Ganglioglioma and migraine headache Amin F. Saad, MD, Kennith F. Layton, MD, MS, S. Sam Finn, MD, and Michael J. Opatowsky, MD, MBA
Mixed neuronal/glial neoplasms represent a minority of intracranial neoplasms, typically associated with a more favorable prognosis than the more common higher-grade glial neoplasms. We describe a young man with headache, confusion, and slurred speech who was found to have a ganglioglioma.
CASE REPORT A 19-year-old man with a history of chronic migraine headaches beginning in his early teens experienced an acute sudden onset of severe headache without provocation. Until this point the patient’s headaches lacked atypical symptoms and responded well to over-the-counter medication, and thus he had not undergone previous imaging evaluation. His family noticed that he was confused and had slurred speech, a marked change in the typical character of his headaches. Upon presentation to the emergency department, a noncontrast brain computed tomography (CT) scan (Figure 1) revealed a 6.9 × 3.2 cm isodense nonhemorrhagic mass centered in the left occipital lobe with scattered coarse calcific deposits, surrounding vasogenic edema, and local mass effect but no midline shift or hydrocephalus. Subsequent magnetic resonance imaging (MRI) of the brain further characterized the extent of the mass and revealed conspicuous cystic components (Figure 2a). Mild heterogeneous enhancement was shown on the postgadolinium T1-weighted sequences (Figure 2b). On the MRI, calcific deposits appeared as hypointense foci on the susceptibility weighted sequence (Figure 2c). The patient underwent a craniotomy and the mass was resected. Histologic examination revealed findings compatible with a World Health Organization grade 1 ganglioglioma. The patient did well postoperatively without focal neurologic deficit and will follow up for routine surveillance imaging. DISCUSSION Gangliogliomas are an uncommon lesion, accounting for 0.4% to 0.9% of all intracranial neoplasms and 1% to 4% of pediatric central nervous system neoplasms (1). Despite being uncommon, they are the most common mixed neuronal-glial neoplasm (2). Gangliogliomas have a peak incidence in patients 10 to 20 years old, with 80% being found in individuals younger than 30 years. In a case series of 99 proven gangliogliomas, 38% occurred in the Proc (Bayl Univ Med Cent) 2014;27(3):215–216
Figure 1. Axial noncontrast CT images demonstrate a left parieto-occipital mass with coarse calcific deposits (arrowhead) and cystic areas (arrows).
temporal lobe, 30% in the parietal lobe, and 18% in the frontal lobe (3). Provenzale et al presented a series of 25 gangliogliomas, including two occipital lobe lesions (8%). Gangliogliomas are the most common neoplastic etiology (40%) for chronic temporal lobe epilepsy, particularly partial complex seizures (1). The imaging appearance of gangliogliomas is variable, with most presenting as mixed solid and cystic lesions (52%) (4, 5). A superficial cortical site of involvement is most commonly encountered (2, 5, 6). On CT, these lesions are most commonly hypoattenuating (38%), followed by mixed density (32%) and hyperattenuating (15%) (4). Calcific deposits most commonly occur in association with mixed solid/cystic masses and are seen in approximately 30% of gangliogliomas (2, 5). Based on CT appearance alone, calcified gangliogliomas may be indistinguishable from oligodendrogliomas. The MRI appearance of gangliogliomas has classically been described as a cystic mass with an enhancing mural nodule (4, 5). The presence of enhancement, however, is not indicative of a higher-grade neoplasm. Provenzale et al described a trend toward increasing tumor volume and prevalence of cystic components in those gangliogliomas occurring in early childhood From the Departments of Diagnostic Radiology (Saad, Layton, Opatowsky) and Neurosurgery (Finn), Baylor University Medical Center at Dallas. Corresponding author: Amin F. Saad, MD, 1551 Oak Lawn Avenue #176, Dallas, TX 75207 (e-mail: [email protected]). 215
a
b
c
Figure 2. MR imaging. (a) Coronal T2-weighted image reveals a heterogeneous mass with prominent cystic components (arrow) centered within the left occipital lobe in the region of the calcarine fissure, with vasogenic edema extending to the posterior temporal and inferior parietal lobes. (b) Axial gadolinium enhanced T1weighted image reveals moderate heterogeneous enhancement of the mass (arrow). (c) Axial susceptibility weighted image reveals punctate foci of diminished signal (arrow) compatible with calcific deposits seen on the noncontrast CT.
(average age 5.5 years) compared with in young adulthood (average age 25.6 years) (6). The preferred treatment for gangliogliomas is complete surgical resection. Adjuvant chemotherapy and radiation are typically reserved for malignant lesions, with gangliogliomas demonstrating a rate of malignant transformation as high as 6%. Optimal technical surgical resections usually result in the complete resolution of seizure activity with only rare tumor recurrences (7). Gangliogliomas and other mixed glioneuronal tumors may harbor BRAFV600E mutations, an area of potential targeted therapy with agents such as vemurafenib. 1. Koeller KK, Henry JM. From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Radiographics 2001; 21(6):1533–1556.
216
2.
3.
4. 5.
6.
7.
Shin JH, Lee HK, Khang SK, Kim DW, Jeong AK, Ahn KJ, Choi CG, Suh DC. Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics 2002;22(5):1177–1189. Johnson JH Jr, Hariharan S, Berman J, Sutton LN, Rorke LB, Molloy P, Phillips PC. Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years. Pediatr Neurosurg 1997;27(4):203–207. Dorne HL, O’Gorman AM, Melanson D. Computed tomography of intracranial gangliogliomas. AJNR Am J Neuroradiol 1986;7(2):281–285. Castillo M, Davis PC, Takei Y, Hoffman JC Jr. Intracranial ganglioglioma: MR, CT, and clinical findings in 18 patients. AJNR Am J Neuroradiol 1990;11(1):109–114. Provenzale JM, Ali U, Barboriak DP, Kallmes DF, Delong DM, McLendon RE. Comparison of patient age with MR imaging features of gangliogliomas. AJR Am J Roentgenol 2000;174(3):859–862. Silver JM, Rawlings CE 3rd, Rossitch E Jr, Zeidman SM, Friedman AH. Ganglioglioma: a clinical study with long-term follow-up. Surg Neurol 1991;35(4):261–266.
Baylor University Medical Center Proceedings
Volume 27, Number 3
Mixed neuronal/glial neoplasms represent a minority of intracranial neoplasms, typically associated with a more favorable prognosis than the more common higher-grade glial neoplasms. We describe a young man with headache, confusion, and slurred speech who was found to have a ganglioglioma.
CASE REPORT A 19-year-old man with a history of chronic migraine headaches beginning in his early teens experienced an acute sudden onset of severe headache without provocation. Until this point the patient’s headaches lacked atypical symptoms and responded well to over-the-counter medication, and thus he had not undergone previous imaging evaluation. His family noticed that he was confused and had slurred speech, a marked change in the typical character of his headaches. Upon presentation to the emergency department, a noncontrast brain computed tomography (CT) scan (Figure 1) revealed a 6.9 × 3.2 cm isodense nonhemorrhagic mass centered in the left occipital lobe with scattered coarse calcific deposits, surrounding vasogenic edema, and local mass effect but no midline shift or hydrocephalus. Subsequent magnetic resonance imaging (MRI) of the brain further characterized the extent of the mass and revealed conspicuous cystic components (Figure 2a). Mild heterogeneous enhancement was shown on the postgadolinium T1-weighted sequences (Figure 2b). On the MRI, calcific deposits appeared as hypointense foci on the susceptibility weighted sequence (Figure 2c). The patient underwent a craniotomy and the mass was resected. Histologic examination revealed findings compatible with a World Health Organization grade 1 ganglioglioma. The patient did well postoperatively without focal neurologic deficit and will follow up for routine surveillance imaging. DISCUSSION Gangliogliomas are an uncommon lesion, accounting for 0.4% to 0.9% of all intracranial neoplasms and 1% to 4% of pediatric central nervous system neoplasms (1). Despite being uncommon, they are the most common mixed neuronal-glial neoplasm (2). Gangliogliomas have a peak incidence in patients 10 to 20 years old, with 80% being found in individuals younger than 30 years. In a case series of 99 proven gangliogliomas, 38% occurred in the Proc (Bayl Univ Med Cent) 2014;27(3):215–216
Figure 1. Axial noncontrast CT images demonstrate a left parieto-occipital mass with coarse calcific deposits (arrowhead) and cystic areas (arrows).
temporal lobe, 30% in the parietal lobe, and 18% in the frontal lobe (3). Provenzale et al presented a series of 25 gangliogliomas, including two occipital lobe lesions (8%). Gangliogliomas are the most common neoplastic etiology (40%) for chronic temporal lobe epilepsy, particularly partial complex seizures (1). The imaging appearance of gangliogliomas is variable, with most presenting as mixed solid and cystic lesions (52%) (4, 5). A superficial cortical site of involvement is most commonly encountered (2, 5, 6). On CT, these lesions are most commonly hypoattenuating (38%), followed by mixed density (32%) and hyperattenuating (15%) (4). Calcific deposits most commonly occur in association with mixed solid/cystic masses and are seen in approximately 30% of gangliogliomas (2, 5). Based on CT appearance alone, calcified gangliogliomas may be indistinguishable from oligodendrogliomas. The MRI appearance of gangliogliomas has classically been described as a cystic mass with an enhancing mural nodule (4, 5). The presence of enhancement, however, is not indicative of a higher-grade neoplasm. Provenzale et al described a trend toward increasing tumor volume and prevalence of cystic components in those gangliogliomas occurring in early childhood From the Departments of Diagnostic Radiology (Saad, Layton, Opatowsky) and Neurosurgery (Finn), Baylor University Medical Center at Dallas. Corresponding author: Amin F. Saad, MD, 1551 Oak Lawn Avenue #176, Dallas, TX 75207 (e-mail: [email protected]). 215
a
b
c
Figure 2. MR imaging. (a) Coronal T2-weighted image reveals a heterogeneous mass with prominent cystic components (arrow) centered within the left occipital lobe in the region of the calcarine fissure, with vasogenic edema extending to the posterior temporal and inferior parietal lobes. (b) Axial gadolinium enhanced T1weighted image reveals moderate heterogeneous enhancement of the mass (arrow). (c) Axial susceptibility weighted image reveals punctate foci of diminished signal (arrow) compatible with calcific deposits seen on the noncontrast CT.
(average age 5.5 years) compared with in young adulthood (average age 25.6 years) (6). The preferred treatment for gangliogliomas is complete surgical resection. Adjuvant chemotherapy and radiation are typically reserved for malignant lesions, with gangliogliomas demonstrating a rate of malignant transformation as high as 6%. Optimal technical surgical resections usually result in the complete resolution of seizure activity with only rare tumor recurrences (7). Gangliogliomas and other mixed glioneuronal tumors may harbor BRAFV600E mutations, an area of potential targeted therapy with agents such as vemurafenib. 1. Koeller KK, Henry JM. From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Radiographics 2001; 21(6):1533–1556.
216
2.
3.
4. 5.
6.
7.
Shin JH, Lee HK, Khang SK, Kim DW, Jeong AK, Ahn KJ, Choi CG, Suh DC. Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics 2002;22(5):1177–1189. Johnson JH Jr, Hariharan S, Berman J, Sutton LN, Rorke LB, Molloy P, Phillips PC. Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years. Pediatr Neurosurg 1997;27(4):203–207. Dorne HL, O’Gorman AM, Melanson D. Computed tomography of intracranial gangliogliomas. AJNR Am J Neuroradiol 1986;7(2):281–285. Castillo M, Davis PC, Takei Y, Hoffman JC Jr. Intracranial ganglioglioma: MR, CT, and clinical findings in 18 patients. AJNR Am J Neuroradiol 1990;11(1):109–114. Provenzale JM, Ali U, Barboriak DP, Kallmes DF, Delong DM, McLendon RE. Comparison of patient age with MR imaging features of gangliogliomas. AJR Am J Roentgenol 2000;174(3):859–862. Silver JM, Rawlings CE 3rd, Rossitch E Jr, Zeidman SM, Friedman AH. Ganglioglioma: a clinical study with long-term follow-up. Surg Neurol 1991;35(4):261–266.
Baylor University Medical Center Proceedings
Volume 27, Number 3
