|Year : 2007 | Volume
| Issue : 2 | Page : 69-71
Congenital glioblastoma multiforme: A case report and review of literature
Manish K Kasliwal1, Deepak K Gupta1, Ashok K Mahapatra1, Mehar C Sharma2
1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi - 110 029, India
2 Department of Neuropathology, All India Institute of Medical Sciences, New Delhi - 110 029, India
Deepak K Gupta
Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Congenital brain tumors are very rare neoplasms. The diagnosis of glioblastoma multiforme in a newborn infant is a rarity. Prognosis of congenital glioblastoma has been uniformly fatal with most of the cases reported in literature having a very short survival in spite of all modalities of treatment. The authors report a case of congenital glioblastoma involving the pan right cerebral hemisphere in a two month-old infant who expired six months after tumor decompression. Literature is reviewed in light of the present case and various treatment modalities for this rare tumor are highlighted.
Keywords: Congenital, glioblastoma, infancy, outcome, tumors
|How to cite this article:|
Kasliwal MK, Gupta DK, Mahapatra AK, Sharma MC. Congenital glioblastoma multiforme: A case report and review of literature. J Pediatr Neurosci 2007;2:69-71
Congenital glioblastoma is a malignant pediatric brain tumor with an comprises about 2-9% of all congenital brain tumors. , Very few cases of congenital glioblastoma multiforme (GBM) have been described as discussed in an extensive review of literature by the authors. ,,,,,,,,, The authors report a probably congenital glioblastoma as per the classification of Solitare and Krigman,  which was treated by surgical decompression. The young age of these patients, absence of a well-accepted protocol and the very nature of this tumor make the management very challenging with a poor outcome.
| Case Report|| |
A two month-old infant presented in emergency with progressive increase in head size for 4-5 days and decreased crying for one day. There was no significant family history or history of any other sibling suffering from similar complaints. The perinatal course was uneventful and there was probably no teratogenic exposure of the mother during the course of pregnancy. On examination, the child was found to be comatose with bilateral pupils 4 mm and not reacting to light. The anterior fontanalle was open and tense and there was left-sided hemiparesis on examination. Contrast-enhanced computed tomography (CT) showed a supratentorial hemispheric tumor with heterogenous contrast enhancement and hydrocephalus [Figure - 1] Magnetic resonance imaging (MRI) revealed a large solid cum cystic mass lesion [Figure - 2]. Right frontoparietal horseshoe osteoplastic flap craniotomy and tumor decompression were done. The child had massive blood loss intraoperatively; the hemostasis was difficult in the end and a part of the tumor extending to the basal ganglion was left behind. The child had an uneventful postoperative period and recovered well over the next three days; hence, was discharged on the 8 th postoperative day. Biopsy was compatible with the diagnosis of glioblastoma multiforme [Figure - 3]. Immunostaining was positive for vimentin while it was negative for epidermal growth factor receptor (EGFR). The patient improved neurologically with residual hemiparesis and was due for chemotherapy. The child however had gradual deterioration and finally expired after six months of tumor decompression.
| Discussion|| |
Congenital brain tumors are very uncommon tumors with an incidence of about 1.1 to 3.4 per million live births. , Their mode of presentation, location, histology, behavior, response to therapy and outcome differ from those of older children and adults. Teratomas are the most common of congenital brain tumors with glioblastoma accounting for only 2-9% of all congenital brain tumors. ,,, In fact, a large series of infant brain tumors studied by Brown et al . reported only one case of Glioblastoma.  Glioblastomas can be divided into three categories as per Solitare and Krigman: i) definitely congenital-presenting or producing symptoms at birth, ii) probably congenital-presenting or producing symptoms within the first week of life or iii) possibly congenital-present or producing symptoms within the first few months of life.  Our case belonged to the probably congenital category, having been diagnosed at two months of age. However, considering the giant size of the tumor, the likelihood of it being present right from birth can't be ruled out as the patient belonged to a poor socioeconomic stratum of society and the mother didn't have any fetal ultrasonography in utero or any other imaging antenatally. Most of the cases of congenital GBM including this case occur supratentorialy as opposed to the infratentorial location of a majority of pediatric brain tumors. ,,,,, Symptoms and signs of raised intracranial pressure form the main presentation. However, due to the ability of the infant's skull to expand and compensate for the rising intracranial pressure and the inability of these infants to complain about specific symptoms, the diagnosis is delayed to some extent giving the tumor adequate time to expand and grow to a large size, making surgical resection almost impossible. 
The prognosis for all these children has been uniformly grim with most of the cases reported in literature dying within two months ,,,,,, and only a few cases surviving for more than two years. , Fetal ultrasonography and fetal MRI had been used to diagnose some of these cases in utero and congenital glioblastoma has been diagnosed from a gestational age of 28 weeks to term. Although congenital glioblastoma can have varied appearance on prenatal imaging and histopathology is the gold standard for diagnosis of this tumor, recognition of some characteristic findings can help in antenatal diagnosis of this tumor. ,,,,,,, This should aid in the appropriate counseling of parents regarding the outcome of the pregnancy and prognosis of the fetus. The prognosis of GBM has improved with the availability of newer chemotherapeutic agents like temezolamide. Currently, aggressive surgical resection with adjuvant concomitant chemoradiation forms the treatment modality of choice for GBM. However, younger age at diagnosis prevents the use of postoperative radiotherapy in congenital GBM, further complicating and affecting the management adversely.
To conclude, inspite of an improvement in prenatal diagnosis, modern imaging facilities and appropriate pre- and postoperative management, the prognosis of congenital GBM is extremely poor. Although rare, congenital brain tumors, especially GBM, can have significant medical, social and emotional effects and a holistic approach addressing this issues should be followed in the management of this tumor.
| References|| |
|1.||Lee Dy, Kim YM, Yoo SJ, Cho BK, Chi JG, Kim IO, et al . Congenital glioblastoma diagnosed by fetal ultrasonography. Childs Nerv Syst 1999;15:197-201. [PUBMED] [FULLTEXT]|
|2.||Nakayama K, Nakamura T. Localization of congenital glioblastomas in the Japanese: A case report and review of the literature. Childs Nerv Syst 2002;18:149-52. |
|3.||Chuang YM, Guo WY, Ho DM, Wong TT, Hung JH, Chen SJ, et al . Skew ocular deviation: A catastrophic sign on MRI of fetal glioblastoma. Childs Nerv Syst 2003;19:371-5. [PUBMED] [FULLTEXT]|
|4.||Heckel S, Favre R, Gasser B, Christmann D. Prenatal diagnosis of a congenital astrocytoma: A case report and literature review. Ultrasound Obstet Gynecol 1995;5:63-6. [PUBMED] [FULLTEXT]|
|5.||Mazzone D, Magro G, Lucenti A, Grasso S. Report of a case of congenital glioblastoma multiforme: An immunohistochemical study. Childs Nerv Syst 1995;11:311-3. [PUBMED] |
|6.||McConachie NS, Twining P, Lamb MP. Case report: Antenatal diagnosis of congenital glioblastoma. Clin Radiol 1991;44:121-2. [PUBMED] |
|7.||Morof DF, Levine D, Stringer KF, Grable I, Folkerth R. Congenital glioblastoma multiforme: Prenatal diagnosis on the basis of sonography and magnetic resonance imaging. J Ultrasound Med 2001;20:1369-75. [PUBMED] [FULLTEXT]|
|8.||Riboni G, De Simoni M, Leopardi O, Molla R. Ultrasound appearance of a glioblastoma in a 33 week fetus in utero. J Clin Ultrasound 1985;13:345-6. [PUBMED] |
|9.||Shimamura N, Asano K, Ogane K, Yagihashi A, Ohkuma H, Suzuki S. A case of definitely congenital glioblastoma manifested by intratumoral hemorrhage. Childs Nerv Syst 2003;19:778-81. [PUBMED] [FULLTEXT]|
|10.||Winters JL, Wilson D, Davis DG. Congenital Glioblastoma multiforme: A report of three cases and a review of the literature. J Neurol Sci 2001;188:13-9. [PUBMED] [FULLTEXT]|
|11.||Solitare GB, Krigman MR. Congenital intracraial neoplasm: A case report and review of the literature. J Neuropathol Exp Neurol 1964;23:280-92. [PUBMED] |
|12.||Wakai S, Arai T, Nagai M. Congenital brain tumors. Surg Neurol 1984;21:597-609. [PUBMED] |
|13.||Brown K, Mapstone TB, Oakes WJ. A modern analysis of intracranial tumors of infancy. Pediatr Neurosurg 1997;26:25-32. [PUBMED] |
|14.||Rivera-Luna R, Medina-Sanson A, Leal-Leal C, Pantoja-Guillen F, Zapata-Tarrιs M, Cardenas-Cardos R, et al . Brain tumors in children under 1 year of age: Emphasis on the relationship of prognostic factors. Childs Nerv Syst 2003;19:311-4. |
|15.||Buetow PC, Smirniotopoulos, Done S. Congenital brain tumors: A review of 45 cases. AJNR Am J Neuroradiol 1990;11:793-9. |
[Figure - 1], [Figure - 2], [Figure - 3]