Introduction

Primary glioblastoma (GBM) of the spinal cord is a rare condition that contributes to just 1.5% of all spinal tumors. This neoplasm most commonly affects the cervical spine or the cervico-thoracic junction in at least 60% of the cases and usually occurs during the second decade of life. Treatment approaches emulate protocols for intracranial high-grade lesions. Leptomeningeal spread is a marker of poor prognosis and usually manifests as patchy lesions in the caudal cord or intracranially. Secondary spinal cord tumors are extremely rare and may occur after radiation exposure to the spine or surrounding soft tissue structures. Currently, there is limited data supporting a standardized treatment of these lesions. Due to the infiltrative nature of these tumors, and the high eloquence of the cervical cord, the extent of resection is usually limited. Thus the goals of surgery are also variable depending on the location of the tumor and the patient’s starting neurological status. Aggressive resection of malignant spinal cord tumors has been debated on conferring a benefit to survival and carries significant neurological morbidity. Moreover, degree of survival improvement after resection followed by radiotherapy alone or with chemotherapy remains controversial. In this chapter, we present the case of a patient with a cervical cord malignancy after exposure to radiotherapy.

Example case

  • Chief complaint: weakness and numbness

  • History of present illness: This is a 45-year-old male patient with a history of multiple myeloma and plasmacytoma diagnosed in 2010. He underwent radiotherapy. He underwent treatment of an additional lesion in 2016, and at that time received radiation and bone marrow transplant. Additionally, he was started on Revlimid. He presented with 4 months of worsening weakness and numbness. He has thrombocytopenia with a platelet count of 50,000. As part of this workup, the patient underwent a magnetic resonance of the cervical spine that showed an intraaxial lesion of the cervical cord with associated mass effect ( Fig. 56.1 ).

    Fig. 56.1, Preoperative magnetic resonance image (MRI) of the cervical spine. (A) Sagittal T2 image demonstrating an intramedullary lesion extending from C6 to T2 levels that has a high signal intensity and is causing a diffuse spinal cord expansion. This is more pronounced at the C7-T1 level and associated with mild tumor-related edema spreading inferiorly (T2). (B) Sagittal and (C) axial T1 with contrast images demonstrating an intramedullary space-occupying mass with avid enhancement. The lesion is slightly heterogenous and displays ill-defined borders.

  • Medications: dexamethasone, pantorpazole, gabapentin

  • Allergies: no known drug allergies

  • Past medical and surgical history: plasmacytoma, multiple myeloma, tumor resection, bone marrow transplant

  • Family history: noncontributory

  • Social history: retired engineer, no smoking, no alcohol use

  • Physical examination: awake, alert, and oriented to person, place, and time; cranial nerves II–XII intact; bilateral deltoids/triceps/biceps 3/5; interossei 2/5; iliopsoas/knee flexion/knee extension/dorsi, and plantar flexion 5/5

  • Reflexes: 3+ in bilateral biceps/triceps/brachioradialis with negative Hoffman; 2+ in bilateral patella/ankle; no clonus or Babinski; sensation decreased in left hemibody

  • Ignacio Barrenechea, MD

  • Neurosurgery

  • Grupo Gamma

  • Rosario, Santa Fe, Argentina

  • George I. Jallo, MD

  • Neurosurgery

  • Johns Hopkins All Children’s

  • Tampa, Florida, United States

  • Jorge Navarro Bonnet, MD

  • Neurosurgery

  • Medica Sur, Mexico City, Mexico

  • Nicholas Theodore, MD

  • A. Karim Ahmed, MD

  • Ann Liu, MD

  • Neurosurgery

  • Johns Hopkins University

  • Baltimore, Maryland, United States

Preoperative
Additional tests requested
  • MRI brain and complete spine

  • SSEP

  • Cardiology evaluation

  • Infectious disease evaluation

  • CT chest/abdomen/pelvis

  • MRI brain and complete spine

  • Lumbar puncture for CSF analysis

  • Flexion-extension cervical spine x-rays

  • CT C-spine

  • PET

  • Swallowing evaluation

  • NCS/EMG/SSEP

  • CT C- and T-spine

  • PET

  • Obtain prior medical records

Surgical approach selected C5-T2 laminoplasty for resection of intramedullary tumor C6-T1 laminectomy or laminoplasty for resection of tumor C6-T1 laminectomy for resection of tumor with C6-T1 posterior fusion C6-T1 laminectomy for resection of tumor and C5-T2 posterior fusion
Goal of surgery Gross total resection if possible, preservation of neurological function, spinal instability Establish diagnosis, debulk tumor Establish diagnosis, spinal cord decompression Establish diagnosis, prevent further neurological decline
Perioperative
Positioning Prone with pins Prone with pins Prone with no pins Prone with pins
Surgical equipment
  • IOM (MEP/SSEP)

  • Surgical microscope

  • Ultrasonic aspirator

  • Ultrasound

  • IOM (MEP/SSEP/D-wave)

  • Fluoroscopy

  • Surgical microscope

  • Ultrasonic aspirator

  • IOM

  • Ultrasonic bone scalpel

  • Surgical microscope

  • Ultrasonic aspirator

  • O-arm

  • IOM (MEP/SSEP/D-wave)

  • Ultrasonic bone scalpel

  • Surgical microscope

Medications Steroids, maintain MAP Steroids None Steroids, maintain MAP >85
Anatomical considerations Spinal cord anatomy (posterior median sulcus for myelotomy), spinal cord arteries Spinal cord anatomy (posterior median sulcus for myelotomy) Spinal cord anatomy (posterior median sulcus for myelotomy), pedicles Spinal cord
Complications feared with approach chosen Neurological injury, CSF leak, instability, epidural scarring Injury to corticospinal tract and dorsal columns Spinal cord injury, bleeding Neurological injury, poor wound healing, CSF leak, adjacent segment disease
Intraoperative
Anesthesia General General General General
Exposure C5-T2 C6-T1 C6-T1 C5-T2
Levels decompressed C5-T2 C6-T1 C6-T1 C6-T1
Levels fused None None C6-T1 C5-T2
Surgical narrative Position prone, posterior longitudinal skin incision, expose posterior superficial cervical fascia for a few centimeters bilaterally, dissection along nuchal ligament, skeletonize attachments of cervical muscles to spinous process and lamina, expose lamina and part of lateral masses, laminotomy using high-speed drill, cut lamina caudal to cranial following a line between each spinous process and zygapophyseal joint, cut interspinous ligaments at rostral and caudal-most levels, store osteoligamentous complex in saline and gentamicin, open dura under loupe magnification, tack dura, peel arachnoid membrane off pia under microscopic magnification, identify posterior median sulcus with IOM, open midline and displace posterior columns up and down with bipolar forces to completely expose tumor, resect tumor slowly with ultrasonic aspirator, work cleavage plane between tumor and posterior columns with bipolar cautery, care taken to avoid injuring anterior spinal artery and branches, use IOM to guide resection if no cleavage plane identified, watertight dural closure with fibrin glue, laminoplasty, layered closure Position prone, obtain IOM baseline, x-ray to confirm levels, perform three- to four-level laminectomy or laminoplasty, ultrasound to confirm tumor exposure, open dura and visualize tumor, perform midline myelotomy to identify tumor unless comes to surface, obtain frozen pathology to assess if planes are present based on histology, work inside-out if no tumor planes and debulk as much as possible, utilize MEP and SSEP to guide resection, avoid cauterizing if possible, watertight dural closure, multilayer closure with possible laminoplasty Preflip IOM, position prone, posterior midline incision from C6-T1, linear fascia opening, subperiosteal dissection, lateral extension to expose pedicle/facet articular professes for instrumentation, C6-T1 laminotomy with ultrasonic bone scalpel, midline dural opening with microscope, open midline posterior sulcus to avoid posterior columns, biopsy lesion for histopathological diagnosis, safe maximal resection with IOM if pathology shows oncologic features, C6-T1 transpedicular screw fixation with O-arm with bone graft if needed, layered closure Position prone with Mayfield pins, posterior midline incision from C5-T2, subperiosteal dissection, placement of C5–6 lateral mass screws and T1 and T2 pedicle screws, x-ray to evaluate position of screws, ultrasonic bone scalpel or drill to perform C6-T1 laminectomies, microsurgical intradural exploration, midline myelotomy if there is no exophytic component, aim for exophytic component or else midline myelotomy to enter cord, biopsy and/or resect lesion depending on consistency and ease of resection, watertight dural closure, rods and caps are placed, decorticate facets, place demineralized bone matrix or autograft on bone surfaces, irrigation of wound, vancomycin in cavity, layered closure with drain
Complication avoidance Laminoplasty, preserve articular capsule during laminoplasty, identify posterior median sulcus with IOM, completely open posterior columns to expose tumor, care taken to avoid injuring anterior spinal artery, use IOM to guide resection if no cleavage plane identified Laminectomy or laminoplasty, ultrasound to confirm exposure, valuate if comes to surface or else midline myelotomy, inside-out debulking if no planes, resection guided by monitoring, avoid cauterizing Preflip IOM, access lesion through midline posterior sulcus to preserve dorsal columns, biopsy to guide further resection, O-arm for instrumentation Excisional biopsy, D-wave IOM, access exophytic component or perform midline myelotomy, resection based on ease of resection
Postoperative
Admission ICU ICU Floor ICU
Postoperative complications feared Neurological injury, CSF leak, instability, wound infection CSF leak, motor or sensory deficit, spinal deformity Hematoma, spinal cord edema Neurological injury, CSF leak, adjacent segment disease, infection poor wound healing
Anticipated length of stay 5 days 3–4 days 2 days 4–5 days
Follow-up testing
  • MRI C-T spine within 24 hours, 2 months, 6 months, yearly after surgery

  • C-spine sitting x-rays prior to discharge, 3 months after surgery

MRI C-spine within 48 hours of surgery
  • MRI C-spine within 48 hours of surgery

  • C and T-spine x-rays 6 weeks after surgery

  • CT within 24 hours of surgery

  • C- and T-spine x-rays 6 weeks after surgery

  • MRI C-spine 3 months after surgery

Bracing None None None None
Follow-up visits 2 weeks, 1 month, 3 months, 6 months, 1 year after surgery 10–14 days after surgeryOncology evaluation 2 weeks after surgery 2 weeks and 6 weeks after surgery
CSF , cerebrospinal fluid; CT , computed tomography; EMG , electromyogram; ICU , intensive care unit; IOM , intraoperative monitoring; MAP , mean arterial pressure; MEP , motor evoked potential; MRI , magnetic resonance imaging; NCS , nerve conduction study; PET , positron emission tomography; SSEP , somatosensory evoked potential.

Differential diagnosis

  • Primary intramedullary spinal cord tumor

  • Secondary intramedullary spinal cord tumor

  • Demyelinating disease

  • Radiation necrosis

  • Leptomeningeal disease

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