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 Table of Contents  
CASE REPORTS
Year : 2022  |  Volume : 5  |  Issue : 2  |  Page : 235-240

Epidural angiolipoma and Andersson lesion: A rare co-occurence—A case report and literature review


1 Spine Unit, Department of Neurosurgery, I-NK, Institute of Neurosciences, Kolkata, West Bengal, India
2 Department of Neuropathology, I-NK, Institute of Neurosciences, Kolkata, West Bengal, India

Date of Submission23-May-2021
Date of Decision24-Jun-2021
Date of Acceptance17-Jul-2021
Date of Web Publication08-Jun-2022

Correspondence Address:
Selvin Prabhakar Vijayan
Spine Unit, Department of Neurosurgery, I-NK, Institute of Neurosciences, 185, Acharya Jagadish Chandra Bose Road, Elgin, Kolkata 700020, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/isj.isj_47_21

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  Abstract 

Spinal angiolipomas (SALs) are very rare benign extradural neoplasms, representing 0.14%–1.2% of all spinal tumors. Andersson lesion is a localized vertebral or disco-vertebral lesion in ankylosing spondylitis. Co-occurrence of these lesions is very rare and has never been reported. We are reporting one such case of a 61-year-old man presented with features of dorsal compressive myelopathy. On magnetic resonance imaging (MRI), he was diagnosed to have a SAL at D6–D9 and Andersson lesion at D7–D8 and L1–L2. He was managed surgically with a long-segment fixation and decompression and gross total excision of the tumor. Diagnosis of a SAL was confirmed in a biopsy, and postoperatively, he showed good clinical improvement. In conclusion, given the rarity of co-occurrence of these pathologies, a strong clinical suspicion is required. MRI and CT scan help in diagnosis. Both these pathologies warrant surgical management. When diagnosed early and managed appropriately, they have a good prognosis.

Keywords: Andersson lesion, ankylosing spondylitis, extradural, spinal angiolipomas, spondylodiscitis, treatment


How to cite this article:
Vijayan SP, Gerber C, Basu A, Basu G, Mhatre R. Epidural angiolipoma and Andersson lesion: A rare co-occurence—A case report and literature review. Indian Spine J 2022;5:235-40

How to cite this URL:
Vijayan SP, Gerber C, Basu A, Basu G, Mhatre R. Epidural angiolipoma and Andersson lesion: A rare co-occurence—A case report and literature review. Indian Spine J [serial online] 2022 [cited 2022 Dec 4];5:235-40. Available from: https://www.isjonline.com/text.asp?2022/5/2/235/346968




  Introduction Top


Spinal angiolipomas (SALs) are very rare extradural benign neoplasms, histologically composed of mature fat cells and anomalous vascular channels.[1],[2] It constitutes 2%–3% of all extradural spinal tumors.[2],[3] Given their rarity, it is often misdiagnosed or under-reported.[4] Usually, they are slow-growing lesions, causing chronic progressive symptoms. Magnetic resonance imaging (MRI) is the gold standard diagnostic method, but confirmatory diagnosis is by histopathological examination. Surgical excision is the standard treatment option and has a good prognosis following surgery.[3] Though there are some reports of a SAL occurring in combination with other pathological conditions, to our knowledge, there has not been any reports of its co-occurrence with Andersson lesion (AL).

Ankylosing spondylitis (AS) is a chronic seronegative inflammatory arthropathy that primarily affects the spine and sacroiliac joints, causing progressive ossification and finally leads to an ankylosed spine. Andersson, in 1937, described a localized vertebral or disco-vertebral lesion in AS, which later came to be known as AL, also called as “disco-vertebral lesion” or “spondylodiscitis.” Its prevalence ranges from 1.5% to over 28%.[5] Multiple theories for etiology are being proposed, all leading to a final pathway of abnormal mechanical stress preventing fusion at the affected level, leading to pseudoarthrosis. Computed tomography (CT) and MRI both aid in diagnosis and surgical planning. The treatment for AL may differ depending on the degree of the lesion; a symptomatic three-column spinal lesion needs surgical stabilization.

Our case is an unusual presentation of a SAL co-occurring with multiple ALs requiring surgical management. Performing a search of any similar case report in open literature using the Pubmed search engine was unfruitful. To the best of our knowledge, this is the first such reported case.


  Case Report Top


Our patient was a 61-year-old man, a known diabetic and hypertensive, presented with complaints of low backache and disbalance while walking for three years with acute worsening of symptoms over the past one month. There was associated tingling and tightness of both lower limbs. There was no history of any trauma. On examination, there was severe spasticity (Modified Ashworth Scale grade 2). The gross motor power and sensations were intact. Superficial reflexes were absent, deep tendon reflexes were exaggerated, and bilateral Babinski showed extensor response. He had a spastic, assisted (walking with a stick) bipedal gait with a Nurick score of grade 4 and modified Japanese Orthopaedic Association score of 14. Radiological investigations including MRI and CT scans were done. MRI revealed a crescent-shaped posterior epidural space-occupying lesion extending from D6 to D9 levels, which was hyperintense on T1, T2 images, and enhancing on contrast image with flow voids, probably suggesting epidural angiolipoma. Endplate changes at D7–D8 and L1–L2 levels suggested AL, and other findings pertaining to AS were also seen [Figure 1]. CT scan showed endplate irregularities, osteophytes, absence of fusion of posterior elements, and vacuum sign in disc space at both levels, all suggestive of pseudoarthrosis. Bony changes involved all three columns at both D7–D8 and L1–L2 levels, causing a potentially unstable lesion [Figure 2]. His HLA-B27 test done on admission was positive. Thus, he was diagnosed to have a two-level AL with an epidural space-occupying lesion, probably a SAL, and was planned for surgical management. As there were ALs at two levels, a long-segment fixation from D6 to L3 was carried out [Figure 3]. A D6–D9 laminectomy was done based on the extent of the tumor on the posterior epidural aspect. Under an operating microscope, the lesion appeared to be a grayish red vascular tissue mass adhered to the dural surface. We performed gross total excision of the lesion using spinal microinstruments after coagulating it using a bipolar cautery prior to dissection. Intraoperative multimodal neuromonitoring with somatosensory evoked potential and motor evoked potential was used, and no significant alterations of signals were noted during surgery. The tissue was then sent for histopathological examination.
Figure 1: T1, T2, and contrast sagittal images showing ankylosis of entire spinal column, endplate changes with AL at D7–D8 and L1–L2 level, crescent-shaped epidural lesion at D6–D9 with T1 and T2 hyperintensity, and heterogenous contrast enhancement suggesting an epidural SAL. T2, contrast axial images show angiolipoma on the dorsal aspect of canal compressing cord

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Figure 2: Sagittal, axial, and 3D reconstruction CT images showing irregular end plates, osteophytes, and vacuum sign suggesting pseudoarthrosis at D7–D8 and L1–L2 levels involving all three spinal columns with a visible gap

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Figure 3: Postoperative antero-posterior and lateral x-ray showing D6–L3 pedicle screw fixation and D6–D9 laminectomy

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The patient was started on physiotherapy the following day. The biopsy showed mature adipose tissue intermingled with thick- and thin-walled blood vessels, and immunohistochemistry showed the vascular endothelial cells [Figure 4]. Thus, the biopsy confirmed the diagnosis of angiolipoma. At three months followup he was able to walk well (Nurick grade 2 and modified Japanese Orthopaedic Association scale score of 16); spasticity and tingling had reduced significantly.
Figure 4: Microphotograph showing lobules of mature adipose tissue intermingled with thick- and thin-walled blood vessels (A, B). Immunohistochemistry for CD31 highlights the vascular endothelial cells (C). Magnification: Obj. ×10

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  Discussion Top


Angiolipomas are benign lesions occurring most commonly in the subcutaneous plane, very rarely in the epidural space. They represent 0.14%–1.2% of all spinal tumors, about 2%–3% of epidural spinal tumors and 16%–35% of spinal lipomas.[6] In 1960, Howard and Helwig established it as a separate pathological entity.[7] Till now, only about 200 cases have been reported in literature.[8] They commonly occur in fourth to sixth decade with a female predominance.[9] The most common site is thoracic followed by lumbar, sacral, and cervical.[10] About 90% of lesions arise in the posterior epidural spaces, extending over several vertebral bodies. Our case was a male in his 6th decade with a thoracic lesion extending from D6 to D9 in the posterior epidural space. Many physiopathological mechanisms of angiolipomas were proposed. The most accepted among them is that it arises from pluripotent mesenchymal stem cells and differentiates under the influence of certain unknown stimulating factors.[8]

Lin and Lin first described SALs as two types: non-infiltrating and infiltrating.[11] The majority are non-infiltrating type; they are encapsulated, do not invade into adjacent tissues or vertebra, and have a benign course.[2] In contrast, infiltrating types are non-encapsulated and may extend into adjacent structures.[9] Clinical features of SALs vary depending on the site of lesion, degree of compression, and structures compressed. Several factors such as trauma, inflammatory disorders and hormonal imbalance (premenopause, pregnancy), and excessive weight gain have been known to accelerate the growth of SALs.[12] When sudden deterioration of symptoms is present, tumor thrombosis and/or hemorrhage should be suspected.[13]

A commonly associated complication with AS namely disco-vertebral lesions was first described by Andersson[14] in 1937 and hence called AL. The incidence rate mentioned in the literature ranges from 1%[15] to as high as 28%.[16] It is usually a solitary lesion, and the most affected site is the thoracolumbar junction. Our case had a two-level lesion, one thoracic and one thoracolumbar. Like SALs, there are multiple theories of etiology for AL as well. Important ones are inflammatory endplate changes and high mechanical stress at the last unfused segment causing abnormal mobility, thus preventing ankylosis and stress fracture due to trivial trauma caused by severe osteoporosis associated with AS. These different mechanisms finally lead to pseudoarthrosis at the level of AL. Depending on the degree of involvement, Cawley et al.[17] in 1972 categorized AL into type A to type E, with type E being the most severe form involving all three columns causing an unstable lesion, like in our case. AL is seen mostly in middle-aged males (63%–86%), with longstanding AS unlike our case who was diagnosed newly on admission. They usually present with mechanical back pain. Sometimes it can present with features of myelopathy/radiculopathy when there is compression to neural structures due to abnormal mobility or an associated deformity.[5]

Various imaging modalities help in the diagnosis of AL. Plain radiographs in late stages usually show an osteolytic lesion with a surrounding zone of reactive sclerosis and osteophytes.[18] It can either be transvertebral, transdiscal, or a combined disco-vertebral lesion. However, radiographs in SALs are of little value, except in infiltrative type where any bony erosion can be seen. In patients with AL, CT scan is superior to conventional radiographs and MRI in identifying the extent of the lesion, posterior elements and facet joint status, and visualization of vacuum phenomena.[19] Even in our case, the lesion at L1–L2 was not very obvious on MRI but was very evident on CT. SALs in CT scans are seen as low- or intermediate-density lesions. CT also helps in the visualization of any tumor calcification, trabeculation, and erosion of the vertebral body and pedicle.[9]

MRI is an important investigation in the above pathologies. A SAL is seen as a spindle-shaped lesion in the sagittal view causing the shifting of the spinal cord. In SALs, the adipose tissue appears hyperintense on T1 and T2 images, whereas blood vessels are hypointense on T1 and hyperintense on T2 images with a postcontrast enhancement.[9],[20] MRI helps in visualizing AL with the highest sensitivity.[21] On T1 image, disc space is hypointense compared with vertebral body and shows contrast enhancement during stages of active inflammation. On T2 images, increased signal intensity in the adjacent area is noticeable. Contrast films help to differentiate acute inflammation from a chronic lesion with fatty infiltration.[22] MRI also helps to evaluate the spinal canal encroachment from various structures like hypertrophic ligamentum flavum, facet joints, or hypertrophic callus formation, all leading to compression of neural structures. In spite of all these MRI features, there is a high rate of misdiagnosis of AL as infective spondylodiscitis.[5] Especially in an endemic country for tuberculosis, like in India, tubercular spondylodiscitis remains an important radiological differential for AL.

The gold standard management for SALs is surgical resection. Total resection should be attempted whenever possible, but even otherwise when only subtotal removal is possible, the prognosis is still good. SALs are slow-growing and do not undergo malignant changes; hence, no adjuvant therapy is recommended.[23] Non-infiltrative type is usually removed en masse, but it might be difficult in infiltrative type, as they have no capsule. In our case, though there is no clear-cut radiological evidence of infiltrative type, the lesion was found adhered to the dura; hence, gross total resection was performed. No preoperative embolization was done in our case, as recommended by some authors. There was no excessive intraoperative blood loss. The recurrence of SALs after complete removal is very rare, and only one case has been reported in the literature.[24]

AL, on the other hand, can be managed with a conservative trial in selected cases, where spinal stability is maintained and when there is no gross deformity or neurological deficit. Usually, a brace or plaster cast is used along with rest and nonsteroidal anti-inflammatory drugs. A majority of them develop severe pain, progressive deformity, or neurological deficit, requiring surgical management. AL should be treated like an unstable fracture in an ankylosing spondylosis patient. They require long-segment fixation, and osteoporosis should also be taken into consideration during surgical planning. Other problems during surgical management in an AS patients such as difficult intubation, positioning, increased risk of iatrogenic durotomy, postoperative wound complication, and lung complications should be anticipated and tackled accordingly. The approach used can be either an anterior, posterior, or combined, and concurrent correction of deformity should also be attempted if present.[25] In our case, a long-segment (D6–L3) pedicle screw fixation via a posterior approach with decompression at D6–D9 and gross total excision of the SAL and a posterolateral fusion was done.


  Conclusion Top


It is a very rare scenario for an epidural angiolipoma to occur at the same level as AL in a patient with ankylosing spondylosis, which has not been reported so far. A strong clinical suspicion is required for the diagnosis of both these pathologies as they can easily be missed or confused with other differentials. When in suspicion, contrast MRI and CT scan are helpful in diagnosis. Both SAL and advanced AL warrant surgical management. If diagnosed early and managed appropriately, the patient usually has a good prognosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Shweikeh F, Sangtani A, Steinmetz MP, Zahos P, Chopko B Spinal angiolipomas: A puzzling case and review of a rare entity. J Craniovertebr Junction Spine2017;8:91-6.  Back to cited text no. 1
    
2.
Carrasco Moro R, Gutiérrez Cierco JA, Martínez San Millán JS, Pian H, Martínez Rodrigo MA Spinal extradural angiolipomas: 7 new cases and review of the literature. Neurologia (Engl Ed) 2019;34:98-104.  Back to cited text no. 2
    
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Si Y, Wang Z, Pan Y, Lin G, Yu T Spinal angiolipoma: Etiology, imaging findings, classification, treatment, and prognosis. Eur Spine J 2014;23:417-25.  Back to cited text no. 3
    
4.
Benvenutti-Regato M, De la Garza-Ramos R, Caro-Osorio E Thoracic epidural spinal angiolipoma with coexisting lumbar spinal stenosis: Case report and review of the literature. Int J Spine Surg 2015;9:67.  Back to cited text no. 4
    
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Bron JL, de Vries MK, Snieders MN, van der Horst-Bruinsma IE, van Royen BJ Discovertebral (Andersson) lesions of the spine in ankylosing spondylitis revisited. Clin Rheumatol 2009;28:883-92.  Back to cited text no. 5
    
6.
Gelabert-Gonzalez M, Garcia-Allut A Spinal extradural angiolipoma: Report of two cases and review of the literature. Eur Spine J 2009;18:324-35.  Back to cited text no. 6
    
7.
Howard WR, Helwig EB Angiolipoma. Arch Dermatol 1960;82:924-31.  Back to cited text no. 7
    
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Rkhami M, Kedous MA, Achoura S, Zehani A, Bahri K, Zammel I Epidural angiolipoma: A rare cause of spinal cord compression. Int J Surg Case Rep 2018;45:72-6.  Back to cited text no. 8
    
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Wang R, Tang H Spinal angiolipoma: A report of two cases and review of the literature. J Int Med Res 2020;48:300060520954690.  Back to cited text no. 9
    
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Dufrenot L, Pele E, Cursolle JC, Coindre JM, Lepreux S Spinal epidural angiolipoma: A case report. Ann Pathol 2010;30:30-2.  Back to cited text no. 10
    
11.
Lin JJ, Lin F Two entities in angiolipoma. A study of 459 cases of lipoma with review of literature on infiltrating angiolipoma. Cancer 1974;34:720-7.  Back to cited text no. 11
    
12.
Mohammed ZI, Ahmed MM Spinal extradural angiolipoma manifested after normal vaginal delivery. BMC Res Notes 2016;9:132.  Back to cited text no. 12
    
13.
Horiuchi K, Yamada T, Sakai K, Okawa A, Arai Y Hemorrhagic sudden onset of spinal epidural angiolipoma. Case Rep Orthop 2018;2018:5231931.  Back to cited text no. 13
    
14.
Andersson O Rontgenbilden vid spondylarthritis ankylopoetica. Nord Med Tidskr 1937;14:2000-2.  Back to cited text no. 14
    
15.
Forestier J, Jacqueline F, Rotesquerol J Ankylosing spondyloarthritis with painless spinal evolution. Rev Rhum Mal Osteoartic 1951;18:486-7.  Back to cited text no. 15
    
16.
Jacqueline F Disorders of bone structure and destructive lesions during ankylosing spondylarthritis. J Radiol Electrol Arch Electr Medicale 1956;37:887-93.  Back to cited text no. 16
    
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Cawley MI, Chalmers TM, Kellgren JH, Ball J Destructive lesions of vertebral bodies in ankylosing spondylitis. Ann Rheum Dis 1972;31:345-58.  Back to cited text no. 17
    
18.
Chan FL, Ho EK, Fang D, Hsu LC, Leong JC, Ngan H Spinal pseudarthrosis in ankylosing spondylitis. Acta Radiol 1987;28: 383-8.  Back to cited text no. 18
    
19.
Chan Ho EK, Chau EM Spinal pseudarthrosis complicating ankylosing spondylitis: Comparison of CT and conventional tomography. Am J Roentgenol 1988;150:611-4.  Back to cited text no. 19
    
20.
Provenzale JM, McLendon RE Spinal angiolipomas: MR features. AJNR Am J Neuroradiol 1996;17:713-9.  Back to cited text no. 20
    
21.
Kenny JB, Hughes PL, Whitehouse GH Discovertebral destruction in ankylosing spondylitis: The role of computed tomography and magnetic resonance imaging. Br J Radiol 1990;63:448-55.  Back to cited text no. 21
    
22.
Shih TT, Chen PQ, Li YW, Hsu CY Spinal fractures and pseudoarthrosis complicating ankylosing spondylitis: MRI manifestation and clinical significance. J Comput Assist Tomogr 2001;25:164-70.  Back to cited text no. 22
    
23.
Tsutsumi S, Nonaka Y, Abe Y, Yasumoto Y, Ito M Spinal angiolipoma in a pregnant woman presenting with acute epidural hemorrhage. J Clin Neurosci 2011;18:849-51.  Back to cited text no. 23
    
24.
Bender JL, Landingham JH, Manno NJ Epidural lipoma producing spinal cord compression. J Neurosurg 1974;41:100-03.  Back to cited text no. 24
    
25.
Marsh CH Internal fixation for stress fractures of the ankylosed spine. J R Soc Med 1985;78:377-9.  Back to cited text no. 25
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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