title: Desmoplastic infantile gangliogliomas: an approach to therapy.
abstract: Desmoplastic infantile gangliogliomas are massive cystic tumors, typically occurring in the cerebral hemispheres of infants. They are remarkable pathologically for a prominent desmoplasia and, in some cases, for a cellular mitotically active component that can be readily interpreted as a malignant neoplasm. Four children less than 1 year of age were diagnosed with desmoplastic infantile gangliogliomas in the Pediatric Oncology Group infant brain tumor study (Protocol number 8633). All had been diagnosed by their respective institutions as having malignant tumors, i.e., Grade III astrocytoma, malignant meningioma, leptomeningeal fibrosarcoma, and gliosarcoma. All had increased intracranial pressure, and two had seizures. The tumors were extremely large, with one measuring 12 x 9 x 9 cm. None had evidence of metastatic disease. One patient had a gross total resection, and the other three had debulking procedures. All four children were treated with chemotherapy (cyclophosphamide, 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 radiation therapy. All children are alive with progression-free survivals after diagnosis of more than 36, 42, 48, and 60 months, respectively. 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 disease is present, chemotherapy appears to be an effective form of therapy.(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: State University of New York, Buffalo School of Medicine.
authors.forename: P K
authors.initials: PK
authors.lastname: Duffner
authors.forename: P C
authors.initials: PC
authors.lastname: Burger
authors.forename: M E
authors.initials: ME
authors.lastname: Cohen
authors.forename: R A
authors.initials: RA
authors.lastname: Sanford
authors.forename: J P
authors.initials: JP
authors.lastname: Krischer
authors.forename: R
authors.initials: R
authors.lastname: Elterman
authors.forename: P A
authors.initials: PA
authors.lastname: Aronin
authors.forename: J
authors.initials: J
authors.lastname: Pullen
authors.forename: M E
authors.initials: ME
authors.lastname: Horowitz
authors.forename: A
authors.initials: A
authors.lastname: Parent
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D001921:Brain / Q000473:pathology; D001932:Brain Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D003131:Combined Modality Therapy; D003399:Craniotomy; D005354:Fibrosarcoma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D005500:Follow-Up Studies; D018303:Ganglioglioma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D005909:Glioblastoma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D018316:Gliosarcoma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D008577:Meningeal Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D008579:Meningioma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D008938:Mitosis / Q000187:drug effects / Q000502:physiology; D009367:Neoplasm Staging
chemical_list: D014408:Biomarkers, Tumor; D005904:Glial Fibrillary Acidic Protein