Subcutaneous and skeletal chordomoid nodules in an infant

Multicentric subcutaneous and skeletal nodules with histologic features of chordoma developed over a period of 3 months in a black infant. Radiolucent intracranial lesions were demonstrated by pneumoencephalogram and computerized tomography scan. The patient received chemotherapy and the nodules regressed. After a follow-up of 7 years, there has been no recurrence of these chordomoid lesions and the child enjoys good health and normal growth and development. In retrospect, these nodules appear to be a benign, self-limited heterotopias and not malignancies. The cells with physaliphorous characteristics may represent unusual differentiation of fibroblasts rather than indicating notochordal origin.