Case report: Mesenchymal chondrosarcoma of the lumbar spine in a child.

ABSTRACT

BACKGROUND: Chondrosarcomas of the spine constitute 4% to 10% of all primary spinal bone tumors and approximately 70% of the cases occur during the second or third decade of life. Mesenchymal chondrosarcoma is a rare aggressive variant of chondrosarcoma. The prognosis of mesenchymal chondrosarcoma is usually poor with a tendency for late local recurrence and metastasis. CASE DESCRIPTION We describe a case of primary mesenchymal chondrosarcoma affecting the L5 vertebra of a 9-year-old girl. The patient underwent a staged circumferential resection of the tumor after three rounds of neoadjuvant chemotherapy. The patient had additional chemotherapy and radiation therapy as an intralesional margin was achieved during the procedure. At 9 years followup, the patient was asymptomatic, neurologically intact, and remained in remission. LITERATURE REVIEW We identified only four previously published cases of spinal mesenchymal chondrosarcoma in childhood, two of which had relatively early recurrence and poor survival, and two survived but with only short followup. PURPOSES AND CLINICAL RELEVANCE As the clinical and radiographic findings of mesenchymal chondrosarcoma are nonspecific, the diagnosis of this rare tumor requires careful histopathologic review of the specimens. We suggest the differential diagnosis of every primary intraspinal tumor include tumors of mesenchymal origin. The prognosis is apparently not uniformly poor.