Ganglioglioneurocytoma of the Posterior Fossa

Abstract

Objective and Importance: Ganglioglioneurocytoma is not yet a well defined clinical and histopathological entity; recent reports outline the histopathological features of this very rare trimorphous tumor, under different names with its basic components of gangliocytoma, neurocytoma and glioma. Four previous reports described this tumor in eleven patients. This is the first case report describing ganglioglioneurocytoma in the posterior fossa. Clinical Presentation and Intervention: A 31-year old male with midline posterior fossa ganglioglioneurocytoma is described, providing the neuroimages and histopathological studies. Conclusion: Ganglioglioneurocytoma is a rare mixed neuronal and glial tumor that can also occur in the posterior fossa. Can. J. Neurol. Sci. 1999; 26: 207-210 Mixed cell tumors in the CNS containing both glial and neuronal elements are rare. Gangliogliomas account for about 1 % of all brain neoplasms, which usually present with seizures in children. They have been reported in all levels of the neuroaxis, but were more common in the temporal lobe. The tumor contains a glial component usually astrocytic of variable cellularity and a ganglionic component of large often binucleated neurons scattered or in clusters that immunostain with synaptophysin and chromogranin. These tumors usually have a good prognosis following surgical resection.' Central neurocytomas are rare tumors of the CNS, classically affect young adults, and arise in the lateral ventricles near the foramen of Monro. However, they have been reported away from the ventricles, 2 in the cerebellum 3 and in the spinal cord. 4 The tumor consists of neurocytes; small round cells with perinuclear halo formation and stain with synaptophysin. Under the electron microscope, neurosecretory granules in the perinuclear cytoplasm and neurites are characteristic. This neoplasm is usually curable with total resection whenever possible. Glioneurocytomas are another mixed tumor with glial and neurocytic components. They have been reported with similar clinical and pathological findings. 6 Since then, a total of eleven cases were reported describing tumors with such a morphology, usually associated with glial component of well differentiated astrocytes. In this paper we describe the 12th case, which to our knowledge is the first to be reported arising in the posterior fossa. CASE REPORTA 25-year-old previously healthy male presented with a one year history of progressive headache and blurring of vision. Physical exam showed papilledema and ataxic gait but normal cranial nerve function. CT scan images revealed a midline posterior fossa mass at the level of the fourth ventricle with a large dorsal cystic component and a smaller solid ventral component with calcification. The solid mass partially enhanced with IV contrast. It seemed to originate from the cerebellum and compressed the fourth ventricle causing obstructive hydrocephalus (Figure 1). The patient underwent surgery for gross total resection and had an uneventful recovery. Postoperativ...

Ganglioneuroma Originating From the Trigeminal Nerve in the Middle Cranial Fossa

“…2,[6][7][8]10) Ganglioneuroma is another tumor entity derived from ganglion cells of the peripheral nerve. The term`g anglioneuroma'' has been used incorrectly as a synonym for gangliocytoma.…”

Skull Base Neurocytoma: Case Report and Review of the Literature of Extraventricular Neurocytomas

“…Subsequently, several additional cases have been published. 6,16,[23][24][25] Because these extremely rare tumors contain all three cellular components, some have speculated that ganglioglioneurocytomas may represent the differentiation of neurocytoma. 25 Ganglioglioneurocytomas are characterized by the presence of large mature binucleated neurons that stain with synaptophysin and chromogranin A, small round cells with perinuclear halos that stain with synaptophysin, and the proliferation of pleomorphic astrocytes.…”

“…25 Ganglioglioneurocytomas are characterized by the presence of large mature binucleated neurons that stain with synaptophysin and chromogranin A, small round cells with perinuclear halos that stain with synaptophysin, and the proliferation of pleomorphic astrocytes. 23 Prognosis seems to follow that of neuronal rather than glial neoplasms, with good outcomes following complete surgical resection. However, others argue that ganglioglioneurocytomas are differentiated from neuroblastomas or medulloblastomas.…”

“…However, others argue that ganglioglioneurocytomas are differentiated from neuroblastomas or medulloblastomas. 23…”