DESMOPLASTIC INFANTILE GANGLIOGLIOMAS - AN APPROACH TO THERAPY

DESMOPLASTIC INFANTILE GANGLIOGLIOMAS are massive cystic tumors, typic ally occurring in the cerebral hemispheres of infants. They are remark able pathologically for a prominent desmoplasia and, in some cases, fo r a cellular mitotically active component that can be readily interpre ted as a malignant neoplasm. Four children less than 1 year of age wer e diagnosed with desmoplastic infantile gangliogliomas in the Pediatri c Oncology Group infant brain tumor study (Protocol number 8633). All had been diagnosed by their respective institutions as having malignan t tumors, i.e., Grade III astrocytoma, malignant meningioma, leptomeni ngeal fibrosarcoma, and gliosarcoma. All had increased intracranial pr essure, and two had seizures. The tumors were extremely large, with on e measuring 12 x 9 x 9 cm. None had evidence of metastatic disease. On e patient had a gross total resection, and the other three had debulki ng procedures. All four children were treated with chemotherapy (cyclo phosphamide, vincristine, cisplatinum, etoposide) for periods ranging from 12 to 24 months. Of those with postoperative measurable disease, one child had a complete response, one a partial response, and one had stable disease at the conclusion of chemotherapy. No child received r adiation therapy. All children are alive with progression-free surviva ls after diagnosis of more than 36, 42, 48, and 60 months, respectivel y. Although desmoplastic infantile gangliomas are rare, recognition of this tumor type is essential because, despite their massive size and pathologically malignant appearance, they may have a relatively benign clinical course. If total surgical resection can be achieved, further therapy may not be indicated. In those patients in whom residual dise ase is present, chemotherapy appears to be an effective form of therap y. With improved recognition of this entity, future reports should hel p clarify its biological behavior and the efficacy of various treatmen t regimens.