Ganglioglioma in a Survivor of Infantile Glioblastoma.

Congenital tumors account for 2% to 4% of all pediatric central nervous system tumors. Glioblastoma multiforme (GBM) represents a small subset of these tumors. Despite harboring histologic features similar to older patients, infants with GBM exhibit improved survival and respond more favorably to surgery and chemotherapy. To highlight this tumor's unique behavior, we report the case of a survivor of infantile GBM who developed a recurrent tumor in the surgical bed 6 months after diagnosis. The tumor was ultimately resected and was a ganglioglioma. This case shows both a favorable clinical outcome to an infantile GBM and this tumor's natural history.

Congenital glioneuronal tumor with neuropil-like islands.

The glioneuronal tumor with neuropil-islands is considered a rare variant of an astrocytoma. Congenital cases of glioneuronal tumor with neuropil-islands, which typically arise in adults, have not been reported to our knowledge. We report an autopsy case of an in-utero demise of a 38-week-gestation female fetus in a 29-year-old female. At autopsy, a previously detected supratentorial tectal mass (by fetal MRI) was identified. Histology showed a biphasic neoplasm marked by island of gray matter-like parenchyma rimmed by mature-appearing neuronal cells intermixed with variably cellular areas resembling a low grade astrocytoma. Focally, the tumor was noted to involve the overlying meninges. The neuronal cell components were highlighted with synaptophysin and neuN antibodies and the glioma areas stained with glial fibrillary acidic protein antibody. The tumor did not stain with isocitrate dehydrogenase 1 (R132H) antibody and had a low Ki-67 labeling index (1.4%), in keeping with a low grade tumor. The pathologic findings were interpreted as representing a low grade glioneuronal tumor with neuropil-like islands. There have been few reports of this tumor arising in children, with most of those developing in the spinal cord. To our knowledge, this is the first reported congenital case of this tumor described in the literature.

Low-grade recurrence of a congenital high-grade supratentorial tumor with astrocytic features in the absence of adjuvant therapy.

The biological behavior of pediatric gliomas and embryonal tumors can be highly variable. A few case reports have described differentiation of primitive neuroectodermal tumors (PNETs) and medulloblastomas, presumably induced by adjuvant chemotherapy and/or radiation. Herein we describe a case of a congenital supratentorial high-grade tumor with astrocytic features that, after near-total surgical resection, was not treated with adjuvant therapies. Thirteen years later the patient presented with recurrent tumor at the original surgical site. The recurrent tumor had completely different morphology compared to the original, with evidence of ganglion cell differentiation and changes more reminiscent of a low-grade pleomorphic xanthoastrocytoma. To the authors' knowledge, this is the first documented case of an untreated high-grade pediatric tumor that spontaneously differentiated into a low grade tumor. The clinical and biological implications of this are briefly discussed.

Successful chemotherapy for congenital malignant gliomas: a report of two cases.

We describe the cases of 2 patients with a congenital malignant glioma that responded to chemotherapy. In the first case, a 2-month-old boy had a conjugate deviation to the right side and nystagmus. A T(1)-weighted gadolinium-enhanced magnetic resonance image showed a large tumor in his right frontal lobe. The tumor was partially resected, and the histological diagnosis was malignant ganglioglioma. The child then underwent 6 cycles of chemotherapy (mainly carboplatin and etoposide), and the residual tumor shrank. The tumor was then partially resected during a second operation, after which the patient underwent 5 cycles of chemotherapy (a combination of carboplatin, etoposide, vincristine, ifosfamide, cisplatin and cyclophosphamide). The tumor has not recurred in more than 8.5 years. In the second case, a 2-month-old boy had bulging of the anterior fontanel. The T(1)-weighted gadolinium-enhanced magnetic resonance image showed a large suprasellar tumor. The tumor was partially resected, and the histological diagnosis was anaplastic astrocytoma. The patient underwent 8 cycles of chemotherapy (MCNU, carboplatin and etoposide) and the tumor has not recurred in more than 7.5 years. Our experience indicates that, if surgical removal and chemotherapy are done aggressively for malignant gliomas in neonates and infants, long-term survival is possible.

Congenital ganglioglioma.

Congenital neoplasms of brain presenting at birth are extremely uncommon. We report a case of congenital ganglioglioma presenting at birth with hydrocephalus. Ventriculoperitoneal shunt and surgical debulking of the tumour along with histopathological confirmation were done at 6 months of age. On follow-up at 18 months, the child's hydrocephalus is static and his assessed developmental age is 10-12 months.

Congenital ganglioglioma: report of a case with an unusual imaging appearance.

A newborn presented with a cerebral ganglioglioma. This is a rare diagnosis in a neonate. The imaging appearance (hyperdense on CT, hyperintense on T1-weighted and hypointense on T2-weighted MRI) is very unusual for this type of tumor.