Pediatric Mesenchymal Chondrosarcoma

Background: Mesenchymal chondrosarcoma of the head and neck is a rare entity that has been associated with an aggressive behavior and propensity for late recurrence. It has been shown to occur in all age groups, but little is known about the behavior of this tumor in the pediatric population.

Methods: A retrospective chart review was conducted on patients presenting with the diagnosis of chondrosarcoma to St Jude Children's Research Hospital in the past 20 years to identify those with mesenchymal subtype. Data to be evaluated included clinical and radiographic characteristics, response to treatment, patterns of failure and survival in children with mesenchymal chondrosarcoma.

Results: Of 16 patients presenting with chondrosarcoma, 10 patients (62.5%) were found to have the mesenchymal subtype. There were 7 females and 3 males with median age of 13.65 years (range: 1.23 years – 19.67 years). All but one patient presented with local disease, and 6 patients had disease arising in the head and neck region (60%). Involved locations included the orbit (2), maxilla, nasal cavity, pterygopalatine fossa, skull base, and cervical spine. Average tumor size was 5.05 cm (range: 2 cm to 10.4 cm).

Surgery was performed in all patients. Subtotal resection was performed in 1 patient, and gross total resection was achieved in 8 patients. 9 patients underwent radiation with a mean cumulative dose of 57.01 Gray (range: 45–70 Gy). Chemotherapy was administered to all but one patient; ifosfamide and doxorubicin were the most commonly used agents. Complications to therapy included Fanconi syndrome, chronic renal insufficiency, and therapy-related myelodysplastic syndrome/acute myeloid leukemia. One patient who presented with pulmonary metastatic disease progressed 9 months after diagnosis.

Nine patients were alive at a median follow-up period of 35.5 months (range:5.6 months to 120.6 months); 8 of these patients had no evidence of disease. Two patients died, one from progressive disease and one from therapy-related complications. No recurrences have occurred to date in patients who presented with localized disease.

Conclusions: Children with mesenchymal chondrosarcoma are able to be successfully treated with aggressive local control measures and chemotherapy, with a low risk of recurrence compared with published rates in the adult literature.