AUTHOR(S): Pagni, Carlo Alberto, M.D.; Canavero, Sergio, M.D. Institute of Neurosurgery, University of Turin, Ospedale S.G.B.C.T. "Molinette," Turin, Italy Neurosurgery 31; 758-764, 1992 ABSTRACT: THREE CASES OF spinal epidural angiolipoma, all affecting middle-aged women, are reported. Spinal epidural angiolipomas are considered a separate entity from the more common lipomas involving the same space. Although these tumors are considered very rare, the occurrence of three cases in less than 2 years in the same geographical area raises the question of their frequency. The computed tomographic scan has been misleading in two of our patients, whereas magnetic resonance imaging was highly suggestive. Two of these tumors were apparently exceptional, being lumbar and anterior. The patients were admitted with typical sciatic symptoms; one tumor eroded the vertebral body. Spinal epidural angiolipomas may go unreported because their pathogenetic potential is not fully recognized. We suggest that both magnetic resonance imaging and the operating microscope should have a more significant place in the evaluation and treatment of sciatica. KEY WORDS: Angiolipoma; Epidural; Epidural spinal tumor; Spinal cord Spinal angiolipoma is a benign tumor with an apparently restricted growth to the epidural space. It reportedly accounts for 0.14% of all spinal axis tumors, rendering this entity a rare lesion (7,10,13,38). It has been a matter of recent interest (19,23,38). In the last 2 years, we have operated on three women with spinal epidural angiolipoma (SEA), all from the same geographical area. This report critically addresses their features, with reference to the literature. Patient 1 has been the subject of a previous work (23). PATIENT 1 This 42-year-old woman underwent previous surgery for an uterovescical prolapse. Starting in July 1988, she developed progressive lower extremity sensory deficits, beginning with paresthesias of the left toe. Subsequently, it spread to involve both lower extremities and the trunk. She found walking increasingly difficult. Whole-spine magnetic resonance imaging (MRI) revealed a D5-D6 posterior epidural lesion, with both lipomatous and vascular components (23). A physical examination at the time of admission demonstrated paraparesis, polykinetic knee jerks, ankle clonus, pain-touch hypoesthesia of the lower trunk and both lower extremities, and

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bilateral Babinski's sign, but no tenderness of the spine. During an operation, a D3-D7 laminectomy was fashioned. The bone was unaffected; schisis was absent. Under magnification, a dark-yellow lobulated mass with several small vessels was seen girding the dural sac. This was removed piecemeal and dissected from the dura, in spite of profuse bleeding. The mass merged into normal peridural fat at its poles. At the end, the sac remained fixed in its lateral position, as if it had been displaced for a long time (malformation?). The postoperative course was uneventful. She is currently free of her previous symptoms. The pathological response was angiolipoma (8). PATIENT 2 This 56-year-old woman underwent an operation for colecystosis. Since 1978, she suffered from recurring episodes of low-back and left sciatic pain, with worsening shortly before hospitalization. Computed tomography (CT) revealed a circumscribed round mass affecting the body of L3, with sharp homogeneous contrast enhancement (Fig. 1b). The mass filled and widened the left portion of the vertebral canal and homolateral intervertebral foramen; it sharply eroded into the posterior wall and left pedicle of L3 (Fig. 1a). No trabeculation of the vertebral body was seen. A diagnosis of left L4 root neurinoma was entertained. At the time of admission, this patient complained of a typical left L5 sciatica. The raising-leg test was positive on the left; the Wassermann-Neri maneuver elicited no pain. The left lower limb was slightly hyposthenic. The remaining physical findings were normal. A L3-L4 foraminotomy was shaped. The intervertebral foramen was filled with fatty tissue. Under magnification, a slightly protruding L3-L4 disc was seen and excised; however, given its size, it could not account for the L5 symptoms. The L3 lamina was cut away, and bleeding ocher fatty tissue present in the foramen was partially aspirated. The root was normal. At this point, medial displacement of the dural sac revealed a small, anterior, ocher-yellow mass with three to four fatty lobules and a few vessels. This was completely removed, which permitted the identification of the CT-visualized L3 erosion. The postoperative course was regular; 2 months later, the patient was fully asymptomatic. A pathological examination revealed an angiolipoma (Fig. 2). PATIENT 3 This 59-year-old woman suffered from congenital hip dislocation, chronic pancreatitis, and multiple abdominal cysts (one adrenal and two hepatic). Since the age of 32, she kept complaining of low-back pain. One year before admission, this worsened and spread to the left lower extremity. Medical treatment was unrewarding. A CT scan revealed a strip of parenchymatous tissue at L4-L5, filling the whole anterior portion of the vertebral canal (Fig. 3a); this extended left and up, to the posterior wall of L4. A diagnosis of hernia was entertained. At the time of admission, she complained of a left sciatica, with electric shock-like sensations in the L5 territory; the neurological examination was normal, except for a

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Neurosurgery 1992-98 October 1992, Volume 31, Number 4 758 Spinal Epidural Angiolipoma: Rare or Unreported? Case Report

DISCUSSION SEA have been considered separate entities from their nonvascular counterparts (13,38), with the following features: 1) they are found exclusively in the epidural space, never intradurally (13,38); 2) they are generally dorsal or dorsolateral (7,10,38); 3) they have a general thoracic localization, with no purely lumbar lesion to be found in the literature (2-38); 4) they have no age or sex predominance (10,38); 5) they usually are accompanied by lower limb weakness or symptoms due to epidural compression (10,38); 6) their history is less than 1 year in about two thirds of patients (10); and 7) a subset may infiltrate the vertebral bone (reviewed in reference (19)). SEA may have a dysembryogenetic origin, differing only in degree from congenital lipomas. In fact, they are, in at least a portion of cases, associated with maldevelopmental lesions (10). Our Patient 3 had been affected by congenital hip dislocation and multiple abdominal cysts, a unique association in the literature. On the other hand, it has been pointed out that SEA may represent an intermediate step of a spectrum ranging from lipoma to full-rank angioma (38) ; this being true, they should not be considered separately from other lipomas. All of our patients were middle-aged women, a finding at odds with the asserted absence of sex and age predominance (10,38) but in agreement with another report (13). Although SEA are usually dorsal and thoracic, the lesions of Patients 2 and 3 had an extraordinarily unusual pure lumbar anterior localization. This explained the atypical clinical presentation; in fact, both patients complained of low-back and sciatic pain, ascribable to a discal hernia. The CT diagnosis in Patient 3 was indicative of a discal hernia tout court. We are not aware of other similar reports in the literature. It is a matter of fact that a spinal tumor may sometimes be misdiagnosed as herniated intervertebral disk and vice versa (1); in our patient, this was compounded by the finding of an equivalent CT density value between the SEA and hernia. Although the acute symptoms of SEA may be explained on the grounds of intralesional thrombosis, hemorrhage, or steal phenomena (30,38) with rapidly evolving symptoms (30), the course of SEA may be apparently very long, 12 and 27 years in Patients 2 and 3, respectively. Long courses with fluctuating symptoms are well described (6,10). A small subset of SEA is characterized by a marked infiltration of the vertebral body (19,33,38). This

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infiltrative potential, not found in ordinary lipomas (38) , is also well recognized for angiolipomas in other body districts (19,38). Infiltrative SEA may be still a different class of tumors from their noninfiltrating counterparts. Our Patient 2 was admitted with an erosion of the vertebral body, as were patients from other cases in the literature (36). It might be speculated that, if lipomas and hemangiomas truly represent the limits of the same pathologic spectrum (38), angiolipoma, angiolipoma with erosion, and infiltrating angiolipoma may represent intermediate steps. We described MRI findings of SEA elsewhere (23); they should be suspected if a spinal extradural mass with isohyperintensity in T1-weighted images and hyperintensity in T2-weighted images plus early contrast enhancement are seen. Only two other reports describing MRI findings of SEA exist (19,24). CT findings have been reported only recently (13,19,23, 24,38) . Slightly hyperdense irregular but defined extradural masses, with diffuse homogeneous contrast enhancement, were seen in our Patients 1 and 2. Patient 2 was admitted with an erosion. These are all unspecific findings. A CT scan may be misleading, as previously discussed. It has been pointed out that minimal CT contrast enhancement distinguishes the small subgroup of infiltrating SEA (six reported up to now; [19]) from hemangiomas of the vertebral body (19,38). SEA are considered very rare: less than 50 cases have been reported up until now (2-38). Since the first histologically proven SEA, dating back to 1890 (4), an average of about one report every other year is found. The finding of three SEA in less than 2 years from the same geographical area, as presently discussed, casts some doubt on this assumption of rarity. In 1945, Ehni and Love (3) wrote: "Extradural lipoma, however, is of a commoner cut, needing no special explanation, exciting less interest and more often going unreported" (7). In 1976, Giuffrè (10) came to the same conclusion: "Presumably the finding of a fatty tumor in such a common site for adipose tissue as the epidural space has been regarded as a routine matter of little interest." This is compounded by the fact that, even at small sizes, SEA can produce symptoms (as demonstrated by our Patients 2 and 3). SEA, and extradural lipomas in general, may thus go unreported because of their not being well recognized by the surgical team. In particular, we suggest that several "typical" sciaticas, operated on without magnification, go unexplained or are considered "functional". Although CT was introduced in 1973, no report describing the associated findings of SEA is to be found up to 1985 (38; see reference (14)), emphasizing the role played by myelography in suspected spinal pathologies up to that time. Yet, the diffusion of CT scanners have not resulted in a substantial increase of reports. As discussed, CT is not specific for SEA and may be misleading. This is compounded by the infrequent use of the surgical microscope in the treatment of sciatica. This latter helps recognize small lesions in the midst of the peridural fat, even when this is particularly abundant. However, even for suspected spinal lesions operated

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positive leg-raising test at 60 degrees on the right and 30 degrees on the left plus left ankle hyporeflexia. A partial L4-L5 foraminotomy was fashioned, and an operative microscope was used. A few veins were seen in the peridural space. The discal space showed no hernia. However, a small, soft, yellow mass faced the dural sac, extending below the left L5 root. Removal was next to radical because of the profuse bleeding of the mass; large surrounding veins were coagulated. A pathological examination disclosed an angiolipoma (Fig. 4). Three months later, the patient was back to normal. A CT scan showed some fatty tissue left at L5 (Fig. 3b).

11.

ACKNOWLEDGMENT We gratefully acknowledge Drs. M. Forni and E. Gaidolfi for contributing their pathological and neuroradiological comments.

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Received, August 28, 1991. Accepted, March 11, 1992. Reprint requests: Prof. C. A. Pagni, Istituto di Neurochirurgia, Università di Torino, Ospedale S.G.B.C.T. "Molinette," Via Cherasco 15, 10126, Torino, Italy.

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on under magnification, if the surgeon is unaware of the possibility of a SEA, both dorsal and anterior, the final result of surgery will be compromised. We make a strong point in favor of MRI as the imaging modality of choice in the initial evaluation of sciatic symptoms, in particular now that costs are rapidly cut and that this is gaining widespread use. The prognosis of SEAs, even if infiltrating, is good (19) . No radiotherapy should be given to patients with these otherwise benign lesions. Surgery with a posterior approach should be generally elected.

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COMMENTS Spinal epidural angiolipomas are a rarely reported type of tumor. There are two subgroups. The more well-known tumors are classically thoracic in location, dorsal to the thecal sac, and do not infiltrate, although they may erode bony elements. The other subgroup is predominantly anterior in location with extensive infiltration of the vertebral body. They display a trabeculated appearance on plain radiographs and computed tomographic scan, virtually indistinguishable from the more common hemangioma. Their failure to enhance with contrast administration and the abundance of fatty stroma distinguish this group from hemangiomas. The prominence of smooth muscle elements has resulted in some of these tumors also being called angiomyolipomas. The authors report 3 patients, the first with a typical thoracic, dorsal angiolipoma. Patients 2 and 3 were found to have angiolipomas in the lumbar spine, a rare location leading to an unusual presentation. As

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29.

Figure 2. Mature adipose tissue interspersed with small- and medium-caliber blood vessels is shown, indicative of an angiolipoma (Patient 2); hematoxylin and eosin.

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Figure 1. CT scan, Patient 2. A round homogeneous mass (a) with brisk, complete contrast enhancement (b) is seen. This widens the left intervertebral foramen and erodes into the posterior wall of L3 (arrow).

Figure 4. The features of a typical angiolipoma are again shown (Patient 3); hematoxylin and eosin.

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Figure 3. CT scan, Patient 3. A strip of tissue mimicking a herniated intervertebral disk is seen in the L4-L5 space (a). Postoperative CT scan: only a small portion of the tumor is left (b).

Table 1. SEA: No CT Data Redistribution of this article permitted only in accordance with the publisher’s copyright provisions. Downloaded from https://academic.oup.com/neurosurgery/article-abstract/31/4/758/2752053 by Washington University in St. Louis user on 04 June 2018

Table 3. SEA: Cases with No Data Available to the Authors, Dubious Cases, and Cases Included in Other Series

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Table 2. SEA: CT Data and Eventual MRIa

Spinal epidural angiolipoma: rare or unreported?

Three cases of spinal epidural angiolipoma, all affecting middle-aged women, are reported. Spinal epidural angiolipomas are considered a separate enti...
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