When managing metastatic spinal tumors, it is essential to select therapeutic methods based on adequate consideration of the features of primary lesions and the expected prognosis.
Two cases of sacral chordoma in a 7-year, 9-month-old boy and a 3-year, 4-month-old boy are presented. In addition to the typical histology of conventional chordoma, both tumors showed the less differentiated sarcomatoid appearance of atypical chordoma in the major portion. Immunohistochemically, in both cases neoplastic cells in areas of conventional as well as atypical chordoma were positive for keratins (CAM 5.2, AE1 and AE3), epithelial membrane antigen, vimentin, S-100 protein, carcinoembryonic antigen, and glial fibrillary acidic protein. Both patients underwent resection of the tumor and chemotherapy. In comparison with conventional chordomas in adults, however, these two tumors showed more aggressive clinical course and were less amenable to therapeutic control. The older boy died of multiple metastasis 1 year after initial diagnosis. At the last follow-up, 15 months after initial diagnosis, the younger boy was alive, but with recurrent and metastatic disease of the left parasacral area and chest wall. Our studies of these two cases and the reported cases suggest that sacral chordoma in children has distinctive clinicopathologic features denoting a highly aggressive tumor and that it should be treated as such.
Observations on 13 patients with primary malignant fibrous histiocytoma of bone were reported. Included were nine male and four female patients, ranging in age from 6 to 81 years (mean, 44.8 years). Pain was the most common complaint. The interval from the first symptom to the initial treatment varied from 2 months to 20 years. Seven tumors arose in the knee region. Roentgenologically, most of the lesions presented with an osteolytic and destructive appearance. Histologically, highly variable morphologic features existed. The storiform‐pleomorphic pattern was found in every tumor, although it was not necessarily pathognomonic for malignant fibrous histiocytoma of bone. Surgery, radical or incomplete, was the primary treatment for all but one patient. Lymph node metastasis was present in three. Five patients died of the disease from 3 to 79 months after the diagnosis (mean, 28 months), all exhibiting metastasis to the lung. Gaucher's body‐like structure demonstrated in the electron microscopic study shows the histiocytic quality of malignant fibrous histiocytoma of bone.
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