Neurology India, Vol. 52, No. 3, July-September, 2004, pp. 384-386
Desmoplastic infantile ganglioglioma - A case report
Balasubramanian D, Ramesh VG, Deiveegan K, Ghosh Mitra, Mallikarjuna VS, Annapoorneswari TP, Chidambaranathan N, Ramani KVN
Departments of Neurosurgery, Apollo Hospitals, Tondiarpet, Chennai
Correspondence Address:350/4, Lloyds Road, Gopalapuram, Chennai - 600 086
Code Number: ni04125
ABSTRACTDesmoplastic infantile ganglioglioma is a very rare supratentorial tumor occurring in the first two years of life. A five-month-old female infant presented with recurrent seizures, large head and loss of acquired milestones. Computerized Tomographic Scan of brain showed a large subarachnoid cyst with a solid intensely contrast enhancing tumor in the right temporoparietal region with severe degree of mass effect. Craniotomy and total excision of the tumor followed subsequently by subduro-peritoneal shunt for the extracerebral fluid collection was done. The child made good recovery. Histopathology revealed features of desmoplastic infantile ganglioglioma, viz., marked desmoplastic component with glial and neuronal elements. Immunohistochemistry showed positive staining for glial fibrillary acidic protein (GFAP) with areas of synaptophysin and chromogranin positivity. Desmoplastic infantile ganglioglioma is a rare tumor of infancy, which has excellent prognosis after total excision. No adjuvant therapy is required. This is the first Indian report of desmoplastic infantile ganglioglioma out of less than fifty cases reported worldwide.
KEY WORDS: Desmoplastic infantile ganglioglioma, supratentorial neuroepithelial tumors of infancy, gangliogliomas
Desmoplastic infantile ganglioglioma was first described by Vanden Berg et al in 1987. Together with superficial astrocytoma, they are also grouped as desmoplastic supratentorial neuroepithelial tumors of infancy. Desmoplastic infantile gangliogliomas are extremely rare and less than 50 cases have been reported in the literature. A 5-month-old infant with right temporoparietal desmoplastic infantile ganglioglioma which was successfully managed is presented in this report and the relevant literature has been reviewed.
A 5-month-old Bengali female child presented with enlargement of head, repeated generalized tonic-clonic seizures and loss of acquired milestones of one-month duration. At the time of admission, the child was irritable. The head circumference was 51 cm. And the anterior fontanelle was wide and tense. There was no neurological deficit. Computerized Tomographic scan (CT scan) of brain showed a large cystic tumor in the right frontotemporoparietal regions, with a moderate-sized, isodense and intensely enhancing surface mass [Figure - 1] and [Figure - 2].
A right frontotemporoparietal craniotomy was performed. The subarachnoid cyst contained xanthochromic fluid. The solid tumor was approximated to the dura. It was firm and relatively avascular and measured about 6 cm x 5 cm x 3 cm. The tumor was excised completely. The postoperative course was uneventful.
Histopathology showed the tumor with marked desmoplastic component with neoplastic astrocytes and a few scattered neuronal ele ments [Figure - 3]. Immunohistochemistry showed strong reaction with Glial Fibrillary Acidic Protein (GFAP) with areas showing posi tive reaction for Synaptophysin and Chromogranin. The picture was consistent with desmoplastic infantile ganglioglioma.
Desmoplastic supratentorial neuroepithelial tumors of infancy form a rare but distinctive group of tumors occurring in children less than 2 years of age. Vanden Berg et al (1987), named these tumors as desmoplastic cerebral astrocytoma of infancy and desmoplastic infantile ganglioglioma (DIG), on the basis of immunohistochemistry and electron microscopy. Both these groups of tumors occur in children less than 2 years of age. They have identical clinical and radiological features and both have favorable outcome following successful excision. Subsequently, these groups of tumors have been included as a distinctive group, in the revised World Health Organization (WHO) classification of brain tumors (Kleihues et al1993).
Less than 50 cases of desmoplastic infantile ganglioglioma (DIG) have been reported in the literature. Several authors have reported single case reports and small series of cases of DIG., The youngest patient has been 4 months of age. All these children presented with macrocephaly, seizures, psychomotor delay, either in isolation or in combination. All these patients have similar Computerized Tomographic scan (CT) and Magnetic Resonance Imaging (MRI) characteristics (Martin et al 1991, Tenreiro-Picon et al, 1995). The tumors commonly occur in frontal or parietal lobes, with a solid component closely attached to the dura, which intensely enhances with contrast, surrounded by a parenchymal cystic component and mass effect. Most of these tumors are amenable to surgical excision because of their surface location and distinct firmness with clear demarcation from the surrounding normal brain. No adjuvant therapy is recommended after total excision, despite the fact that the lesion has a low malignant potential. Long-term prognosis after total surgical excision has been excellent in all the reported series.
Histologically, these tumors have intense desmoplasia, with neoplastic astrocytes with occasional neuronal elements. Desmoplastic cerebral astrocytoma of infancy and DIG are difficult to differentiate under light microscopy. Immunohistochemistry plays a very important role in the diagnosis of DIG. In DIG, the tumor shows intense positivity for Glial Fibrillary Acidic Protein (GFAP) which is a glial marker, with areas positive for synaptophysin or neurofilament immunostain, which are neuronal markers. In desmoplastic,, cerebral astrocytoma, there is positivity for GFAP only.
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