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Extradural metastases from paraganglionomas, report of two cases
ClinicalRadiology (1988) 39, 65-68
Extradural Metastases from Paraganglionomas, Report of Two Cases A . P. P A R N E L L
a n d D . J. D I C K
Departments" of Neuroradiology and Neurology, Newcastle General Hospital, Newcastle upon Tyne
Two cases of vertebral extradural metastases from paraganglionomas are presented. The significance of both is discussed with particular reference to abnormal catecholamine secretion in one and sclerotic bone metastases in the other case.
Paraganglionomas of the carotid and allied bodies are u n c o m m o n t u m o u r s w h i c h in d e f e r e n c e t o t h e i r p h y s i o l o g i c a l r o l e a r e a l s o k n o w n as c h e m o d e c t o m a s . T h e y have a low malignancy and only rarely spread. Metast a s e s h a v e b e e n e s t i m a t e d t o o c c u r in 1 6 % o f v a g a l b o d y t u m o u r s ( K a h n , 1976) a n d in a b o u t 6 % o f c a r o t i d b o d y t u m o u r s ( S t a a t s et al., 1966), a l t h o u g h in half of t h e cases s p r e a d w a s c o n f i n e d to t h e r e g i o n a l l y m p h n o d e s ; d i s t a n t d e p o s i t s w e r e m o s t l y in t h e l u n g s and skeleton. In the two cases of extradural metas t a s e s r e p o r t e d in t h i s p a p e r , d i r e c t s p r e a d f r o m t h e a d j a c e n t v e r t e b r a l b o d y h a d o c c u r r e d in o n e , w h i l e in the other a solitary extradural deposit was discovered in t h e a b s e n c e o f d i s e a s e e l s e w h e r e .
CASE REPORTS Case 1. A 45-year-old woman presented in 1965 to an ear, nose and throat surgeon with a 5 year history of an enlarging swelling behind the right mandible and a lower motor neurone lesion of the right hypoglossal nerve. The neck was explored and a deep-seated plum coloured tumour was seen behind the parotid gland. A biopsy was reported as tissue from a chemodectoma and, because a nerve was found in the wall of the specimen, the lesion was thought to represent a glomus intravagale tumour. A right common carotid angiogram performed after the operation revealed a vascular tumour 6x4×0.4 cm in extent displacing the internal carotid artery anteriorly, appearances consistent with a vagal body tumour (Fig. 1). There was no evidence of invasion of the skull base. A fluctuating blood pressure, at times as high as 220/110mmHg, was recorded during the post operative period and the urinary catecholamine levels were found to be elevated (Hydroxy-methylmandelic acid=18mg/24 h; the normal range below 12mg/24 h). The patient was discharged and given a course of 55 Gy radiotherapy to the tumour as an outpatient. Over the following 18 months the patient developed right lower cranial nerve palsies and a mild spastic quadriparesis, which was attributed at that time to a radiation myelopathy. Her blood pressure remained raised and a further elevated urinary catecholamine level was recorded. Attempts to identify a phaeochromocytoma including an excretory urogram and retro-peritoneal air insuffiation proved fruitless; the increased catecholamine secretion was assumed to originate from the chemodectoma. Treatment with alpha methyltyrosine was started and blood pressure levels became stable. In 1973 at the age of 53 the patient was readmitted with increasing difficulty in walking. She was found to have a spastic paraparesis with a sensory level at T8. A myelogram showed a complete extradural block at T9. A T8--9 laminectomy was performed and an Correspondence to: Dr A. P. Parnell, Cardiology X-Ray Department, Freeman Hospital, Freeman Road, Newcastle upon Tyne NE7 7DN.
extradural tumour was totally excised apart from a finger of tumour extending outwards through an intervertebral foramen. The histology of the tumour was reported as similar to the biopsy material obtained from the original vagal body tumour and consistent with either a chemodectoma or a phaeochromocytoma. Three years later the patient developed a progressive right sided deafness and loss of balance, and conventional tomography revealed a lytic area in the right petrous temporal bone (Fig. 2). A further course of radiotherapy (41.8 Gy) was applied to the skull base to treat this local tumour extension. In 1985 she was readmitted with a 5 year history of increasing ataxia of gait. Computed tomography (CT) showed the right petrous temporal bone to be partly destroyed by an isodense tumour which enhanced strongly and extended into the posterior fossa (Fig. 3). A right common carotid angiogram was performed showing the internal and external carotid arteries to be displaced forwards by a vascular tumour which was supplied by both branches of the carotid and by the vertebral artery. This tumour extended into the right petrous temporal bone. The patient was considered unsuitable for further operative treatment or radiotherapy and embolisation was not deemed possible. She was therefore treated conservatively. Owing to the multiple cranial nerve palsies she developed increasing difficulty in swallowing and aspiration became a serious problem. In June 1986 she was readmitted with an aspiration pneumonia from which she died. Autopsy examination revealed a 3 cm turnout projecting from the right petrous temporal bone and compressing the right cerebellar hemisphere. The lower cranial nerves were intimately involved with the turnout which extended through the petrous temporal bone to the jugular foramen. Tumour tissue was also identified in the extradural space at T9, where it was adherent to the dura and invaded the vertebral body. It did not extend through the intervertebral foramen and the sympathetic chain at T9 was normal. No paraganglionomas, phaeochromocytoma or other metastases were discovered. Case 2. A 19-year-old girl in 1970 developed a swelling on the right side of her neck and was found on examination to have a right hypoglossal nerve palsy and a Horner's syndrome. A right common carotid angiogram revealed occlusion of the right internal carotid artery at its origin in the neck and a turnout circulation, which suggested that the mass was a chemodectoma (Fig. 4). A biopsy was obtained but it proved inconclusive and the patient received a course of radiotherapy totalling 63.7 Gy. The swelling regressed and the patient remained well for about a year. Thereafter she became unwell and a chest radiograph showed multiple opacities. A Kveim test was positive. The diagnosis of sarcoidosis was made but her symptoms resolved without any specific treatment. The patient remained well until 1982 when, at the age of 31, she developed several tender nodules on her scalp. A skull radiograph revealed several sclerotic lesions (Fig. 5), but a CT brain scan was normal. One year later when seen for the first time in the Regional Neurological Centre, she complained of visual obscurations and was found to have bilateral papilloedema. A CT scan was again normal but a moderate increase was recorded in the cerebrospinal fluid pressure (285 mm water). Four vessel angiography revealed that the right common carotid artery was now completely occluded, the intraeranial circulation time was slowed and there was no filling of either lateral sinus; the right internal jugular vein was occluded. It was concluded that the patient's visual obscurations may have been caused by defective venous drainage. The obscurations continued, despite trials of treatment with diuretics and acetazolamide, until 1985 when she presented with unsteadiness of gait and a patchy sensory disturbance in both lower limbs. Examination revealed a mild spastic paraparesis and a vague sensory level at the level of the umbilicus. Spine radiographs showed sclerotic deposits in several thoracic and lumbar vertebral
66
CLINICAL RADIOLOGY
R Fig. 1 - (Case 1). Right common carotid angiogram showing anterior displacement of the internal carotid artery by a vascular tumour. These appearances are consistent with a vagal body tumour.
Fig. 4 - (Case 2). Right common carotid angiogram showing occlusion of the internal carotid artery and a vascular tumour at the carotid bifurcation consistent with a carotid body tumour.
Fig. 2 - (Case 1). Frontal tomogram showing a lytic area in the right petrous temporal bone due to extension of the chemodectoma.
Fig. 5 - (Case 2). Sclerotic deposits of the skull.
Fig. 3 - (Case 1). Contrast enhanced CT scan of the posterior fossa showing an enhancing tumour eroding the petrous temporal bone and extending into the cerebellopontine angle.
bodies, the pelvis (Fig. 6) and the occiput. A small paraspinal mass was present at the TI0 level (Fig. 7). Myelography revealed an incomplete extradural obstruction at the level of the T I 0 / l l disc space. A laminectomy was performed and a tumour was found. Histological examination showed it to be moderately cellular and to be invading the bone. The cells contained vacuolated clear cytoplasm with pleomorphic nuclei; Grimclius' stain showed the presence of intracytoplasmic argyrophilic granules. These features were highly suggestive of a metastasis from a carotid body tumour. Subsequent testing of the urine for hydroxy-methylmandelic acid was normal. Local" radiotherapy was given to the spine and one year later the patient remains well.
METASTASES FROM PARAGANGLIONOMAS
Fig. 6 - (Case 2). Sclerotic deposits in the lumbar spine and pelvis.
Fig. 7. - (Case 2). Coned view of T10 showing sclerosis of the body and both pediclcs of T10 with a small paraspinal mass at the level of the extradural block.
DISCUSSION
Both of the patients reported here had undergone radiotherapy for chemodectomas arising in the neck, one from the vagal body and the other from the carotid body. Both subsequently developed extradural metastases in the thoracic spine. Only one case of isolated extradural metastases from a chemodectoma appears to have been reported previously in the literature (Whimster and Masson, 1970), although extradural metastases associated with vertebral body
67
deposits have been described (Donald and Crile, 1948; Sessions et al., 1959; Say et al., 1973). Case 1 is particularly interesting in that the evidence of metastatic disease was confined to the extradural space, despite a 13 year follow-up after the laminectomy and a detailed post-mortem examination. In this respect, the case reported by Whimster and Masson (1970) is similar, viz. a carotid body tumour associated with two extradural metastases and no evidence of tumour dissemination elsewhere. These authors suggested that the tumour may have spread by the deep cervical and vertebral venous plexuses to the internal spinal veins. However, they followed up their patient for 3 months only and no post-mortem study was available, therefore clinically silent disease may have been present elsewhere in the body. Multicentric origin of primary paraganglionomas is well recognised; Kahn (1976) found a second chemodectoma in 8 of 57 cases of primary vagal body tumours. Although numerous sites of origin of primary chemodectomas are described, no reports exist of these turnouts arising in the extradural space. In Case 1 the vagal body tumour and the extradural deposit were macroscopically, histologically and ultrastructurally very similar, consequently it is likely that the extradural tumour was a metastasis rather than a second primary tumour. In Case 2 there is no doubt that the multiple lesions were metastases. Catecholamine secretion by a vagal body tumour is very unusual and only one case has previously been reported (Tannir et al., 1983). This case was not submitted to selective venous catheterisation (White and Hickson, 1979) or histochemical analysis (Hamberger et al., 1967). However, the strong clinical and biochemical evidence of abnormal catecholamine secretion disappeared completely after surgical resection of the chemodectoma and this strongly suggests that the chemodectoma was secreting catecholamines. While the above-mentioned diagnostic criteria, applied to the vagal body tumour in Case 1 to show that it secreted catecholamines, are not fulfilled, there was good clinical and biochemical evidence of abnormal catecholamine secretion. Since no other source of this abnormal secretion was discovered despite thorough investigation, prolonged follow-up and a complete post-mortem examination, it is reasonable to assume that it originated from the vagal body tumour. Case 2 is remarkable for the extensive sclerotic deposits in the occiput, spine and pelvis. Bone metastases from chemodectomas are usually lytic (Pendergrass and Kirsch, 1947; Romanski, 1954; Gustilo et al., 1965; Brown el al., 1967; Coulson, 1970). Only five cases of sclerotic bone metastases from chemodectomas have been described (Donald and Crile, 1948; Goormantigh and Patyn, 1954; Sessions et al., 1959; Say et al., 1973; Rangwalla et al., 1978), only the cases reported by Donald and Crile and Goormantigh and Patyn have been confirmed by histology. Fragments of sclerotic bone obtained at laminectomy in Case 2 contained tumour tissue compatible with a metastatic deposit from a carotid body tumour. Although glomus jugulare tumours not uncommonly extend into the posterior fossa and these tumours may arise from the superior vagal ganglion in the jugular fossa (Conley and Clairmont, 1977), invasion through the skull base by vagal body tumours is much less
68
CLINICAL RADIOLOGY
f r e q u e n t ( K e e n e r , 1959; P e r e z et al., 1960; M u r p h y et al., 1970). It occurs at a l a t e r s t a g e o f t h e d i sease p r o c e s s , as was e x e m p l i f i e d by C a s e 1 in w h i ch t h e t e m p o r a l b o n e was i n v a d e d 11 y e a r s a f t e r th e original diagnosis h ad b e e n m a d e .
Acknowledgements. We thank Dr J. B. Foster, Dr A. Appleby and Mr P. J. Crawford for their permission to report these cases.
REFERENCES Brown, JW, Burton, RC & Dahlin, DC (1967). Chemodectoma with skeletal metastases. Mayo Clinic Proceedings', 42, 551-555. Conley, JJ & Clairmont, AA (1977). Glomus intravagale. Laryngoscope, 87, 2096-2100. Coulson, WF (1970). A metastasising carotid body tumour. Journal of Bone and Joint Surgery, 52A, 355-360. Donald, RA & Crile, G (1948). Tumours of the carotid body. American Journal of Surgery, 75, 435-440. Goormantigh, N & Patyn, S (1954). A presumably benign tumour and a proved malignant tumour of the carotid body. American Journal of Pathology, 30, 679-693. Gustilo, RM, Lober, PH & Salovich, EC (1965). Chemodectoma (Carotid body tumour) metastasising to bone. Case report. Journal of Bone and Joint Surgery, 47A, 155-160. Hamberger, CA, Hamberger, CB, WersaU, J & Wagermark, J (1967). Malignant catecholamine producing tumour of the carotid body. Acta pathologica et microbiologica Scandinavia, 69, 489-492. Kahn, LB (1976). Vagal body tumour (non chromaffin paraganglionoma, and carotid body like tumour) with cervical node metastasis and familial association. Cancer, 38, 2367-2377.
Keener, EB (1959). Chemodectomas of the vagal body. Canadian Medical Association Journal, 80, 173-179. Murphy, TE, Huvos, AG & Frazell, EL (1970). Chemodectomas of the glomus intravagale: vagal body turnouts, non chromaffin paraganglionomas of the nodose ganglion of the vagus nerve. Annals of Surgery, 172, 246-255. Perez, PE, Harrison, EG & ReMine, WH (1960). Vagal body tumour (Chemodectoma of the glomus intravagale). New England Journal of Medicine, 263, 1116--1121. Pendergrass, EP & Kitsch, D (1947). Roentgen manifestations in the skull of metastatic carotid body turnout (paraganglionoma), of meningioma and of mucocoele. A report of three unusual cases. American Journal of Roentgenology, 57, 417-428. Rangwalla, AF, Sylvia, LC & Becker, SM (1978). Soft tissue metastasis of a chemodectoma, A case report and a review of the literature. Cancer, 42, 2865-2869. Romanski, R (1954). Chemodectoma (non-chromaffin paraganglionoma) of the carotid body with distant metastases. With illustrative case. American Journal of Pathology, 30, 1-14. Say, CC, Hori, J & Spratt, J (1973). Chemodectoma with distant metastasis: Case report and review of the literature. The American Surgeon, 39, 333-341. Sessions, RT, McSwait, B, Carlson, RI & Scott, HW (1959). Surgical experiences with tumours of the carotid body, glomus jugulare and retroperitoneal non-chromaffin paraganglia. Annals" of Surgery, 150, 808-823. Staats EF, Brown, RL & Smith, RR (1966). Carotid body tumours, benign and malignant. Laryngoscope, 76, 907-916. Tannir, NM, Cortas, NM & Allam, C (1983). A functioning catecholamine secreting vagal body tumour. A case report and a review of the literature. Cancer, 52, 932-935. Whimster, WF & Masson, AF (1970). Malignant carotid body tumour with extradural metastases. Cancer, 26, 239-244. White, MC & Hickson, BR (1979). Multiple paraganglionomata secreting catecholamines and calcitonin with intermittent hypercalcemia. Journal of the Royal Society of Medicine, 72, 532-538.
Extradural Metastases from Paraganglionomas, Report of Two Cases A . P. P A R N E L L
a n d D . J. D I C K
Departments" of Neuroradiology and Neurology, Newcastle General Hospital, Newcastle upon Tyne
Two cases of vertebral extradural metastases from paraganglionomas are presented. The significance of both is discussed with particular reference to abnormal catecholamine secretion in one and sclerotic bone metastases in the other case.
Paraganglionomas of the carotid and allied bodies are u n c o m m o n t u m o u r s w h i c h in d e f e r e n c e t o t h e i r p h y s i o l o g i c a l r o l e a r e a l s o k n o w n as c h e m o d e c t o m a s . T h e y have a low malignancy and only rarely spread. Metast a s e s h a v e b e e n e s t i m a t e d t o o c c u r in 1 6 % o f v a g a l b o d y t u m o u r s ( K a h n , 1976) a n d in a b o u t 6 % o f c a r o t i d b o d y t u m o u r s ( S t a a t s et al., 1966), a l t h o u g h in half of t h e cases s p r e a d w a s c o n f i n e d to t h e r e g i o n a l l y m p h n o d e s ; d i s t a n t d e p o s i t s w e r e m o s t l y in t h e l u n g s and skeleton. In the two cases of extradural metas t a s e s r e p o r t e d in t h i s p a p e r , d i r e c t s p r e a d f r o m t h e a d j a c e n t v e r t e b r a l b o d y h a d o c c u r r e d in o n e , w h i l e in the other a solitary extradural deposit was discovered in t h e a b s e n c e o f d i s e a s e e l s e w h e r e .
CASE REPORTS Case 1. A 45-year-old woman presented in 1965 to an ear, nose and throat surgeon with a 5 year history of an enlarging swelling behind the right mandible and a lower motor neurone lesion of the right hypoglossal nerve. The neck was explored and a deep-seated plum coloured tumour was seen behind the parotid gland. A biopsy was reported as tissue from a chemodectoma and, because a nerve was found in the wall of the specimen, the lesion was thought to represent a glomus intravagale tumour. A right common carotid angiogram performed after the operation revealed a vascular tumour 6x4×0.4 cm in extent displacing the internal carotid artery anteriorly, appearances consistent with a vagal body tumour (Fig. 1). There was no evidence of invasion of the skull base. A fluctuating blood pressure, at times as high as 220/110mmHg, was recorded during the post operative period and the urinary catecholamine levels were found to be elevated (Hydroxy-methylmandelic acid=18mg/24 h; the normal range below 12mg/24 h). The patient was discharged and given a course of 55 Gy radiotherapy to the tumour as an outpatient. Over the following 18 months the patient developed right lower cranial nerve palsies and a mild spastic quadriparesis, which was attributed at that time to a radiation myelopathy. Her blood pressure remained raised and a further elevated urinary catecholamine level was recorded. Attempts to identify a phaeochromocytoma including an excretory urogram and retro-peritoneal air insuffiation proved fruitless; the increased catecholamine secretion was assumed to originate from the chemodectoma. Treatment with alpha methyltyrosine was started and blood pressure levels became stable. In 1973 at the age of 53 the patient was readmitted with increasing difficulty in walking. She was found to have a spastic paraparesis with a sensory level at T8. A myelogram showed a complete extradural block at T9. A T8--9 laminectomy was performed and an Correspondence to: Dr A. P. Parnell, Cardiology X-Ray Department, Freeman Hospital, Freeman Road, Newcastle upon Tyne NE7 7DN.
extradural tumour was totally excised apart from a finger of tumour extending outwards through an intervertebral foramen. The histology of the tumour was reported as similar to the biopsy material obtained from the original vagal body tumour and consistent with either a chemodectoma or a phaeochromocytoma. Three years later the patient developed a progressive right sided deafness and loss of balance, and conventional tomography revealed a lytic area in the right petrous temporal bone (Fig. 2). A further course of radiotherapy (41.8 Gy) was applied to the skull base to treat this local tumour extension. In 1985 she was readmitted with a 5 year history of increasing ataxia of gait. Computed tomography (CT) showed the right petrous temporal bone to be partly destroyed by an isodense tumour which enhanced strongly and extended into the posterior fossa (Fig. 3). A right common carotid angiogram was performed showing the internal and external carotid arteries to be displaced forwards by a vascular tumour which was supplied by both branches of the carotid and by the vertebral artery. This tumour extended into the right petrous temporal bone. The patient was considered unsuitable for further operative treatment or radiotherapy and embolisation was not deemed possible. She was therefore treated conservatively. Owing to the multiple cranial nerve palsies she developed increasing difficulty in swallowing and aspiration became a serious problem. In June 1986 she was readmitted with an aspiration pneumonia from which she died. Autopsy examination revealed a 3 cm turnout projecting from the right petrous temporal bone and compressing the right cerebellar hemisphere. The lower cranial nerves were intimately involved with the turnout which extended through the petrous temporal bone to the jugular foramen. Tumour tissue was also identified in the extradural space at T9, where it was adherent to the dura and invaded the vertebral body. It did not extend through the intervertebral foramen and the sympathetic chain at T9 was normal. No paraganglionomas, phaeochromocytoma or other metastases were discovered. Case 2. A 19-year-old girl in 1970 developed a swelling on the right side of her neck and was found on examination to have a right hypoglossal nerve palsy and a Horner's syndrome. A right common carotid angiogram revealed occlusion of the right internal carotid artery at its origin in the neck and a turnout circulation, which suggested that the mass was a chemodectoma (Fig. 4). A biopsy was obtained but it proved inconclusive and the patient received a course of radiotherapy totalling 63.7 Gy. The swelling regressed and the patient remained well for about a year. Thereafter she became unwell and a chest radiograph showed multiple opacities. A Kveim test was positive. The diagnosis of sarcoidosis was made but her symptoms resolved without any specific treatment. The patient remained well until 1982 when, at the age of 31, she developed several tender nodules on her scalp. A skull radiograph revealed several sclerotic lesions (Fig. 5), but a CT brain scan was normal. One year later when seen for the first time in the Regional Neurological Centre, she complained of visual obscurations and was found to have bilateral papilloedema. A CT scan was again normal but a moderate increase was recorded in the cerebrospinal fluid pressure (285 mm water). Four vessel angiography revealed that the right common carotid artery was now completely occluded, the intraeranial circulation time was slowed and there was no filling of either lateral sinus; the right internal jugular vein was occluded. It was concluded that the patient's visual obscurations may have been caused by defective venous drainage. The obscurations continued, despite trials of treatment with diuretics and acetazolamide, until 1985 when she presented with unsteadiness of gait and a patchy sensory disturbance in both lower limbs. Examination revealed a mild spastic paraparesis and a vague sensory level at the level of the umbilicus. Spine radiographs showed sclerotic deposits in several thoracic and lumbar vertebral
66
CLINICAL RADIOLOGY
R Fig. 1 - (Case 1). Right common carotid angiogram showing anterior displacement of the internal carotid artery by a vascular tumour. These appearances are consistent with a vagal body tumour.
Fig. 4 - (Case 2). Right common carotid angiogram showing occlusion of the internal carotid artery and a vascular tumour at the carotid bifurcation consistent with a carotid body tumour.
Fig. 2 - (Case 1). Frontal tomogram showing a lytic area in the right petrous temporal bone due to extension of the chemodectoma.
Fig. 5 - (Case 2). Sclerotic deposits of the skull.
Fig. 3 - (Case 1). Contrast enhanced CT scan of the posterior fossa showing an enhancing tumour eroding the petrous temporal bone and extending into the cerebellopontine angle.
bodies, the pelvis (Fig. 6) and the occiput. A small paraspinal mass was present at the TI0 level (Fig. 7). Myelography revealed an incomplete extradural obstruction at the level of the T I 0 / l l disc space. A laminectomy was performed and a tumour was found. Histological examination showed it to be moderately cellular and to be invading the bone. The cells contained vacuolated clear cytoplasm with pleomorphic nuclei; Grimclius' stain showed the presence of intracytoplasmic argyrophilic granules. These features were highly suggestive of a metastasis from a carotid body tumour. Subsequent testing of the urine for hydroxy-methylmandelic acid was normal. Local" radiotherapy was given to the spine and one year later the patient remains well.
METASTASES FROM PARAGANGLIONOMAS
Fig. 6 - (Case 2). Sclerotic deposits in the lumbar spine and pelvis.
Fig. 7. - (Case 2). Coned view of T10 showing sclerosis of the body and both pediclcs of T10 with a small paraspinal mass at the level of the extradural block.
DISCUSSION
Both of the patients reported here had undergone radiotherapy for chemodectomas arising in the neck, one from the vagal body and the other from the carotid body. Both subsequently developed extradural metastases in the thoracic spine. Only one case of isolated extradural metastases from a chemodectoma appears to have been reported previously in the literature (Whimster and Masson, 1970), although extradural metastases associated with vertebral body
67
deposits have been described (Donald and Crile, 1948; Sessions et al., 1959; Say et al., 1973). Case 1 is particularly interesting in that the evidence of metastatic disease was confined to the extradural space, despite a 13 year follow-up after the laminectomy and a detailed post-mortem examination. In this respect, the case reported by Whimster and Masson (1970) is similar, viz. a carotid body tumour associated with two extradural metastases and no evidence of tumour dissemination elsewhere. These authors suggested that the tumour may have spread by the deep cervical and vertebral venous plexuses to the internal spinal veins. However, they followed up their patient for 3 months only and no post-mortem study was available, therefore clinically silent disease may have been present elsewhere in the body. Multicentric origin of primary paraganglionomas is well recognised; Kahn (1976) found a second chemodectoma in 8 of 57 cases of primary vagal body tumours. Although numerous sites of origin of primary chemodectomas are described, no reports exist of these turnouts arising in the extradural space. In Case 1 the vagal body tumour and the extradural deposit were macroscopically, histologically and ultrastructurally very similar, consequently it is likely that the extradural tumour was a metastasis rather than a second primary tumour. In Case 2 there is no doubt that the multiple lesions were metastases. Catecholamine secretion by a vagal body tumour is very unusual and only one case has previously been reported (Tannir et al., 1983). This case was not submitted to selective venous catheterisation (White and Hickson, 1979) or histochemical analysis (Hamberger et al., 1967). However, the strong clinical and biochemical evidence of abnormal catecholamine secretion disappeared completely after surgical resection of the chemodectoma and this strongly suggests that the chemodectoma was secreting catecholamines. While the above-mentioned diagnostic criteria, applied to the vagal body tumour in Case 1 to show that it secreted catecholamines, are not fulfilled, there was good clinical and biochemical evidence of abnormal catecholamine secretion. Since no other source of this abnormal secretion was discovered despite thorough investigation, prolonged follow-up and a complete post-mortem examination, it is reasonable to assume that it originated from the vagal body tumour. Case 2 is remarkable for the extensive sclerotic deposits in the occiput, spine and pelvis. Bone metastases from chemodectomas are usually lytic (Pendergrass and Kirsch, 1947; Romanski, 1954; Gustilo et al., 1965; Brown el al., 1967; Coulson, 1970). Only five cases of sclerotic bone metastases from chemodectomas have been described (Donald and Crile, 1948; Goormantigh and Patyn, 1954; Sessions et al., 1959; Say et al., 1973; Rangwalla et al., 1978), only the cases reported by Donald and Crile and Goormantigh and Patyn have been confirmed by histology. Fragments of sclerotic bone obtained at laminectomy in Case 2 contained tumour tissue compatible with a metastatic deposit from a carotid body tumour. Although glomus jugulare tumours not uncommonly extend into the posterior fossa and these tumours may arise from the superior vagal ganglion in the jugular fossa (Conley and Clairmont, 1977), invasion through the skull base by vagal body tumours is much less
68
CLINICAL RADIOLOGY
f r e q u e n t ( K e e n e r , 1959; P e r e z et al., 1960; M u r p h y et al., 1970). It occurs at a l a t e r s t a g e o f t h e d i sease p r o c e s s , as was e x e m p l i f i e d by C a s e 1 in w h i ch t h e t e m p o r a l b o n e was i n v a d e d 11 y e a r s a f t e r th e original diagnosis h ad b e e n m a d e .
Acknowledgements. We thank Dr J. B. Foster, Dr A. Appleby and Mr P. J. Crawford for their permission to report these cases.
REFERENCES Brown, JW, Burton, RC & Dahlin, DC (1967). Chemodectoma with skeletal metastases. Mayo Clinic Proceedings', 42, 551-555. Conley, JJ & Clairmont, AA (1977). Glomus intravagale. Laryngoscope, 87, 2096-2100. Coulson, WF (1970). A metastasising carotid body tumour. Journal of Bone and Joint Surgery, 52A, 355-360. Donald, RA & Crile, G (1948). Tumours of the carotid body. American Journal of Surgery, 75, 435-440. Goormantigh, N & Patyn, S (1954). A presumably benign tumour and a proved malignant tumour of the carotid body. American Journal of Pathology, 30, 679-693. Gustilo, RM, Lober, PH & Salovich, EC (1965). Chemodectoma (Carotid body tumour) metastasising to bone. Case report. Journal of Bone and Joint Surgery, 47A, 155-160. Hamberger, CA, Hamberger, CB, WersaU, J & Wagermark, J (1967). Malignant catecholamine producing tumour of the carotid body. Acta pathologica et microbiologica Scandinavia, 69, 489-492. Kahn, LB (1976). Vagal body tumour (non chromaffin paraganglionoma, and carotid body like tumour) with cervical node metastasis and familial association. Cancer, 38, 2367-2377.
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