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Case report: Painless thoracic scoliosis due to dumb-bell ganglioneuroma — CT and MRI appearances
Clinical Radiology (1991) 44, 359-360
Case Report: Painless Thoracic Scoliosis Due to Dumb-Bell Ganglioneuroma- CT and MRI Appearances M. A. S A M P S O N ,
R. M I T C H E L L
a n d T. R. M O R E L Y *
Departments of Radiology and *Orthopaedics, Royal National Orthopaedic Hospital, Stanmore W e report a case of a child with a painless progressive scoliosis, initially diagnosed as idiopathic, who was demonstrated on computed tomography and magnetic resonance to have an extensive dumb-bell tumour of the thoracic spine, subsequently shown to be a ganglioneuroma. S a m p s o n , M . A . , M i t c h e l l , R. & M o r e l y , T . R . (1991). Clinical Radiology 44, 3 5 9 - 3 6 0 . C a s e R e p o r t : P a i n l e s s T h o r a c i c Scoliosis D u e to D u m b - B e l l G a n g l i o n e u r o m a - C T a n d M R I A p p e a r ances
CASE REPORT This 12-year-old girl presented with 18 months painless thoracic scoliosis, noticed by her mother and ballet teacher to be progressive. Her previous development had been normal with no significant past history of illness. On examination she was found to be tall and slim with an upper thoracic scoliosis convex to tlle right. There were no other deformities and no skin lesions or other visible stigmata. Radiographs showed a thoracic scoliosis to the right associated with a paravertebral mass (Fig. 1) confirmed on the lateral view. Computed tomography (CT) using a GE 9000 scanner and 5 mm slice thickness demonstrated bony erosion associated with the mass, which was of soft tissue density, along with widening of the spinal canal (Fig. 2). The aorta was displaced and encased but not obviously involved. Magnetic resonance imaging (MRI) on a 0.5 T machine using both short (TE26) and long (TE80) echo times and a slice thickness of 5 mm in the axial, and 10 mm in the sagittal and coronal planes was carried out. These features were confirmed and, in addition, demonstrated that the mass had an extensive intraspinal component, deviating the cord to the left, and was continuous with the extraspinal component via the enlarged exit foramina from T4 to T7 (Fig. 3). Open biopsy was performed, and histology showed a well differentiated ganglioneuroma. A joint thoracic and neurosurgical approac h is planned. At present, the patient is neurologically normal and on the stage as a dancer. This further compounds the difficulty in the timing of operation. The risk of paraplegia is estimated at approximately 15%; prevarication may lead to added problems in the attempt to resect the tumour.
DISCUSSION Ganglioneuromas c o m p r i s e a p p r o x i m a t e l y 1% o f t u m o u r s l o c a t e d at o r n e a r the spinal c o r d ( N i t t n e r , 1975). They share a common origin with neuroblastomas and g a n g l i o n e u r o b l a s t o m a s f r o m the p r i m i t i v e cells o f the n e u r a l crest. N e u r o b l a s t o m a s are p r i m i t i v e , u n d i f f e r e n t i a t e d cells w i t h a h i g h m a l i g n a n t p o t e n t i a l ; g a n g l i o n e u r o m a s are b e n i g n t u m o u r s at the o t h e r e n d o f t h e s p e c t r u m - o f d i f f e r e n t i a t i o n a n d are c o m p o s e d o f g a n g l i o n cells a n d n e r v e b u n d l e s . G a n g l i o n e u r o b l a s t o m a s are i n t e r m e d i a t e between the two. Approximately 50% of ganglioneuromas occur below the d i a p h r a g m , o f t e n j u x t a p o s e d to t h e a d r e n a l gland_ Sixty p e r c e n t o c c u r b e f o r e the age o f 20 a n d t h e r e is a slight m a l e p r e p o n d e r a n c e ( S t o v e n s , 1957). O n l y o n e p r e v i o u s c a s e o f g a n g l i o n e u r o m a a n d spinal scoliosis has b e e n r e p o r t e d in t h e l i t e r a t u r e to t h e k n o w l e d g e o f t h e Correspondence to: Dr M. A. Sampson, Department of Radiology, Southampton General Hospital, Tremona Road, Southampton.
Fig. 1 - AP view of the thoracic spine showing a scoliosis convex to the right with a paravertebral soft tissue mass. a u t h o r s ( B a u e r et al., 1989). In this case a 16-year-old girl was d i a g n o s e d a s i d i o p a t h i c scoliosis a n d t r e a t e d w i t h a cast-brace and physiotherapy for 4 years before destruct i o n o f the s e c o n d , t h i r d a n d f o u r t h l u m b a r v e r t e b r a l b o d i e s was seen a n d the true n a t u r e o f the lesion
360
CLINICAL RADIOLOGY
Fig. 2 CT image at the level of the carina showing the paravertebral soft tissue mass, bony erosion of the right side of the vertebral body, pedicle and transverse process and widening of the spinal~canal.
(0 Fig. 3 (cont.)
(a)
i n v e s t i g a t e d . I n t e r e s t i n g l y , in this p r e v i o u s l y r e p o r t e d case the progressive scoliosis was p a i n f u l , a n d u l t i m a t e l y d e m o n s t r a t e d n e u r o l o g i c a l signs. T h e case p r e s e n t e d here was also initially believed to be i d i o p a t h i c , b u t was painless. B o t h w r o n g d i a g n o s i s a n d clinical m a n a g e m e n t in the d u m b - b e l l g a n g l i o n e u r o m a s h a v e b e e n r e p o r t e d ( S h e p h a r d a n d S u t t o n , 1958; B a u e r et al_, 1989). T h e need to e x a m i n e the i n t r a s p i n a l s i t u a t i o n i n all cases b y C T m y e l o g r a p h y has b e e n e m p h a s i z e d ( K l i m m e r a n d H e c k e r , 1988). W e suggest t h a t if M R I is a v a i l a b l e this s h o u l d be the i n v e s t i g a t i o n o f choice in d e l i n e a t i n g the e x t e n t o f i n t r a s p i n a l disease a n d the state o f the spinal cord, as this assists the surgical p l a n n i n g . REFERENCES
(b) Fig. 3 Spin echo TR 1000 TE 26 ms MR images in the (a) axial, (b) coronal and (c) sagittal planes, demonstrating the extensive intra- and extraspinal extent of tumour passing through the intervertebral foramina. The compressed thoracic cord deviated to the left side of the spinal canal is shown in (a) (arrow).
Bauer, BL, Bauer, H, Griss, P, Lutcke, A, Maroske, D, Mennel, HD el al. (1989). Dumb-bell ganglioneuroma of the spine misinterpreted as progressive idiopathic scoliosis. Archives of Orthopaedic and Traumatic Surgery, 108, 189-I94. Nittner, K (1975)i Spinal meningiomas, neuromas and neurofibromas, and hourglass tumours. In: Handbook of Clinical Neurology, Tumours of the Spine and Spinal Cord, Part II, Chap. 7, eds. Vinken, PJ & Bruyn, GW, pp. 177 322. Springer-Verlag, Berlin. Shephard, RH & Sutton, D (1958). Dumb-bell ganglioneuromata of the spine with a report of four cases. British Journal of Surgery, 45, 305317. Stovens, D (1957). Neuroblastoma and related tumours. Archives of Pathology, 63, 451. Klimmer, M & Hecker, WC (1988). Zur Klinik van Neuroblastomen und Ganglioneuromen mit-intraspinalar Beteiligung. Zeitschriftfiir Kinderchirurgie, 43, 343-346. (English abstract on Medline.)
Case Report: Painless Thoracic Scoliosis Due to Dumb-Bell Ganglioneuroma- CT and MRI Appearances M. A. S A M P S O N ,
R. M I T C H E L L
a n d T. R. M O R E L Y *
Departments of Radiology and *Orthopaedics, Royal National Orthopaedic Hospital, Stanmore W e report a case of a child with a painless progressive scoliosis, initially diagnosed as idiopathic, who was demonstrated on computed tomography and magnetic resonance to have an extensive dumb-bell tumour of the thoracic spine, subsequently shown to be a ganglioneuroma. S a m p s o n , M . A . , M i t c h e l l , R. & M o r e l y , T . R . (1991). Clinical Radiology 44, 3 5 9 - 3 6 0 . C a s e R e p o r t : P a i n l e s s T h o r a c i c Scoliosis D u e to D u m b - B e l l G a n g l i o n e u r o m a - C T a n d M R I A p p e a r ances
CASE REPORT This 12-year-old girl presented with 18 months painless thoracic scoliosis, noticed by her mother and ballet teacher to be progressive. Her previous development had been normal with no significant past history of illness. On examination she was found to be tall and slim with an upper thoracic scoliosis convex to tlle right. There were no other deformities and no skin lesions or other visible stigmata. Radiographs showed a thoracic scoliosis to the right associated with a paravertebral mass (Fig. 1) confirmed on the lateral view. Computed tomography (CT) using a GE 9000 scanner and 5 mm slice thickness demonstrated bony erosion associated with the mass, which was of soft tissue density, along with widening of the spinal canal (Fig. 2). The aorta was displaced and encased but not obviously involved. Magnetic resonance imaging (MRI) on a 0.5 T machine using both short (TE26) and long (TE80) echo times and a slice thickness of 5 mm in the axial, and 10 mm in the sagittal and coronal planes was carried out. These features were confirmed and, in addition, demonstrated that the mass had an extensive intraspinal component, deviating the cord to the left, and was continuous with the extraspinal component via the enlarged exit foramina from T4 to T7 (Fig. 3). Open biopsy was performed, and histology showed a well differentiated ganglioneuroma. A joint thoracic and neurosurgical approac h is planned. At present, the patient is neurologically normal and on the stage as a dancer. This further compounds the difficulty in the timing of operation. The risk of paraplegia is estimated at approximately 15%; prevarication may lead to added problems in the attempt to resect the tumour.
DISCUSSION Ganglioneuromas c o m p r i s e a p p r o x i m a t e l y 1% o f t u m o u r s l o c a t e d at o r n e a r the spinal c o r d ( N i t t n e r , 1975). They share a common origin with neuroblastomas and g a n g l i o n e u r o b l a s t o m a s f r o m the p r i m i t i v e cells o f the n e u r a l crest. N e u r o b l a s t o m a s are p r i m i t i v e , u n d i f f e r e n t i a t e d cells w i t h a h i g h m a l i g n a n t p o t e n t i a l ; g a n g l i o n e u r o m a s are b e n i g n t u m o u r s at the o t h e r e n d o f t h e s p e c t r u m - o f d i f f e r e n t i a t i o n a n d are c o m p o s e d o f g a n g l i o n cells a n d n e r v e b u n d l e s . G a n g l i o n e u r o b l a s t o m a s are i n t e r m e d i a t e between the two. Approximately 50% of ganglioneuromas occur below the d i a p h r a g m , o f t e n j u x t a p o s e d to t h e a d r e n a l gland_ Sixty p e r c e n t o c c u r b e f o r e the age o f 20 a n d t h e r e is a slight m a l e p r e p o n d e r a n c e ( S t o v e n s , 1957). O n l y o n e p r e v i o u s c a s e o f g a n g l i o n e u r o m a a n d spinal scoliosis has b e e n r e p o r t e d in t h e l i t e r a t u r e to t h e k n o w l e d g e o f t h e Correspondence to: Dr M. A. Sampson, Department of Radiology, Southampton General Hospital, Tremona Road, Southampton.
Fig. 1 - AP view of the thoracic spine showing a scoliosis convex to the right with a paravertebral soft tissue mass. a u t h o r s ( B a u e r et al., 1989). In this case a 16-year-old girl was d i a g n o s e d a s i d i o p a t h i c scoliosis a n d t r e a t e d w i t h a cast-brace and physiotherapy for 4 years before destruct i o n o f the s e c o n d , t h i r d a n d f o u r t h l u m b a r v e r t e b r a l b o d i e s was seen a n d the true n a t u r e o f the lesion
360
CLINICAL RADIOLOGY
Fig. 2 CT image at the level of the carina showing the paravertebral soft tissue mass, bony erosion of the right side of the vertebral body, pedicle and transverse process and widening of the spinal~canal.
(0 Fig. 3 (cont.)
(a)
i n v e s t i g a t e d . I n t e r e s t i n g l y , in this p r e v i o u s l y r e p o r t e d case the progressive scoliosis was p a i n f u l , a n d u l t i m a t e l y d e m o n s t r a t e d n e u r o l o g i c a l signs. T h e case p r e s e n t e d here was also initially believed to be i d i o p a t h i c , b u t was painless. B o t h w r o n g d i a g n o s i s a n d clinical m a n a g e m e n t in the d u m b - b e l l g a n g l i o n e u r o m a s h a v e b e e n r e p o r t e d ( S h e p h a r d a n d S u t t o n , 1958; B a u e r et al_, 1989). T h e need to e x a m i n e the i n t r a s p i n a l s i t u a t i o n i n all cases b y C T m y e l o g r a p h y has b e e n e m p h a s i z e d ( K l i m m e r a n d H e c k e r , 1988). W e suggest t h a t if M R I is a v a i l a b l e this s h o u l d be the i n v e s t i g a t i o n o f choice in d e l i n e a t i n g the e x t e n t o f i n t r a s p i n a l disease a n d the state o f the spinal cord, as this assists the surgical p l a n n i n g . REFERENCES
(b) Fig. 3 Spin echo TR 1000 TE 26 ms MR images in the (a) axial, (b) coronal and (c) sagittal planes, demonstrating the extensive intra- and extraspinal extent of tumour passing through the intervertebral foramina. The compressed thoracic cord deviated to the left side of the spinal canal is shown in (a) (arrow).
Bauer, BL, Bauer, H, Griss, P, Lutcke, A, Maroske, D, Mennel, HD el al. (1989). Dumb-bell ganglioneuroma of the spine misinterpreted as progressive idiopathic scoliosis. Archives of Orthopaedic and Traumatic Surgery, 108, 189-I94. Nittner, K (1975)i Spinal meningiomas, neuromas and neurofibromas, and hourglass tumours. In: Handbook of Clinical Neurology, Tumours of the Spine and Spinal Cord, Part II, Chap. 7, eds. Vinken, PJ & Bruyn, GW, pp. 177 322. Springer-Verlag, Berlin. Shephard, RH & Sutton, D (1958). Dumb-bell ganglioneuromata of the spine with a report of four cases. British Journal of Surgery, 45, 305317. Stovens, D (1957). Neuroblastoma and related tumours. Archives of Pathology, 63, 451. Klimmer, M & Hecker, WC (1988). Zur Klinik van Neuroblastomen und Ganglioneuromen mit-intraspinalar Beteiligung. Zeitschriftfiir Kinderchirurgie, 43, 343-346. (English abstract on Medline.)