- Email: [email protected]
Brain Tumors in Childhood
Brain Tumors in Childhood FRANK J. OTENASEK, M.D., F.A.C.S.*
ONE may be inclined to conclude from the experiences and impressions of older pediatricians and physicians that intracranial neoplasms occur relatively rarely in infancy and childhood. There are, moreover, few reliable sources of information which might tend to confirm or refute the assumption that such lesions are found only infrequently. Many of the estimates concerning the incidence of these lesions are derived from studies of series of patients made before the advent of Dandy's introduction of ventriculography as a 10ca1izing diagnostic procedure. Stern,! for example, records only 62 verified intracranial tumors in 2808 consecutive necropsies in the London Hospital for Sick Children, an incidence of only 2.2 per cent of the total. From the experience of Cushing, it was estimated that cerebral tumors occur only about one-sixth as frequently in childhood as they do in adult life. More recent observations would seem to indicate that such tumors are found in nearly the same proportion in children as they are in adults,2 or at least, that the number of intracranial tumors in childhood is greater than previously generally believed. 3 Pathological studies of the various types of intracranial tumors in children have revealed an uncomfortably high percentage of tumor types which are generally associated, in the average physician's mind, with incurability. Thus, in the oldest reports, the incidence of tuberculomas was so high that the diagnosis of a brain tumor in a child was almost synonymous with this infectious process and therefore with death. In an analysis by Starr,4 more than 50 per cent of the tumors were tuberculomas. Fiori's5 concepts of the symptomatology of posterior fossa tumors, published in 1911, were derived mostly from localized subtentorial tuberculous infections. The work of Critchley,6 in 1925, points up the tubercle as the most common intracranial tumor in childhood,
From the Division of Neurological Surgery, The Johns Hopkins University School of Medicine and The Johns Hopkins Hospital, Baltimore, Maryland.
* Assistant Professor of Neurosurgery, The Johns Hopkins University School Medicine; Visiting Neurosurgeon, The Johns Hopkins Hospital. 1363
0'
1364
Frank J. Otenasek
while as recently as 1933, Garland and Armitage7 felt that-at least in Leeds-about 66 per cent of such tumors were of acid-fast origin. However, Stern1 in 1937, reports that the incidence of intracranial tubercles was only in the neighborhood of 6 per cent. This was also the estimate of Cushing. Act~ally, at present the percentage of such lesions (in the United States at least) is so much lower that only rarely need serious consideration of an intracranial tuberculoma enter into the processes of differential diagnosis. Moreover, some of the recent encouraging reports on the effectiveness of streptomycin in tuberculosis of the central nervous system may help to allay the ancient prognostic fears associated with such a diagnosis. Consideration of intracranial pathological processes of expanding nature, other than infectious in type" occurring among children, reveal a striking preponderance of tumors of neuroepithelial origin, largely gliomas. Numerous .authorities place t~e incidence of these neoplasms in children in the rieighborh60d of 75' per cent. The term glioma, in the mind of the average pediatrician, is practically synonymous with death. And thus many physicians are loathe to subject a child, in whom they have made this diagnosis, to the "whims" of the neurosurgeon. Such an attitude of fatalism cannot help but be fostered by perusal of the best known treatise on intracranial tumors of infancy in childhood,3 from which the following may be quoted (page 440): "Ford concludes his study of intracranial tumors in childhood rather sadly by stating that 'the chances of discovering a benign tumor at operation are relatively small.' If by 'benign' he means a tumor which can be successfully and permanently removed without leaving the patient too crippled, the pathologist must agree with him. Not only do the records of surgical clinics prove it, but also the study of pathology teaches that it must be so." (Italics ours.) Obviously, careful pathological studies are of paramount importance in establishing the precise nature of tumors, but such studies alone do not justify far-reaching prognostic conclusions. Before such conclusions can be validly drawn there must be a combination of immense clinical experience and superb surgical technic with knowledge of the biology of the tumor in question. Such a combination is rare indeed. It is the primary purpose of this clinic to de-emphasize the fact that most intracranial tumors in children are malignant and inoperable, to show that some of those deemed so before operation prove, surprisingly enough, to be curable, and to emphasize that even tumors which at operation are believed to be "hopelessly inoperable" are sometimes compatible with some years of happy living. I shall endeavor to illustrate with a few cases, picked almost at random from the files of The Johns Hopkins Hospital.
Brain Tumors in Childhood
1365
CASE I. M. H., a white girl of 13 years, daughter of a physician, was admitted to the neurosurgical servive of Dr. Walter E. Dandy on July 30, 1942 (Unit!No. 265396). The chief complaint at the time of admission was headache and vomiting. Apparently nearly 2 years before her admission to the hospital the child had suffered moderately severe headaches occurring in episodes lasting for 1 or 2 days. The headaches were of dual character, being either generalized or localized largely to the occipital area. In February 1941 she was seen by an ophthalmologist in her home town and normal findings were recorded. For an indefinite period of time, probably almost a year, the patient was noticed to have had a mild tendency to stagger and also exhibited some degree of clumsiness. For 7 months she has had occasional tinnitus of the right ear. In January 1942, approximately 6 months before her admission, all the symptoms seemed to become considerably worse. About 10 days before her entry into the hospital here she had gone swimming and following this noticed very severe headaches, associated with constant vomiting. Physical examination on admission revealed a well-developed and well~ nourished young girl of 13. The general physical examination contributed little to her present illness. There was a suggestive cracked-pot sound over the calvarium. The inion did not appear to be higher than the expected position and therefore, the posterior fossa did not seem to be large. There was about 3 diopters of papilledema with hemorrhages in the right fundus. Her gait was a little unsteady. The Romberg test was negative. There was very slight bilateral ataxia and dysdiadochokinesis. The child was thought to have a probable cerebellar tumor although the possibility of a stricture at the aqueduct of Sylvius was discussed. A ventriculogram was done and this was followed by a bilateral suboccipital approach. A large cystic astrocytoma was found and the mural nodule removed from the posterior medial aspect of the right cerebellar hemisphere. The child made an uneventful recovery and is now well 10 years after the operative-procedure was carried out.
It is interesting that the presumptive diagnosis in this patient following ventriculography was medulloblastoma of the cerebellum because of the absence of lateralizing signs in the examination. While it is generally supposed that such cystic astrocytomas as this patient had are the most favorable of all intracranial tumors of childhood, it is not generally known that some neurosurgeons, on the basis of a presumptive diagnosis of a midline medulloblastoma of the cerebellum, will only half-heartedly search for such a tumor through a small midline suboccipital opening and then only for purposes of a biopsy. Such a course of action in the case cited would have been fatal. Yet the tumor was benign and removable. CASE II. J. S., a Belgian boy of 10 years, was admitted to the Harriet Lane Home of The Johns Hopkins Hospital on May 3, 1948 (Unit No. A 62575). This child was admitted to the Pediatric Department because of a complaint of headache and vomiting. Apparently, about 8 months before, he had begun to have morning vomiting occurring nearly daily, at first only after breakfast. Several weeks later this began to occur several times a day. About 3 months before his admission he developed severe generalized headaches. There were no other symptoms of any kind.
1366
Frank J. Otenasek
Physical examination showed a boy well developed and well nourished in spite of his frequent vomiting. Examination of the head revealed a cracked-pot sound and x-ray evidence of separation of the sutures. There was bilateral papilledema with hemorrhages. On deviation of the eyes to the right side there were horizontal nystagmoid movements of small amplitude. There was no definite weakness or ataxia, but station and gait were definitely unsteady and the child could not stand on one foot with the eyes open. A clinical diagnosis of posterior fossa tumor, probably medulloblastoma, was made, although because of the patient's age the possibility of astrocytoma was entertained. A ventriculogram confirmed the location of the lesion and at operation a medulloblastoma of the posterior fossa was subtotally removed. The child did well postoperatively and after 10 days deep x-ray therapy was begun, being given to each of 3 ports of the cerebellar and occipital area, while the entire spine was divided into 3 equal ports for purposes of irradiation. During his stay in the hospital and while undergoing radiotherapy the count of white blood cells in the blood dropped to 3000 and x-ray therapy had to be discontinued. After his discharge from the hospital, the course of therapy was con.tinued elsewhere. The child has now long· since returned to Europe and a letter from his father early in 1952, nearly 3 years after his operation, reveals that the child is attending school, is doing well, and leading a perfectly normal life.
This patient represents an instance of a child, hopelessly ill with a malignant tumor, who is still subjectively and objectively normal nearly four years after incomplete removal of the neoplasm. Although no one would be so brash as to assume that there is a significant chance of permanent cure in this case, the fact that the child is leading a normal life with hope of continuing so for a while longer leaves no doubt that the treatment accorded produced a result beyond the expectations of mere statistical tables. Not every medulloblastoma necessarily means death within a year. CASE III. M. H., a white boy of 6, was admitted to The Johns Hopkins Hospital, February 20, 1947 (Unit No. 414406), because of failing vision. His past history was not contributory. Apparently about 18 months before his hospital admission, the parents first noticed that the patient was unable to see clearly. He would grope about for objects when attempting to pick them up and he did not seem to be able to locate them accurately. Shortly thereafter, he began to bump into things at times, as though he had not noticed them. About a year before his admission, it was noted by the mother that his eyes were continually jerking from side to side and it was felt that he was becoming nervous. Six months before he came to the hospital he began to complain of headaches which were associated with vomiting. These symptoms gradually progressed to the time of his admission. Physical examination revealed a well-developed and well-nourished child who did not appear generally ill. The head was of normal size, shape, and configuration. There was a cracked-pot sound on the head to percussion over the coronal sutures. Examination of the eyes revealed grossly defective vision, being less than 5/50 in each eye. Small objects were not seen well. Ophthalmoscopically the disks were dead white and flat. There was no evidence of blurring of the
Brain Tumors in Childhood
1367
margins. The extraocular movements were well performed but there was a constant slow nystagmus of equal amplitude and rate in each direction. It was present at all points of fixation and no point of rest could be brought out. The remainder of the neurological examination was normal. X-rays of the skull confirmed the separation of the sutures and also showed erosion of the sella turcica, particularly of the anterior clinoid processes. The preoperative diagnosis 'was tumor of the optic chiasm and hypothalamus. A right frontal approach was carried out. As we slipped down under the frontal lobe in the neighborhood of the chiasm we encountered dense, hard, grayish tumor which was very vascular and filled the whole area. Neither optic nerve could be identified. According to the operative description, "It was clearly a hopeless tumor. Small portions of it were taken for biopsy but there will be no relief of either vision or hydrocephalus." The tumor was an astrocytoma. The child did well postoperatively. The parents were given a completely hopeless prognosis and it was felt that radiotherapy should be given more. for the benefit of the parents than actually for the benefit of the patient himself. He was next seen in October of 1947 at which time he looked well, had no headache or vomiting. The vision had improved both subjectively and objectively. Deep therapy was still being given by Dr. George Farber. No cracked-pot sound could be elicited. The patient was last seen in December 1951, at which time his vision was 5/40 in the right eye and 3/30 in the left. There was marked optic atrophy in both eyes. His nystagmus persisted. At this time he was attending a school for the blind and leading a happy life. This case illustrates the not uncommon pitfall of calling a tumor inoperable from its gross appearance, and even from its microscopic appearance when the histological picture does not suggest radiosensitivity. Furthermore, it was diagnosed preoperatively as a tumor of the optic chiasm and hypothalamus, while according to our authority,3 "Those (gliomas) of the optic chiasm and hypothalamus ... should be diagnosed only to be let alone, regardless of their structure." (Italics ours.) This child is alive and relatively well five years after the "hopeless" tumor was exposed. He and his family are happy with the result. In spite of the inadequacy of his vision, his intelligence is normal and his attendance at the School for the Blind may enable him to be useful if the lesion does not recur in the future. Again we emphasize that not all "hopeless" tumors are incompatible with the pursuit of happiness. CASE IV. K. B., a white girl of 16, was admitted to The Johns Hopkins Hospital April 17, 1947 (Unit No. 419559) because of headache, unsteadiness, and difficulty with vision. Apparently about 3 years before she developed some frontal headache which appeared to come on once or twice a month and lasted for perhaps half a day. These gradually became more severe and more frequent. About 2 years before her admission to the hospital, she noticed some difficulty in seeing the blackboard at school. Glasses were prescribed by an optometrist and apparently the reading defect was corrected. She was then fairly well until approximately 6 or 7 months before her hospital admission when the headaches became much more severe. They seemed to occur about once every 2 or 3 weeks
<
1368
Frank J. Otenasek
and were accompanied by nausea but no vomiting. Within a short time, the headaches became as frequent as once a week and required her to stay 1 to 2 days a week in bed. Blurring of vision and diplopia became severe in December of 1946 at which time vomiting began and was most severe in the morning. This was apparently arrested by a change in diet. For 3 months prior to her admission she noticed some unsteadiness on walking which was apparent to the patient as well as to other people. Physical examination showed early papilledema with normal and full visual fields. There was nystagmus on looking to either side, with the quick phase in the lateral position and the slow phase toward the midline. The remaining cranial nerves were entirely normal. There was no motor weakness or sensory loss. There was no evidence of ataxia or adiadochokinesis. The patient showed a little unsteadiness on turning, with a slight tendency to drift toward the right. A venticular air injection was done. On tapping there was a spurt of fluid under considerable pressure. Fluid in the amount of 120 cc. was obtained and a similar amount of air injected. The films showed a 'moderately advanced hydrocephalus. There was an outline of tumor in the fourth ventricle. A bilateral suboccipital approach was made. The cerebellar tonsils were found far down in the spinal canal. They were separated and as the operator entered the fourth ventricle a grayish, solid tumor, seemingly well circumscribed, was encountered. The vermis was split over a distance of nearly 2 cm. There was tumor everywhere infiltrating into both cerebellar hemispheres, a little more perhaps on the left side. The tumor was soft and friable. It was removed piece-' meal. Infiltration of the tumor took place into both hemispheres as well as into the floor of the fourth ventricle so that no line of cleavage could be discovered. The conclusion was reached that the tumor had probably arisen in the brain stem. It extended high up into the ventricle and it was impossible to obtain a free flow of fluid from the aqueduct of Sylvius. The patient had a very stormy postoperative course. Histological sections of the tumor revealed it to be an astrocytoma. She is now left with a residuum of rather marked ataxia of the left side and considerable difficulty in walking without support. However, she dances well, is able to drive her own car, and spent 1 year on the continent of Europe. It is now 5 years since the time of her operation. She has been leading a relatively normal and certainly a happy life. She was treated elsewhere with polysaccharides and teropterin, but the effect of these treatments cannot properly be evaluated. Nevertheless the fact remains' that the patient is now 21 years of age and is alive. This is simply another instance in which even failure to relieve hydrocephalus by operation did not prevent a happy five year survival. CASE V. M. S., a white girl of 5, was admitted to the Harriet Lane Home on May 12, 1948 (Unit No. A 46571). This child was admitted to the Pediatric Department because of jerking of the left arm. She was an only child and apparently 4 months before her admission there had been a transitory closing of the left hand lasting about 4 minutes. This recurred 1 month later. About 2 weeks before admission she had attacks of weakness of the left arm and the left side of her face which were quite transient. For 2 weeks also she complained of double vision. Occasionally there was a complaint of pain in the left hand and the left side of her mouth.
Brain Tumors in Childhood
1369
Examination on admission showed a well-developed and well-nourished child. There was bilateral papilledema with hemorrhages in the right eye. There was a left sixth nerve palsy. No motor weakness or sensory loss or reflex change could be demonstrated. There was no cracked-pot sound. A ventriculogram was done revealing a right parietal tumor. At right craniotomy a large cellular meningioma, weighing 55.4 gm., was removed. The patient was discharged about 2 weeks later in good condition. The patient was readmitted to the hospital on August 9, 1948 and it was stated that 2 days before she had developed weakness of the left arm, following a fall. Following this there were headaches, drowsiness and vomiting. She now developed a cracked-pot sound and had an almost complete left hemiplegia. Reflexes were hyperactive on the left and there was a positive Babinski on that side. The old bone flap was again turned down and a soft, friable, recurrent tumor was removed. Again the patient did well following this procedure. X-ray therapy was instituted and although the child had a residual hemiplegia she was able to run about and play in fairly normal fashion for two and a half years, until overtaken by death in December of 1951.
Here is a case of malignant meningioma in a child, treated by irradiation with a survival period of three years. The mother of the child was a high-strung, perfectionistic, emotional woman, who almost literally "lived for her only baby." Psychiatrists predicted that the death of the patient would probably produce a most severe mental reaction in the mother and quite possibly suicide. The nearly three year survival period of the patient enabled another pregnancy to take place, with the result that, when death came for the patient, the blow was softened for both parents by the presence of another child, nearly two years of age. From a humanitarian point of view, surely the painless, not unhappy survival period was a boon rather than a burden. SUMMARY AND CONCLUSION
The purpose of this presentation has been to emphasize the fact that intracranial tumors in childhood are not necessarily hopeless, regardless of their apparent preoperative or even operative incurability. Tumors which are deemed inoperable by reason of their location or their statistically assumed histologic nature may sometimes be attacked by the neurosurgeon with gratifying results. The five cases described illustrated that the principles of humanitarianism demand operative exposure of all primary brain tumors in children. REFERENCES 1. Stern, R. 0.: Cerebral Tumors in Children. A Pathologic Report. Arch. Dis. Childhood. 12: 291, 1937. 2. Ford, F. R.: Diseases of the Nervous System in Infancy, Childhood, and Adolescence. 2nd Ed. Springfield, Ill., C. C Thomas, 1944, Chapter VIII.
1370
Frank J. Otenasek
3: 'Bailey, P., Buchanan, D. N. and Bucy, P. C.: Intracranial Tumors of Infancy
""'i'
and Childhood. Chicago, University of Chicago Press, 1939. 4: iStarr, M. A.: Tumors of the Brain in Childhood. Med. News 54: 29,1889. 5. Fiori, G.: La Sintomatologia del tumore cerebellare nei bambino. Riv. spero di freniat. 37: 407, 1911. 6. Critchley, MacD.: Brain Tumors in Children. Brit. J. Child. Dis. 22: 251,1925. 7. Garland, H. S. and Armitage, G.: Intracranial Tuberculoma. J. Path. & Bact. 37: 461, 1933.
ONE may be inclined to conclude from the experiences and impressions of older pediatricians and physicians that intracranial neoplasms occur relatively rarely in infancy and childhood. There are, moreover, few reliable sources of information which might tend to confirm or refute the assumption that such lesions are found only infrequently. Many of the estimates concerning the incidence of these lesions are derived from studies of series of patients made before the advent of Dandy's introduction of ventriculography as a 10ca1izing diagnostic procedure. Stern,! for example, records only 62 verified intracranial tumors in 2808 consecutive necropsies in the London Hospital for Sick Children, an incidence of only 2.2 per cent of the total. From the experience of Cushing, it was estimated that cerebral tumors occur only about one-sixth as frequently in childhood as they do in adult life. More recent observations would seem to indicate that such tumors are found in nearly the same proportion in children as they are in adults,2 or at least, that the number of intracranial tumors in childhood is greater than previously generally believed. 3 Pathological studies of the various types of intracranial tumors in children have revealed an uncomfortably high percentage of tumor types which are generally associated, in the average physician's mind, with incurability. Thus, in the oldest reports, the incidence of tuberculomas was so high that the diagnosis of a brain tumor in a child was almost synonymous with this infectious process and therefore with death. In an analysis by Starr,4 more than 50 per cent of the tumors were tuberculomas. Fiori's5 concepts of the symptomatology of posterior fossa tumors, published in 1911, were derived mostly from localized subtentorial tuberculous infections. The work of Critchley,6 in 1925, points up the tubercle as the most common intracranial tumor in childhood,
From the Division of Neurological Surgery, The Johns Hopkins University School of Medicine and The Johns Hopkins Hospital, Baltimore, Maryland.
* Assistant Professor of Neurosurgery, The Johns Hopkins University School Medicine; Visiting Neurosurgeon, The Johns Hopkins Hospital. 1363
0'
1364
Frank J. Otenasek
while as recently as 1933, Garland and Armitage7 felt that-at least in Leeds-about 66 per cent of such tumors were of acid-fast origin. However, Stern1 in 1937, reports that the incidence of intracranial tubercles was only in the neighborhood of 6 per cent. This was also the estimate of Cushing. Act~ally, at present the percentage of such lesions (in the United States at least) is so much lower that only rarely need serious consideration of an intracranial tuberculoma enter into the processes of differential diagnosis. Moreover, some of the recent encouraging reports on the effectiveness of streptomycin in tuberculosis of the central nervous system may help to allay the ancient prognostic fears associated with such a diagnosis. Consideration of intracranial pathological processes of expanding nature, other than infectious in type" occurring among children, reveal a striking preponderance of tumors of neuroepithelial origin, largely gliomas. Numerous .authorities place t~e incidence of these neoplasms in children in the rieighborh60d of 75' per cent. The term glioma, in the mind of the average pediatrician, is practically synonymous with death. And thus many physicians are loathe to subject a child, in whom they have made this diagnosis, to the "whims" of the neurosurgeon. Such an attitude of fatalism cannot help but be fostered by perusal of the best known treatise on intracranial tumors of infancy in childhood,3 from which the following may be quoted (page 440): "Ford concludes his study of intracranial tumors in childhood rather sadly by stating that 'the chances of discovering a benign tumor at operation are relatively small.' If by 'benign' he means a tumor which can be successfully and permanently removed without leaving the patient too crippled, the pathologist must agree with him. Not only do the records of surgical clinics prove it, but also the study of pathology teaches that it must be so." (Italics ours.) Obviously, careful pathological studies are of paramount importance in establishing the precise nature of tumors, but such studies alone do not justify far-reaching prognostic conclusions. Before such conclusions can be validly drawn there must be a combination of immense clinical experience and superb surgical technic with knowledge of the biology of the tumor in question. Such a combination is rare indeed. It is the primary purpose of this clinic to de-emphasize the fact that most intracranial tumors in children are malignant and inoperable, to show that some of those deemed so before operation prove, surprisingly enough, to be curable, and to emphasize that even tumors which at operation are believed to be "hopelessly inoperable" are sometimes compatible with some years of happy living. I shall endeavor to illustrate with a few cases, picked almost at random from the files of The Johns Hopkins Hospital.
Brain Tumors in Childhood
1365
CASE I. M. H., a white girl of 13 years, daughter of a physician, was admitted to the neurosurgical servive of Dr. Walter E. Dandy on July 30, 1942 (Unit!No. 265396). The chief complaint at the time of admission was headache and vomiting. Apparently nearly 2 years before her admission to the hospital the child had suffered moderately severe headaches occurring in episodes lasting for 1 or 2 days. The headaches were of dual character, being either generalized or localized largely to the occipital area. In February 1941 she was seen by an ophthalmologist in her home town and normal findings were recorded. For an indefinite period of time, probably almost a year, the patient was noticed to have had a mild tendency to stagger and also exhibited some degree of clumsiness. For 7 months she has had occasional tinnitus of the right ear. In January 1942, approximately 6 months before her admission, all the symptoms seemed to become considerably worse. About 10 days before her entry into the hospital here she had gone swimming and following this noticed very severe headaches, associated with constant vomiting. Physical examination on admission revealed a well-developed and well~ nourished young girl of 13. The general physical examination contributed little to her present illness. There was a suggestive cracked-pot sound over the calvarium. The inion did not appear to be higher than the expected position and therefore, the posterior fossa did not seem to be large. There was about 3 diopters of papilledema with hemorrhages in the right fundus. Her gait was a little unsteady. The Romberg test was negative. There was very slight bilateral ataxia and dysdiadochokinesis. The child was thought to have a probable cerebellar tumor although the possibility of a stricture at the aqueduct of Sylvius was discussed. A ventriculogram was done and this was followed by a bilateral suboccipital approach. A large cystic astrocytoma was found and the mural nodule removed from the posterior medial aspect of the right cerebellar hemisphere. The child made an uneventful recovery and is now well 10 years after the operative-procedure was carried out.
It is interesting that the presumptive diagnosis in this patient following ventriculography was medulloblastoma of the cerebellum because of the absence of lateralizing signs in the examination. While it is generally supposed that such cystic astrocytomas as this patient had are the most favorable of all intracranial tumors of childhood, it is not generally known that some neurosurgeons, on the basis of a presumptive diagnosis of a midline medulloblastoma of the cerebellum, will only half-heartedly search for such a tumor through a small midline suboccipital opening and then only for purposes of a biopsy. Such a course of action in the case cited would have been fatal. Yet the tumor was benign and removable. CASE II. J. S., a Belgian boy of 10 years, was admitted to the Harriet Lane Home of The Johns Hopkins Hospital on May 3, 1948 (Unit No. A 62575). This child was admitted to the Pediatric Department because of a complaint of headache and vomiting. Apparently, about 8 months before, he had begun to have morning vomiting occurring nearly daily, at first only after breakfast. Several weeks later this began to occur several times a day. About 3 months before his admission he developed severe generalized headaches. There were no other symptoms of any kind.
1366
Frank J. Otenasek
Physical examination showed a boy well developed and well nourished in spite of his frequent vomiting. Examination of the head revealed a cracked-pot sound and x-ray evidence of separation of the sutures. There was bilateral papilledema with hemorrhages. On deviation of the eyes to the right side there were horizontal nystagmoid movements of small amplitude. There was no definite weakness or ataxia, but station and gait were definitely unsteady and the child could not stand on one foot with the eyes open. A clinical diagnosis of posterior fossa tumor, probably medulloblastoma, was made, although because of the patient's age the possibility of astrocytoma was entertained. A ventriculogram confirmed the location of the lesion and at operation a medulloblastoma of the posterior fossa was subtotally removed. The child did well postoperatively and after 10 days deep x-ray therapy was begun, being given to each of 3 ports of the cerebellar and occipital area, while the entire spine was divided into 3 equal ports for purposes of irradiation. During his stay in the hospital and while undergoing radiotherapy the count of white blood cells in the blood dropped to 3000 and x-ray therapy had to be discontinued. After his discharge from the hospital, the course of therapy was con.tinued elsewhere. The child has now long· since returned to Europe and a letter from his father early in 1952, nearly 3 years after his operation, reveals that the child is attending school, is doing well, and leading a perfectly normal life.
This patient represents an instance of a child, hopelessly ill with a malignant tumor, who is still subjectively and objectively normal nearly four years after incomplete removal of the neoplasm. Although no one would be so brash as to assume that there is a significant chance of permanent cure in this case, the fact that the child is leading a normal life with hope of continuing so for a while longer leaves no doubt that the treatment accorded produced a result beyond the expectations of mere statistical tables. Not every medulloblastoma necessarily means death within a year. CASE III. M. H., a white boy of 6, was admitted to The Johns Hopkins Hospital, February 20, 1947 (Unit No. 414406), because of failing vision. His past history was not contributory. Apparently about 18 months before his hospital admission, the parents first noticed that the patient was unable to see clearly. He would grope about for objects when attempting to pick them up and he did not seem to be able to locate them accurately. Shortly thereafter, he began to bump into things at times, as though he had not noticed them. About a year before his admission, it was noted by the mother that his eyes were continually jerking from side to side and it was felt that he was becoming nervous. Six months before he came to the hospital he began to complain of headaches which were associated with vomiting. These symptoms gradually progressed to the time of his admission. Physical examination revealed a well-developed and well-nourished child who did not appear generally ill. The head was of normal size, shape, and configuration. There was a cracked-pot sound on the head to percussion over the coronal sutures. Examination of the eyes revealed grossly defective vision, being less than 5/50 in each eye. Small objects were not seen well. Ophthalmoscopically the disks were dead white and flat. There was no evidence of blurring of the
Brain Tumors in Childhood
1367
margins. The extraocular movements were well performed but there was a constant slow nystagmus of equal amplitude and rate in each direction. It was present at all points of fixation and no point of rest could be brought out. The remainder of the neurological examination was normal. X-rays of the skull confirmed the separation of the sutures and also showed erosion of the sella turcica, particularly of the anterior clinoid processes. The preoperative diagnosis 'was tumor of the optic chiasm and hypothalamus. A right frontal approach was carried out. As we slipped down under the frontal lobe in the neighborhood of the chiasm we encountered dense, hard, grayish tumor which was very vascular and filled the whole area. Neither optic nerve could be identified. According to the operative description, "It was clearly a hopeless tumor. Small portions of it were taken for biopsy but there will be no relief of either vision or hydrocephalus." The tumor was an astrocytoma. The child did well postoperatively. The parents were given a completely hopeless prognosis and it was felt that radiotherapy should be given more. for the benefit of the parents than actually for the benefit of the patient himself. He was next seen in October of 1947 at which time he looked well, had no headache or vomiting. The vision had improved both subjectively and objectively. Deep therapy was still being given by Dr. George Farber. No cracked-pot sound could be elicited. The patient was last seen in December 1951, at which time his vision was 5/40 in the right eye and 3/30 in the left. There was marked optic atrophy in both eyes. His nystagmus persisted. At this time he was attending a school for the blind and leading a happy life. This case illustrates the not uncommon pitfall of calling a tumor inoperable from its gross appearance, and even from its microscopic appearance when the histological picture does not suggest radiosensitivity. Furthermore, it was diagnosed preoperatively as a tumor of the optic chiasm and hypothalamus, while according to our authority,3 "Those (gliomas) of the optic chiasm and hypothalamus ... should be diagnosed only to be let alone, regardless of their structure." (Italics ours.) This child is alive and relatively well five years after the "hopeless" tumor was exposed. He and his family are happy with the result. In spite of the inadequacy of his vision, his intelligence is normal and his attendance at the School for the Blind may enable him to be useful if the lesion does not recur in the future. Again we emphasize that not all "hopeless" tumors are incompatible with the pursuit of happiness. CASE IV. K. B., a white girl of 16, was admitted to The Johns Hopkins Hospital April 17, 1947 (Unit No. 419559) because of headache, unsteadiness, and difficulty with vision. Apparently about 3 years before she developed some frontal headache which appeared to come on once or twice a month and lasted for perhaps half a day. These gradually became more severe and more frequent. About 2 years before her admission to the hospital, she noticed some difficulty in seeing the blackboard at school. Glasses were prescribed by an optometrist and apparently the reading defect was corrected. She was then fairly well until approximately 6 or 7 months before her hospital admission when the headaches became much more severe. They seemed to occur about once every 2 or 3 weeks
<
1368
Frank J. Otenasek
and were accompanied by nausea but no vomiting. Within a short time, the headaches became as frequent as once a week and required her to stay 1 to 2 days a week in bed. Blurring of vision and diplopia became severe in December of 1946 at which time vomiting began and was most severe in the morning. This was apparently arrested by a change in diet. For 3 months prior to her admission she noticed some unsteadiness on walking which was apparent to the patient as well as to other people. Physical examination showed early papilledema with normal and full visual fields. There was nystagmus on looking to either side, with the quick phase in the lateral position and the slow phase toward the midline. The remaining cranial nerves were entirely normal. There was no motor weakness or sensory loss. There was no evidence of ataxia or adiadochokinesis. The patient showed a little unsteadiness on turning, with a slight tendency to drift toward the right. A venticular air injection was done. On tapping there was a spurt of fluid under considerable pressure. Fluid in the amount of 120 cc. was obtained and a similar amount of air injected. The films showed a 'moderately advanced hydrocephalus. There was an outline of tumor in the fourth ventricle. A bilateral suboccipital approach was made. The cerebellar tonsils were found far down in the spinal canal. They were separated and as the operator entered the fourth ventricle a grayish, solid tumor, seemingly well circumscribed, was encountered. The vermis was split over a distance of nearly 2 cm. There was tumor everywhere infiltrating into both cerebellar hemispheres, a little more perhaps on the left side. The tumor was soft and friable. It was removed piece-' meal. Infiltration of the tumor took place into both hemispheres as well as into the floor of the fourth ventricle so that no line of cleavage could be discovered. The conclusion was reached that the tumor had probably arisen in the brain stem. It extended high up into the ventricle and it was impossible to obtain a free flow of fluid from the aqueduct of Sylvius. The patient had a very stormy postoperative course. Histological sections of the tumor revealed it to be an astrocytoma. She is now left with a residuum of rather marked ataxia of the left side and considerable difficulty in walking without support. However, she dances well, is able to drive her own car, and spent 1 year on the continent of Europe. It is now 5 years since the time of her operation. She has been leading a relatively normal and certainly a happy life. She was treated elsewhere with polysaccharides and teropterin, but the effect of these treatments cannot properly be evaluated. Nevertheless the fact remains' that the patient is now 21 years of age and is alive. This is simply another instance in which even failure to relieve hydrocephalus by operation did not prevent a happy five year survival. CASE V. M. S., a white girl of 5, was admitted to the Harriet Lane Home on May 12, 1948 (Unit No. A 46571). This child was admitted to the Pediatric Department because of jerking of the left arm. She was an only child and apparently 4 months before her admission there had been a transitory closing of the left hand lasting about 4 minutes. This recurred 1 month later. About 2 weeks before admission she had attacks of weakness of the left arm and the left side of her face which were quite transient. For 2 weeks also she complained of double vision. Occasionally there was a complaint of pain in the left hand and the left side of her mouth.
Brain Tumors in Childhood
1369
Examination on admission showed a well-developed and well-nourished child. There was bilateral papilledema with hemorrhages in the right eye. There was a left sixth nerve palsy. No motor weakness or sensory loss or reflex change could be demonstrated. There was no cracked-pot sound. A ventriculogram was done revealing a right parietal tumor. At right craniotomy a large cellular meningioma, weighing 55.4 gm., was removed. The patient was discharged about 2 weeks later in good condition. The patient was readmitted to the hospital on August 9, 1948 and it was stated that 2 days before she had developed weakness of the left arm, following a fall. Following this there were headaches, drowsiness and vomiting. She now developed a cracked-pot sound and had an almost complete left hemiplegia. Reflexes were hyperactive on the left and there was a positive Babinski on that side. The old bone flap was again turned down and a soft, friable, recurrent tumor was removed. Again the patient did well following this procedure. X-ray therapy was instituted and although the child had a residual hemiplegia she was able to run about and play in fairly normal fashion for two and a half years, until overtaken by death in December of 1951.
Here is a case of malignant meningioma in a child, treated by irradiation with a survival period of three years. The mother of the child was a high-strung, perfectionistic, emotional woman, who almost literally "lived for her only baby." Psychiatrists predicted that the death of the patient would probably produce a most severe mental reaction in the mother and quite possibly suicide. The nearly three year survival period of the patient enabled another pregnancy to take place, with the result that, when death came for the patient, the blow was softened for both parents by the presence of another child, nearly two years of age. From a humanitarian point of view, surely the painless, not unhappy survival period was a boon rather than a burden. SUMMARY AND CONCLUSION
The purpose of this presentation has been to emphasize the fact that intracranial tumors in childhood are not necessarily hopeless, regardless of their apparent preoperative or even operative incurability. Tumors which are deemed inoperable by reason of their location or their statistically assumed histologic nature may sometimes be attacked by the neurosurgeon with gratifying results. The five cases described illustrated that the principles of humanitarianism demand operative exposure of all primary brain tumors in children. REFERENCES 1. Stern, R. 0.: Cerebral Tumors in Children. A Pathologic Report. Arch. Dis. Childhood. 12: 291, 1937. 2. Ford, F. R.: Diseases of the Nervous System in Infancy, Childhood, and Adolescence. 2nd Ed. Springfield, Ill., C. C Thomas, 1944, Chapter VIII.
1370
Frank J. Otenasek
3: 'Bailey, P., Buchanan, D. N. and Bucy, P. C.: Intracranial Tumors of Infancy
""'i'
and Childhood. Chicago, University of Chicago Press, 1939. 4: iStarr, M. A.: Tumors of the Brain in Childhood. Med. News 54: 29,1889. 5. Fiori, G.: La Sintomatologia del tumore cerebellare nei bambino. Riv. spero di freniat. 37: 407, 1911. 6. Critchley, MacD.: Brain Tumors in Children. Brit. J. Child. Dis. 22: 251,1925. 7. Garland, H. S. and Armitage, G.: Intracranial Tuberculoma. J. Path. & Bact. 37: 461, 1933.