Desmoplastic infantile gangliogliomas

An approach to therapy

Patricia K. Duffner, Peter C. Burger, Michael E. Cohen, Robert A. Sanford, Jeffrey P. Krischer, Roy Elterman, Patricia A. Aronin, Jeanette Pullen, Marc E. Horowitz, Andrew Parent, Paul Martin, Larry E. Kun

Research output: Contribution to journalArticle

76 Citations (Scopus)

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 12×9×9 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.

Original languageEnglish (US)
Pages (from-to)583-589
Number of pages7
JournalNeurosurgery
Volume34
Issue number4
StatePublished - Jan 1 1994

Fingerprint

Ganglioglioma
Neoplasms
Drug Therapy
Gliosarcoma
Therapeutics
Fibrosarcoma
Astrocytoma
Intracranial Pressure
Cerebrum
Meningioma
Vincristine
Etoposide
Brain Neoplasms
Cyclophosphamide
Disease-Free Survival
Seizures
Radiotherapy
Pediatrics

Keywords

  • Brain tumor
  • Cerebral neoplasm
  • Ganglioglioma
  • Infant brain tumors

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Duffner, P. K., Burger, P. C., Cohen, M. E., Sanford, R. A., Krischer, J. P., Elterman, R., ... Kun, L. E. (1994). Desmoplastic infantile gangliogliomas: An approach to therapy. Neurosurgery, 34(4), 583-589.

Desmoplastic infantile gangliogliomas : An approach to therapy. / Duffner, Patricia K.; Burger, Peter C.; Cohen, Michael E.; Sanford, Robert A.; Krischer, Jeffrey P.; Elterman, Roy; Aronin, Patricia A.; Pullen, Jeanette; Horowitz, Marc E.; Parent, Andrew; Martin, Paul; Kun, Larry E.

In: Neurosurgery, Vol. 34, No. 4, 01.01.1994, p. 583-589.

Research output: Contribution to journalArticle

Duffner, PK, Burger, PC, Cohen, ME, Sanford, RA, Krischer, JP, Elterman, R, Aronin, PA, Pullen, J, Horowitz, ME, Parent, A, Martin, P & Kun, LE 1994, 'Desmoplastic infantile gangliogliomas: An approach to therapy', Neurosurgery, vol. 34, no. 4, pp. 583-589.
Duffner PK, Burger PC, Cohen ME, Sanford RA, Krischer JP, Elterman R et al. Desmoplastic infantile gangliogliomas: An approach to therapy. Neurosurgery. 1994 Jan 1;34(4):583-589.
Duffner, Patricia K. ; Burger, Peter C. ; Cohen, Michael E. ; Sanford, Robert A. ; Krischer, Jeffrey P. ; Elterman, Roy ; Aronin, Patricia A. ; Pullen, Jeanette ; Horowitz, Marc E. ; Parent, Andrew ; Martin, Paul ; Kun, Larry E. / Desmoplastic infantile gangliogliomas : An approach to therapy. In: Neurosurgery. 1994 ; Vol. 34, No. 4. pp. 583-589.
@article{df5329ba2c5047b7ba0210194728eee2,
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 12×9×9 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.",
keywords = "Brain tumor, Cerebral neoplasm, Ganglioglioma, Infant brain tumors",
author = "Duffner, {Patricia K.} and Burger, {Peter C.} and Cohen, {Michael E.} and Sanford, {Robert A.} and Krischer, {Jeffrey P.} and Roy Elterman and Aronin, {Patricia A.} and Jeanette Pullen and Horowitz, {Marc E.} and Andrew Parent and Paul Martin and Kun, {Larry E.}",
year = "1994",
month = "1",
day = "1",
language = "English (US)",
volume = "34",
pages = "583--589",
journal = "Neurosurgery",
issn = "0148-396X",
publisher = "Lippincott Williams and Wilkins Ltd.",
number = "4",

}

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 - 1994/1/1

Y1 - 1994/1/1

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

M3 - Article

VL - 34

SP - 583

EP - 589

JO - Neurosurgery

JF - Neurosurgery

SN - 0148-396X

IS - 4

ER -