Paravertebral “Round Cell” Tumors in Children

Abstract

Arecent review of the records of the Division of Radiotherapy, Children's Hospital Medical Center, and of the Children's Cancer Research Foundation of Boston has disclosed 5 patients who had a malignant neoplasm which appeared to be unusual because of its histologic characteristics and its clinical course in relation to the site of origin at diagnosis. In each child, the epidural space of a portion of spinal cord was involved and the adjacent paravertebral soft tissues were infiltrated to varying degrees. Symptoms of cord compression were due to extradural pressure. Except for a congenital abnormality of a vertebral body, one patient exhibited no radiographic evidence of bone alteration at the local site. The other patients were found to have radiographic changes in the neural arch of a vertebral body at the site of tumor involvement. These changes were due to pressure by extrinsic tumor and secondary bone reaction. Case Reports CASE I: M. K., a girl, was born with a lumbar hemivertebra at L-2 and resultant scoliosis. The patient was treated for pulmonary tuberculosis at two and one-half years of age. At age six years, she was admitted to Children's Hospital Medical Center because of pain in the lower back and weakness in the left lower extremity. Myelography at this time revealed complete obstruction to the free flow of contrast material at the level of L-4. At laminectomy, an extradural tumor which extended through the neural foramen and infiltrated adjacent soft tissues about the lumbar column was removed. Inspection of the retroperitoneal space at a subsequent laparotomy revealed no masses, although a portion of excised psoas muscle did reveal tumor infiltration. Following surgery, the child received 3,060 rads2 in twenty-four elapsed days to the lumbar spine from L-1 to L-5, using opposed 45° oblique posterior portals. Chemotherapy3 was used concomitantly (nitrogen mustard) and later as maintenance (Cytoxan). The patient remained well until eighteen months later when weakness developed in the left leg, followed within twenty-four hours by weakness in the right lower extremity. Physical examination disclosed that the patient dragged the left leg because of weakness, but no sensory impairment was noted. Left knee and ankle reflexes were hyperactive and she had bilateral Babinski reflexes. Radiographs of the spine revealed no new findings. The hemivertebral abnormality and scoliosis were apparent as before, and there was no bone destruction or soft-tissue mass (Fig. 1). A myelogram revealed a total extradural block to the flow of contrast medium at the level of the T-9 vertebral body. It was thought that the previous tumor had extended upward by direct continuity. Conservative irradiation management was elected. Since this was prior to our policy of initial high-dose irradiation (31), the girl received an initial low dose of 20 rads to the cord from T-l to T-12.