Craniopharyngiomas

Introduction

Craniopharyngiomas are rare intracranial tumors with an incidence of 1 to 2 cases per million persons per year that are derived from pituitary gland embryonic tissue, and have a bimodal clinical presentation in which it most commonly occurs in children between the ages of 5 to 14 years (30%–50%), but can also occur in adults 50 to 74 years. ^, Childhood-onset craniopharyngiomas are typically adamantinomatous in histology and have an 87% to 95% 20-year overall survival rate, but are associated with a poor quality of life owing to injury of the optic apparatus and hypothalamic-pituitary axis, whereas adult-onset craniopharyngiomas are typically papillary in histology and have a 19-fold higher cerebrovascular mortality than the general population. Childhood craniopharyngiomas usually present late with prolonged symptoms of mass effect (headaches, nausea, vomiting), visual impairment, and endocrine deficits, whereas adults typically present with hormonal deficits (growth hormone, gonadotropins, adrenocorticotropic hormone, thyroid stimulating hormone). Surgery is typically first-line therapy in most patients; however, there is controversy over the best treatment strategy (gross total resection vs. partial resection and radiation therapy, as well as endoscopic vs. transcranial approaches). In this chapter, we present a case of a patient with a craniopharyngioma.

Example case

Chief complaint: headaches

History of present illness

A 14-year-old, right-handed boy with no significant past medical history presented with worsening headaches. For the past 2 to 3 months, he complained of worsening headaches that were exacerbated by activity and associated with nausea and vomiting. Primary care ordered imaging that showed a large brain lesion ( Fig. 62.1 ).

Medications: None.
Allergies: No known drug allergies.
Past medical and surgical history: Tonsillectomy.
Family history: No history of intracranial malignancies.
Social history: Eighth grade honors student, no smoking or alcohol.
Physical examination: Awake, alert, oriented to person, place, time; Cranial nerves II to XII intact, except bitemporal hemianopsia; Moves all extremities with good strength.
Pituitary labs: Prolactin, insulin-like growth factor-1, cortisol, thyroid-stimulating hormone/T4 all within normal limits.

	Peter Bullock, FRCS, MRCP, London Clinic, London, England	Edward R. Laws, MD, Daniel Donoho, MD, Brigham and Women’s Hospital, Boston, MA, United States	James Rutka, MD, PhD, University of Toronto Sick Kids, Toronto, Canada	Theodore H. Schwartz, MD, Cornell University, New York, NY, United States
Preoperative
Additional tests requested	CT Neuroophthalmology evaluation Neuroendocrinology evaluation Neuropsychological assessment	CT and CTA Neuroophthalmology evaluation (papilledema, OCT) Neuropsychological assessment BMI and diabetes insipidus evaluation	CT Neuroophthalmology evaluation Neuroendocrinology evaluation Neuropsychological Neurology evaluation	CT Neuroophthalmology evaluation (visual fields) Neuroendocrinology evaluation Neuropsychological assessment
Surgical approach selected	Expanded endoscopic endonasal	Right frontotemporal craniotomy with IOM (MEP/SSEP)	Endoscopic endonasal	EVD and endoscopic endonasal
Other teams involved during surgery	ENT	None	ENT	ENT
Anatomic corridor	Transsphenoidal, transplanum, transtuberculum	Right frontotemporal, trans-Sylvian	Transsphenoidal	Transsphenoidal, transplanum, transtubercular
Goal of surgery	Gross total resection, maintain or improve vision, preserve endocrine function	Diagnosis, optic compression, maximal safe resection, cyst decompression	Gross total resection	EVD to relieve hydrocephalus, decompression of optic chiasm, gross total resection without injuring hypothalamus
Perioperative
Positioning	Supine with pins with left rotation	Supine with left rotation	Supine neutral in pins	Supine neutral in pins
Surgical equipment	Surgical navigation Endoscopes Ring curettes	Surgical navigation IOM (MEP/SSEP) Surgical microscope Ultrasonic aspirator Ring curettes	Surgical navigation Endoscopes	Surgical navigation Endoscopes with scope holder Tissue biting device
Medications	Steroids	Antiepileptics Mannitol if necessary	None	Steroids
Anatomic considerations	Sella, clivus, carotid prominences, optic canals, medial and lateral optico-carotid recesses	ICA	Optic nerves and chiasm, pituitary gland and stalk, ICA/ACA, hypothalamus	Tuberculum, sella, ICAs, optic chiasm and nerves
Complications feared with approach chosen	Diabetes insipidus, vision loss, CSF leak, ICA injury, meningitis	Visual deficit	Injury to hypothalamus, optic apparatus, cerebrovasculature, CSF leak	Injury to optic nerves, hypothalamic injury, CSF leak, anosmia, panhypopituitarism
Intraoperative
Anesthesia	General	General	General	General
Skin incision	None	Pterional	None	None
Bone opening	Anterior wall of sphenoid sinus, posterior ethmoidectomy, sella, planum tuberculum	Frontotemporal	Anterior wall of sphenoid sinus, sella	Sphenoid rostrum, anterior sphenoid sinus wall, posterior ethmoids, sellar face, tuberculum, planum
Brain exposure	Sella, tuberculum, planum	Frontotemporal	Sella	Sella, tuberculum, planum
Method of resection	ENT starts the approach, sphenoid sinus opened widely, nasoseptal flap raised and tucked into nasopharynx, identification of ICA/optic canals, removal of tuberculum and sellar bone, neurosurgeon exposes medial edge of cavernous sinuses and thins bone over planum and tuberculum with drill, remove medial aspects of borth optic canals, carotid and ophthalmic arteries identified with Doppler, dura opened with diamond knife and coagulating and dividing intercavernous sinus, suprasellar space entered and diaphragm opened, chiasm and supraclinoid ICA identified, cyst decompressed on left, chiasm dissected from tumor making sure to preserve perforators to the chiasm and pituitary, extra capsular dissection of solid elements with internal tumor debulking, stalk may need to be sacrificed to compelte resection, peel superior cyst wall from inside ventricle, close with a multi-layer reconstruction using nasoseptal flap rotated into position and held in position with surgicell / tissue glue / nasopore	Pterional craniotomy, dural opening, Sylvian fissure opening, mannitol if necessary, tumor resection of solid and cystic component with sparing hypothalamus	Nasal septal flap, exposure of sphenoid sinus, remove sellar face, puncture and drain cyst, mobilize capsule, dissect capsule from surrounding neurovasculature structures, preserve pituitary stalk, angled endoscope to observe for residual, closure of sella with bone, fat, and nasoseptal flap nasal packing	EVD placement, ENT approach with harvesting of nasoseptal flap and opening of sphenoid sinus posterior ethmoids, partial removal of left middle turbinate, removal of the keel and then entry into sphenoid sinus, remove all mucosa within the sphenoid sinus, open bone from the top of the sella just below the superior intercavernous sinus to the planum from medial OCR to medial OCR, opening of dura starting below intercavernous sinus with dural removal, identification of SHA and dissected away from tumor, tumor entered and debulked, capsule dissected free from arachnoid, stalk identified and attempted at preservation unless it compromises complete resection, tumor removed from optic chiasm and hypothalamus with caution, dura reconstructed with alloderm of fascia lata and countersunk with medpore implant and nasoseptal flap
Complication avoidance	Wide bony opening, drain cyst early, extracapsular dissection, nasal septal flap and multi-layered closure	Sylvian fissure opening, leave tumor adherent to hypothalamus	Drain cyst first, dissect capsule from surrounding structures but care to hypothalamus and pituitary stalk, nasoseptal flap	EVD, nasoseptal flap, remove all sphenoid sinus mucosa, identify SHA, leave piece along hypothalamus if cannot be dissected, countersinking layered closure, leave EVD at opening pressure of 0 to reduce intracranial pressure for 48 hours
Postoperative
Admission	High dependency unit	ICU	ICU	ICU
Postoperative complications feared	CSF leak, hypopituitarism, diabetes insipidus, visual decline	Hypopituitarism, hyponatremia, visual loss, injury to arteries and hypothalamus, obesity, memory loss	CSF leak, panhypopituitarism, visual impairment	Diabetes insipidus, adrenal insufficiency, CSF leak, vision loss
Follow-up testing	Maintain strict fluid balance Vision checked every 4 hours MRI within 24 hours after surgery	Sodium and cortisol 1 week after surgery Full endocrine panel 6 weeks after surgery MRI 3 months after surgery	MRI within 24 hours after surgery Neuroophthalmology evaluation	EVD MRI within 48 hours after surgery after EVD removal
Follow-up visits	2 weeks and 3 months after surgery with neurosurgery 1 week after surgery with ENT	3 months after surgery	4–6 weeks after surgery	7 days after surgery with ENT and endocrine 3 months after surgery with neurosurgery
Adjuvant therapies recommended for residual	Radiation	Radiation, BRAF inhibitor, or repeat surgery if necessary	Radiation therapy or radiosurgery, repeat surgery if necessary	Observation for gross total resection, early radiation for subtotal resection