The Journal of Laryngology and Otology December 1992, Vol. 106, pp. 1086-1088
Cavernous haemangioma of the temporal bone D. S. BUCHANAN, F.R.A.C.S.,* P. A. FAGAN, F.R.A.C.S., F.R.C.S.,* J. TURNER, F.R.C.P.A.** (Sydney, Australia)
Abstract Cavernous haemangioma of the temporal bone is a rare lesion and nearly always of limited extent. A case of a large and rapidly progressive temporal bone haemangioma in a child is presented. Negative angiography led to biopsy at which profuse and troublesome haemorrhage was encountered. Subsequent Red Blood Cell labelled scan demonstrated blood pooling in the lesion. The combination of positive RBC labelled scan and negative angiography can only occur in lesions in which blood pooling takes place, enabling biopsy with its hazards to be avoided. The tumour was resected using a skull base, infratemporal approach. Morbidity was minimal. There was no evidence of recurrence at a 15 month follow-up.
recurrent intermittent left otitis media with effusion. She was treated effectively with oral antibiotics. On routine follow up, a dull, red mass in the left middle ear cavity was noted. There was an associated left sensori-neural hearing loss. High resolution computerised tomography (CT) revealed a large mass replacing the temporal bone and eroding the lateral semi-circular canal (Figures 1 a & b). A provisional diagnosis of glomus tumour was made, but when angiography showed no tumour blush, a tympanotomy was carried out. A fleshy vascular tumour (which bled profusely on biopsy) was noted. Histopathological examination revealed a cavernous haemangioma. At radiotherapy consultation, this mode of treatment was felt to be inappropriate. A decision to observe the patient was made.
Introduction Haemangioma of the temporal bone is an uncommon lesion. Nearly all are small and are found either in the internal auditory canal (IAC) or at the geniculate ganglion. We present a case of a large cavernous haemangioma which had replaced the temporal bone. The diagnosis, aggressive nature and surgical management of the lesion are highlighted. Case report A five-year-old girl presented with a 15-month history of
FIG. la Axial CT scan. Bone window view of the temporal bone. Arrowheads outline the intracranial, extradural extent of the tumour. Arrow points to lateral semi-circular canal involvement. The middle ear and horizontal facial nerve are free of tumour.
FIG. lb Coronal CT scan (bone windows). Soft tissue mass (arrowhead) seen in the middle ear cleft in close proximity to cochlea.
From the Departments of Otolaryngology* and Pathology**, St Vincent's Hospital, Sydney, Australia. Accepted for publication: 23 September 1992. 1086
CLINICAL RECORDS
Six months later, the child developed a complete left lower motor neurone facial nerve palsy which resolved in three weeks. There were no other neurological features. A CT scan showed significant progression since the previous imaging study with almost complete replacement of the temporal bone by the tumour (Fig. 2). The patient was then referred to the senior author (PAF). Red Blood Cell (RBC) labelled scan confirmed the presence of a vascular tumour (Fig. 3). Magnetic resonance imaging (MRI) demonstrated the gross extent of the tumour, with intracranial extra-dural extension (Fig. 4). A Fisch skull base approach (Type A) (Fisch et al., 1984) was carried out. Particular attention was paid to ligation of all possible feeding vessels in the neck. The tumour was found to be invading the epineurium of the vertical and horizontal portions of the facial nerve, from which it was stripped. Bipolar diathermy was used to remove macroscopic remnants. The vagus and accessory nerves were also involved by tumour but it was possible to separate nerve from tumour in each case. The dura of the middle and posterior fossa was found to be infiltrated with tumour. Macroscopic removal followed by extensive bipolar diathermy of the dura was carried out. It was judged at the time of surgery that the morbidity of resecting a large sheet of dura would be unacceptable. Tumour involvement of the IAC proved to be extra dural. Removal in this area was complete and without damage to the facial nerve. Blood loss for the whole procedure was approximately 300 ml (15 per cent of the total blood volume) and this was replaced intra-operatively. Histology confirmed a cavernous haemangioma, involving and destroying bone with some reactive new bone formation. Many vascular spaces contained thrombus and others showed evidence of part thrombosis with papillary endothelial hyperplasia (Fig. 5). Post-operatively, the child had a mild left facial palsy, which recovered completely, a normal cough and she was eating and drinking normally without aspiration at four days. She was discharged from hospital on the sixth day. Follow-up at 15 months showed no evidence of tumour recurrence on CT examination. There was still a minimal weakness of the mandibular branch of the left facial nerve and of the sternomastoid and trapezius muscles.
FIG. 2 Comparable view to Figure 1 a, taken six months later. The temporal bone has been largely replaced by the tumour. The semi-circular canals have been destroyed (arrow) and the mastoid air cells filled withfluiddensity material. The destructive process has reached the petrous apex (arrowhead). The middle ear is filled which proved at surgery to be tumour.
1087 Discussion Haemangioma is an uncommon lesion of the temporal bone (Mangham et al, 1981). It occurs mainly in the IAC or at the geniculate ganglion, with rarer sites being the middle ear cavity and the petrous apex (Fisch and Ruttner, 1976; Mangham et al., 1981; Glasscock et al, 1983; Mazzoni et al., 1988). All such reported tumours have been small. Large intra-osseous cavernous haemangioma are even more infrequent (Sullivan, 1934; Irgens, 1939). Such tumours, reported prior to CT, may have been glomus tumours (Mangham et al., 1981). There is an early report of a large haemangioma of the temporal bone (Kessler et al., 1957) but prior to the advent of CT, determination of exact size was difficult if not impossible. These last aside, it is our belief that with cavernous haemangioma of the temporal bone neither such a large tumour nor such aggressive behaviour has been previously reported. Haemangioma is classified on the basis of the dominant vessel, either capillary or cavernous (Fisch and Ruttner, 1976; Sundaresan et al., 1976). It is said that the capillary type can become cavernous with time, but capillary haemangioma has not been reported in the temporal bone (Glasscock et al., 1983). Cavernous haemangioma exhibits aggressive behaviour and does not spontaneously involute (Mangham et al., 1981; Glasscock et al., 1983), making treatment mandatory. It has been said that there are no imaging features that help differentiate haemangioma from other temporal bone tumours (Glasscock et al, 1983). CT scanning did delineate the extent of the tumour very accurately and documented the rapid progression that took place over six months. However, in this patient, angiography was negative, but RBC labelled scans were positive, which, as reported previously (Front et al, 1982) is a combination that can only be seen in vascular lesions in which blood flow is very sluggish. Treatment is necessary because of the rapid growth features and the effect on surrounding vital structures. Steroids have been used for cavernous haemangiomata elsewhere in the body, but as far as can be ascertained, no study has reported their use in the temporal bone. Radiation therapy has been used for unresectable skull base lesions of all types, or in those patients medically unsuitable for surgical treatment (Mangham et al, 1981; Glasscock et al, 1983). Radiotherapy was considered in this case, but because of the possible growth retarding effect (Plowman et al, 1991) and later malignant change (Fredrickson et al, 1979) it was deemed unsuitable. The skull base approach of Fisch has made it possible
FIG. 3 Red blood cell labelled scan. There is increased vascularity in the left temporal bone (arrowheads). Venous flow distal to the tumour is equivalent to the contralateral side.
1088
D. S. BUCHANAN, P. A. FAGAN, J. TURNER
FIG. 5 Histological section showing cavernous vascular spaces. The space at left contains organizing thrombus (arrow). Reactive bone is seen at right (arrowhead). H & E, original magnification X10.
Tobias, of the Paediatric Anatomical Pathology Departments of Prince of Wales Hospital.
FIG. 4 Axial Magnetic Resonance Imaging. Tl weighted view with Gadolinium (TR:TE = 2,000:90). The tumour is extradural and compressing the cerebellum (arrows). It is in intimate contact with the horizontal portion of the internal carotid artery (arrowhead) as far as petrous apex.
to resect lesions of this type which were previously thought to be unresectable. Even though angiography had shown no major feeding vessels, ligation of the external carotid artery and its branches resulted in a relatively bloodless field. This is in contrast to the profuse bleeding noted at the earlier biopsy and previously reported (Irgens, 1939). Pre-operative radiological studies showed the tumour to be extra dural and at surgery there were few dural feeding blood vessels noted. Clearance of the tumour from the dura was possible without the need for excision of dura with the consequent increase in morbidity of that procedure. Conclusion Cavernous haemangioma of the temporal bone are rare and mostly small in size. Conventional CT imaging outlines the tumour extent, but does not show features that can make the diagnosis pre-operatively. The combination of negative angiography and positive RBC labelled scan may be helpful in establishing the diagnosis before surgery, thereby avoiding biopsy and troublesome haemorrhage. The aggressive behaviour of cavernous haemangioma and involvement of surrounding vital structures make treatment necessary. The Fisch Type A skull base approach has been shown in this case report to be effective in removing the tumour with minimal morbidity. Acknowledgment Our thanks to Dr Robert Smee, of the Radiotherapy, and Dr V.
Key words: Haemangioma, cavernous; Temporal bone
References Fisch, U., Fagan, P. A., Valvanis, A. (1984) Infratemporal fossa approach for the lateral skull base. Otolaryngologic Clinics of North America, 17: 513-552. Fisch, U., Ruttner, J. (1976) Pathology of intratemporal tumours involving the facial nerve in facial nerve surgery. Aesculapius, Birmingham, Alabama, p. 448-456. Fredrickson, J. M., Haight, J. S. H., Noyek, A. M. (1979) Radiation induced carcinoma in a haemangioma. Otolaryngology-Head and Neck Surgery, 87: 584-586. Front, D., Israel, O., Kleinhaus, U., Gdal-On, M. (1982) Tc-99mlabelled red blood cells in the evaluation of haemangiomas of the skull and orbit: Concise communication, Journal of Nuclear Medicine, 23: 1080-1084. Glasscock, M. E., Smith, P. G., Schwaber, M. K., Wissen, A. J. (1983) Clinical aspects of osseous haemangiomas of the skull base. Laryngoscope, 94: 869-873. Irgens, E. R. (1939) Haemangioma of skull involving right petrous and occipital bones. Archives of Otolaryngology, 20: 709-712. Kessler, L. A., Lubic, L. G., Koskoff, Y. D. (1957) Epidural haemorrhage secondary to cavernous haemangioma of the petrous portion of the temporal bone, Journal ofNeurosurgery, 14:329-331. Mangham, C. A., Carberry, J. N., Brackmann, D. E. (1981) Management of intratemporal vascular tumours. Laryngoscope, 91: 867-875. Mazzoni, A., Pareschi, R., Calabrese, V. (1988) Intratemporal vascular tumours. Journal of Laryngology and Otology, 102: 353-356. Plowman, P. N., McElwain, T. J., Meadows, A. T. (1991) Complications of cancer management. Butterworth-Heinemann, Oxford, 95-113. Sullivan, J. A. (1934) Haemangio-endothelioma of the temporal bone. Archives of Otolaryngology, 20: 61-67. Sundaresan, N., Eller, T., Ciric, I. (1976) Haemangioma of the internal auditory canal. Surgery and Neurology, 6: 119—121. Address for correspondence: Dr P. A. Fagan, 352 Victoria Street, Darlinghurst 2010, Sydney, Australia
Cavernous haemangioma of the temporal bone D. S. BUCHANAN, F.R.A.C.S.,* P. A. FAGAN, F.R.A.C.S., F.R.C.S.,* J. TURNER, F.R.C.P.A.** (Sydney, Australia)
Abstract Cavernous haemangioma of the temporal bone is a rare lesion and nearly always of limited extent. A case of a large and rapidly progressive temporal bone haemangioma in a child is presented. Negative angiography led to biopsy at which profuse and troublesome haemorrhage was encountered. Subsequent Red Blood Cell labelled scan demonstrated blood pooling in the lesion. The combination of positive RBC labelled scan and negative angiography can only occur in lesions in which blood pooling takes place, enabling biopsy with its hazards to be avoided. The tumour was resected using a skull base, infratemporal approach. Morbidity was minimal. There was no evidence of recurrence at a 15 month follow-up.
recurrent intermittent left otitis media with effusion. She was treated effectively with oral antibiotics. On routine follow up, a dull, red mass in the left middle ear cavity was noted. There was an associated left sensori-neural hearing loss. High resolution computerised tomography (CT) revealed a large mass replacing the temporal bone and eroding the lateral semi-circular canal (Figures 1 a & b). A provisional diagnosis of glomus tumour was made, but when angiography showed no tumour blush, a tympanotomy was carried out. A fleshy vascular tumour (which bled profusely on biopsy) was noted. Histopathological examination revealed a cavernous haemangioma. At radiotherapy consultation, this mode of treatment was felt to be inappropriate. A decision to observe the patient was made.
Introduction Haemangioma of the temporal bone is an uncommon lesion. Nearly all are small and are found either in the internal auditory canal (IAC) or at the geniculate ganglion. We present a case of a large cavernous haemangioma which had replaced the temporal bone. The diagnosis, aggressive nature and surgical management of the lesion are highlighted. Case report A five-year-old girl presented with a 15-month history of
FIG. la Axial CT scan. Bone window view of the temporal bone. Arrowheads outline the intracranial, extradural extent of the tumour. Arrow points to lateral semi-circular canal involvement. The middle ear and horizontal facial nerve are free of tumour.
FIG. lb Coronal CT scan (bone windows). Soft tissue mass (arrowhead) seen in the middle ear cleft in close proximity to cochlea.
From the Departments of Otolaryngology* and Pathology**, St Vincent's Hospital, Sydney, Australia. Accepted for publication: 23 September 1992. 1086
CLINICAL RECORDS
Six months later, the child developed a complete left lower motor neurone facial nerve palsy which resolved in three weeks. There were no other neurological features. A CT scan showed significant progression since the previous imaging study with almost complete replacement of the temporal bone by the tumour (Fig. 2). The patient was then referred to the senior author (PAF). Red Blood Cell (RBC) labelled scan confirmed the presence of a vascular tumour (Fig. 3). Magnetic resonance imaging (MRI) demonstrated the gross extent of the tumour, with intracranial extra-dural extension (Fig. 4). A Fisch skull base approach (Type A) (Fisch et al., 1984) was carried out. Particular attention was paid to ligation of all possible feeding vessels in the neck. The tumour was found to be invading the epineurium of the vertical and horizontal portions of the facial nerve, from which it was stripped. Bipolar diathermy was used to remove macroscopic remnants. The vagus and accessory nerves were also involved by tumour but it was possible to separate nerve from tumour in each case. The dura of the middle and posterior fossa was found to be infiltrated with tumour. Macroscopic removal followed by extensive bipolar diathermy of the dura was carried out. It was judged at the time of surgery that the morbidity of resecting a large sheet of dura would be unacceptable. Tumour involvement of the IAC proved to be extra dural. Removal in this area was complete and without damage to the facial nerve. Blood loss for the whole procedure was approximately 300 ml (15 per cent of the total blood volume) and this was replaced intra-operatively. Histology confirmed a cavernous haemangioma, involving and destroying bone with some reactive new bone formation. Many vascular spaces contained thrombus and others showed evidence of part thrombosis with papillary endothelial hyperplasia (Fig. 5). Post-operatively, the child had a mild left facial palsy, which recovered completely, a normal cough and she was eating and drinking normally without aspiration at four days. She was discharged from hospital on the sixth day. Follow-up at 15 months showed no evidence of tumour recurrence on CT examination. There was still a minimal weakness of the mandibular branch of the left facial nerve and of the sternomastoid and trapezius muscles.
FIG. 2 Comparable view to Figure 1 a, taken six months later. The temporal bone has been largely replaced by the tumour. The semi-circular canals have been destroyed (arrow) and the mastoid air cells filled withfluiddensity material. The destructive process has reached the petrous apex (arrowhead). The middle ear is filled which proved at surgery to be tumour.
1087 Discussion Haemangioma is an uncommon lesion of the temporal bone (Mangham et al, 1981). It occurs mainly in the IAC or at the geniculate ganglion, with rarer sites being the middle ear cavity and the petrous apex (Fisch and Ruttner, 1976; Mangham et al., 1981; Glasscock et al, 1983; Mazzoni et al., 1988). All such reported tumours have been small. Large intra-osseous cavernous haemangioma are even more infrequent (Sullivan, 1934; Irgens, 1939). Such tumours, reported prior to CT, may have been glomus tumours (Mangham et al., 1981). There is an early report of a large haemangioma of the temporal bone (Kessler et al., 1957) but prior to the advent of CT, determination of exact size was difficult if not impossible. These last aside, it is our belief that with cavernous haemangioma of the temporal bone neither such a large tumour nor such aggressive behaviour has been previously reported. Haemangioma is classified on the basis of the dominant vessel, either capillary or cavernous (Fisch and Ruttner, 1976; Sundaresan et al., 1976). It is said that the capillary type can become cavernous with time, but capillary haemangioma has not been reported in the temporal bone (Glasscock et al., 1983). Cavernous haemangioma exhibits aggressive behaviour and does not spontaneously involute (Mangham et al., 1981; Glasscock et al., 1983), making treatment mandatory. It has been said that there are no imaging features that help differentiate haemangioma from other temporal bone tumours (Glasscock et al, 1983). CT scanning did delineate the extent of the tumour very accurately and documented the rapid progression that took place over six months. However, in this patient, angiography was negative, but RBC labelled scans were positive, which, as reported previously (Front et al, 1982) is a combination that can only be seen in vascular lesions in which blood flow is very sluggish. Treatment is necessary because of the rapid growth features and the effect on surrounding vital structures. Steroids have been used for cavernous haemangiomata elsewhere in the body, but as far as can be ascertained, no study has reported their use in the temporal bone. Radiation therapy has been used for unresectable skull base lesions of all types, or in those patients medically unsuitable for surgical treatment (Mangham et al, 1981; Glasscock et al, 1983). Radiotherapy was considered in this case, but because of the possible growth retarding effect (Plowman et al, 1991) and later malignant change (Fredrickson et al, 1979) it was deemed unsuitable. The skull base approach of Fisch has made it possible
FIG. 3 Red blood cell labelled scan. There is increased vascularity in the left temporal bone (arrowheads). Venous flow distal to the tumour is equivalent to the contralateral side.
1088
D. S. BUCHANAN, P. A. FAGAN, J. TURNER
FIG. 5 Histological section showing cavernous vascular spaces. The space at left contains organizing thrombus (arrow). Reactive bone is seen at right (arrowhead). H & E, original magnification X10.
Tobias, of the Paediatric Anatomical Pathology Departments of Prince of Wales Hospital.
FIG. 4 Axial Magnetic Resonance Imaging. Tl weighted view with Gadolinium (TR:TE = 2,000:90). The tumour is extradural and compressing the cerebellum (arrows). It is in intimate contact with the horizontal portion of the internal carotid artery (arrowhead) as far as petrous apex.
to resect lesions of this type which were previously thought to be unresectable. Even though angiography had shown no major feeding vessels, ligation of the external carotid artery and its branches resulted in a relatively bloodless field. This is in contrast to the profuse bleeding noted at the earlier biopsy and previously reported (Irgens, 1939). Pre-operative radiological studies showed the tumour to be extra dural and at surgery there were few dural feeding blood vessels noted. Clearance of the tumour from the dura was possible without the need for excision of dura with the consequent increase in morbidity of that procedure. Conclusion Cavernous haemangioma of the temporal bone are rare and mostly small in size. Conventional CT imaging outlines the tumour extent, but does not show features that can make the diagnosis pre-operatively. The combination of negative angiography and positive RBC labelled scan may be helpful in establishing the diagnosis before surgery, thereby avoiding biopsy and troublesome haemorrhage. The aggressive behaviour of cavernous haemangioma and involvement of surrounding vital structures make treatment necessary. The Fisch Type A skull base approach has been shown in this case report to be effective in removing the tumour with minimal morbidity. Acknowledgment Our thanks to Dr Robert Smee, of the Radiotherapy, and Dr V.
Key words: Haemangioma, cavernous; Temporal bone
References Fisch, U., Fagan, P. A., Valvanis, A. (1984) Infratemporal fossa approach for the lateral skull base. Otolaryngologic Clinics of North America, 17: 513-552. Fisch, U., Ruttner, J. (1976) Pathology of intratemporal tumours involving the facial nerve in facial nerve surgery. Aesculapius, Birmingham, Alabama, p. 448-456. Fredrickson, J. M., Haight, J. S. H., Noyek, A. M. (1979) Radiation induced carcinoma in a haemangioma. Otolaryngology-Head and Neck Surgery, 87: 584-586. Front, D., Israel, O., Kleinhaus, U., Gdal-On, M. (1982) Tc-99mlabelled red blood cells in the evaluation of haemangiomas of the skull and orbit: Concise communication, Journal of Nuclear Medicine, 23: 1080-1084. Glasscock, M. E., Smith, P. G., Schwaber, M. K., Wissen, A. J. (1983) Clinical aspects of osseous haemangiomas of the skull base. Laryngoscope, 94: 869-873. Irgens, E. R. (1939) Haemangioma of skull involving right petrous and occipital bones. Archives of Otolaryngology, 20: 709-712. Kessler, L. A., Lubic, L. G., Koskoff, Y. D. (1957) Epidural haemorrhage secondary to cavernous haemangioma of the petrous portion of the temporal bone, Journal ofNeurosurgery, 14:329-331. Mangham, C. A., Carberry, J. N., Brackmann, D. E. (1981) Management of intratemporal vascular tumours. Laryngoscope, 91: 867-875. Mazzoni, A., Pareschi, R., Calabrese, V. (1988) Intratemporal vascular tumours. Journal of Laryngology and Otology, 102: 353-356. Plowman, P. N., McElwain, T. J., Meadows, A. T. (1991) Complications of cancer management. Butterworth-Heinemann, Oxford, 95-113. Sullivan, J. A. (1934) Haemangio-endothelioma of the temporal bone. Archives of Otolaryngology, 20: 61-67. Sundaresan, N., Eller, T., Ciric, I. (1976) Haemangioma of the internal auditory canal. Surgery and Neurology, 6: 119—121. Address for correspondence: Dr P. A. Fagan, 352 Victoria Street, Darlinghurst 2010, Sydney, Australia
