Glioblastoma multiforme in children GEOI~CE J. DOHRMANN, M.D., ProD., JACQUELINE R. FARWELL,M.D., AND JOHN T. FLANNERY, B.S. Sections of Neurosurgery and Neuropathology. and Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut Of 488 children with central nervous system neoplasms, 43 (8.8%) had glioblastomas, 22 of which were in the cerebral hemispheres, 16 in the brain stem, two in the cerebellum, and three in the spinal cord. The male to female ratio was 3:2. Glioblastoma multiforme of the cerebral hemispheres occurred at a mean age of 12.7 years, and the frontal lobe was the most commonly involved. Main presenting symptoms included headache (85%), nausea or vomiting (65%), and seizures (35%). Papilledema (45%) was the most common physical finding. The longest survivals were achieved by a combination of operation and radiation (22 months). Brain stem glioblastomas occurred at a mean age of 6.7 years, with the pons as the most frequent site. Nausea or vomiting (50%) and headache (36%) were the main presenting symptoms; the major physical findings were ataxia (43%), cranial nerve palsies (28%), and paresis (28%). The length of survival was greatest with radiation alone (10.5 months). The period of survival of children with glioblastoma multiforme was significantly increased with steroid therapy. Glioblastoma multiforme behaves similarly in children and adults. Intracranial glioblastomas have a more rapidly fatal course than that of other similarly situated gliomas in childhood. KEy W o R o s brain t u m o r
9 glioblastoma multiforme 9 spinal cord tumor
LIOBLASTOMA multiforme occurs less | , ~ often in children than in adults.,8 38 In large series of patients with glioblastomas, less than 3% occurred in children (Table 1). Glioblastomas accounted for approximately 7% of all intracranial neoplasms of childhood (Table 2). Because the number of glioblastoma patients younger than 20 years of age is so small, the natural history and survival of children with glioblastoma multiforme have not been well documented. The purpose of the present study is to review and analyze cases of glioblastoma multiforme that have occurred in children in Connecticut during a 39-year period (1935 to 1973). 442
9
childhood glioblastoma
9
TABLE 1 Incidence ofglioblastoma multiforme in children reported in nine large series Author, Year
Total Cases
Children No. 7o
Busch& Christensen, 1947 Davis, et al., 1949 LCken,1952 Marsh, 1956 Earle, et al., 1957 Frankel & German, 1958 Bouchard & Pierce, 1960 Taveras, et al., 1962 Jelsma& Bucy, 1967 total
102 211 164 272 167 219
2 4 13 10 7 2
2.0 1.9 7.9 3.7 4.2 0.9
125
1
0.8
425 162 1847
8 1 48
1.9 0.6 2.6
J. Neurosurg. / Volume 44 / April, 1976
Glioblastoma multiforme in children Materials and Methods T h e files o f t h e C o n n e c t i c u t T u m o r R e g i s t r y , 7 w h i c h c o n t a i n r e p o r t s o f all d i a g n o s e d cancer patients in C o n n e c t i c u t hospitals and their lifelong follow-up, were searched for cases o f h i s t o l o g i c a l l y c o n f i r m e d g l i o b l a s t o m a m u l t i f o r m e occurring in persons younger t h a n 20 y e a r s o f age over a 39-year
p e r i o d (1935 to 1973). E a c h c a s e was analyzed in relation to age, sex, l o c a t i o n o f n e o p l a s m , d a t e o f onset of s y m p t o m s , d a t e o f diagnosis, presenting symptoms, main physical findings, t r e a t m e n t s , and p e r i o d o f survival after diagnosis. In instances where the diagnosis o f g l i o b l a s t o m a m u l t i f o r m e was not m a d e by a n e u r o p a t h o l o g i s t , the slides, when available, were reviewed by one o f the authors.
TABLE 2
Results
b~cidence of glioblastoma multifi)t~Te in 18 large series oJ' #ltracranial neoplasms i~t children*
O f a t o t a l o f 488 central nervous system n e o p l a s m s in children, 43 g l i o b l a s t o m a s (8.8%) w e r e n o t e d . F o r t y o c c u r r e d int r a c r a n i a l l y (8.6% o f c h i l d h o o d i n t r a c r a n i a l tumors), and three g l i o b l a s t o m a s were intraspinal. The m a l e to female r a t i o was 3:2. T h i r t y - f o u r children were t r e a t e d with o p e r a tion a n d / o r r a d i a t i o n and nine children were not treated. A l l affected children have died (Table 3).
Author, Year
Age (yrs)
Cushing, 1927 Z(ilch, 1937 Bailey, et al., 1939 French, 1948 Keith, et al., 1949 Walker & Hopple, 1949 L~bken, 1952 Miller, et al., 1952 Bodian & Lawson, 1953 Odom, et al., 1956 Katsura, et al., 1959 Troupp, 1960 Backus & Millichap, 1962
< 15
9 glioblastoma multiforme 9 spinal cord tumor
LIOBLASTOMA multiforme occurs less | , ~ often in children than in adults.,8 38 In large series of patients with glioblastomas, less than 3% occurred in children (Table 1). Glioblastomas accounted for approximately 7% of all intracranial neoplasms of childhood (Table 2). Because the number of glioblastoma patients younger than 20 years of age is so small, the natural history and survival of children with glioblastoma multiforme have not been well documented. The purpose of the present study is to review and analyze cases of glioblastoma multiforme that have occurred in children in Connecticut during a 39-year period (1935 to 1973). 442
9
childhood glioblastoma
9
TABLE 1 Incidence ofglioblastoma multiforme in children reported in nine large series Author, Year
Total Cases
Children No. 7o
Busch& Christensen, 1947 Davis, et al., 1949 LCken,1952 Marsh, 1956 Earle, et al., 1957 Frankel & German, 1958 Bouchard & Pierce, 1960 Taveras, et al., 1962 Jelsma& Bucy, 1967 total
102 211 164 272 167 219
2 4 13 10 7 2
2.0 1.9 7.9 3.7 4.2 0.9
125
1
0.8
425 162 1847
8 1 48
1.9 0.6 2.6
J. Neurosurg. / Volume 44 / April, 1976
Glioblastoma multiforme in children Materials and Methods T h e files o f t h e C o n n e c t i c u t T u m o r R e g i s t r y , 7 w h i c h c o n t a i n r e p o r t s o f all d i a g n o s e d cancer patients in C o n n e c t i c u t hospitals and their lifelong follow-up, were searched for cases o f h i s t o l o g i c a l l y c o n f i r m e d g l i o b l a s t o m a m u l t i f o r m e occurring in persons younger t h a n 20 y e a r s o f age over a 39-year
p e r i o d (1935 to 1973). E a c h c a s e was analyzed in relation to age, sex, l o c a t i o n o f n e o p l a s m , d a t e o f onset of s y m p t o m s , d a t e o f diagnosis, presenting symptoms, main physical findings, t r e a t m e n t s , and p e r i o d o f survival after diagnosis. In instances where the diagnosis o f g l i o b l a s t o m a m u l t i f o r m e was not m a d e by a n e u r o p a t h o l o g i s t , the slides, when available, were reviewed by one o f the authors.
TABLE 2
Results
b~cidence of glioblastoma multifi)t~Te in 18 large series oJ' #ltracranial neoplasms i~t children*
O f a t o t a l o f 488 central nervous system n e o p l a s m s in children, 43 g l i o b l a s t o m a s (8.8%) w e r e n o t e d . F o r t y o c c u r r e d int r a c r a n i a l l y (8.6% o f c h i l d h o o d i n t r a c r a n i a l tumors), and three g l i o b l a s t o m a s were intraspinal. The m a l e to female r a t i o was 3:2. T h i r t y - f o u r children were t r e a t e d with o p e r a tion a n d / o r r a d i a t i o n and nine children were not treated. A l l affected children have died (Table 3).
Author, Year
Age (yrs)
Cushing, 1927 Z(ilch, 1937 Bailey, et al., 1939 French, 1948 Keith, et al., 1949 Walker & Hopple, 1949 L~bken, 1952 Miller, et al., 1952 Bodian & Lawson, 1953 Odom, et al., 1956 Katsura, et al., 1959 Troupp, 1960 Backus & Millichap, 1962
< 15
