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Spinal hemangioblastoma
Introduction
Hemangioblastoma of the spine is an infrequent, benign (World Health Organization [WHO] I), and highly vascularized tumor accounting for about 3% of all intramedullary spinal tumors and 2% to 15% of all the spinal cord neoplasms. Among the intramedullary tumors of the spinal cord, hemangioblastomas come third in frequency, just after ependymomas and astrocytomas. Sporadic neoplasms represent the majority of cases (70%–80%), whereas those associated to von Hippel Lindau disease (VHL) account for a third of the cases (20%–30%). Most of the patients become affected during the fourth decade of life, and it has a predilection for the male population. VHL is characterized by early onset of diagnosis and/or presence of multiple lesions. Sporadic lesions are often extraspinal tumors, specifically in the cerebellum. Despite these differences, hemangioblastomas of the spine share identical clinical and histological features. Overall, most of the spinal hemangioblastomas originate in the cervical and thoracic segments. Moreover, the majority of symptomatic lesions are found in the cervical spine. The most common associated findings are a tumor cyst or a syrinx, which can be found in up to 90% of the occurrences. Mass effect causes most symptoms including pain and sensory disturbances. A rapid progression of symptoms is not uncommon. Surgical resection is the definitive and curative treatment for symptomatic lesions. However, preferred treatment of asymptomatic, sporadic, or multiple lesions is less clear. In this chapter, we present the case of a young female patient with a history of VHL disease and sensory disturbances in both lower extremities.
Example case
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Chief complaint: bilateral lower extremity paresthesias
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History of present illness: This is a 20-year-old female patient with a known family history of VHL disease who presents with numbness and tingling of her bilateral lower extremities for 1 month. This has progressively worsened and she also complains of some dyscoordination when exercising. As part of her evaluation, the patient underwent a magnetic resonance image of the thoracic spine, which demonstrated an intramedullary lesion exerting mass effect at the level of T8-T9 ( Fig. 66.1 ).
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Medications: none
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Allergies: no known drug allergies
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Past medical and surgical history: none
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Family history: von Hippel Lindau disease
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Social history: college student, no smoking, no alcohol
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Physical examination: awake, alert, and oriented to person, place, and time; cranial nerves II–XII intact; bilateral deltoids/triceps/biceps 5/5; interossei 5/5; iliopsoas/knee flexion/knee extension/dorsi, and plantar flexion 5/5
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Reflexes: 2+ in bilateral biceps/triceps/brachioradialis with negative Hoffman; 3+ in bilateral patella/ankle; left foot clonus, bilateral Babinski; sensation diminished in both lower extremities with a sensory level at T10
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| Preoperative | ||||
| Additional tests requested |
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MRI brain and complete spine |
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| Surgical approach selected | T9 laminoplasty and gross-total resection | T8-9 laminectomy and tumor resection | T8-9 laminectomy and hemangioblastoma resection | T8-9 laminectomy and hemangioblastoma resection |
| Goal of surgery | Gross total resection, preservation of neurological function, diagnosis, spinal cord decompression | Tumor resection | Gross total resection | Gross total resection |
| Perioperative | ||||
| Positioning | Prone, no pins | Prone on Jackson table, no pins | Prone | Prone on Jackson table, no pins |
| Surgical equipment |
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| Medications | Steroids, maintain MAP | Steroids, MAP >80 | Steroids, maintain MAP | Steroids, maintain MAP > 75 |
| Anatomical considerations | Posterior columns of spinal cord, surface vasculature | Midline spinal cord structures (midline artery, posterior sulcus) | Hemangioblastoma vasculature, dorsal root associated with hemangioblastoma | Ascending and descending motor and sensory tracts |
| Complications feared with approach chosen | Paraparesis, paraplegia, urinary/fecal incontinence/retention | Spinal cord injury, neurological worsening | Spinal instability, CSF leak | Spinal cord injury |
| Intraoperative | ||||
| Anesthesia | General | General | General | General |
| Exposure | T9 | T8-9 | T8-9 | T8-9 |
| Levels decompressed | T9 | T8-9 | T8-9 | T8-9 |
| Levels fused | T9 | None | None | None |
| Surgical narrative | Position prone, vertical midline posterior incision one level above and below level of interest down to spinous processes, dissection of paraspinal muscles, confirm level with fluoroscopy, bilateral laminotomy to span level with high-speed drills, remove ligamentum flavum and adipose tissue, surgical microscope brought into field, confirm lesion with ultrasound, study dorsal midline to identify median raphe with ultrasound, dura opened and tacked open, dorsal midline myelotomy, use IOM probe to help identify neural tissue, save dorsal vascular tissues by dissecting and rotating, coagulate feeding arteries, lesion removed en bloc if possible, intraoperative ultrasound to confirm extent of resection, watertight dural closure and fibrin glue, laminoplasty with plate and screws, nonabsorbable sutures to fix lamina to cranial and caudal interspinal ligaments and paraspinal muscles, layered closure with drain | Position prone on Jackson table, mark levels using anatomical landmarks and intraoperative fluoroscopy, laminectomy intraoperative ultrasound to confirm level and assess spinal cord compression, midline dural opening, baseline MEP, examine dorsal surface of spinal cord to identify midline and lateral side for possible entry into the tumor where it reaches the surface of the spinal cord, expose tumor and stay out of lesion, try to go around the lesion to achieve a complete resection, serial MEP throughout, send tissue for pathology examination, another ultrasound view to determine decompression of spinal cord, watertight dural closure, application of fibrin glue, closure in layers | Position prone, x-ray for level verification, midline subperiosteal dissection, laminectomy over enhancing nodule, ½ level above and ½ level below, ultrasound to confirm correct level and adequate exposure, midline dural incision, tack dura to muscle, arachnoid dissection under microscope visualization, identify orange surface of hemangioblastoma, dissect and coagulate feeding surface vessels, sharp dissection of normal pia from pia of hemangioblastoma, extend myelotomy as needed to identify rostral and caudal poles, extracapsular dissection from gliotic spinal cord, coagulate and ligate vessels, coagulate and ligate draining vein last, en bloc removal, dural closure with compressed Gelfoam and fibrin glue, layered closure | Position prone, skin incision localized with fluoroscopy, lamina are exposed and correct level confirmed with fluoroscopy, standard laminectomy, microscope brought into field, dura opened and tacked up, myelotomy where lesion comes closest to dorsal surface and eccentric on left, en bloc resection, dura closed with suture and fibrin glue, layered closure |
| Complication avoidance | One-level laminotomy, ultrasound to confirm exposure, identify median raphe, IOM probe to identify neural tissue, save dorsal vascular structures, en bloc resection, fibrin glue | Surgical navigation, intraoperative ultrasound, examine for midline, enter tumor at most superficial aspect, stay out of lesion, continuous MEP, fibrin glue | Intraoperative ultrasound, sharp dissection, extracapsular dissection, coagulate and ligate draining vein last, en bloc resection | Myelotomy at most superficial aspect, en bloc resection, fibrin glue |
| Postoperative | ||||
| Admission | ICU | Stepdown unit | ICU | ICU |
| Postoperative complications feared | Hematoma, CSF leak, neurological injury, vascular injury, urinary/fecal incontinence, neuropathic pain, sensory loss | Neurological compromise, CSF leak | CSF leak, neurological deficit, subtotal removal | Spinal cord injury, CSF leak, infection |
| Anticipated length of stay | 3–4 days | 7 days | 3–4 days | 2–3 days |
| Follow-up testing | CT T-spine within 24 hours of surgeryMRI T-spine within 24 hours of surgery | Physical therapyMRI 48 hours after surgery if worsening deficit, 3 months after surgery | MRI within 48 hours of surgery | Maintain MAP >75 for 24 hoursMRI T-spine before discharge and 1 year after surgery |
| Bracing | Thoracolumbar brace for 6 weeks | None | None | None |
| Follow-up visits | 2 weeks, 6 weeks, 3 months, 6 months, 12 months after surgery | 1 month after surgery | 3–4 weeks, 3–4 months, 1 year after surgery | 10–14 days, 6 weeks, 1 year after surgery |
CSF , Cerebrospinal fluid; CT , computed tomography; ICU , intensive care unit; IOM , intraoperative monitoring; VHL , von-Hippel Lindau; MAP , mean arterial pressure; MEP , motor evoked potential; MRI , magnetic resonance imaging; SSEP , somatosensory evoked potential.
Differential diagnosis
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Ependymoma
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Astrocytoma
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Simplex syringomyelia
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Arteriovenous malformation
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