Abstract
Introduction
Desmoplastic infantile gangliogliomas (DIG) are rare cerebral glioneural tumors usually occurring in early childhood. DIGs are generally benign although rare cases with poor outcome are known. Total resection, if possible, is the treatment of choice, without further adjuvant therapy. After incomplete resection, adjuvant chemo-and/or radiotherapy is generally applied, despite the potential negative side effects in such young patients.
Case reports
We describe two girls with DIG, one who twice underwent subtotal resection at 3 and 5 months, the other who underwent total resection at 2 years. Neither had adjuvant therapy and there was no tumor recurrence.
Conclusions
Our own experience and a review of the literature suggest that in most DIGs adjuvant therapy is not justified even after incomplete resection. After tumor recurrence a second surgical intervention should be considered instead of adjuvant therapy. An exception may be made for rare, deep-seated DIGs, which are more aggressive and have a poorer outcome.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G, Sciot R (2002) Desmoplastic infantile ganglioglioma: a potentially malignant tumor? Am J Surg Pathol 26:1515–1522
Duffner PK, Burger PC, Cohen ME, Sanford RA, Krischer JP, Elterman R, Aronin PA, Pullen J, Horowitz ME, Parent A, Martin P (1994) Desmoplastic infantile ganglioglioma: an approach to therapy. Neurosurgery 34:583–589
Duffner PK, Krischer JP, Horowitz ME, Cohen ME, Burger PC, Friedman HS, Kun LE (1998) Second malignancies in young children with primary brain tumors following treatment with prolonged postoperative chemotherapy and delayed irradiation: a Pediatric Oncology Group Study. Ann Neurol 44:313–316
Fan X, Larson TC, Jennings MT, Tulipan NB, Ton SA, Johnson MD (2001) December 2000: 6 month old boy with 2 week history of progressive lethargy. Brain Pathol 11:265–266
Galatioto S, Gullotta F (1996) Desmoplastic non-infantile ganglioglioma. J Neurosurg Sci 40:235–238
Komori T, Scheithauer BW, Parisi JE, Watterson J, Priest JR (2001) Mixed conventional and desmoplastic infantile ganglioglioma: an autopsied case with 6-year follow up. Mod Pathol 14:720–726
Kros JM, Delwel EJ, de Jong TH, Tanghe HL, van Run PR, Vissers K, Alers JC (2002) Desmoplastic infantile astrocytoma and ganglioglioma: a search for genomic characteristics. Acta Neuropathol (Berl) 104:144–148
Kuchelmeister K, Steinhauser A, Korf B, Wagner D, Prey N, Schachenmayr W (1996) Anaplastic desmoplastic infantile ganglioglioma. Clin Neuropathol 15:280
Kuchelmeister K, Schonmeyr R, Albani M, Schachenmayr W (1998) Anaplastic desmoplastic infantile ganglioglioma. Clin Neuropathol 17:269–270
Louis DN, von Deimling A, Dickersin GR, Dooling EC, Seizinger BR (1992) Desmoplastic cerebral astrocytomas of infancy: a histopathologic, immunohistochemical, ultrastructural, and molecular genetic study. Human Pathol 23:1402–1409
Mallucci C, Lellouch A, Salazar C, Cinalli G, Renier D, Sainte-Rose C, Kahn A, Zerah M (2000) The management of desmoplastic neuroepithelial tumors in childhood. Childs Nerv Syst 16:8–14
Marti A, Almostarchid B, Maher M, Saidi A (2000) Desmoplastic non-infantile ganglioglioma. Case report. J Neurosurg Sci 44:150–154
Parisi JE, Scheithauer BW, Priest JR, Okazaki H, Komori T (1992) Desmoplastic infantile ganglioglioma (DIG): a form of ganglio-gliomatosis. J Neuropathol Exp Neurol 51:365
Rout P, Santosh V, Mahadevan A, Kolluri VR, Yasha TC, Shankar SK (2002) Desmoplastic infantile ganglioglioma—clinicopathological and immunohistochemical study of four cases. Childs Nerv Syst 18:463–467
Setty SN, Miller DC, Camras L, Charbel F, Schmidt ML (1997) Desmoplastic infantile astrocytoma with metastases at presentation. Mod Pathol 10:945–951
Taratuto AL, Monges J, Lylyk P, Leiguarda R (1984) Superficial cerebral astrocytoma attached to dura. Report of six cases in infants. Cancer 54:2505–2512
Taratuto AL, VandenBerg SR, Rorke LB (2000) Desmoplastic infantile astrocytoma and ganglioglioma. In: Kleihues P, Cavanee WK (eds) Pathology and genetics of tumours of the nervous system. IARC, Lyon, pp 99–102
VandenBerg SR (1993) Desmoplastic infantile ganglioglioma and cerebral astrocytoma of infancy. Brain Pathol 3:275–281
VandenBerg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, Collins VP, Park TS (1987) Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential ("desmoplastic infantile gangliogliomas"). Report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 66:58–71
Woesler B, Kuwert T, Kurelmann G, Morgenroth C, Probst-Cousin S, Lerch H, Gulotta F, Wassmann H, Schober O (1998) High amino acid uptake in a low grade desmoplastic infantile ganglioglioma in a 14-year-old patient. Neurosurg Rev 21:31–35
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Bächli, H., Avoledo, P., Gratzl, O. et al. Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview. Childs Nerv Syst 19, 359–366 (2003). https://doi.org/10.1007/s00381-003-0754-9
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-003-0754-9