to septic shock, the treatment-related complication, and one died due to acute lymphoblastic leukemia, the secondary malignancy.
CONCLUSION
Craniofacial osteosarcoma in childhood is a very uncommon bone malignancy, and there is no study about that in Korea. The further study for more patients with craniofacial osteosarcoma or the study with multicenter analysis, for the better understanding of clinical features and the establishment of better treatment plan will be needed.