On-line version ISSN 1678-4227
Arq. Neuro-Psiquiatr. vol.61 no.2A São Paulo June 2003
Angiolipoma lombosacral: relato de caso
Antonio Aversa do SoutoI; Flavio S. DominguesI; Leila ChimelliII; Armando M. LemosI
Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro RJ, Brasil
IServiço de Neurocirurgia
Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro RJ, Brasil
IIDepartamento de Patologia
We present a case of a 46-year old woman with a ventral epidural angiolipoma at the lumbosacral level with erosion of the sacrum. About ninety cases of spinal angiolipomas have been previously described in the literature, most of them situated on the thoracic region, dorsal to the dural sac. Angiolipomas can be radically excised with a good prognosis even in the presence of bone erosion. We did not find any other angiolipoma at the sacral level surgically explored in the review of the literature.
Key words: angiolipoma, spinal tumors, sacral tumors.
Descrevemos o caso de uma mulher de 46 anos com um angiolipoma lombosacral ventral ao saco dural que erodia o sacro. Cerca de noventa casos de angiolipomas foram publicados na literatura, sendo na maioria localizados na região torácica e dorsal ao saco dural. Angiolipomas podem ser ressecados de maneira radical, com bom prognóstico mesmo na presença de infiltração óssea. Não encontramos, em revisão da literatura, nenhum outro caso semelhante de angiolipoma lombosacral com erosão do sacro abordado cirurgicamente.
Palavras-chave: angiolipoma, tumores medulares, tumores sacrais.
Spinal angiolipoma is a benign tumor of the epidural space. It is a rare cause of spinal cord compression, accounting for 0.14% to 1.2% of the spinal tumors1. It is considered a distinct clinical and pathological entity traditionally grouped as a variant of lipoma1. Characteristically the tumor lies over the dorsal aspect of the dura at the thoracic level2-7. Its port-wine color or dark brown appearance contrasts very well with the normal epidural fat1,8,9. Sometimes the tumor can be more aggressive and invade the contiguous bone and adjacent soft tissues10,11. We report a patient with a lumbosacral angiolipoma with bone erosion associated with a L4-L5 left sided disc herniation.
A 46-year old female with a history of ten years of low back pain had a worsening of the symptoms in the three months before diagnosis. The pain radiated down the posterior aspect of the left thigh, calf and ankle, and increased with walking and physical strength. The patient also referred a progressive numbness of the perineum. A neurological examination demonstrated a mild paresis of the plantar flexion of the left toe and hypoactive left jerk reflex. A positive straight leg-raising test at ten degree at the left side could be elicited. Superficial hypoesthesia at the lateral aspect of the left foot, buttocks and perineum was noted. An X-ray of the lumbar spine and sacrum showed erosion of the posterior aspect of the sacrum and widening of the sacral canal. A MRI scan revealed an epidural mass displacing the dural sac posteriorly, eroding the bone and projecting to the anterior sacral foramina with the sacral root. The mass was isointense in T1-weighted and hyperintense in T2-weighted and showed a homogeneous and intense enhancement with gadolinium infusion. There was also a left sided disc herniation associated with the upper limit of the tumor at the level of L4-L5 space (Fig 1).
She was submitted to a L4-L5 laminectomy and a posterior opening of the sacral canal with a wide exposure of the dura and sacral roots bilaterally. The dural sac was pushed back and ventrally compressed by a firm and large port-wine highly vascularized mass, which partially encased the sacral roots and infiltrated the sacrum (Fig 2). The mass was totally resected with preservation of the roots. The herniated disc was dissected from the inferior aspect of the left L5 root and resected. Subcutaneous fat was harvest and then used in hemostasis and for filling the large epidural space ventral to the dural sac.
The patient recovered completely of the previous symptoms and could return to her previous activities. Two repeated MRI studies six and eighteen months after the surgical procedure confirmed the total resection and did not show any evidence of tumor recurrence (Fig 3). Histopathological analysis was characteristic of an angiolipoma and showed that the lesion was composed by adult fatty tissue intermingled with many thin walled blood vessels (Fig 4).
Berenbruch in 1890 described the first case of angiolipoma in a sixteen-year-old male confirmed in an autopsy12. Since then about ninety cases of angiolipoma have been reported at the literature. In the past, the finding of fatty tumors in the epidural space would be considered a matter of little interest and many cases would be unreported13. With the advance of MRI and the widespread use of operating microscope, even those small tumors have been distinguished from the epidural fat, and the number of cases being reported is increasing13. Otherwise those slow growing tumors would be unrecognized by many surgeons1,13.
In the review of Preul1 spinal angiolipomas account for 0.14% to 1.2% of all spinal tumors and 2% to 3% of extradural spinal tumors. Males and females are almost equally affected. The mean age of patients is 41.6 years (the youngest was 6 years and the oldest 72 years old)1. If the lipomas secondary to prolonged high corticosteroid intake or following epidural injections are excluded, most of the symptomatic fatty tumors in the epidural space are angiolipomas4,14. Spinal angiolipoma typically presents as a slow growing mass causing compression of the spinal cord. Lower-extremity numbness, back pain and leg weakness are frequent initial complaints 1. The mean duration of the symptoms is usually longer than one year4,15,16. There were rare described cases of sudden installation of the symptoms or abrupt neurological deterioration17. Increasing of the body weight and pregnancy appear to exacerbate the symptoms due to changes in the tumor mass and volume1,4,16. The concomitant presence of spinal tumors and discal hernia is probably rare. However, before the introduction of CT and MRI studies, spinal tumors might be misdiagnosed as herniated intervertebral disk and vice-versa13. In the present case the simultaneous occurrence of a lumbar disc herniation could be crucial in the development of the symptoms of radicular compression. The sacral location of the tumor in this case explained the atypical clinical presentation. The association of spinal angiolipoma and lumbar disc herniation was found in two previous reported cases9,13.
Histopathologically angiolipomas are characterized by mature adipose tissue containing copious vascular elements that vary from sinusoids, thin-walled vessels or thick-walled vessels with proliferation of the smooth muscle layer4. Mitotic figures are infrequent and malignant changes have not been identified18. They have a gross aspect that vary in color from whitish-yellow to a grayish-purple and can be usually well distinguished from the normal epidural fat1,9,13,19. The pathogenesis of angiolipomas is unknown. They may result from abnormal development of the primitive, pluripotential mesenchymal cells from which adipose tissue and vascular endothelium arise or may be hamartomatous in nature1,16,17.
Almost all-spinal angiolipomas are located at the epidural space. The great majority of the epidural angiolipomas are located in the thoracic region at the posterior surface of the dura1,4,13,16,20-23. There have been described eight cases of angiolipomas involving the lumbar spine16,24. They were frequently located anteriorly in the spinal canal in contrast with the regular dorsally located angiolipomas at the thoracic levels1,15, 17. Kasper et al. in 1929 reported an autopsy study with angiolipomas in the lumbosacral area (from L3-S3) and in the cervical spine canal in a 6-years old boy25. Nishiura et al. described a case of left L5 and S1 radicular compression pre-operatively diagnosed as disc herniations that was explored through foraminotomies9. They found a hemorrhagic mass similar to our case compressing the L5 and S1 roots which was subtotally removed and the final diagnosis was hemangiolipoma. Palkovic et al.28 reported the first case of intramedullary angiolipoma and Preul et al.1 described another similar case.
In 1966 Gonzalez-Crussi et al described the first case of infiltrating spinal angiolipoma27. They considered that bone infiltration was associated with a more aggressive behavior and a worse prognosis. Subsequent studies failed to demonstrate differences in prognosis in the infiltrating angiolipomas in comparison with the non-infiltrating group16. These invasive angiolipomas are more common at the ventral aspect of the dura in the lumbar spinal canal13,27. Total resection is the goal of the surgical treatment and could be achieved less frequently in the infiltrating angiolipomas specially if they are anterior and anterolateral in location1,28. However, angiolipomas sub-totally resected have also a good prognosis with rare recurrences described even in the infiltrating variant12. Almost all the patients have improvement of the neurological deficits after the surgery with total or near-total recovery17.
Plain X-rays are normal in the majority of the cases. Computed tomography studies are not conclusive in angiolipomas, with the epidural mass more often isodense or slightly hyperdense with light or absent homogeneous contrast enhancement1,29. In MRI studies the extradural components of angiolipomas are isointense or hyperintense in T1-weighted images, probably due to the fat component and usually hyperintense in T2-weighted images. Those areas show an early enhancement after gadolinium administration and are considered the vascular component1,16. Slow-growing masses dorsally located in the thoracic spinal canal with these MRI findings should be suspected of angiolipoma. With the widespread availability of MRI, spinal angiolipomas have been more frequently recognized and diagnosed.
In conclusion, spinal angiolipomas are rare tumors with a benign behavior. When localized in the thoracic spinal canal they have usually a well-defined clinical-radiological presentation with MRI studies. The authors describe a case of a lumbosacral angiolipoma diagnosed in vivo and totally resected with a good outcome. To the best of our knowledge we could not find any other angiolipoma with erosion of the sacrum reported in the literature.
1. Preul MC, Leblanc R, Tampieri D, Robitaille Y, Pokrupa R. Spinal angiolipomas: report of three cases. J Neurosurg 1993;78:280-286. [ Links ]
2. Aguiar PH, Plese JP, Rosemberg S, et al. Thoracic spinal angiolipoma: case report. Arq Bras Neurocirurg 1996;15:103-107. [ Links ]
3. Balbo RJ, Araujo JF, Melro CA, Iafigliola MG, Valvassore FR. Thoracic epidural angiolipoma: case report. Arq Neuropsiquiatr 1995;53:659-661. [ Links ]
4. Haddad FS, Abla A, Allam CK. Extradural spinal angiolipoma. Surg Neurol 1986;26:473-486. [ Links ]
5. Obrador S, Villarejo F, Deblas A. Angiolipoma extradural causa infrequente de compression medullar. Rev Clin Esp 1977;146:395-396. [ Links ]
6. Padovani R, Tognetti F, Speranza S, Pozzati E. Spinal extrathecal hemangiolipomas: report of two cases and review of the literature. Neurosurgery 1982;11:674-677. [ Links ]
7. Turgut M. Spinal angiolipomas: report of a case and review of the cases published since the discovery of the tumor in 1890. Br J Neurosurg 1999;13:30-40. [ Links ]
8. Machado JAF, Fabião OM Neto, Ordovas CA, Hack I, Gallo P. Spinal angiolipoma: case report. Arq Bras Neurocirurg 1993;12:141-144. [ Links ]
9. Nishiura I, Kubo Y, Koyama T. Spinal haemangiolipoma: three case reports. Neurochirurgia (Stuttg) 1986;29:63-66. [ Links ]
10. Rivkind A, Margulies JY, Lebenstart P, Sherman Y, Robin GC. Anterior approach for removal of spinal angiolipoma: a case report. Spine 1986;11:623-625. [ Links ]
11. Sakaida H, Waga S, Kojima T, Kubo Y, Matsubara T, Yamamoto J. Thoracic spinal angiolipoma with extracanal extension to the thoracic cavity: a case report. Spine 1998;23:391-394. [ Links ]
12. Stookey B. Intradural spinal angiolipoma: report of case and symptoms for 10 years in child aged 11: review of literature. Arch Neurol Psychiatry 1927;18:16-43. [ Links ]
13. Pagni CA, Canavero S. Spinal epidural angiolipoma: rare or unreported? Neurosurgery 1992;31:758-764. [ Links ]
14. Von Hanwehr R, Apuzzo MLJ, Ahmadi J, Chandrasoma P. Thoracic spinal angiomyolipoma: case report and literature review. Neurosurgery 1985;16:406-411. [ Links ]
15. Pearson J, Stellar S, Feigin I. Angiolipoma: long term cure following radical approach to malignant-appearing benign intraspinal tumor. J Neurosurg 1993;78:280-286. [ Links ]
16. Trabulo A, Cerqueira L, Monteiro P, Roque P, Reis FC, Coelho MR. Spinal angiolipomas revisited: two case repoerts. Acta Neurochir (Wien) 1996;138:1311-1319. [ Links ]
17. Labram EK, el-Shunnar K, Hilton DA, Robertson NJ. Spinal angiolipoma: three additional cases. Br J Neurosurg 1999;13:25-29. [ Links ]
18. Lin JJ, Lin F. Two entities in angiolipoma: a study of 459 cases of lipomas with review of literature on infiltrating angiolipoma. Cancer 1974;34:720-727. [ Links ]
19. Boockvar JA, Black K, Malik S, Stanek A, Tracey KJ. Subacute paraparesis induced by venous thrombosis of a spinal angiolipoma: a case report. Spine 1997;22:2304-2308. [ Links ]
20. Bender JL, Van Landingham JH, Manno NJ. Epidural lipoma producing spinal cord compression. Report of two cases. J Neurosurg 1974;41:100-103. [ Links ]
21. Bouramas D, Korres D, Roussos L, Mantzilas T, Anagnostopoulos D. Spinal extradural angiolipoma. J Spinal Disord 1995;8:324-327. [ Links ]
22. Costa FA, Balaguez HM, Ferreira LC, et al. Dorsal spinal epidural angiolipoma: case report. Arq Bras Neurocirurg 1998;17:48-50. [ Links ]
23. Rubin G, Gornish M, Sandbank J, Shevach I, Rappaport ZH. Spinal extradural angiolipoma: case report and review of the literature. Spine 1992;17:719-723. [ Links ]
24. Provencale JM, McLendon RE. Spinal angiolipomas: MR features. AJNR 1996;17:713-719. [ Links ]
25. Kasper GA, Cowan A. Extradural lipoma of the spinal cord. Arch Pathol 1929;8:800-802. [ Links ]
26. Palkovic S, Wassman H, Bonse R, Kashab M. Angiolipoma of the spinal cord. Magnetic resonance imaging and microsurgical management. Surg Neurol 1988;29:243-245. [ Links ]
27. Gonzales-Cruzzi F, Ennecking WF, Arean WM. Infiltrating angiolipoma. J Bone Joint Surg 1966;48:1111-1124. [ Links ]
28. Kuroda S, Abe H, Akino M, Iwasaki Y, Nagashima K. Infiltrating spinal angiolipoma causing myelopathy: case report. Neurosurgery 1990;27:315-318. [ Links ]
29. Michilli R, Tzonos P, Iglesias-Rozas JR. Spinal extradural angiolipoma: case report and literature review. Neurochirurgia (Stuttg) 1993;36:63-65. [ Links ]
Received 23 July 2002, received in final form 23 October 2002
Accepted 6 November 2002
Dr. Antonio Aversa do Souto - Serviço de Neurocirurgia - Hospital Universitário UFRJ - Avenida Brigadeiro Trompowski s/n/10º andar - 21941-590 Rio de Janeiro RJ - Brasil. E-mail: email@example.com