Surg Neurol 1990;34:345-51
345
Spinal Extradural Cavernous Hemangioma Jen-Pei Lee, M.D., Alexander Dah-Jium Wang, M.D., Ph.D., Yau-Yau Wai, M.D., and Y a t - S e n H o , M . D . Division of Neurosurgery, Departments of Radiology and Pathology, Chang Gung Medical College & Chang Gung Memorial Hospital, Taipei, Taiwan, Republic of China
Lee J-P, Wang A D-J, Wai Y-Y, Ho Y-S. Spinal extradural cavernous hemangioma. Surg Neurol 1990;34:345-51.
Case R e p o r t s Case I
Three cases of isolated spinal extradural cavernous hemangiomas are reported, two in the thoracic and o n e in the lumbar region. One of them manifested as acute cord compression, the other two as chronic progressive myelopathy or radiculopathy. A total excision or subtotal excision with irradiation was performed. All of them had good functional recovery. The clinical picture, radiological diagnosis, and optimal method of treatment are discussed. The relevant literature is reviewed.
This 25-year-old man presented with a typical history of progressive spinal cord compression. Two weeks before admission, he began to have complaints of interscapular pain, which was aggravated by sneezing, followed by numbness and weakness of both legs 1 week later. Urinary retention and constipation developed 3 days before admission. On admission, he was alert. The general physical examination was normal. The neurological examination revealed spastic paraparesis with hyperreflexia and prominent ankle clonus in the lower limbs. Bilateral positive Babinski's signs were found. He could stand with support but could not walk. The pinprick sensation was abolished below the level of T-7. No impairment of posterior column sensation was noted. His anal tone was preserved but there was urine retention. Plain film of the thoracic spine showed no bony abnormality. X-ray examination of the chest was negative. Myelography performed via the lumbar route showed extradural compression with complete block at the level of T-7 (Figure 1 A). A bilateral laminectomy from T-4 through T-7 was performed under the impression of an extradural lesion with cord compression. A dark red mass was encountered in the posterior surface of the dura mater. It was not encapsulated, extending around both sides of the spinal cord anteriorly from T-5 to the upper margin of T-7. Massive bleeding from dilated vascular channels was encountered during dissection of the lesion from the dura mater. The lesion was gradually removed in one piece from the posterior dural sec. Histopathological examination of the lesion disclosed a typical picture of cavernous hemangioma (Figure 2). The size of the blood vessels varied. These irregular vascular spaces were filled with thrombi and blood. Their walls were lined by a single layer of endothelium and thickened by fibrous and hyaline materials. A course of cobalt 60 radiation therapy was given with 3800 rads to T-4 through T-8. The patient had an
KEY WORDS: Angioma; Cavernous hemangioma; Spinal canal; Vascular malformation
Introduction Cavernous hemangiomas are uncommon vascular malformations of the central nervous system. They are characterized by hemorrhage, seizure, or focal neurological deficits [6]. Although generally located in the cerebral hemisphere, these malformations may be found in any part of the neuraxis. In the spine, the majority of these lesions are from secondary extensions of vertebral hemangiomas [9]. The occurrence of a primary extradural cavernous hemangioma is relatively rare. We report three cases of spinal extradural cavernous hemangiomas without vertebral foci, two in the thoracic and one in the lumbar spine.
Address reprint requests to: Jen-Pei Lee, M.D., Division of Neurosurgery, Chang Gung Medical College & Chang Gung Memorial Hospital, 199, Tung Hwa North Road, Talpei, Taiwan, Republic of China 10591. Received August 29, 1989; accepted May 16, 1990.
© 1990 by ElsevierSciencePublishingCo., Inc.
0090-3019/90/$3.50
346
Surg Neurol 1990;34:345-51
Lee et al
zamide computed tomography (CT) study of the spine (Figure 3) revealed a soft tissue mass in the extradural space of the spinal canal, mainly on the left side from T8 to T-10, with displacement of the cord to the right. A bilateral laminectomy from T-8 through T-10 was performed under the impression of an extradural mass with cord compression. The bone appeared normal. An encapsulated dark purple, lobulated mass was found on the posterior surface of the dura mater. It extended from T-8 to T-10, exclusively on the left side. The mass was clearly defined and easily removed. Histopathological examination confirmed the hemangiomatous nature of the lesion (Figure 4). The patient had a good recovery after surgery. H e has been followed for 1 year. His neurological examinations, including sensory modalities, gait, and sphincters, are normal. H e has gone back to work.
Case 3
Figure 1. Case 1: (A) Myelogram with myodil injected by the lumbar route showing extradural compression with complete block at the level of T-7. (B) Metrizamide CT scan through T-6, performed 5 years after surgery, revealed no recurrence of tumor.
uneventful postoperative recovery. H e was followed up for 5 years; no neurological deficit was found, and he has returned to work. The follow-up radiological study revealed no recurrence of the lesion (Figure 1 B).
Case 2 A 60-year-old, right-handed man had a history of progressive right leg weakness and numbness for 1 year. One month before admission, left leg weakness was also noted. On examination, the patient was alert. The cranial nerves and arms were normal. Paraparesis was present with muscle power at grade 4. Hyperactive deep tendon reflexes were observed in both legs. Hypesthesia was detected below the level ofT-10. Urinary retention was verified. X-ray examination of the spine showed diffuse spondylitic change, more evident in the lumbosacral area. Plain film of the chest was negative. Myelography performed via the lumbar route disclosed a complete block of the opaque contrast medium at T-10. The metri-
This 27-year-old female student was admitted due to complaints o f pain over the posterior aspect o f her right lower leg and sole for I month. She had similar episodes several times in the 6 months prior to admission. N o definite history of trauma was disclosed. On admission, she was alert. The general physical examination was normal. The cranial nerves and her arms were normal. A decrease in pinprick sensation was detected over the S- 1 dermatome on the right. The straight leg raising test was positive at 70 ° on the right side. The sphincter functions were preserved. Myelography performed via the lumbar route disclosed lumbar dura ectasia and poor delineation of the right S-1 root. Metrizamide CT study of the spine revealed a lobulated soft tissue mass in the epidural space at L-5 on the right, with extension through the intervertebral foramina to the paravertebral region. A neurofibroma was first suspected. During surgery, many dilated venous channels in the paraspinal region and epidural space were found. Since the macroscopic appearance of the lesion was of an angiomatous pattern, complete extirpation was postponed. A more detailed workup of the vascular lesion was arranged. A C T study with intravenous contrast enhancement was then performed and revealed a heterogenously enhanced soft tissue mass. The paraspinal muscles in the right side were also enhanced (Figure 5). Spinal angiography performed by catheterization of the femoral artery showed no abnormality. The second operation was performed under the impression of a cavernous hemangioma. A dark red, wormlike mass was found both on the posterior surface of the dura mater, extending through the intervertebral foramina to the paravertebral region on the right, and on the anterior aspect o f the dural sac. Massive venous bleeding from the dilated vascular channels was encoun-
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Figure 2. Case 1: Histological appearance of cavernous hemangioma. Note
Figure 3. Case 2: Metrizamide CT scan through T-9 revealed a soft
the irregular cavernous vascular spaces, some of which contain blood and thrombi (arrow). The walls of vascular spaces are lined by a single layer of endothelium and are thickened by fibrous and hyaline material (hematoxylin and eosin stain x 66).
tissue mass in the left extradural space with extension into the intervertebral foramen. The cord was displaced to the right.
tered during dissection of the lesion. The tumor was gradually removed in one piece from the posterior dural sac and amputated from the paravertebral region. Cavernous hemangioma was confirmed by histopathological study (Figure 6). Additional radiation therapy was suggested, but was refused by the patient. She was discharge.d with no neurological deficits and was back to school in 1 month. She was followed for 1 year. The patient is currently still symptom free. Discussion Cavernous hemangioma or cavernoma is a purely descriptive term, signifying the gross and microscopic appearance of those lesions that are composed of irregular, sinusoidal vascular spaces. These lesions have also been called "cavernous angioma," and "cavernous real-
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Figure 4. Case 2: Typical histological appearance of cavernous hemangioma (hematoxylin and eosin stain × 165).
formation." The etiology of cavernous hemangioma has been unclear. Although previous authors have considered this lesion to be a vascular neoplasm [1,21], current classification schemes define the cavernous hemangioma as a vascular malformation [9,14]. Wyburn-Mason [23] regarded cavernous hemangioma as a developmental form of telangiectasis. Russell and Rubinstein [19] divided vascular hamartoma into capillary telangiectasis, cavernous angioma, venous malformation, and arteriovenous malformation. These lesions are considered to be of congenital origin and are clearly differentiated from vascular neoplasms. Cavernous hemangioma is an uncommon vascular malformation that may affect any part of the neuraxis but is generally seen intracranially [20]. The spinal cavernous hemangioma has been estimated to represent 5 % - 1 2 % of all spinal vascular anomalies [9]. The majority of these lesions arise within the vertebral bodies and may extend into the extradural space [7]. Only a few cases of primary extradural cavernous hemangiomas without vertebral focus have been reported. Padovani et
Lee et al
Figure 5. Case 3." CT scan with intravenous contrast enhancement through L-5 revealed heterogenously enhanced mass in the right paravertebral region with extension to the spinal canal The paraspinal muscles in the right were also enhanced (arrows).
Spinal Extradural Cavernous Hemangioma
al [16] could only accumulate a series of 27 cases o f hemangioma o f the spinal epidural space from the literature. W e have encountered three cases of epidural cavernous hemangioma, two in the thoracic and one in the lumbar region. From the review of the reported cases, the most common clinical course o f an extradural hemangioma does not differ from that o f other spinal space-occupying lesions with slow but progressive spinal cord compression. T h e rare, acute clinical course is due either to extrathecal hemorrhage [ 10,11] or to thrombotic occlusion within the structure of the vascular lesion causing sudden increase o f the bulk of the lesion [ 15 ]. Richardson and Cerullo [17] reported a case o f cervical epidural hemangioma presenting as radicular pain following minor trauma, which was probably due to microhemorrhage within the lesion. In our cases, two manifested as mass lesions with cord or root compression, one as acute myelopathy. Wyburn-Mason [23] claimed that T-2 and T-6 were the most c o m m o n spinal levels for hemangiomas. This is confirmed by most reported cases. T h r e e cases of epidural hemangioma have been found in the cervical region
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Figure 6. Case 3: Photomicroscoptc picture shows the typical finding of cavernous hemangioma. Note some blood in the irregular vascular spaces (arrows) (hematoxylin and eosin stain x 165).
[15,17,22]. Only one case was reported in the lumbar region, at the level o f L-1 [2]. The lesion in our last case was located in the level o f L-5, below the level of the conus meduUaris, which might be the lowest level o f reported occurrence. Cavernous hemangiomas, although usually solitary, may associate with the same lesion in other organ systems [22] or in other parts o f the central nervous system [12]. Multiple lesions are found incidentally in approximately one third of the postmortem cases. Occasionally, multiple cavernous hemangiomas in the central nervous system are accompanied by some congenital anomalies elsewhere, such as multiple telangiectases and small cavernous hemangiomas o f the skin and liver, renal or hepatic cyst, or horseshoe kidney [ 18]. This situation suggested cavernous hemangioma might be a kind of mesenchymal malformative lesion and also gave some evidence of a dysembryogenetic nature. Vascular skin
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dysplasia or fibromatosis in the dermatome corresponding to the spinal lesion has also been observed by Johnston [10] and Kaplan [11]. Such combinations are not encountered in other cases. Abnormal, dilated vascular channels in the paravertebral muscles were found in our last case. From the radiological point of view, standard radiograms of the spine showed abnormalities in 41% of the reported cases [ 16]. There might be erosion of the pedicle, the lamina, and the vertebral bodies, and enlargement of the intervertebral foramina. Chest radiographs would sometimes show a mediastinal extension of hemangioma [ 13]. In our cases, the standard radiogram of the spine and chest did not present any pathological evidence. The myelogram usually reveals a picture of total block of the extradural type in most cases, but it could not provide any information on the nature of the lesion. Our cases, except the last one, presented this common picture. With the advent of modern diagnostic procedures like CT scan and magnetic resonance imaging (MRI), we can clearly visualize the lesion and accurately estimate the extent of cord compression. A C T scan through the affected level following administration of intravenous contrast medium may reveal an enhancing area. The MRI would be more valuable, as the mass would be revealed as a low-intensity signal admixed with small areas of high-intensity signals [4]. High-intensity signals represent old clots or hemosiderin within the lesion. This appearance is considered characteristic and is identical to that of intracranial cavernous hemangiomas. All three of our patients were hospitalized before the date of installation of MRI in our hospital, hence the test was not performed. Spinal angiography is usually normal in these cases, but two cases have been referred to as positive [8,15]. The angiographic study in our last case was normal. The treatment of choice for extradural hemangioma has been controversial. In order to decompress the spinal canal, laminectomy followed by radiation therapy has been suggested, and some of the effects have also been described [3,16]. However, not all the cases were treated effectively. From the review of the reported cases, the patient treated by total excision of the lesion usually had a good outcome [ 5,15,17 ]. Complete resection of the lesion seems to be the best method of treatment. In rare situations, the lesion may extend to the anterior aspect of the dural sac, and total excision of the lesion is impossible [16]. The cavernous hemangioma then has the chance to progressively enlarge via the mechanism of capillary proliferation, budding, vessel dilatation, and repeated hemorrhage, followed by organization and canalization. Guthkelch [7] suggested that irradiation may play an important role in the prolonged symptom-free survival of the patient. He found that the
patients treated with radiation were doing better than those not irradiated. In our first case, only subtotal excision could be accomplished and was followed by radiation therapy; no evidence of recurrence was noted at 5 years follow up. It seems that irradiation is a reliable adjuvant therapy for the patients in whom total excision is not possible. We suggest that complete excision of the cavernous hemangioma is the treatment of choice, but subtotal excision should be followed by radiation therapy for achieving better long-term results.
References 1. Cushing H, Bailey P. Tumors arising from the blood vessels of the brain: angiomarous malformations and hemangioblastomas. Springfield, Ill: Charles C Thomas, 1928. 2. Decker RE, Augustin WS, Epstein JA. Spinal epidural venous angioma causing foraminal enlargement and erosion of the vertebral body. J Neurosurg 1978;49:605-6. 3. Dinakar I, Sarada P, Reddy CCM, Kumari GS, Geshpande RP, Sreenivasulu, Sarma NH. Spinal compression due to hemangiomas. IJR 1981;35:283-7. 4. Fontaine S, Melanson D, Cosgrove R, Bertrand G. Cavernous hemangioma of the spinal cord: MR image. Radiology 1988; 166:839-41. 5. Fukushima M, Nabeshima Y, Shimazaki K, Hirohata K. Dumbbell-shaped spinal extradural hemangioma. Arch Orthop Trauma Surg 1987;106:394-6. 6. Giombini S, Morello G. Cavernous angiomas of the brain: account of fourteen personal cases and review of the literature. Acta Neurochir 1978;40:61-82. 7. Guthkelch AN. Hemangiomas involving spinal epidural space. J Neurol Neurosurg Psychiatry 1948;11:199-210. 8. Hurth M. Les h'emangioblastomes intrarachidiens. Neurochirurgie 1975;21(suppl 1):1-136. 9. Jellinger K. Pathology of spinal vascular malformations and vascular tumors. In: Pia HW, Djindjian R, eds. Spinal angiomas: advance in diagnosis and therapy. New York: Springer, 1978. 10. Johnston LM. Epidural hemangioma with compression of spinal cord. JAMA 1938; 110:119-22. 11. Kaplan A. Acute spinal cord compression following hemorrhage within extradural neoplasm. Am J Surg 1942;57:450. 12. Lindboe CF, Nordal HJ. Multiple neurilemomas of the cauda equina, cavernous hemangioma of the spinal cord and degeneration of the lateral corticospinal tract in a man with a clinical diagnosis of multiple sclerosis. Clin Neuropathol 1985;4:260-4.' 13. Mark L, Smiley GL. Hemangioma of the spinal canal.J Kans Med Assoc 1969;67:825-6. 14. McCormick WF. The pathology of vascular ("arteriovenous") malformations. J Neurosurg 1966;807-16. 15. Padovani R, Poppi M, Pozzati E, Tognetti F, Querzola C. Spinal epidural hemangiomas. Spine 198l;6:336-40. 16. Padovani R, Tognetti F, Proietti D, Pozzati E, Servadei F. Extrathecal cavernous hemangioma. Surg Neurol 1982;18:463-5. 17. Richardson RR, Cerullo LJ. Spinal epidural cavernous hemangiomas. Surg Neurol 1979;12:266-8. 18. Rubinstein LJ. Tumors of the central nervous system. In: Atlas of tumor pathology, series 2, fasc 6. Washington, DC: Armed Forces Institute of Pathology, 1972:245. 19. Russell DS, Rubinstein JL. Pathology of tumors of the nervous system, ed 4. Baltimore: Williams & Wilkins, 1977:116-45.
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20. Simard JM, Bengochea FG, BaUingerWE Jr, Mickle JP, Quisling RG. Cavernous angioma; a review of 126 collected and 12 new clinical cases. Neurosurgery 1986;18:162-72. 21. Turner OA, Kernohan JW. Vascular malformations and vascular tumors involving the spinal cord. Acta Neurol Psychiatry 1941;46:444-63.
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22. Wood MW, White RJ, Kernohan JW. Cavernous hemangiomatosis involving the brain, spinal cord, heart, skin and kidney. Report of case. Proc Staff Meeting Mayo Clin 1957;32:24954. 23. Wyburn-Mason R. Vascular abnormalities and tumors of the spinal cord and its membrane. London: Kimpton, 1943.
345
Spinal Extradural Cavernous Hemangioma Jen-Pei Lee, M.D., Alexander Dah-Jium Wang, M.D., Ph.D., Yau-Yau Wai, M.D., and Y a t - S e n H o , M . D . Division of Neurosurgery, Departments of Radiology and Pathology, Chang Gung Medical College & Chang Gung Memorial Hospital, Taipei, Taiwan, Republic of China
Lee J-P, Wang A D-J, Wai Y-Y, Ho Y-S. Spinal extradural cavernous hemangioma. Surg Neurol 1990;34:345-51.
Case R e p o r t s Case I
Three cases of isolated spinal extradural cavernous hemangiomas are reported, two in the thoracic and o n e in the lumbar region. One of them manifested as acute cord compression, the other two as chronic progressive myelopathy or radiculopathy. A total excision or subtotal excision with irradiation was performed. All of them had good functional recovery. The clinical picture, radiological diagnosis, and optimal method of treatment are discussed. The relevant literature is reviewed.
This 25-year-old man presented with a typical history of progressive spinal cord compression. Two weeks before admission, he began to have complaints of interscapular pain, which was aggravated by sneezing, followed by numbness and weakness of both legs 1 week later. Urinary retention and constipation developed 3 days before admission. On admission, he was alert. The general physical examination was normal. The neurological examination revealed spastic paraparesis with hyperreflexia and prominent ankle clonus in the lower limbs. Bilateral positive Babinski's signs were found. He could stand with support but could not walk. The pinprick sensation was abolished below the level of T-7. No impairment of posterior column sensation was noted. His anal tone was preserved but there was urine retention. Plain film of the thoracic spine showed no bony abnormality. X-ray examination of the chest was negative. Myelography performed via the lumbar route showed extradural compression with complete block at the level of T-7 (Figure 1 A). A bilateral laminectomy from T-4 through T-7 was performed under the impression of an extradural lesion with cord compression. A dark red mass was encountered in the posterior surface of the dura mater. It was not encapsulated, extending around both sides of the spinal cord anteriorly from T-5 to the upper margin of T-7. Massive bleeding from dilated vascular channels was encountered during dissection of the lesion from the dura mater. The lesion was gradually removed in one piece from the posterior dural sec. Histopathological examination of the lesion disclosed a typical picture of cavernous hemangioma (Figure 2). The size of the blood vessels varied. These irregular vascular spaces were filled with thrombi and blood. Their walls were lined by a single layer of endothelium and thickened by fibrous and hyaline materials. A course of cobalt 60 radiation therapy was given with 3800 rads to T-4 through T-8. The patient had an
KEY WORDS: Angioma; Cavernous hemangioma; Spinal canal; Vascular malformation
Introduction Cavernous hemangiomas are uncommon vascular malformations of the central nervous system. They are characterized by hemorrhage, seizure, or focal neurological deficits [6]. Although generally located in the cerebral hemisphere, these malformations may be found in any part of the neuraxis. In the spine, the majority of these lesions are from secondary extensions of vertebral hemangiomas [9]. The occurrence of a primary extradural cavernous hemangioma is relatively rare. We report three cases of spinal extradural cavernous hemangiomas without vertebral foci, two in the thoracic and one in the lumbar spine.
Address reprint requests to: Jen-Pei Lee, M.D., Division of Neurosurgery, Chang Gung Medical College & Chang Gung Memorial Hospital, 199, Tung Hwa North Road, Talpei, Taiwan, Republic of China 10591. Received August 29, 1989; accepted May 16, 1990.
© 1990 by ElsevierSciencePublishingCo., Inc.
0090-3019/90/$3.50
346
Surg Neurol 1990;34:345-51
Lee et al
zamide computed tomography (CT) study of the spine (Figure 3) revealed a soft tissue mass in the extradural space of the spinal canal, mainly on the left side from T8 to T-10, with displacement of the cord to the right. A bilateral laminectomy from T-8 through T-10 was performed under the impression of an extradural mass with cord compression. The bone appeared normal. An encapsulated dark purple, lobulated mass was found on the posterior surface of the dura mater. It extended from T-8 to T-10, exclusively on the left side. The mass was clearly defined and easily removed. Histopathological examination confirmed the hemangiomatous nature of the lesion (Figure 4). The patient had a good recovery after surgery. H e has been followed for 1 year. His neurological examinations, including sensory modalities, gait, and sphincters, are normal. H e has gone back to work.
Case 3
Figure 1. Case 1: (A) Myelogram with myodil injected by the lumbar route showing extradural compression with complete block at the level of T-7. (B) Metrizamide CT scan through T-6, performed 5 years after surgery, revealed no recurrence of tumor.
uneventful postoperative recovery. H e was followed up for 5 years; no neurological deficit was found, and he has returned to work. The follow-up radiological study revealed no recurrence of the lesion (Figure 1 B).
Case 2 A 60-year-old, right-handed man had a history of progressive right leg weakness and numbness for 1 year. One month before admission, left leg weakness was also noted. On examination, the patient was alert. The cranial nerves and arms were normal. Paraparesis was present with muscle power at grade 4. Hyperactive deep tendon reflexes were observed in both legs. Hypesthesia was detected below the level ofT-10. Urinary retention was verified. X-ray examination of the spine showed diffuse spondylitic change, more evident in the lumbosacral area. Plain film of the chest was negative. Myelography performed via the lumbar route disclosed a complete block of the opaque contrast medium at T-10. The metri-
This 27-year-old female student was admitted due to complaints o f pain over the posterior aspect o f her right lower leg and sole for I month. She had similar episodes several times in the 6 months prior to admission. N o definite history of trauma was disclosed. On admission, she was alert. The general physical examination was normal. The cranial nerves and her arms were normal. A decrease in pinprick sensation was detected over the S- 1 dermatome on the right. The straight leg raising test was positive at 70 ° on the right side. The sphincter functions were preserved. Myelography performed via the lumbar route disclosed lumbar dura ectasia and poor delineation of the right S-1 root. Metrizamide CT study of the spine revealed a lobulated soft tissue mass in the epidural space at L-5 on the right, with extension through the intervertebral foramina to the paravertebral region. A neurofibroma was first suspected. During surgery, many dilated venous channels in the paraspinal region and epidural space were found. Since the macroscopic appearance of the lesion was of an angiomatous pattern, complete extirpation was postponed. A more detailed workup of the vascular lesion was arranged. A C T study with intravenous contrast enhancement was then performed and revealed a heterogenously enhanced soft tissue mass. The paraspinal muscles in the right side were also enhanced (Figure 5). Spinal angiography performed by catheterization of the femoral artery showed no abnormality. The second operation was performed under the impression of a cavernous hemangioma. A dark red, wormlike mass was found both on the posterior surface of the dura mater, extending through the intervertebral foramina to the paravertebral region on the right, and on the anterior aspect o f the dural sac. Massive venous bleeding from the dilated vascular channels was encoun-
Spinal Extradural Cavernous Hemangioma
Surg Neurol 1990;34:345-51
347
Figure 2. Case 1: Histological appearance of cavernous hemangioma. Note
Figure 3. Case 2: Metrizamide CT scan through T-9 revealed a soft
the irregular cavernous vascular spaces, some of which contain blood and thrombi (arrow). The walls of vascular spaces are lined by a single layer of endothelium and are thickened by fibrous and hyaline material (hematoxylin and eosin stain x 66).
tissue mass in the left extradural space with extension into the intervertebral foramen. The cord was displaced to the right.
tered during dissection of the lesion. The tumor was gradually removed in one piece from the posterior dural sac and amputated from the paravertebral region. Cavernous hemangioma was confirmed by histopathological study (Figure 6). Additional radiation therapy was suggested, but was refused by the patient. She was discharge.d with no neurological deficits and was back to school in 1 month. She was followed for 1 year. The patient is currently still symptom free. Discussion Cavernous hemangioma or cavernoma is a purely descriptive term, signifying the gross and microscopic appearance of those lesions that are composed of irregular, sinusoidal vascular spaces. These lesions have also been called "cavernous angioma," and "cavernous real-
348
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Figure 4. Case 2: Typical histological appearance of cavernous hemangioma (hematoxylin and eosin stain × 165).
formation." The etiology of cavernous hemangioma has been unclear. Although previous authors have considered this lesion to be a vascular neoplasm [1,21], current classification schemes define the cavernous hemangioma as a vascular malformation [9,14]. Wyburn-Mason [23] regarded cavernous hemangioma as a developmental form of telangiectasis. Russell and Rubinstein [19] divided vascular hamartoma into capillary telangiectasis, cavernous angioma, venous malformation, and arteriovenous malformation. These lesions are considered to be of congenital origin and are clearly differentiated from vascular neoplasms. Cavernous hemangioma is an uncommon vascular malformation that may affect any part of the neuraxis but is generally seen intracranially [20]. The spinal cavernous hemangioma has been estimated to represent 5 % - 1 2 % of all spinal vascular anomalies [9]. The majority of these lesions arise within the vertebral bodies and may extend into the extradural space [7]. Only a few cases of primary extradural cavernous hemangiomas without vertebral focus have been reported. Padovani et
Lee et al
Figure 5. Case 3." CT scan with intravenous contrast enhancement through L-5 revealed heterogenously enhanced mass in the right paravertebral region with extension to the spinal canal The paraspinal muscles in the right were also enhanced (arrows).
Spinal Extradural Cavernous Hemangioma
al [16] could only accumulate a series of 27 cases o f hemangioma o f the spinal epidural space from the literature. W e have encountered three cases of epidural cavernous hemangioma, two in the thoracic and one in the lumbar region. From the review of the reported cases, the most common clinical course o f an extradural hemangioma does not differ from that o f other spinal space-occupying lesions with slow but progressive spinal cord compression. T h e rare, acute clinical course is due either to extrathecal hemorrhage [ 10,11] or to thrombotic occlusion within the structure of the vascular lesion causing sudden increase o f the bulk of the lesion [ 15 ]. Richardson and Cerullo [17] reported a case o f cervical epidural hemangioma presenting as radicular pain following minor trauma, which was probably due to microhemorrhage within the lesion. In our cases, two manifested as mass lesions with cord or root compression, one as acute myelopathy. Wyburn-Mason [23] claimed that T-2 and T-6 were the most c o m m o n spinal levels for hemangiomas. This is confirmed by most reported cases. T h r e e cases of epidural hemangioma have been found in the cervical region
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Figure 6. Case 3: Photomicroscoptc picture shows the typical finding of cavernous hemangioma. Note some blood in the irregular vascular spaces (arrows) (hematoxylin and eosin stain x 165).
[15,17,22]. Only one case was reported in the lumbar region, at the level o f L-1 [2]. The lesion in our last case was located in the level o f L-5, below the level of the conus meduUaris, which might be the lowest level o f reported occurrence. Cavernous hemangiomas, although usually solitary, may associate with the same lesion in other organ systems [22] or in other parts o f the central nervous system [12]. Multiple lesions are found incidentally in approximately one third of the postmortem cases. Occasionally, multiple cavernous hemangiomas in the central nervous system are accompanied by some congenital anomalies elsewhere, such as multiple telangiectases and small cavernous hemangiomas o f the skin and liver, renal or hepatic cyst, or horseshoe kidney [ 18]. This situation suggested cavernous hemangioma might be a kind of mesenchymal malformative lesion and also gave some evidence of a dysembryogenetic nature. Vascular skin
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dysplasia or fibromatosis in the dermatome corresponding to the spinal lesion has also been observed by Johnston [10] and Kaplan [11]. Such combinations are not encountered in other cases. Abnormal, dilated vascular channels in the paravertebral muscles were found in our last case. From the radiological point of view, standard radiograms of the spine showed abnormalities in 41% of the reported cases [ 16]. There might be erosion of the pedicle, the lamina, and the vertebral bodies, and enlargement of the intervertebral foramina. Chest radiographs would sometimes show a mediastinal extension of hemangioma [ 13]. In our cases, the standard radiogram of the spine and chest did not present any pathological evidence. The myelogram usually reveals a picture of total block of the extradural type in most cases, but it could not provide any information on the nature of the lesion. Our cases, except the last one, presented this common picture. With the advent of modern diagnostic procedures like CT scan and magnetic resonance imaging (MRI), we can clearly visualize the lesion and accurately estimate the extent of cord compression. A C T scan through the affected level following administration of intravenous contrast medium may reveal an enhancing area. The MRI would be more valuable, as the mass would be revealed as a low-intensity signal admixed with small areas of high-intensity signals [4]. High-intensity signals represent old clots or hemosiderin within the lesion. This appearance is considered characteristic and is identical to that of intracranial cavernous hemangiomas. All three of our patients were hospitalized before the date of installation of MRI in our hospital, hence the test was not performed. Spinal angiography is usually normal in these cases, but two cases have been referred to as positive [8,15]. The angiographic study in our last case was normal. The treatment of choice for extradural hemangioma has been controversial. In order to decompress the spinal canal, laminectomy followed by radiation therapy has been suggested, and some of the effects have also been described [3,16]. However, not all the cases were treated effectively. From the review of the reported cases, the patient treated by total excision of the lesion usually had a good outcome [ 5,15,17 ]. Complete resection of the lesion seems to be the best method of treatment. In rare situations, the lesion may extend to the anterior aspect of the dural sac, and total excision of the lesion is impossible [16]. The cavernous hemangioma then has the chance to progressively enlarge via the mechanism of capillary proliferation, budding, vessel dilatation, and repeated hemorrhage, followed by organization and canalization. Guthkelch [7] suggested that irradiation may play an important role in the prolonged symptom-free survival of the patient. He found that the
patients treated with radiation were doing better than those not irradiated. In our first case, only subtotal excision could be accomplished and was followed by radiation therapy; no evidence of recurrence was noted at 5 years follow up. It seems that irradiation is a reliable adjuvant therapy for the patients in whom total excision is not possible. We suggest that complete excision of the cavernous hemangioma is the treatment of choice, but subtotal excision should be followed by radiation therapy for achieving better long-term results.
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