Case 5: cervical neurofibroma

posted June 20, 1996

Resident: Bruce Hamilton, MD
Attending: Deepak Awasthi, MD

This 25 white woman presented to the neurosurgery clinic with the chief complaint of right shoulder and neck pain as well as posterior headaches. In addition, she also described increasing difficulty with walking and balance over the course of previous month. She denied any weakness of the upper extremities or any paresthesias or any bowel/bladder difficulties.

PHYSICAL EXAMINATION: Slender healthy appearing woman in moderate distress from neck pain.

Neck exam: firm, immobile, non-tender mass palpated on the right side of the neck- below the mastoid process and posterior to the sternocleidomastoid muscle. Good pulses in the neck. Good range of motion of the neck and supple.

General exam: multiple cafe-au-lait spots and patches on the trunk and extremities. Good rectal tone.

Neuro Exam: awake and alert; cranial nerves intact; no lesions seen in the retina; pupils equal and reactive. Motor: increase tone in both lower extremities; mild weakness (3/5) in the distal muscles of the lower extremities. Sensory: decrease position and vibratory sensation in both lower extremities; able to feel touch and pain normally. Cerebellar: no dysmetria. Gait: very hesitant and spastic. Romberg sign present. Reflexes: hyperactive in both lower extremities with bilateral ankle clonus; plantar reflex- extensor.

DIAGNOSTIC STUDIES: The patient had a CT scan followed by a MRI scan with and without contrast of the cervical spine. These studies showed a large homogeneously enhancing mass at the right C2-3 level extending from the spinal canal through the foramen in a "dumbell-shaped' manner. A selected contrasted enhanced T1-weighted axial image at the level of C2-3 is shown below:

The studies were consistent with the diagnosis of a neurofibroma. Given the strong possibility of NF 1 (neurofibromatosis), the patient also had a MRI scan of the thoracic spine as well as brain to rule out any other lesions.

What would you do now? If surgery, what approach?

Please comment: dawast@lsuhsc.edu

STEPS TAKEN AT LSU: The patient was taken to the OR and through a posterolateral approach, the tumor was completely resected. It was both intradural (extramedullary) and extradural as it extended out of the neural foramina at C3. Below are some select intraoperative photographs.

The incision- longitudinal midline and then extending to the right superiorly

The bulk of the tumor has been removed laterally and above is the tumor (T) extending from the intradural space outwards towards the foramen and overlying the vertebral artery.

The tumor has been grossly resected and the dura closed. Note the remarkable view of the vertebral artery (yellow dot) on the right traversing between C3 and C2. The tumor was overlying the artery. Also note the wide exposure provided by the posterolateral approach. Blue dot: thecal sac; Purple dot: C1

PATHOLOGY: Neurofibroma

POSTOPERATIVE COURSE: Patient did well postoperatively- there were no new neurological deficits. The spastic paraparesis was unchanged. She did report improvement in the right shoulder pain.

The fall issue of NerveCenter highlights the posterolateral approach to the cervical spine.

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