J Neurosurg 48:443-449, 1978
Ipsilateral cerebral atrophy with thalamic tumor of childhood Case report
RYUNGCHAN KWAK, M.D., SHUN-ICHI SASO, M.D., AND JIRO SUZUKI, M . D .
Division of Neurosurgery, Institute of Brain Diseases, Tohoku University School of Medicine, Sendal, Japan v" A case of cerebral tumor with ipsilateral cerebral hemiatrophy is presented and five previously reported cases are reviewed. The etiology of this entity is discussed on the basis of the symptomatological and clinicopathological findings noted in these six cases. It is suggested that ipsilateral cerebral hemiatrophy is due to thinning of the cerebral cortex with degeneration and disappearance of ganglion cells, demyelination in the subcortex, and destruction of axons. The'mechanism proposed for ipsilateral cerebral hemiatrophy due to thalamic tumor is that thalamic tumor causes degeneration and disappearance of thalamic ganglion cells and nerve fibers, with consequent secondary Wallerian degeneration of afferent and projecting fibers from the thalamus as well as retrograde degeneration of efferent fibers, thus resulting in extensive atrophy of the cerebral cortex and subcortical tissue. KEY W o a D s 9 ectopic pinealoma 9 ipsilateral cerebral hemiatrophy thalamic tumor 9 brain tumor in children
HIS report describes a case of brain tumor with ipsilateral cerebral hemiatrophy, and reviews five previously reported cases, T M three of them ours. An explanation is presented for the relationship between slowly growing thalamic tumors and ipsilateral cerebral hemiatrophy.
T
Case Report
In August, 1966, at the age of 8 years, this boy noticed slight paresis of the left arm. Seven months later he developed paresis of his left leg and he was admitted to the pediatric clinic o f T o h o k u University Hospital in M a r c h , 1967. Right-sided cerebral hemiatrophy was suspected on carotid angiogram and pneumoencephalogram,
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but an exact diagnosis was not made. H e was discharged 11/2 m o n t h s later, but his neurological symptoms gradually became more severe. On March 6, 1969, the patient had a transient episode of stupor and was admitted to our clinic the next day. Examination. Hemiparesis and pathological reflexes were noted on the left side, anisocoria (greater on the right than the left), loss of light reflex on the right side, slight paresis on the right oculomotor muscles, and horizontal nystagmus. There were no pathological findings in the cerebrospinal fluid. Cerebral hemiatrophy on the right side was suspected on carotid angiogram (Fig. 1 left), pneumoencephalogram (Fig. 1 right), and pneumotomogram. According to our pre443
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R. Kwak, S.-I. Saso and J. Suzuki
FIG. 1. Left." Right carotid angiogram, anteroposterior view, shows slight shift of anterior cerebral artery from the left to right. Right."Pneumoencephalogram, anteroposterior view, shows slight shift to right side (tumor side) in the lateral and third ventricles. Air was apparent in the subarachnoid space, more on the right side than the left. Cerebral hemiatrophy on the right side was suspected.
FIG. 2. Left: Frontal section of brain at the level of the mamillary body. The tumor is located in the hypothalamus and thalamus on the right side with invasion of the capsula interna, nucleus lentiformis, and nucleus caudatus. Right: Frontal section through the trigone of the lateral ventricle. Dilatation of the ventricle system was marked on the right side. The right hemisphere was smaller than the left, and cerebral hemiatrophy of the right side is obvious. Arrow indicates tumor in the right hypothalamus.
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Cerebral atrophy with thalamic tumors vious report, it was thought that cerebral hemiatrophy with the neurological symptoms on the opposite side indicated ipsilateral thalamic tumor, but tumor shadow was not obvious. The patient was discharged without surgical treatment. His condition deteriorated and he died of acute pneumonia on October 9, 1969. Postmortem Examination. On autopsy, an invasive process was found that had destroyed the thalamus and hypothalamus on the right side (Fig. 2 left). Further diffuse cerebral atrophy and dilatation of the lateral ventricle was observed in the right cerebral hemisphere (Fig. 2 right). In other organs, acute bilateral hemorrhagic pneumonia and marked atrophy of the frontal lobe of the hypophysis, adrenal cortex, and thyroid gland were the main pathological findings. On histological examination, the neoplasm was a two-cell-pattern pinealoma (Fig. 3 left) with giant astrocytes and perivascular roundcell infiltration (Fig. 3 right). In the right cerebral hemisphere, ganglion cells had partly disappeared or degenerated in the cortex (Fig. 4 upper left), and demyelination and destruction of axons (Fig. 4 upper right) were marked in the white matter of the temporal lobe (Fig. 4 lower left). In this area, proliferation of giant astrocytes and glial fibers was remarkable (Fig. 4 lower right). These pathological findings were in agreement with the microscopic diagnosis of cerebral hemiatrophy of the right side. There were no
pathological findings in the pineal gland itself (Fig. 5). Discussion
The six reported cases of brain tumor with overt ipsilateral cerebral hemiatrophy2 5,7 are summarized in Table 1. The onset of the disease was noted when the patients were between 8 and 14 years of age, with a mean of 11 years and 8 months. Duration of illness between onset and admission to the hospital ranged from 1 year and 2 months to 4 years, with an average of a little over 2 years and 1 month, showing a relatively chronic disease progression. Primary signs were marked decline of schoolwork in five cases, hemiparesis in four cases, and an episode of unconsciousness in one case, all of which were progressive throughout the course. The principal symptoms were hemiparesis, hemihypesthesia, character and emotional changes, deterioration of mental faculties, and behavioral abnormalities. It was interesting that none of the cases showed signs or symptoms of increased intracranial pressure before admission, and no papilledema was observed at the time of admission. Ipsilateral cerebral hemiatrophy on the tumor side was evidenced by carotid angiography and pneumoencephalography (Fig. 1) and by exploratory craniotomy. Histopathological examination of the lesion revealed pinealoma in all six cases, of two-cell pattern in five cases and of one-cell pattern in
FIG. 3. Left: Photomicrograph of the tumor, showing the two-cell pattern of a pineaioma. H & E, • 200. Right: Photomicrograph showing giant astroglia in the surrounding tumor. Holzer, • 100.
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R. Kwak, S.-I. Saso and J. Suzuki TABLE I Six cases of ectopic pinealoma with ipsilateral cerebral hemiatrophy
Case No.
Author, Year
Sex, Age on Onset
Period Onset to Admission
Symptoms* Initial Symptoms
Motor Hemiparesis
CharacterOther istic and Demen- Abnormal Emotional tia Behavior Symptoms Change
1 Suzuki, et al., 1968
M, 11 yrs 9 mos
1 yr rt hemi5 mos paresis, schoolwork deteriorated
rt
no
2 Kwak & Suzuki, 1970
M, 13 yrs 4 mos
1 yr It hemi6 mos paresis, schoolwork deteriorated
It
3 Kwak, et al., 1974
M, 8 yrs 7 mos
2 yrs It hemi6 mos paresis, schoolwork deteriorated
It
4 Kwak, et al., 1978
M, 9 yrs 2 mos
1 yr It hemi2 mos paresis, schoolwork deteriorated
It
careless, restless, talkative, superficial, ready wit
5 Numabe & Kamioka, 1963
M, 14 yrs
4 yrs
schoolwork deteriorated
rt
careless, yes restless, argumentative, violent, talkative, cheerful, superficial
6 Nakagawa, et al., 1973
F, 13 yrs
2 yrs
period of unconsciousness
It
careless, restless, superficial, impulsive, ready wit no
no
yes
no
yes
wandering, theft
yes
no
no
wandering, erotic behavior
no
consciousness disturbance
wandering, seizure incendiary, pass water, queer voice
no
It homonymous hemianopsia, diabetes insipidus
*None of the patients had symptoms of increased intracranial pressure.
the other. T h e p i n e a l b o d y was n o t e d to be n o r m a l in all t h r e e a u t o p s y cases; e c t o p i c p i n e a l o m a was a l s o s u s p e c t e d clinically in the t h r e e r e m a i n i n g cases. T h e c o m m o n site o f t u m o r in this series was t h e t h a l a m u s a n d its surrounding areas. T h e m i c r o s c o p i c a l l y p r o m i n e n t features o f the a f f e c t e d a r e a i n c l u d e d p r o l i f e r a t i o n o f g i a n t a s t r o g l i a w i t h i n t h e t u m o r a n d in t h e 446
a d j o i n i n g a r e a s (Fig. 3 right), g e n e r a l t h i c k e n ing o f t h e c e r e b r a l c o r t e x with d e g e n e r a t i o n a n d d e s t r u c t i o n o f g a n g l i o n cells in t h e r e l a t i v e l y s u p e r f i c i a l layer, a m a r k e d s u b c o r tical demyelination, disintegration of axons, and a marked proliferation of neuroglia and n e u r o g l i a l fibers in this a r e a (Fig. 4). It h a s been r e p o r t e d by G r e e n f i e l d , et al., 1 t h a t r e t r o g r a d e a t r o p h y of t h e t h a l a m u s m a y J. Neurosurg. / Volume 48 / March, 1978
Cerebral atrophy with thalamie tumors TABLE 1 (Continued) Examination Pressure of Lumbar Radiological Puncture Findings (ram H~O)
Tumor Histological Findings
Localization
Pineal Gland
Treatment
Clinical Course & Results
280
It cerebral one-cell-pattern hemiatrophy pinealoma
It thalamus temporal & occipital lobe
?
subtotal extirpation 6 ~ irradiation (4750 rads)
120
rt cerebral two-cell-pattern hemiatrophy pinealoma
rt thalamus
?
linac irradiation improved, but (7000 rads) died with uremia after 3 yrs 4 mos; no autopsy
140
rt cerebral two-cell-pattern hemiatrophy pinealoma
rt thalamus & hypothalamus
250
rt cerebral two-cell-pattern hemiatrophy pinealoma
rt thalamus
170
It cerebral two-cell-pattern hemiatrophy pinealoma
It thalamus, hypothalamus, nuc. caudatus, capsula int., corpus mamillare
70
rt cerebral two-cell-pattern hemiatrophy pinealoma
rt lateral vent., normal nuc. caudatus, thalamus, hypothalamus, capsula int., corpus callosum
normal
?
normal
none
improved, no evidence of recurrence after 10 yrs 10 mos
aggravated, died after 3 yrs 2 mos; autopsy
BAR therapy ~ improved, but ~0Co irradiation died after 3 yrs 4 hod:]" 4960 mos; no autopsy rads; spinal cord: 4800 rads; decompressed craniotomy re-irradiation (5200 rads) none
aggravated, died with convulsive attack after 5 yrs; autopsy
irradiation
aggravated, died after 3 yrs, autopsy
~BAR = BUdR-antimetabolite continuous intraarterial infusion-radiation therapy. 6
arise from disorders o f the cerebral cortex. The h i s t o p a t h o l o g i c a l findings noted in our Case 3, described here, seem to support our previously r e p o r t e d view on the pathogenesis of ipsilateral cerebral h e m i a t r o p h y associated with brain t u m o r ? T h a t is: 1) thalamic t u m o r causes d e g e n e r a t i o n and disappearance of thalamic g a n g l i o n cells and nerve fibers; 2) secondary W a l l e r i a n degeneration of afferent
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and projecting fibers f r o m the t h a l a m u s occurs, as well as r e t r o g r a d e d e g e n e r a t i o n o f efferent fibers; thus 3) extensive a t r o p h y o f the cerebral cortex and subcortical tissue results (Fig. 6). In slowly growing unilateral t u m o r s o f the thalamus, we think it is likely t h a t unilateral cerebral h e m i a t r o p h y is b r o u g h t a b o u t by the neoplastic g r o w t h in a n y kind o f t u m o r . In 447
R. Kwak, S.-I. Saso and J. Suzuki
FIG. 4. Upper Left." Photomicrograph showing degenerated ganglion cells in right parietal cortex. Nissl, X 200. Upper Right." Photomicrograph showing damaged axons in the subcortex of the right temporal lobe. Bodian, X 100. Lower Left: Autopsy section showing demyelination of the subcortex of the right temporal lobe. Klfiver-Barrera, X 1.5. Lower Right: Photomicrograph showing proliferation of giant glia cells and glial fibers at demyelinated part in the subcortex of the right temporal lobe. Hol . . . . X 200.
FIG. 5. Photomicrograph of pineal gland shows normal structure. H & E, X 400. 448
FIG. 6. Diagram of our speculated mechanism of ipsilateral cerebral hemiatrophy due to thalamic tumor.
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Cerebral atrophy with thalamie tumors cases such as glioma in the thalamus with rapid growth, however, it seems that the patient's condition usually deteriorates and he succumbs to increased intracranial pressure before the development of cerebral atrophy. References
1. Blackwood W, McMenemey WH, Meyer A, et al: Greenfield's Neuropathology. London: Edward Arnold, 1963, pp 404--405 2. Kwak R, Saso S, Suzuki J, et ah [An autopsy case of ectopic pinealoma with ipsilateral cerebral hemiatrophy.] Clin Neurol 14: 623-630, 1974 (Jpn) 3. Kwak R, Suzuki J: [Brain tumor with ipsilateral cerebral hemiatrophy.] Brain Nerve (Tokyo) 22:1439-1446, 1970 (Jpn) 4. Nakagawa Y, Hamazima I, Iwasaki Y, et ah [Ipsilateral cerebral atrophy caused by ectopic
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pinealoma.] Brain Nerve (Tokyo) 25:69-75, 1973 (Jpn) 5. Numabe T, Kamioka Y: [A case of ectopic pinealoma.] Brain Nerve (Tokyo) 15:793-800, 1963 (Jpn) 6. Sano K, Sato F, Hoshino T, et al: Experimental and clinical studies of radiosensitizers in brain tumors, with special reference to BUdRantimetabolite continuous regional infusionradiation therapy (BAR therapy). Neurol Med Chit 7:51-72, 1965 7. Suzuki J, Kwak R, Takaku A et al: [A case of ectopic pinealoma with a peculiar course.] Brain Nerve (Tokyo) 20:169-174, 1968 (Jpn) Address reprint requests to: Ryungchan Kwak, M.D., Division of Neurosurgery, Institute of Brain Diseases, Tohoku University School of Medicine, 5-13-1, Nagamachi, Sendai, Japan.
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