Spinal epidural cavernous hemangiomas in the lumbar spine: A case report

Abstract

Introduction

Spinal epidural cavernous hemangiomas (SECHs) are relatively rare intradural epidural lesions of the spinal canal, and those occurring in the lumbar spine are even rarer.

Case presentation

A 60-year-old man presented for low back pain with right leg pain. His pee and feces were both normal but symptoms were very similar to a typical lumbar disc herniation. The MRI findings suggest an epidural space of unknown nature in the spinal cord at the L2–3 level and a definite diagnosis of SECHs was made by postoperative pathological examination.

Clinical discussion

Patients who are suspected of having SECHs should undergo initial classification and differential diagnosis based on MRI imaging features. It is crucial to identify the responsible segment in correlation with the presenting symptoms. During surgery, the primary objective should be the complete removal of the mass, while taking utmost care to protect the nerves. Dynamic stabilization systems, utilizing pedicle rods, can be considered as one of the treatment options for such patients.

Conclusion

Patients presenting with low back pain and neurological symptoms should undergo MRI, and diagnosed with SECHs should undergo early surgical intervention. For patients with an intradural mass in the spinal canal, complete resection should be performed while prioritizing nerve protection.

Highlights

• •The manifestation of spinal epidural cavernous hemangiomas (SECHs) in patients can closely mirror that of standard lumbar disc herniation, thereby emphasizing the importance of MRI scanning.
• •Performing a preliminary differential diagnosis of potential SECHs patients based on MRI results holds significant importance.
• •Upon identifying the responsible segment in SECHs patients, surgical action should be swiftly yet carefully undertaken, with a key emphasis placed on the vigilant protection of nerve tissue throughout the procedure.

1. Introduction

Cavernous malformation (CM) is a type of intradural lesion characterized by developmental deformities caused by vascular abnormalities in the central nervous system. Among these lesions, spinal epidural cavernous hemangiomas (SECHs) are relatively uncommon, accounting for only 4 % of all epidural lesions [1]. SECHs can occur anywhere along the spine, with a higher frequency observed in the dorsal thoracic region and a lower incidence in the lumbar region [2]. We outline the treatment provided to a patient admitted to our hospital with SECHs at the L2-L3 level of the lumbar spine.

The work has been reported in line with the SCARE 2020 criteria [3].

2. Presentation of a case

A 60-year-old man experiencing “low back pain with right leg pain for 5 years, aggravated for 1 month” was taken to the hospital. He was diagnosed with “lumbar disc herniation” and his pee and feces were both normal. Examination: Lumbar spine activity was limited, muscle tension on both sides of the L2-L4 spinous processes, tenderness and pain to pressure and percussion (+). Muscle strength of each group of the lower limbs was grade 4 bilaterally, muscle tone was normal. There were no superficial and deep sensory abnormalities in the hip and pre-femoral area of both lower limbs, the knee and the following areas. The right leg raising and strengthening test was 60° (+) and the right femoral nerve pull test was +. The preoperative Japanese Orthopedic Association (JOA) lumbar score was 8, Oswestry Disability Index (ODI) score was 27, and Visual Analogue Scale (VAS) scores were 6 and 7 for lumbar and lower limb pain, Preoperative imaging results are shown in Fig. 1a-h. The preoperative diagnosis was an intradural mass, SECHs?

Fig. 1.

Imaging data of the case

a,b: The lumbar spine was seen to be degenerated, with the presence of overall physiological curvature and rostral osteophyte formation at the anterior margin of the L1–2 vertebral body c,d:Anterior flexion and posterior extension demonstrate fair mobility of the L2–3 segments. e,f,g: MRI showed obvious compression of the spinal cord at the level of L2–3, and an oval-shaped mass was seen in the dorsal aspect of the spinal canal with clear borders, smooth edges and intact periosteum. The mass showed isointense signal on T1WI and high signal on T2WI and compression fat images. h:MRI showed that 75 % of the spinal canal was encroached, and the tumor was on the right side. i: The tumor was removed intact. j,k:The nail rod system was in good position. l,m,n:MRI showed that the spinal cord space at L2–3 was significantly enlarged. o:The L2–3 level showed that the morphology of the cauda equina in the spinal cord was restored and dispersed, surrounded by cerebrospinal fluid, and the intervertebral foraminal space was good bilaterally.

The patient underwent lumbar posterior laminectomy, mass removal and transpedicular internal fixation 3d after admission. Under general anesthesia, a posterior median incision was performed to expose the vertebral plate and the L2-L3 spinous processes. The surface of the mass was smooth, fish-egg shaped, soft, with clear margins, rich in blood supply and intact covering. After leaving the pathology for evaluation, the tumor was complete and thorough removal (Fig. 1i). The patient's vital signs were steady both throughout and following the procedure.

Postoperative pathology confirmed a cavernous hemangioma. The tumor was mainly composed of a large number of thin-walled blood vessels, with a single layer of endothelial cells and collagen fibres in the tube wall, lacking the elastic and muscular layers, and the immunohistochemical results were Vim (+), EMA (+), CD34 (vasculature +), CD31 (vasculature +), Ki-67 (individually +) and SMA (+). At 12 days post-operatively, the patient was discharged from hospital with a lumbar JOA score of 21, an ODI score of 39, and lumbar and lower extremity VAS scores of 3 and 2. At the 4-month postoperative follow-up, the lumbar JOA score of 19, an ODI score of 41, lumbar and lower extremity VAS scores of 2 and 2. Postoperative imaging results are shown in Fig. 1j-o. Patient was satisfied with the results of his treatment.

3. Discussion

Cavernous hemangiomas (CM), first characterized by Globus et al. in 1929, are uncommon yet insidiously developing, tumor-like vascular malformations. Though they typically manifest in the supratentorial region of the brain parenchyma, these formations can indeed occur anywhere within the body [4]. Depending on their proximities to structures such as the dura mater, these vascular malformations can be catagorized into four types [5]: intramedullary, extramedullary intradural, epidural, and vertebral. The instances of spinal epidural cavernous hemangiomas (SECHs) are quite rare, constituting approximately just 4 % of epidural tumors [1]. Whether there exists a significant gender disparity in the prevalence of SECHs remains contentious [1,2]. It is agreed that they are most often found in the thoracic vertebrae - followed by lumbar, then cervical and sacral vertebrae - and most likely to be located dorsal or dorsal-lateral to the spinal canal [2,6].

SECHs were considered as a vascular malformation initiated by dysplasia [7]. Investigations of CM imply genetics [8], trauma [9], hormonal expressions and haemodynamic factors [10] as potential influences on its formation. The clinical symptoms of SECHs depend on their specific genesis site and how they interact with anatomical structures such as the spinal cord and nerve roots. Reviews suggest that up to 86 % of SECHs have been chiefly situated at the dorsal or dorsolateral aspect of the spinal cord, with 9 % ventral and a mere 5 % occurring at the intervertebral foramen [1]. Hence, most patients will still exhibit signs of spinal cord and nerve root compression. SECHs usually follow a course characterized by gradual onset. SECHs with sudden onset being a rarity, potentially resulting from an increased pressure due to intra-tumoral hemorrhage caused by trauma [4]. In this particular case, the patient had a history of 5 years, with symptoms exacerbating gradually and pain concentrated locally and extending to lower extremities – an observation that aligns with previously documented cases.

At present, the clinical diagnoses for SECHs are reliant on MRI and pathological examinations. MRI permits sagittal and coronal scanning, thereby elucidating the location, size, and relationship of the lesion to the surrounding tissues. SECHs typically exhibit an isosignal on T1-weighted images and a slightly lower high signal than that of cerebrospinal fluid (CSF) on T2-weighted images [1,2,11]. When there's an acute rupture and hemorrhage in SECHs due to trauma or various other factors, both T1- and T2-weighted images display high signals on the MRI [11]. Some patients may warrant an enhanced MRI to distinguish it from other intraspinal masses, because SECHs may reflecting inhomogeneous enhancement. In our case, where the posterior aspect of the L2-L3 spinal cord displayed a low signal on T1WI and a high signal on T2WI during the preoperative MRI.

The conclusive diagnosis of spinal epidural cavernous hemangiomas (SECHs) is reliant on pathological results. The initial differential diagnosis can also be made preoperatively on the basis of MRI. It is crucial to differentiate SECHs from other types of epidural lesions, such as metastatic tumors, lymphomas, abscesses, among others [12]. Metastatic tumors usually manifest as sizable soft tissue masses, commonly accompanied by significant vertebral and accessory bone destruction and a history of the principal tumor, and exhibit slightly higher signals on T2-weighted images in MRI scans. Differentiating lymphoma from the epidural type of SECHs can be challenging, especially when they occur as a mass in the epidural space, indicated by a moderate signal on both T1WI and T2WI. Epidural abscesses may display a moderate signal on T1WI and a high signal on T2WI; these patients typically have a history of trauma, surgery, or other infections. SECHs also need to be distinguished from other vascular-rich tumors like spinal tumors, as their signal and enhancement patterns are very similar. It's noteworthy to identify the differences in morphology and growth patterns between these two types of SECHs. Variance in the location is also distinctive of the different types of SECHs. Epidural SECHs, positioned in the epidural space, tend to exhibit a creeping growth pattern, resembling a staggered or elongated oval shape. Conversely, a chordoma is mostly broad-based, slow-growing mass adhering to the dura mater and is primarily situated in the extramedullary subdural space.

The cornerstone of treatment for SECHs is surgical resection. Early diagnosis and timely surgical intervention are imperative. For most SECHs, complete resection is a possibility, particularly for the epidural type. Protective measures for the spinal cord and nerve roots are indispensable during surgery, especially for intramedullary and extra-medullary intradural CM. Additionally, selecting the right fixation method must take into account the lesion's location and the involved segments. In this case, the preoperative X-rays showed minor activity in the L2–3 segment without notable instability. Therefore, we applied the Isobar system to preserve some of the patient's operational segment mobility. The shock-absorbing ring at the center of the Isobar rods offers unique cushioning properties, reducing stress on the rods. The pre-curvature before implantation complements the physiological anterior convexity of the segments, which diminishes stress concentration to some extent. The range of motion (ROM) attached to the rods theoretically maintains segmental activity while reducing the compensatory ROM of neighboring segments [13]. During surgery, the L2–3 vertebral plate was exposed, revealing the tumor along the right side of the dura mater. This location consistent with the symptomatic manifestation. The patient showed a satisfactory functional recovery and no sign of symptom deterioration during the follow-up period.

4. Conclusion

In this study, we discuss a case of spinal epidural cavernous hemangiomas (SECHs) in the lumbar region. The patient's symptoms paralleled that of a traditional lumbar disc herniation, underlining the necessity for a thorough MRI examination when presented with patients showing similar symptomatology. The preliminary differential diagnosis should be rooted in a detailed interpretation of the imaging findings. On ascertaining the accountable segment, surgical intervention should be expedited in a prudent and judicious manner, prioritizing the safeguarding of nerves during the procedure.

Consent

Written informed consent was obtained from the patient for publi-cation of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Sources of funding

No funding was obtained for this study.

Ethical approval

This study was approved by the Medical Ethics Committee of Dongzhimen Hospital, Beijing University of Traditional Chinese Medicine (approval number: 2022DZMEC-085-05).

Research registration

None.

Guarantor

Dingyan Zhao.

Provenance and peer review

Not commissioned, externally peer-reviewed.

CRediT authorship contribution statement

Dingyan Zhao: Writing- Original draft preparation and Methodology; Yukun Ma: Writing- Reviewing and Editing; Xing Yu: Conceptualization, Supervision; Xinliang Yue, Lianyong Bi: Data curation.

Declaration of competing interest

The authors report no conflict of interest.