Hemangioblastoma

Introduction

Hemangioblastomas account for approximately 1.5% to 2.5% of all central nervous system tumors, and 7% to 8% of all posterior fossa tumors, but are the most common primary tumor of the cerebellar hemisphere. ^, The majority of posterior fossa hemangioblastomas involve the cerebellar hemispheres (70%), but other locations include the brainstem (24%), cerebellopontine angle (2%), fourth ventricle (2%), and craniocervical junction (2%). ^, Single tumors may appear sporadically, whereas patients with multiple tumors typically have von Hippel-Lindau disease. ^, Patients can present with symptoms of elevated intracranial pressure, including headaches, nausea, and vomiting, as well as cerebellar symptoms. ^, Surgical resection is the standard of care, but surgery can be associated with significant morbidity, including cranial nerve deficits, postoperative hemorrhage, hydrocephalus, pseudomeningocele, meningitis, and pneumonia, among others. ^, In this chapter, we present a case of a patient with a right cerebellar hemisphere hemangioblastoma.

Example case

Chief complaint: headaches, nausea, vomiting

History of present illness

A 24-year-old, right-handed, female graduate student with no significant past medical history presented with headaches, nausea, and vomiting. She was in the laboratory when she developed acute onset of headaches with nausea and vomiting. She denied any loss of consciousness. She saw her primary care physician who ordered brain imaging that revealed a brain lesion ( Fig. 72.1 ). She was started on dexamethasone.

Medications : Dexamethasone, pantoprazole.
Allergies : No known drug allergies.
Past medical and surgical history : None.
Family history : No history of intracranial malignancies.
Social history : PhD student, no smoking, no alcohol.
Physical examination : Awake, alert, oriented to person, place, time; Cranial nerves II to XII intact; No drift, moves all extremities with good strength; Right greater than left finger-to-nose dysmetria.
Imaging : Retinal examination, abdominal ultrasound, and spine imaging negative for lesions.

	Michael R. Chicoine, MD, Washington University, St. Louis, MO, United States	Evandro de Oliveira, MD, PhD, Joao Paulo Almeida, MD, Institute of Neurological Sciences, São Paulo, SP, Brazil	Michael T. Lawton, MD, Barrow Neurological Institute, Phoenix, AZ, United States	Gerardo D. Legaspi, MD, Philippine General Hospital, Manila, Philippines
Preoperative
Additional tests requested	Urine and serum metanephrines	Transesophageal echocardiogram Anesthesia evaluation	Cerebral angiogram VHL workup	None
Surgical approach selected	Right suboccipital craniotomy	Right suboccipital craniotomy	Preoperative embolization, right suboccipital craniotomy	Right suboccipital craniotomy
Anatomic corridor	Right transcerebellar	Right transcerebellar	Right transcerebellar	Right transcerebellar
Goal of surgery	Gross total resection, diagnosis	Gross total resection, preservation of neurovascular structures	Gross total resection	Gross total resection
Perioperative
Positioning	Prone with slight left rotation	Semisitting	Right lateral	Right supine with left head rotation
Surgical equipment	Surgical navigation Ultrasound Surgical microscope Aneurysm clips	Semisitting equipment Precordial Doppler IOM (SSEP, MEP) Surgical navigation Ultrasound Surgical microscope Brain retractors	Surgical navigation Surgical microscope with ICG	Lumbar drain Surgical navigation Surgical microscope
Medications	Steroids	Steroids	Steroids	None
Anatomic considerations	Transverse and sigmoid sinuses, cerebellum brainstem, fourth ventricle, CPA	Occipital bone, foramen magnum, transverse and sigmoid sinuses, cerebellum, dentate nucleus, AICA/PICA branches	Sigmoid sinus	Dentate nucleus
Complications feared with approach chosen	Cerebral edema, hydrocephalus, brainstem, or cranial nerve injury	Injury to dentate nucleus, vascular injury to PICA or AICA and branches, hydrocephalus	Neurologic deficit	Cerebellar injury
Intraoperative
Anesthesia	General	General	General	General
Skin incision	Linear paramedian from transverse sinus to foramen magnum	Hockey stick incision from inion to C2 with lateral extension over superior nuchal line down to mastoid tip	Linear paramedian	Linear retromastoid (∼6 cm)
Bone opening	Right retrosigmoid suboccipital	Right lateral suboccipital	Right retrosigmoid suboccipital	Right retrosigmoid suboccipital
Brain exposure	Right cerebellar hemisphere	Right cerebellar hemisphere	Right cerebellar hemisphere	Right cerebellar hemisphere
Method of resection	Right retrosigmoid craniotomy, utilize shortest trajectory to lesion, open dura, circumferential dissection around lesion with coagulation and sectioning of arterial feeders, vascular clips on larger arterial feeders, removal of lesion en bloc	Expose superficial fascia, form muscle cuff, work through avascular plane in the midline, retract muscles, right lateral suboccipital craniotomy with exposure of inferior border of transverse sinus and medial border of sigmoid sinus, inverted C-shaped dural opening near transverse and sigmoid sinus, ultrasound and navigation for localization, corticectomy, dissect tumor from surrounding parenchyma without violating tumor capsule and preserving developmental venous anomaly, watertight closure with muscle interposed	Right retrosigmoid craniotomy, transcerebellar dissection, removal of lesion with attempted en bloc	Lumbar drain placement, localize tumor with surgical navigation, linear retromastoid incision, burr hole and craniotomy, localize lesion with surgical navigation and ICG, radial dural incision, cerebellotomy based on navigation, gross total resection, closure in layers
Complication avoidance	Shortest trajectory, en bloc resection, vascular clips for larger arterial feeders	Semisitting position, ultrasound, and navigation to pinpoint location, avoid violating capsule, preserve developmental venous anomaly	Preoperative embolization, en bloc resection, ICG	ICG and navigation to pinpoint location
Postoperative
Admission	ICU	ICU	ICU	Floor
Postoperative complications feared	Cerebellar edema, hydrocephalus, brainstem, or cranial nerve injury	Hydrocephalus, cerebellar stroke, CSF leak	Posterior fossa syndrome	Cerebellar edema
Follow-up testing	MRI 3 months after surgery	MRI within 24 hours after surgery Consider genetics referral for VHL	MRI within 24 hours after surgery	None
Follow-up visits	3–4 weeks after surgery	15 days after surgery 6 months after surgery	14 days after surgery 6 weeks after surgery	1 week after surgery