Spinal hemangioblastoma causing a holocord syrinx: A report of two cases and review of literature

Tungish Bansal; Devasheesh Kamra; Sachin Borkar

doi:10.4103/isj.isj_17_22

CASE REPORT

Year : 2023 | Volume : 6 | Issue : 1 | Page : 110-114

Spinal hemangioblastoma causing a holocord syrinx: A report of two cases and review of literature

Tungish Bansal, Devasheesh Kamra, Sachin Borkar
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India

Date of Submission	22-Feb-2022
Date of Decision	13-May-2022
Date of Acceptance	22-Jul-2022
Date of Web Publication	11-Feb-2023

Correspondence Address:
Sachin Borkar
Department of Neurosurgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/isj.isj_17_22

Abstract

Intramedullary spinal hemangioblastomas are often associated with syringomyelia. However, holocord syrinx associated with such lesions is a rare entity. We present two cases of dorsal spinal hemangioblastoma associated with holocord syrinx. Both the cases were treated with surgical excision which resulted in the resolution of holocord syrinx and good neurological recovery. In this case report, we discuss the various aspects of the diagnosis and surgical management in such cases. We also present an extensive review of literature of such lesions.

Keywords: Hemangioblastoma, holocord, spinal, syringomyelia, syrinx

How to cite this article:
Bansal T, Kamra D, Borkar S. Spinal hemangioblastoma causing a holocord syrinx: A report of two cases and review of literature. Indian Spine J 2023;6:110-4

How to cite this URL:
Bansal T, Kamra D, Borkar S. Spinal hemangioblastoma causing a holocord syrinx: A report of two cases and review of literature. Indian Spine J [serial online] 2023 [cited 2023 Mar 28];6:110-4. Available from: https://www.isjonline.com/text.asp?2023/6/1/110/369569

Introduction

Hemangioblastomas in the spinal cord are uncommon lesions, accounting for only 1–2.5% of all primary spinal cord tumors.^[1] They are highly vascular lesions and are mostly found in the cervical or thoracic region.^[1] While syringomyelia may be found with hemangioblastomas, a holocord syrinx is very rare.^{[2],[3],[4],[5],[6],[7],[8],[9]} We present two cases of dorsal spinal hemangioblastoma with holocord syrinx. We also present a literature review of cases of spinal hemangioblastoma with holocord syrinx.^{[2],[3],[4],[5],[6],[7],[8],[9]}

Case 1

A 32-year-old female presented to us with complaints of weakness in left upper and lower limb with dysesthesia over the left side of the torso for the past two years. The dysesthesia progressed to left arm 1.5 years back and to the left lower limb for the past 2 months. On examination, the tone was normal in all limbs. There was some weakness in the left upper and lower limb with a power of 4/5 and normal power on the right side. There was altered sensation over the C5 to D12 dermatome on the left side. The deep tendon reflexes (DTRs) were normal in all four limbs and plantars were down going.

Magnetic resonance imaging (MRI) was suggestive of a T1 hypointense, T2 hyperintense intramedullary lesion at the D9 vertebral body level with homogeneous post-contrast enhancement [Figure 1]A-[C]. There was a holocord syrinx extending from C2 to D12 [Figure 2]. A differential diagnosis of hemangioblastoma was made. An ultrasound examination of abdomen, fundus examination of the eyes, and imaging of the brain were normal.

Figure 1: Case 1: A: T1, B: T2, and C: contrast-enhanced axial MRI at D9 vertebral body level

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Figure 2: Case 1: Pre-operative cervical and thoracic sagittal MRI showing a holocord syrinx extending from C2 to D12 vertebrae

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The patient was planned for operative intervention. The patient was positioned prone on the operating table and a standard midline exposure was done from D8–D10. A D9 and D10 laminectomy was done. On opening the duramater, there was a reddish subpial tumor. The tumor was highly vascular. Gross total excision of the tumor was done. Dura was closed primarily. The post-operative course was uneventful. The diagnosis was confirmed on histopathology. The patient had relief in the dysesthetic pain, and there was no new neurological deficit at final follow-up at 1 year. Six months follow-up imaging showed near total resolution of the syrinx [Figure 3].

Figure 3: Case 1: Post-operative cervical and thoracic MRI showing near total resolution of the syrinx

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Case 2

A 21-year-old male presented with complaints of urge incontinence for three years, numbness in the left torso region for two years, and numbness in the inner part of hand and fingers for the past 1.5 years. On examination, the patient had a scoliotic deformity of dorsolumbar spine with left convexity. The tone and power were normal in all limbs. The DTRs were normal in the upper limbs and exaggerated in the knee and ankle joints. Babinski test was positive bilaterally. There was a 20% sensory loss in bilateral C8 dermatome and a 70% sensory loss in D6–12 dermatomes on the left side. Radiographs were suggestive of dorsal scoliosis with apex at D9–D10 disc space with a Cobb angle of 46° [Figure 4]. Imaging was suggestive of a subpial intensely enhancing intramedullary lesion at the D6–7 level with holocord syrinx from cervicomedullary junction to D11 vertebrae [Figure 5] and [Figure 6]. The fundal examination, ultrasound abdomen, and MRI brain were normal. A D6–7 laminectomy and durotomy were performed, and a reddish firm and vascular tumor was revealed in the subpial plane. A gross total excision was done for the tumor, and closure was done in standard fashion. The post-operative recovery was uneventful, and the patient had significant relief of symptoms in the post-operative period. At 6 months, the patient had improvement in bladder symptoms with relief in urge incontinence. The sensory loss also improved subjectively. The imaging at 6-month follow-up revealed resolution of syrinx, and no progression of scoliotic curve was seen.

Figure 4: Case 2: Anteroposterior radiograph of the whole spine showing dorsal scoliosis with compensatory lumbar curve. Cobb’s angle for the dorsal curve measured 46° with apex at D9–10 disc space

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Figure 5: Case 2: Sagittal T2 scans through the cervical and thoracic spine showing holocord syrinx

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Figure 6: Case 2: A: T2 axial MRI scan and B: contrast-enhanced axial MRI scan at D6–7 level

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Discussion

Hemangioblastomas are uncommon, benign, highly vascular tumors of the central nervous system.^[1] They are more common in the cerebellum and the retina. Spinal lesions are rare with an incidence of only 1–2% of all primary spinal tumors.^[1] It is important to rule out association with Von Hippel–Lindau (VHL) syndrome in all cases as 20–45% of the cases of spinal hemangioblastomas can be associated with VHL syndrome.^[10],[11] A fundus examination and an MRI of the brain are needed to rule out similar tumors elsewhere. An ultrasonography of the whole abdomen is advised to look for any renal or adrenal gland cysts.^[10],[11] The lesions in our case were sporadic occurrences.

A review of the cases with spinal hemangioblastoma associated with holocord syrinx is presented in [Table 1]. The symptoms in these cases can be highly variable ranging from dysesthesia to quadriparesis, depending on the location, extent, and compression caused by the primary lesion and syrinx.^{[2],[3],[4],[5],[6],[7],[8],[9]} The presence of dysesthetic pain, involvement of all limbs, and mixture of upper and lower motor neuron signs should guide toward imaging the whole spine.

Table 1: A summary of cases of spinal hemangioblastoma presenting with holocord syrinx from literature

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The imaging modality of choice in these cases is an MRI. Hemangioblastomas are classically hypointense on T1, hyperintense on T2, and enhance brightly on contrast imaging, as was seen in our cases.^[1] A whole spine screening MRI can demonstrate the holocord syrinx.

Though benign in nature (WHO grade 1), hemangioblastomas can be associated with significant edema, cystic changes, and extension over a large area of cord.^[1] Symptomatic lesions need to be operated upon, and gross total excision is usually possible especially when tumors are located posteriorly in the cord.^[1] Surgery alone is usually sufficient.^[1]

The formation of syrinx is associated with intramedullary spinal cord tumors such as ependymomas and hemangioblastomas.^[12] While nearly half of the cases of spinal hemangioblastomas are associated with a syrinx, the formation of a holocord syrinx is rare.^[3] Obstruction of cerebrospinal fluid flow and transudation of fluid from abnormal tumor vessels are postulated mechanisms for syrinx formation.^[1],[3] The presence of syrinx has been proposed to favor resectability of the tumor, and patients recover from surgery sooner.^[12],[13] Similar to other cases of syrinx, cases of holocord syrinx resolve with excision of tumor alone, and other measures specifically targeted for syrinx are usually not required. Good outcomes with relief in dysesthetic pain and neurological recovery can be expected in these cases post-surgery [Table 1].

Conclusion

Hemangioblastomas can be rarely associated with a holocord syrinx. The presence of a holocord syrinx should be investigated with whole spine MRI. Good clinical outcomes with resolution of syrinx can be achieved with surgical excision of the hemangioblastoma.

Acknowledgements

Nil.

Financial support and sponsorship

There is no funding source for this publication/study.

Conflicts of interest

The authors declare that they have no conflicts of interest.

Patient consent statement

The authors certify that the study was approved by the Institutional Review Board. All procedures performed in the study were conducted in accordance with the ethics standards given in 1964 Declaration of Helsinki, as revised in 2013. All participants provided written informed consent for the participation in the study. Patient consent was obtained for purpose of the study with due care to maintain his/her privacy.

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