Child’s Brain I: 121 125 (1975)
Brain Tumors of Early Infants O samu S ato , A kira T amura and K eiji S ano Department of Neurosurgery, University of Tokyo, Tokyo
Key Words. Early infancy brain tumors • Teratoma in infancy • Glioma in infancy • Craniopharyngioma in infancy • Choroid plexus papilloma in infancy • Shunt in infancy Abstract. The authors describe their experience with ten cases of brain tumors in children in whom the onset of symptoms occurred within the first year of life, but who were operated on when they were 2 months to 4 years of age. The series in cludes 5 gliomas (4 supratentorial, 1 cerebellar; 3 astrocytomas, 1 spongioblastoma, 1 ependymoblastoma), 2 teratomas of the lateral ventricle, and 3 single cases of third ventricle choroid plexus papilloma, temporal lobe sarcoma, and a parasellar craniopharyngioma. Six cases were partially removed, one of them was shunted. Only biopsy was carried out in two, one of which was shunted. The only case of radical removal was a lateral ventricle teratoma; this patient died at operation. Nine patients survived at surgery, three of whom are alive on the 3rd, 4th, and 7th postoperative year. There were two late deaths: one at 2 years (lateral ventricle astrocytoma, which was only biopsied) and one at 7 years and 3 months (spongio blastoma) following surgery. This series from a 40-year period (1933-1973) represents 0.4°/» of 2,832 brain tu mors and 1.9°/o of 528 brain tumors in the pediatric age. The authors emphasize the need to treat brain tumors in early infancy and insist on the use of radiotherapy and chemotherapy when the lesions are not radically removed at surgery.
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It is well known that brain tumors in children under 1 year of age are very rare. Consequently, these tumors are of real interest, not only be cause of the low incidence, considering the overall distribution of tumors in older ages, but because of the real difficulties which a correct clinical diagnosis presents. In addition to this, the anatomical pathological char acteristics, the site of origin, the distribution, classification, and prognosis are unknown factors.
122
Sato/T amura/S ano
During the 40-year period from 1933 to 1973 we have seen ten such tumors. As a point of reference we have arbitrarily chosen the age of on set of the initial symptom or sign rather than the age of final diagnosis at operation for chronological classification. This to us appears to be a more precise and natural criterion for brain tumors in infants. The frequency of these tumors is 0.4% (of a total of 2,832 tumors diagnosed by us) and 1.9% (of a total of 528 tumors) in children less than 15 years of age. We have selected two cases as being representative of particular points of interest from our total series of ten cases. Case 1. An 11-month-old female who suffered generalized convulsions at 4 months of age, and who developed proptosis at 5 months of age, subsequently suf fered fever and vomiting before becoming lethargic. Lumbar puncture revealed tur bid cerebrospinal fluid which was xanthochromic and contained an elevated number of cells. The tentative diagnosis of ‘purulent meningitis' was made and the baby was treated at a children’s hospital with antibiotics. There was an initial improvement in the signs suggesting meningitis, but the proptosis worsened and the eye movements became limited. At 11 months of age the child developed polyuria, polydipsia and obesity. For these reasons a tentative diagnosis of parasellar tumor was made and she was referred to our clinic. On admission to hospital she was found to be obese and to suffer polydipsia. She had proptosis on the right, a complete right ophthalmoplegia, hypesthesia in the first division of the fifth cranial nerve on the right, and a left homonymous hemianopia. Plain skull films revealed an enlarged, eroded sella turcica. Pneumoence phalography revealed a mass within the sella turcica and a filling defect of the an terior portion of the 3rd ventricle. Cerebral angiography revealed an elevated inter nal carotid artery (clinoid portion), posterior displacement of the basilar artery, and upward displacement of the posterior communicating artery. This latter vessel was also stretched and tapered. A diagnosis of a tumor occupying the sella turcica and extending into the parasellar area was made. A right frontal temporal craniotomy was performed and a yellowish brown solid tumor was partially removed. The his tologic diagnosis was ependymoblastoma. The cell pattern was characteristic in that there were rosette formations with tubules and that some of the cells contained cilia. Cobalt therapy was given. 14 months after surgery the child's obesity and ophthalmoplegia were very much improved. Four years after surgery the child is healthy and has no signs of a recurrence of her tumor.
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Case 2. This 5-month-old male was well until 2 months of age, at which time there was bulging in the left temporal area and a progressive increase in head size. The baby was referred to the neurosurgery clinic with a tentative diagnosis of sub dural hygroma. A left carotid angiography revealed only signs compatible with the diagnosis of internal hydrocephalus. It is noteworthy that there was no displacement of vascular structures and no evidence of tumor blush. The anterior fontanel was punctured bi laterally, and xanthochromic fluid was obtained from the left, whereas crystal-clear
123
Brain Tumors in Infancy Table I Case
Sex Age at onset months
M.K. 9 M.M. 9 N.K. $
Age at operation months
Location
Pathology
Operation
Prognosis
11 7 7
14 11 10
lateral ventricle frontoparietal cerebellar
teratoma spongioblastoma astrocytoma
operative death 7 years 3 months
choroid plexus papilloma sarcoma teratoma astrocytoma ependymoblastoma astrocytoma craniopharyngioma
total partial biopsy and decomp. partial and shunt partial partial biopsy
A.H.
11 O
2
II
third ventricle
N.S. N.K. D.l. E.F.
9 Ö o
9
12 2 1 4
16 2 5 11
temporal lateral ventricle lateral ventricle parasellar
K.Y. H.K.
9 9
3 6
58 31
third ventricle parasellar
3 months 7 years 4 months* 5 days 15 days 2 years
partial partial
4 years 1 month* 3 years 10 months'
shunt
9 months (* alive)
Osamu Sato, Departmenl of Neurosurgery, University of Tokyo and University of Tokyo Hospital, Hongo,
Bunkyo-Ku, Tokyo (Japan)
Downloaded by: University of Leeds 129.11.21.2 - 5/8/2018 1:25:29 AM
fluid was obtained from the right. Air was injected separately into each side. These findings suggested to us that the child had a chronic subdural hematoma on the left. Following irrigation of the hematoma, a subdural-atrial shunt was inserted. It was subsequently necessary to revise the shunt on two different occasions. Because of these persistent occurrences, air was again injected into what had been considered the subdural space and X-rays were obtained. The findings were not consistent with the diagnosis of subdural hematoma. A left temporal parietal craniotomy was performed. On opening the dura, which was thin, we observed a thin cerebral cortex, realizing that what was considered to be a subdural space was a markedly dilated lateral ventricle. The medial portion of the wall of this lateral ventricle was opened, exposing a pinkish cauliflower-like mass, which we diagnosed to be a choroid plexus papilloma. A sub-total resection was performed. There was histological confirmation of this diagnosis. A ventriculo-peritoneal shunt was inserted following craniotomy, and the child has continued to be normal now over a period of eight years. It is of interest that there are still no signs of noncompensated hydrocephalus. At the time this child was diagnosed, we did not have access to isotope studies in our country. We speculate at the present time whether these would have facilitated the diagnosis.
124
Sato/T amura/S ano
Downloaded by: University of Leeds 129.11.21.2 - 5/8/2018 1:25:29 AM
The data of our ten cases is summarized in table I. Seven of our ten cases were females. The age of onset of symptoms ranged between 1 and 12 months with the mean at 5 months. The age at operation ranged be tween 2 and 31 months with the mean at 12 months; excluded is a 4-yearold girl who had suffered generalized convulsions, anorexia, and loss in weight during the third month of life, but otherwise had no further symp toms until the fourth year of life. It is of particular interest to note that the patient was referred to neu rosurgery by pediatricians with the diagnosis of hydrocephalus in four cases, subdural effusion in two, meningitis in one and dyspepsia in one case. Brain tumor was suspected in only 2 of our 10 cases. This is unfor tunate. The increase in size or deformity of the head generally leads to a clinical diagnosis of hydrocephalus, chronic subdural hematoma, or, on occasion, subdural hygroma. Convulsions, hemiparesis, and neurologcial signs accompanied by some diminution of conscious level, or respiratory difficulties more often suggest brain tumor. It appears, therefore, that brain tumor should be suspected in all children who are referred with a tentative diagnosis of hydrocephalus, chronic subdural hematoma or hy groma. In our ten cases, focal neurological signs such as hemiparesis were seen in eight, and convulsions in six cases. These signs, when associated with an increase in intracranial pressure and vomiting (8 cases) or papil ledema (3 cases), are of particular significance in suspecting an intra cranial tumor. In addition, it must be stressed that utilization of the most modern diagnostic techniques is essential, not only to the correct diagno sis of brain tumor, but also to ascertain its precise localization. With the exception of a single cerebellar spongioblastoma, all tumors were located in the supratentorial compartment. This is quite different from what has long been observed in tumors of childhood. Five of the nine supratentorial tumors were located within or immediately adjacent to the lateral ventricles. Five of our tumors were gliomas, and three were congenital tumors. Among the gliomas, four were well differentiated and one (an epcndymoblastoma) was pathologically immature. The congenital tumors included two teratomas and one craniopharyngioma. All cases were operated on. Except for the child with craniopharyn gioma, who was in very poor condition, a direct operative attack through craniotomy was the procedure used. Total removal of the tumor was im possible in eigth cases because either of the histological characteristics or the anatomical location of the tumor. An exception was the case of a tera
Brain Tumors in Infancy
125
toma of the lateral ventricle. This patient died, probably because of his poor preoperative general condition. In three cases adjuvant therapy of the residual tumor was used; sys temic administration of anti-cancer drugs in a case of spongioblastoma, radiation therapy in a case of ependymoblastoma, and intracystic BAR therapy in a case of astrocytoma. The operative mortality was 1 out of 9 upon whom a craniotomy was performed. At the discharge from hospital three children died, two re mained unchanged, and five have improved. It is of interest to note that one child died after 2 years, and one after 7 years. Three of the children remain alive more than 3 years after surgery and are entirely without symptoms or signs of recurrence. These observations suggest that the prognosis of brain tumors in early infancy may be better than one would expect, especially if adjuvant therapy is used. In conclusion, we wish to stress that every effort should be made to treat infants with brain tumors - just as every efforts is made to treat adults with brain tumors.
Downloaded by: University of Leeds 129.11.21.2 - 5/8/2018 1:25:29 AM
Dr. O samu Sato, Department of Neurosurgery, University of Tokyo and University of Tokyo Hospital, Hongo. Bunkyo-ku, Tokyo (Japan)
Brain Tumors of Early Infants O samu S ato , A kira T amura and K eiji S ano Department of Neurosurgery, University of Tokyo, Tokyo
Key Words. Early infancy brain tumors • Teratoma in infancy • Glioma in infancy • Craniopharyngioma in infancy • Choroid plexus papilloma in infancy • Shunt in infancy Abstract. The authors describe their experience with ten cases of brain tumors in children in whom the onset of symptoms occurred within the first year of life, but who were operated on when they were 2 months to 4 years of age. The series in cludes 5 gliomas (4 supratentorial, 1 cerebellar; 3 astrocytomas, 1 spongioblastoma, 1 ependymoblastoma), 2 teratomas of the lateral ventricle, and 3 single cases of third ventricle choroid plexus papilloma, temporal lobe sarcoma, and a parasellar craniopharyngioma. Six cases were partially removed, one of them was shunted. Only biopsy was carried out in two, one of which was shunted. The only case of radical removal was a lateral ventricle teratoma; this patient died at operation. Nine patients survived at surgery, three of whom are alive on the 3rd, 4th, and 7th postoperative year. There were two late deaths: one at 2 years (lateral ventricle astrocytoma, which was only biopsied) and one at 7 years and 3 months (spongio blastoma) following surgery. This series from a 40-year period (1933-1973) represents 0.4°/» of 2,832 brain tu mors and 1.9°/o of 528 brain tumors in the pediatric age. The authors emphasize the need to treat brain tumors in early infancy and insist on the use of radiotherapy and chemotherapy when the lesions are not radically removed at surgery.
Downloaded by: University of Leeds 129.11.21.2 - 5/8/2018 1:25:29 AM
It is well known that brain tumors in children under 1 year of age are very rare. Consequently, these tumors are of real interest, not only be cause of the low incidence, considering the overall distribution of tumors in older ages, but because of the real difficulties which a correct clinical diagnosis presents. In addition to this, the anatomical pathological char acteristics, the site of origin, the distribution, classification, and prognosis are unknown factors.
122
Sato/T amura/S ano
During the 40-year period from 1933 to 1973 we have seen ten such tumors. As a point of reference we have arbitrarily chosen the age of on set of the initial symptom or sign rather than the age of final diagnosis at operation for chronological classification. This to us appears to be a more precise and natural criterion for brain tumors in infants. The frequency of these tumors is 0.4% (of a total of 2,832 tumors diagnosed by us) and 1.9% (of a total of 528 tumors) in children less than 15 years of age. We have selected two cases as being representative of particular points of interest from our total series of ten cases. Case 1. An 11-month-old female who suffered generalized convulsions at 4 months of age, and who developed proptosis at 5 months of age, subsequently suf fered fever and vomiting before becoming lethargic. Lumbar puncture revealed tur bid cerebrospinal fluid which was xanthochromic and contained an elevated number of cells. The tentative diagnosis of ‘purulent meningitis' was made and the baby was treated at a children’s hospital with antibiotics. There was an initial improvement in the signs suggesting meningitis, but the proptosis worsened and the eye movements became limited. At 11 months of age the child developed polyuria, polydipsia and obesity. For these reasons a tentative diagnosis of parasellar tumor was made and she was referred to our clinic. On admission to hospital she was found to be obese and to suffer polydipsia. She had proptosis on the right, a complete right ophthalmoplegia, hypesthesia in the first division of the fifth cranial nerve on the right, and a left homonymous hemianopia. Plain skull films revealed an enlarged, eroded sella turcica. Pneumoence phalography revealed a mass within the sella turcica and a filling defect of the an terior portion of the 3rd ventricle. Cerebral angiography revealed an elevated inter nal carotid artery (clinoid portion), posterior displacement of the basilar artery, and upward displacement of the posterior communicating artery. This latter vessel was also stretched and tapered. A diagnosis of a tumor occupying the sella turcica and extending into the parasellar area was made. A right frontal temporal craniotomy was performed and a yellowish brown solid tumor was partially removed. The his tologic diagnosis was ependymoblastoma. The cell pattern was characteristic in that there were rosette formations with tubules and that some of the cells contained cilia. Cobalt therapy was given. 14 months after surgery the child's obesity and ophthalmoplegia were very much improved. Four years after surgery the child is healthy and has no signs of a recurrence of her tumor.
Downloaded by: University of Leeds 129.11.21.2 - 5/8/2018 1:25:29 AM
Case 2. This 5-month-old male was well until 2 months of age, at which time there was bulging in the left temporal area and a progressive increase in head size. The baby was referred to the neurosurgery clinic with a tentative diagnosis of sub dural hygroma. A left carotid angiography revealed only signs compatible with the diagnosis of internal hydrocephalus. It is noteworthy that there was no displacement of vascular structures and no evidence of tumor blush. The anterior fontanel was punctured bi laterally, and xanthochromic fluid was obtained from the left, whereas crystal-clear
123
Brain Tumors in Infancy Table I Case
Sex Age at onset months
M.K. 9 M.M. 9 N.K. $
Age at operation months
Location
Pathology
Operation
Prognosis
11 7 7
14 11 10
lateral ventricle frontoparietal cerebellar
teratoma spongioblastoma astrocytoma
operative death 7 years 3 months
choroid plexus papilloma sarcoma teratoma astrocytoma ependymoblastoma astrocytoma craniopharyngioma
total partial biopsy and decomp. partial and shunt partial partial biopsy
A.H.
11 O
2
II
third ventricle
N.S. N.K. D.l. E.F.
9 Ö o
9
12 2 1 4
16 2 5 11
temporal lateral ventricle lateral ventricle parasellar
K.Y. H.K.
9 9
3 6
58 31
third ventricle parasellar
3 months 7 years 4 months* 5 days 15 days 2 years
partial partial
4 years 1 month* 3 years 10 months'
shunt
9 months (* alive)
Osamu Sato, Departmenl of Neurosurgery, University of Tokyo and University of Tokyo Hospital, Hongo,
Bunkyo-Ku, Tokyo (Japan)
Downloaded by: University of Leeds 129.11.21.2 - 5/8/2018 1:25:29 AM
fluid was obtained from the right. Air was injected separately into each side. These findings suggested to us that the child had a chronic subdural hematoma on the left. Following irrigation of the hematoma, a subdural-atrial shunt was inserted. It was subsequently necessary to revise the shunt on two different occasions. Because of these persistent occurrences, air was again injected into what had been considered the subdural space and X-rays were obtained. The findings were not consistent with the diagnosis of subdural hematoma. A left temporal parietal craniotomy was performed. On opening the dura, which was thin, we observed a thin cerebral cortex, realizing that what was considered to be a subdural space was a markedly dilated lateral ventricle. The medial portion of the wall of this lateral ventricle was opened, exposing a pinkish cauliflower-like mass, which we diagnosed to be a choroid plexus papilloma. A sub-total resection was performed. There was histological confirmation of this diagnosis. A ventriculo-peritoneal shunt was inserted following craniotomy, and the child has continued to be normal now over a period of eight years. It is of interest that there are still no signs of noncompensated hydrocephalus. At the time this child was diagnosed, we did not have access to isotope studies in our country. We speculate at the present time whether these would have facilitated the diagnosis.
124
Sato/T amura/S ano
Downloaded by: University of Leeds 129.11.21.2 - 5/8/2018 1:25:29 AM
The data of our ten cases is summarized in table I. Seven of our ten cases were females. The age of onset of symptoms ranged between 1 and 12 months with the mean at 5 months. The age at operation ranged be tween 2 and 31 months with the mean at 12 months; excluded is a 4-yearold girl who had suffered generalized convulsions, anorexia, and loss in weight during the third month of life, but otherwise had no further symp toms until the fourth year of life. It is of particular interest to note that the patient was referred to neu rosurgery by pediatricians with the diagnosis of hydrocephalus in four cases, subdural effusion in two, meningitis in one and dyspepsia in one case. Brain tumor was suspected in only 2 of our 10 cases. This is unfor tunate. The increase in size or deformity of the head generally leads to a clinical diagnosis of hydrocephalus, chronic subdural hematoma, or, on occasion, subdural hygroma. Convulsions, hemiparesis, and neurologcial signs accompanied by some diminution of conscious level, or respiratory difficulties more often suggest brain tumor. It appears, therefore, that brain tumor should be suspected in all children who are referred with a tentative diagnosis of hydrocephalus, chronic subdural hematoma or hy groma. In our ten cases, focal neurological signs such as hemiparesis were seen in eight, and convulsions in six cases. These signs, when associated with an increase in intracranial pressure and vomiting (8 cases) or papil ledema (3 cases), are of particular significance in suspecting an intra cranial tumor. In addition, it must be stressed that utilization of the most modern diagnostic techniques is essential, not only to the correct diagno sis of brain tumor, but also to ascertain its precise localization. With the exception of a single cerebellar spongioblastoma, all tumors were located in the supratentorial compartment. This is quite different from what has long been observed in tumors of childhood. Five of the nine supratentorial tumors were located within or immediately adjacent to the lateral ventricles. Five of our tumors were gliomas, and three were congenital tumors. Among the gliomas, four were well differentiated and one (an epcndymoblastoma) was pathologically immature. The congenital tumors included two teratomas and one craniopharyngioma. All cases were operated on. Except for the child with craniopharyn gioma, who was in very poor condition, a direct operative attack through craniotomy was the procedure used. Total removal of the tumor was im possible in eigth cases because either of the histological characteristics or the anatomical location of the tumor. An exception was the case of a tera
Brain Tumors in Infancy
125
toma of the lateral ventricle. This patient died, probably because of his poor preoperative general condition. In three cases adjuvant therapy of the residual tumor was used; sys temic administration of anti-cancer drugs in a case of spongioblastoma, radiation therapy in a case of ependymoblastoma, and intracystic BAR therapy in a case of astrocytoma. The operative mortality was 1 out of 9 upon whom a craniotomy was performed. At the discharge from hospital three children died, two re mained unchanged, and five have improved. It is of interest to note that one child died after 2 years, and one after 7 years. Three of the children remain alive more than 3 years after surgery and are entirely without symptoms or signs of recurrence. These observations suggest that the prognosis of brain tumors in early infancy may be better than one would expect, especially if adjuvant therapy is used. In conclusion, we wish to stress that every effort should be made to treat infants with brain tumors - just as every efforts is made to treat adults with brain tumors.
Downloaded by: University of Leeds 129.11.21.2 - 5/8/2018 1:25:29 AM
Dr. O samu Sato, Department of Neurosurgery, University of Tokyo and University of Tokyo Hospital, Hongo. Bunkyo-ku, Tokyo (Japan)
![[Brain tumors in infants].](/assets/img/hold.png)
