Abstract

Background: Spinal cord hemangioblastomas (HBs) account for 2–15% of all spinal cord neoplasms. They are the third most common primary intramedullary tumor (1–5%). Here, 72-year-old female presented with a thoracic intramedullary spinal HB that responded well to surgery.

Case Description: A 72-year-old female presented with a 3–4 years of progressive paresthesias and paraparesis. On examination, she exhibited diffuse distal weakness of the lower extremities. The magnetic resonance scan showed an intramedullary expansive lesion at the T1–T2 level that markedly enhanced with contrast with both proximal and distal hydromyelia. Surgery included a C7 partial and T1–T2 total laminectomies performed under microscope visualization with intraoperative monitoring. At surgery, there was a well-documented cleavage plane between the tumor and the cord; excision was facilitated using the cavitron ultrasonic surgical aspirator device.

Conclusion: Surgery is the gold standard treatment for treating/resecting HBs and should include utilization of an operating microscope and intraoperative monitoring.

Keywords: Hemangioblastoma, Neurosurgery, Spine surgery, Spine, Spine tumor

INTRODUCTION

Spinal hemangioblastomas (HBs) are benign and highly vascular tumors that can occur anywhere throughout the central nervous system. They account for 2–15% of all spinal cord neoplasm and are the third most common primary intramedullary tumors (1–5%). Notably, they most commonly involve the cervical spine and are often associated with perilesional edema, and/or peritumoral cysts.[ 4 , 8 , 9 ] Clinically, they contribute to varying degrees of myelopathy that corresponds with their size/location.[ 2 ] Here, a 72-year-old female presented with an expansive intramedullary HB at the T1–T2 spinal level resulting in a significant paraparesis that resolved following tumor resection.

CASE REPORT

Clinical presentation

A 72-year-old female presented with a 6 month history of progressive paresthesias and paraparesis. On examination, she exhibited distal lower extremity weakness (4/5 level dorsiflexion along with diffuse lower extremity hyperreflexia and bilateral Babinski signs). The magnetic resonance imaging (MRI) demonstrated an intramedullary expansive lesion at the T1–T2 level that markedly enhanced with contrast; it was also accompanied by cephalad and caudad hydromyelia [ Figures 1 and 2 ].

Figure 1:

Magnetic resonance imaging of the cervical spine. (a) Sagittal T2-weighted revealed a syrinx with an intramedullary expansive lesion. (b) Sagittal T1-weighted dixon Gadolinium (Gd) enhancement image revealing an intramedullary intradural hyperintense mass (red arrow) at the T1-T2 level.

Figure 2:

Axial T1-weighted image with Gd enhancement demonstrates the posterolateral hyperintense lesion infiltrating the spinal cord located in the posterior arch of T1.

Surgery

The patient underwent for a C7 partial and T1–T2 total laminectomies. Using microscope visualization and intraoperative monitoring, the dura was opened revealing an expansive and very vascular intramedullary cord lesion [ Figure 3 ]. A cleavage plane between the tumor and cord facilitated gross-total operative resection of the lesion using the cavitron ultrasonic surgical aspirator device [ Figure 4 ].

Figure 3:

Posterolateral sulcus approach for the thoracic spinal cord intramedullary tumor. Tumor (yellow arrow).

Figure 4:

Macroscopic complete resection of the spinal cord lesion.

Pathology

The histopathological examination confirmed the diagnosis of an HB. The tumor included a rich vascular network and vacuolated cells with mild nuclear enlargement and clear cytoplasm. Immunohistochemical staining revealed stromal cells positive for inhibin A, S100, and CD31, and was immunonegative for CD56, and AE1-3 [ Figure 5 ].

Figure 5:

(a and b) Histopathological examination with hematoxylin and eosin staining revealed a large and vacuolated stromal cells, and intertwined with thin-walled capillaries, and clear cytoplasm. Immunohistochemical staining with inhibin A showing marked positivity of the neoplastic cells.

Postoperatively

Postoperatively, the patient’s paraparesis markedly improved. The postoperative MRI revealed that the tumor had been totally removed, and the syrinxes/hydromyelia cephalad/ caudad were diminished. One-year later, the patient was fully neurologically intact, and the magnetic resonance (MR) continued to confirm the lack of tumor recurrence along with further reduction of the cephalad/caudad syrinxes/ hydromyelia [ Figure 6 ].

Figure 6:

(a and b) Follow-up magnetic resonance imaging showing gross total removal of the hemangioblastoma (HBL) and reduction of the syrinx.

DISCUSSION

Patients typically present with symptoms/signs of spinal HB during the fourth decade of life.[ 9 ] Approximately 70% of spinal hemangioblastoma (HB) are sporadic single lesions, with the remaining 30% representing von Hippel-Lindau (VHL)-associated familial cases.[ 10 ] The posterior fossa is the most common location for HB (45–50%), followed by the spinal cord (40–45%, where they typically extend over 1–2 vertebral levels) and the brain stem (5–10%). Over 75% of spinal HB are intramedullary (75%) and comprise the third most common intramedullary spinal cord neoplasm, following ependymomas and astrocytomas.[ 4 , 7 ] Approximately 60% of intramedullary HBs are located in the cervical spine, 40% involve the thoracic cord, followed by the lumbar spine (5–10%) and cauda equina (<1%) [ Table 1 ].[ 3 , 5 ]

Table 1:

Summary of the cited cases, including the present case report.

MRI gold standard examination for HB

MRI remains the gold standard for diagnosing HB; lesions are typically iso- to hypointense on T1-weighted sequences, and iso- to hyperintense on T2-weighted studies; further, they markedly enhance with contrast.[ 1 ] For patients with HB, it is critical to obtain MRI studies of the entire spine and brain before proceeding with definitive spinal surgery.[ 8 ]

Indications for surgery

For patients with HB, the clinical and radiographic findings both determine whether surgical intervention is warranted. The optimal surgical management includes gross-total resection that is typically achieved in over 90% of the cases; this usually results in a 96% incidence of full functional recovery of the cases. Postoperatively, patients’ symptoms often regress within 1–2 postoperative weeks, while follow-up contrast MR studies help confirm complete tumor resection, along with documenting regression of cephalad/caudad cysts (i.e., syrinxes/hydromyelia over 3–6 months).[ 2 , 6 ]

CONCLUSION

A 72-year-old female presented with myelopathy/paraparesis attributed to a T1–T2 HB. Following complete tumor resection, the patient regained normal neurological function, and the follow-up MR up to 6 months lateral confirmed no tumor recurrence and regression of the cephalad/caudad syrinxes/hydromyelia.

Declaration of patient consent

Patient’s consent not required as patient’s identity is not disclosed or compromised.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

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