TY - JOUR
T1 - Desmoplastic infantile gangliogliomas
T2 - An approach to therapy
AU - Duffner, Patricia K.
AU - Burger, Peter C.
AU - Cohen, Michael E.
AU - Sanford, Robert A.
AU - Krischer, Jeffrey P.
AU - Elterman, Roy
AU - Aronin, Patricia A.
AU - Pullen, Jeanette
AU - Horowitz, Marc E.
AU - Parent, Andrew
AU - Martin, Paul
AU - Kun, Larry E.
PY - 1994/4
Y1 - 1994/4
N2 - 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 12×9×9 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. With improved recognition of this entity, future reports should help clarify its biological behavior and the efficacy of various treatment regimens.
AB - 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 12×9×9 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. With improved recognition of this entity, future reports should help clarify its biological behavior and the efficacy of various treatment regimens.
KW - Brain tumor
KW - Cerebral neoplasm
KW - Ganglioglioma
KW - Infant brain tumors
UR - http://www.scopus.com/inward/record.url?scp=0028288227&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0028288227&partnerID=8YFLogxK
U2 - 10.1227/00006123-199404000-00003
DO - 10.1227/00006123-199404000-00003
M3 - Article
C2 - 8008154
AN - SCOPUS:0028288227
SN - 0148-396X
VL - 34
SP - 583
EP - 589
JO - Neurosurgery
JF - Neurosurgery
IS - 4
ER -