The Neuroradiology Journal 20: 61-66, 2007
www. centauro. it
Central Neurocytoma: Case Report of an 81-Year-Old and Histopathologic Findings G. BRANT-ZAWADZKI*, M.S. MATHEWS*****, C.M. DUMA*, B. KIM***, P. CHEN***, S. HEINEMANN****, M. BRANT-ZAWADZKI** * Departments of Neurosurgery, ** Radiology, *** Radiation Oncology, and **** Pathology, Hoag Memorial Hospital Presbyterian; Newport Beach, CA, USA ***** Department of Neurosurgery, University of California; Irvine, CA, USA
Key words: central neurocytoma, elderly, 81-year-old, gamma knife, histopathology
SUMMARY – Central Neurocytomas are rare and usually benign tumors found primarily in the lateral ventricles of the brain. Central Neurocytomas are composed of uniform round cells exhibiting neuronal differentiation and are found almost exclusively in young adults between 15 and 60 years of age. The authors report the case of an 81-year-old man with a history of severe psychosis and depression, who presented with progressive confusion and ataxia likely unrelated to a central neurocytoma of his right lateral ventricle. The patient underwent a stereotactic biopsy of the lesion, followed by Gamma Knife radiosurgery. Histopathology showed immunohistological staining for synaptophysin, neuron specific enolase (NSE) and neuronal nuclear antigen (NeuN). A review of published literature on central neurocytomas revealed that this tumor occurs most frequently in young adults with a median age between 25 and 30 years. This pathology has never been reported in patients over the age of 80. Gamma Knife radiosurgery was successful in decreasing the tumor volume by 20% at the four month follow-up, but the fact that the patient died of unrelated occurrences in the elderly cannot be ruled out. Patients with central neurocytomas commonly present with obstructive hydrocephalus and immediate treatment may be necessary to tide over urgent situations.
Introduction First described by Hassoun et Al in 1982 3, central neurocytomas (CN) are rare and mostly benign tumors found primarily in the lateral ventricles of the brain 8. CNs are composed of uniform round cells exhibiting neuronal differentiation and are found almost exclusively in young adults between 15 and 60 years of age. Although frequently mistaken for oligodendrogliomas in the past, the use of synaptophysin immunoreaction and electron microscopy in pathologic diagnosis has revealed a significant increase in the number of reported cases of CN from 30 cases recorded between 1982 and 1992, to 127 cases in 1995 alone 4. Still, these tumors only compose 118
Hassoun et Al, 1993
29 (Median)
20-40
>127
Kim et Al, 1996
31 (Median)
15-60
>113
Schild et Al, 1997
27 (Median)
14-57
>132
Ian R. A, Mackenzie, 1998
27 (Median)
19-57
>115
Metellus et Al, 2000
29 (Median)
18-67
>>207
Ashkan et Al, 2000
27 (Median)
12-56
>112
Rades et Al, 2005
27 (Median)
13-76
>121
Marc C. Chamberlain, 2002
23 (Median)
15-72
>150
Schmidt et Al, 2004
29 (Median)
20-40
>127
a comprehensive review of the literature on CN and analyzed some of the largest studies and case reports of the disease. They cited studies showing a mean age of presentation of 29 years, with 70% of all patients presenting between the ages of 20 and 40 years. Furthermore, the review claims that due to the limited size of most clinical trials, there is no clear optimal treatment for CN, as management ranges from surgery (subtotal or total resection), standard external beam radiation, radiosurgery, and chemotherapy. Often, according to their findings, physicians will administer multiple treatment options for optimal results. The authors cite a 1997 article by Schild et Al showing five year local control for 100% of patients who underwent gross total resection and 80% survival rate 7. For patients who received a subtotal resection followed by radiotherapy (RT) the study found five year control rate of 100% compared to a rate of 50% when RT was not administered. The typical CT imaging findings for CN are an isointense to slightly hyperdense mass in the lateral ventricle, moderate contrast enhancement, hypodense areas corresponding to cystic degeneration, and evidence of transependymal fluid with obstructive hydrocephalus. MRI imaging typically shows an inhomogeneous isointense mass on T1-weighted images, low signal on T1 corresponding to vessels or calcium, isointense or hyperintense on T2-weighted sequences, and moderate to strong gadolinium enhancement.
Our case was atypical from the imaging perspective. Most reported cases demonstrate predominantly intraventricular tumors, with some demonstrating prominent calcification 1,8. Cystic components were not a typical feature. Our case had a more central location and the often seen attachment to the septum pelucidum was not present. To our knowledge, the previous oldest presenting CN patient was 76 (6), while 70% of reported central neurocytoma cases involved patients ranging from 20 to 40 years, and a mean age of 29 8. Our own review of ten major articles representing the culmination of well over 250 cases revealed similar findings (table 1). The gross appearance of the tumor specimen suggested oligodendroglioma or central neurocytoma. Because of the patient’s age we also considered lymphoma and metastatic tumors. Strong diffuse cytoplasmic staining for synaptophysin confirmed the diagnosis of central neurocytoma. A small percentage of oligodendrogliomas have scattered synaptophysin positive cells 8. The degree of synaptophysin positivity seen in our case would be inconsistent with a primarily glial tumor. However, the presence of a few GFAP positive cells suggests that either the tumor has some capacity for glial differentiation, or it may represent reactive gliosis. This has been previously reported for central neurocytoma 8. The diagnosis of central neurocytoma is further supported by detection of neuron specific enolase (NSE) and neuronal nuclear antigen (NeuN) in the tumor cells, spe65
Central Neurocytoma: Case Report of an 81-Year-Old and Histopathologic Findings
cific markers for neuronal differentiation. The treatment of this tumor using single-fraction radiosurgery is warranted considering the historical experience using radiation therapy for this tumor. Furthermore the margins of this tumor are well-demarcated and lend themselves to conformational planning. Our patient’s tumor decreased in size by 20% in four months. Unfortunately, the patient died of unrelated causes before further follow-up imaging could be performed.
G. Brant-Zawadzki
Conclusions Although central neurocytomas typically present in young adults they cannot be ruled out in the elderly purely on age. Patients with central neurocytomas commonly present with obstructive hydrocephalus and immediate treatment may be necessary to tide over urgent situations. Gamma knife radiosurgery may prove to be an alternative to surgical resection for these tumors.
References 1 Chamberlain MC: Treatment of Central Neurocytomas. Expert review of Neurotherapeutics 2: 464-468, 2002. 2 Coca S, Moreno M et Al: "Neurocytoma of spinal cord". Acta Neuropathol 87: 537-40, 1994. 3 Hassoun J, Gambarelli D, Grisoli F et Al: Central Neurocytoma. An electron-microscopic study of two cases. Acta Neuropathol 56: 151-6, 1982. 4 Metellus, P, Dufour H et Al: Central neurocytoma revealed by intraventricular hemorrhage. A case report and review of the literature. Neurochirurgie 47: 445-7, 2001. 5 Okamura A, Goto S et Al: "Central Neurocytoma with Hemorrhagic Onset". Surg Neurol 43: 252-5, 1995. 6 Rades D, Schild SE: Value of postoperative stereotactic radiosurgery and conventional radiotherapy for incompletely resected typical neurocytomas. Cancer 106: 1140-3, 2006. 7 Schild SE, Scheithauer BW et Al: "Central Neurocytomas". Cancer 79: 790-5, 1997. 8 Schmidt MH, Gottfried ON, von Koch CS et Al: Central Neurocytoma: a review. J Neurooncol 66: 377-84, 2004.
66
9 Soylemezoglu F, Scheithauer BW et Al: "Atypical Central Neurocytoma". J Neuropathol Exp Neurol 56: 5516, 1997. 10 Tatter SB, Borges LF et Al: "Central Neurocytomas of the Cervical Spinal Cord. Report of two cases". J Neurosurg 81: 288-93, 1994.
Marlon S. Mathews, MD 101 The City Drive South Bldg 56 - Suite 400 Orange, CA 92868, USA Tel.: +1.714.456-6966 Fax: +1.714.456-6966 E-mail: [email protected]
www. centauro. it
Central Neurocytoma: Case Report of an 81-Year-Old and Histopathologic Findings G. BRANT-ZAWADZKI*, M.S. MATHEWS*****, C.M. DUMA*, B. KIM***, P. CHEN***, S. HEINEMANN****, M. BRANT-ZAWADZKI** * Departments of Neurosurgery, ** Radiology, *** Radiation Oncology, and **** Pathology, Hoag Memorial Hospital Presbyterian; Newport Beach, CA, USA ***** Department of Neurosurgery, University of California; Irvine, CA, USA
Key words: central neurocytoma, elderly, 81-year-old, gamma knife, histopathology
SUMMARY – Central Neurocytomas are rare and usually benign tumors found primarily in the lateral ventricles of the brain. Central Neurocytomas are composed of uniform round cells exhibiting neuronal differentiation and are found almost exclusively in young adults between 15 and 60 years of age. The authors report the case of an 81-year-old man with a history of severe psychosis and depression, who presented with progressive confusion and ataxia likely unrelated to a central neurocytoma of his right lateral ventricle. The patient underwent a stereotactic biopsy of the lesion, followed by Gamma Knife radiosurgery. Histopathology showed immunohistological staining for synaptophysin, neuron specific enolase (NSE) and neuronal nuclear antigen (NeuN). A review of published literature on central neurocytomas revealed that this tumor occurs most frequently in young adults with a median age between 25 and 30 years. This pathology has never been reported in patients over the age of 80. Gamma Knife radiosurgery was successful in decreasing the tumor volume by 20% at the four month follow-up, but the fact that the patient died of unrelated occurrences in the elderly cannot be ruled out. Patients with central neurocytomas commonly present with obstructive hydrocephalus and immediate treatment may be necessary to tide over urgent situations.
Introduction First described by Hassoun et Al in 1982 3, central neurocytomas (CN) are rare and mostly benign tumors found primarily in the lateral ventricles of the brain 8. CNs are composed of uniform round cells exhibiting neuronal differentiation and are found almost exclusively in young adults between 15 and 60 years of age. Although frequently mistaken for oligodendrogliomas in the past, the use of synaptophysin immunoreaction and electron microscopy in pathologic diagnosis has revealed a significant increase in the number of reported cases of CN from 30 cases recorded between 1982 and 1992, to 127 cases in 1995 alone 4. Still, these tumors only compose 118
Hassoun et Al, 1993
29 (Median)
20-40
>127
Kim et Al, 1996
31 (Median)
15-60
>113
Schild et Al, 1997
27 (Median)
14-57
>132
Ian R. A, Mackenzie, 1998
27 (Median)
19-57
>115
Metellus et Al, 2000
29 (Median)
18-67
>>207
Ashkan et Al, 2000
27 (Median)
12-56
>112
Rades et Al, 2005
27 (Median)
13-76
>121
Marc C. Chamberlain, 2002
23 (Median)
15-72
>150
Schmidt et Al, 2004
29 (Median)
20-40
>127
a comprehensive review of the literature on CN and analyzed some of the largest studies and case reports of the disease. They cited studies showing a mean age of presentation of 29 years, with 70% of all patients presenting between the ages of 20 and 40 years. Furthermore, the review claims that due to the limited size of most clinical trials, there is no clear optimal treatment for CN, as management ranges from surgery (subtotal or total resection), standard external beam radiation, radiosurgery, and chemotherapy. Often, according to their findings, physicians will administer multiple treatment options for optimal results. The authors cite a 1997 article by Schild et Al showing five year local control for 100% of patients who underwent gross total resection and 80% survival rate 7. For patients who received a subtotal resection followed by radiotherapy (RT) the study found five year control rate of 100% compared to a rate of 50% when RT was not administered. The typical CT imaging findings for CN are an isointense to slightly hyperdense mass in the lateral ventricle, moderate contrast enhancement, hypodense areas corresponding to cystic degeneration, and evidence of transependymal fluid with obstructive hydrocephalus. MRI imaging typically shows an inhomogeneous isointense mass on T1-weighted images, low signal on T1 corresponding to vessels or calcium, isointense or hyperintense on T2-weighted sequences, and moderate to strong gadolinium enhancement.
Our case was atypical from the imaging perspective. Most reported cases demonstrate predominantly intraventricular tumors, with some demonstrating prominent calcification 1,8. Cystic components were not a typical feature. Our case had a more central location and the often seen attachment to the septum pelucidum was not present. To our knowledge, the previous oldest presenting CN patient was 76 (6), while 70% of reported central neurocytoma cases involved patients ranging from 20 to 40 years, and a mean age of 29 8. Our own review of ten major articles representing the culmination of well over 250 cases revealed similar findings (table 1). The gross appearance of the tumor specimen suggested oligodendroglioma or central neurocytoma. Because of the patient’s age we also considered lymphoma and metastatic tumors. Strong diffuse cytoplasmic staining for synaptophysin confirmed the diagnosis of central neurocytoma. A small percentage of oligodendrogliomas have scattered synaptophysin positive cells 8. The degree of synaptophysin positivity seen in our case would be inconsistent with a primarily glial tumor. However, the presence of a few GFAP positive cells suggests that either the tumor has some capacity for glial differentiation, or it may represent reactive gliosis. This has been previously reported for central neurocytoma 8. The diagnosis of central neurocytoma is further supported by detection of neuron specific enolase (NSE) and neuronal nuclear antigen (NeuN) in the tumor cells, spe65
Central Neurocytoma: Case Report of an 81-Year-Old and Histopathologic Findings
cific markers for neuronal differentiation. The treatment of this tumor using single-fraction radiosurgery is warranted considering the historical experience using radiation therapy for this tumor. Furthermore the margins of this tumor are well-demarcated and lend themselves to conformational planning. Our patient’s tumor decreased in size by 20% in four months. Unfortunately, the patient died of unrelated causes before further follow-up imaging could be performed.
G. Brant-Zawadzki
Conclusions Although central neurocytomas typically present in young adults they cannot be ruled out in the elderly purely on age. Patients with central neurocytomas commonly present with obstructive hydrocephalus and immediate treatment may be necessary to tide over urgent situations. Gamma knife radiosurgery may prove to be an alternative to surgical resection for these tumors.
References 1 Chamberlain MC: Treatment of Central Neurocytomas. Expert review of Neurotherapeutics 2: 464-468, 2002. 2 Coca S, Moreno M et Al: "Neurocytoma of spinal cord". Acta Neuropathol 87: 537-40, 1994. 3 Hassoun J, Gambarelli D, Grisoli F et Al: Central Neurocytoma. An electron-microscopic study of two cases. Acta Neuropathol 56: 151-6, 1982. 4 Metellus, P, Dufour H et Al: Central neurocytoma revealed by intraventricular hemorrhage. A case report and review of the literature. Neurochirurgie 47: 445-7, 2001. 5 Okamura A, Goto S et Al: "Central Neurocytoma with Hemorrhagic Onset". Surg Neurol 43: 252-5, 1995. 6 Rades D, Schild SE: Value of postoperative stereotactic radiosurgery and conventional radiotherapy for incompletely resected typical neurocytomas. Cancer 106: 1140-3, 2006. 7 Schild SE, Scheithauer BW et Al: "Central Neurocytomas". Cancer 79: 790-5, 1997. 8 Schmidt MH, Gottfried ON, von Koch CS et Al: Central Neurocytoma: a review. J Neurooncol 66: 377-84, 2004.
66
9 Soylemezoglu F, Scheithauer BW et Al: "Atypical Central Neurocytoma". J Neuropathol Exp Neurol 56: 5516, 1997. 10 Tatter SB, Borges LF et Al: "Central Neurocytomas of the Cervical Spinal Cord. Report of two cases". J Neurosurg 81: 288-93, 1994.
Marlon S. Mathews, MD 101 The City Drive South Bldg 56 - Suite 400 Orange, CA 92868, USA Tel.: +1.714.456-6966 Fax: +1.714.456-6966 E-mail: [email protected]
