Novel Insights from Clinical Practice Pediatr Neurosurg 2013;49:33–37 DOI: 10.1159/000353992
Received: February 7, 2013 Accepted after Revision: June 24, 2013 Published online: October 29, 2013
Hemorrhagic Ganglioglioma of the Posterior Fossa: Case Report Dhruve S. Jeevan Jayson A. Neil Avinash Mohan Michael Tobias Division of Pediatric Neurosurgery, Department of Neurological Surgery, Maria Fareri Children’s Hospital at Westchester Medical Center, New York Medical College, Valhalla, N.Y., USA
Established Facts • Gangliogliomas are tumors of mixed glial cells (astrocytes and oligodendrocytes) together with mature ganglion-type neurons, generally occurring in the supratentorial compartment. Infratentorial gangliogliomas are rare. • Infratentorial gangliogliomas can present in a variety of ways depending on the structures involved. Cranial nerve deficits (e.g. hearing loss, intractable facial pain and hemifacial seizures), hemiparises, cerebellar signs, gait disturbance and headache have all been described.
Novel Insights • Hemorrhagic presentation of infratentorial gangliogliomas is extremely rare, but when it does occur, it can have devastating consequences in an otherwise benign disease. • Gangliogliomas are relatively avascular and are rarely necrotic. However, the enhancement pattern of the ganglioglioma may mimic a vascular malformation and, in the presence of hemorrhage, other vascular tumors and arteriovenous malformations should be included in the differential requiring catheter angiography.
Key Words Infratentorial ganglioglioma · Posterior fossa · Hemorrhage
Abstract Gangliogliomas are rare tumors of the central nervous system that are usually found in the supratentorial compartment, although cases throughout the nervous system have
© 2013 S. Karger AG, Basel 1016–2291/13/0491–0033$38.00/0 E-Mail [email protected] www.karger.com/pne
been described. They are generally low-grade malignancies that are amenable to cure by surgical resection. Most manifest as seizures, though, based on location, they can present with focal neurological deficits. We present here a rare case of an infratentorial ganglioglioma presenting with hemorrhage. To our knowledge this is the only reported case of a hemorrhagic ganglioglioma and, as such, we examine its possible prognosis. © 2013 S. Karger AG, Basel
Dhruve S. Jeevan, BMBCH Department of Neurosurgery, New York Medical College Mungar Pavillion, Room 329 Valhalla, N.Y. 10595 (USA) E-Mail dhruve.jeevan @ me.com
Introduction
Gangliogliomas show a mixed proliferation of glial cells (astrocytes and oligodendrocytes) together with mature ganglion-type neurons, generally occurring in the supratentorial compartment. They are normally benign tumors in which complete excision, when possible, constitutes the optimal treatment leading to disease-free survival. Infratentorial gangliogliomas are rare, with only 30 cerebellar gangliogliomas having been reported in the literature [1–26]. We report on a rare case of cerebellar ganglioglioma presenting acutely with hemorrhage. This is, to the best of our knowledge, the first reported case of hemorrhagic ganglioglioma of the posterior fossa. This case adds to the growing knowledge about this entity, and leads us to question the clinical grading of such lesions.
Postoperative Course The postoperative course was uneventful. Immediate postoperative MR imaging did show a small enhancing nodule in the left cerebral hemisphere, which was subsequently resected at 6 months. Pathology confirmed the previous diagnosis of WHO grade I ganglioglioma. At present, 1 year after initial presentation, the patient remains tumor-free with no residual deficits.
Discussion
Case Report History and Findings A 9-year-old female with a history of intermittent headaches over 2–3 weeks presented to our institution acutely with severe headache, nausea and vomiting, unresponsive to analgesics. On arrival in the emergency room, the patient had become progressively lethargic, only opening her eyes to stimulation, and her speech was limited to 1–2 words when questioned. No cranial nerve deficits, nystagmus or limb ataxia were noted from our limited examination, and it was established that there was no family history of gait disturbance. A cranial computerized tomography (CT) was performed which demonstrated a midline posterior fossa hemorrhage with extension into the fourth and third ventricles with surrounding vasogenic edema, obstructive hydrocephalus and effacement of sulci (fig. 1). A CT angiogram completed at the same time did not show any evidence of underlying arteriovenous malformation or aneurysm. Calcification was seen along the left lateral aspect of the vermian hemorrhage, raising the possibility of an underlying lesion. Due to her deteriorating neurological condition, the patient was intubated and an external ventricular drain was placed. Subsequently, a brain magnetic resonance (MR) imaging study was also obtained revealing an enhancing cerebellar lesion with hemorrhagic material (fig. 2). Operation and Pathology The patient subsequently underwent a median suboccipital craniotomy and decompression of hemorrhagic mass with resection of the lesion. The tumor appeared as a hemorrhagic, firm, grayish vascular mass and had a mixed soft and hard consistency. Neuropathological examination revealed the presence of a tumor with large focus of central hemorrhage walled in by tumor tissue with the presence of ganglion-type neurons in a mixed glial background, WHO grade I (fig. 3). The astrocytic background was found to express glial fibrillary acid protein (GFAP) within the cytoplasm and fibrillary processes, while a group of telangiec-
34
tatic vessels along the periphery, resembling an angioma, bordered the central hemorrhagic zone. The cells showed biphenotypic differentiation with several cells expressing glial fibrillary acid protein within the cytoplasm and fibrillary processes, while scattered large uni- or binucleate ganglionic cells with eccentric nuclei and prominent nucleoli that were immunoreactive of synaptophysin and chromogranin were seen. These ganglionic cells showed dystrophic intracytoplasmic accumulation of phosporylated neurofilament. The features were characteristic of a ganglioglioma with neovascularization causing intratumoral hemorrhage. Pathology was confirmed with outside consultation by two neuropathologists.
Pediatr Neurosurg 2013;49:33–37 DOI: 10.1159/000353992
Gangliogliomas of the central nervous system are uncommon and form only 0.4–1.3% of all intracranial neoplasms [26]. Typically, they are supratentorial compartment lesions, with the majority occurring in the temporal lobe, although cases throughout the central nervous system have been described [27]. Peak incidence is between 10 and 20 years of age [28]. Infratentorial gangliogliomas present in a variety of ways depending on the structures involved; cranial nerve deficits (e.g. hearing loss, intractable facial pain and hemifacial seizures), hemiparises, cerebellar signs, gait disturbance and headache have all been described [2, 5, 12, 29– 31]. Interestingly, epilepsy of cerebellar origin has also been reported in patients with cerebellar gangliogliomas [3, 12, 18–20]. In general, cerebellar gangliogliomas are rare, with only 30 cases being reported in the literature [1–26, 31–34], and they usually present with a short history (mean 1.6 years) [35]. To our knowledge, this is the first documented case of a cerebellar ganglioglioma presenting with hemorrhage. Typically, gangliogliomas are thought to be of lowgrade malignancy, grades I–II according to WHO classification. However, anaplastic gangliogliomas have been previously described [36], and are typically due to dedifferentiation of the glial component. It is estimated that
Received: February 7, 2013 Accepted after Revision: June 24, 2013 Published online: October 29, 2013
Hemorrhagic Ganglioglioma of the Posterior Fossa: Case Report Dhruve S. Jeevan Jayson A. Neil Avinash Mohan Michael Tobias Division of Pediatric Neurosurgery, Department of Neurological Surgery, Maria Fareri Children’s Hospital at Westchester Medical Center, New York Medical College, Valhalla, N.Y., USA
Established Facts • Gangliogliomas are tumors of mixed glial cells (astrocytes and oligodendrocytes) together with mature ganglion-type neurons, generally occurring in the supratentorial compartment. Infratentorial gangliogliomas are rare. • Infratentorial gangliogliomas can present in a variety of ways depending on the structures involved. Cranial nerve deficits (e.g. hearing loss, intractable facial pain and hemifacial seizures), hemiparises, cerebellar signs, gait disturbance and headache have all been described.
Novel Insights • Hemorrhagic presentation of infratentorial gangliogliomas is extremely rare, but when it does occur, it can have devastating consequences in an otherwise benign disease. • Gangliogliomas are relatively avascular and are rarely necrotic. However, the enhancement pattern of the ganglioglioma may mimic a vascular malformation and, in the presence of hemorrhage, other vascular tumors and arteriovenous malformations should be included in the differential requiring catheter angiography.
Key Words Infratentorial ganglioglioma · Posterior fossa · Hemorrhage
Abstract Gangliogliomas are rare tumors of the central nervous system that are usually found in the supratentorial compartment, although cases throughout the nervous system have
© 2013 S. Karger AG, Basel 1016–2291/13/0491–0033$38.00/0 E-Mail [email protected] www.karger.com/pne
been described. They are generally low-grade malignancies that are amenable to cure by surgical resection. Most manifest as seizures, though, based on location, they can present with focal neurological deficits. We present here a rare case of an infratentorial ganglioglioma presenting with hemorrhage. To our knowledge this is the only reported case of a hemorrhagic ganglioglioma and, as such, we examine its possible prognosis. © 2013 S. Karger AG, Basel
Dhruve S. Jeevan, BMBCH Department of Neurosurgery, New York Medical College Mungar Pavillion, Room 329 Valhalla, N.Y. 10595 (USA) E-Mail dhruve.jeevan @ me.com
Introduction
Gangliogliomas show a mixed proliferation of glial cells (astrocytes and oligodendrocytes) together with mature ganglion-type neurons, generally occurring in the supratentorial compartment. They are normally benign tumors in which complete excision, when possible, constitutes the optimal treatment leading to disease-free survival. Infratentorial gangliogliomas are rare, with only 30 cerebellar gangliogliomas having been reported in the literature [1–26]. We report on a rare case of cerebellar ganglioglioma presenting acutely with hemorrhage. This is, to the best of our knowledge, the first reported case of hemorrhagic ganglioglioma of the posterior fossa. This case adds to the growing knowledge about this entity, and leads us to question the clinical grading of such lesions.
Postoperative Course The postoperative course was uneventful. Immediate postoperative MR imaging did show a small enhancing nodule in the left cerebral hemisphere, which was subsequently resected at 6 months. Pathology confirmed the previous diagnosis of WHO grade I ganglioglioma. At present, 1 year after initial presentation, the patient remains tumor-free with no residual deficits.
Discussion
Case Report History and Findings A 9-year-old female with a history of intermittent headaches over 2–3 weeks presented to our institution acutely with severe headache, nausea and vomiting, unresponsive to analgesics. On arrival in the emergency room, the patient had become progressively lethargic, only opening her eyes to stimulation, and her speech was limited to 1–2 words when questioned. No cranial nerve deficits, nystagmus or limb ataxia were noted from our limited examination, and it was established that there was no family history of gait disturbance. A cranial computerized tomography (CT) was performed which demonstrated a midline posterior fossa hemorrhage with extension into the fourth and third ventricles with surrounding vasogenic edema, obstructive hydrocephalus and effacement of sulci (fig. 1). A CT angiogram completed at the same time did not show any evidence of underlying arteriovenous malformation or aneurysm. Calcification was seen along the left lateral aspect of the vermian hemorrhage, raising the possibility of an underlying lesion. Due to her deteriorating neurological condition, the patient was intubated and an external ventricular drain was placed. Subsequently, a brain magnetic resonance (MR) imaging study was also obtained revealing an enhancing cerebellar lesion with hemorrhagic material (fig. 2). Operation and Pathology The patient subsequently underwent a median suboccipital craniotomy and decompression of hemorrhagic mass with resection of the lesion. The tumor appeared as a hemorrhagic, firm, grayish vascular mass and had a mixed soft and hard consistency. Neuropathological examination revealed the presence of a tumor with large focus of central hemorrhage walled in by tumor tissue with the presence of ganglion-type neurons in a mixed glial background, WHO grade I (fig. 3). The astrocytic background was found to express glial fibrillary acid protein (GFAP) within the cytoplasm and fibrillary processes, while a group of telangiec-
34
tatic vessels along the periphery, resembling an angioma, bordered the central hemorrhagic zone. The cells showed biphenotypic differentiation with several cells expressing glial fibrillary acid protein within the cytoplasm and fibrillary processes, while scattered large uni- or binucleate ganglionic cells with eccentric nuclei and prominent nucleoli that were immunoreactive of synaptophysin and chromogranin were seen. These ganglionic cells showed dystrophic intracytoplasmic accumulation of phosporylated neurofilament. The features were characteristic of a ganglioglioma with neovascularization causing intratumoral hemorrhage. Pathology was confirmed with outside consultation by two neuropathologists.
Pediatr Neurosurg 2013;49:33–37 DOI: 10.1159/000353992
Gangliogliomas of the central nervous system are uncommon and form only 0.4–1.3% of all intracranial neoplasms [26]. Typically, they are supratentorial compartment lesions, with the majority occurring in the temporal lobe, although cases throughout the central nervous system have been described [27]. Peak incidence is between 10 and 20 years of age [28]. Infratentorial gangliogliomas present in a variety of ways depending on the structures involved; cranial nerve deficits (e.g. hearing loss, intractable facial pain and hemifacial seizures), hemiparises, cerebellar signs, gait disturbance and headache have all been described [2, 5, 12, 29– 31]. Interestingly, epilepsy of cerebellar origin has also been reported in patients with cerebellar gangliogliomas [3, 12, 18–20]. In general, cerebellar gangliogliomas are rare, with only 30 cases being reported in the literature [1–26, 31–34], and they usually present with a short history (mean 1.6 years) [35]. To our knowledge, this is the first documented case of a cerebellar ganglioglioma presenting with hemorrhage. Typically, gangliogliomas are thought to be of lowgrade malignancy, grades I–II according to WHO classification. However, anaplastic gangliogliomas have been previously described [36], and are typically due to dedifferentiation of the glial component. It is estimated that
