Esthesioneuroblastoma

Introduction

Esthesioneuroblastoma (ENB), which is also known as an olfactory neuroblastoma, is an uncommon sinonasal malignancy that has a propensity for local invasion into surrounding paranasal structures, including the eye and brain, as well as systemic metastases. These tumors are thought to arise from neuroendocrine cells in the olfactory epithelium, and account for approximately 3% to 6% of all sinonasal cancers. They usually present with symptoms of nasal obstruction and epistaxis, but can also result in symptoms of cerebral mass effect (headaches, nausea, vomiting) and vision loss. The outcomes for patients with ENB are dictated by the extent of disease and tumor grade at presentation, in which the overall 5- and 10-year survival rates were 62.1% and 45.6%, respectively. The typical goal of treatment for ENB is complete surgical resection with negative margins, and in cases in which negative margins are not obtained or in patients with distant metastases, adjuvant radiation and chemotherapy are often offered. However, treatment plans should be administered on an individual basis. In this chapter, we present a case of a patient with an ENB.

Example case

Chief complaint: acute vision loss

History of present illness

A 73-year-old, right-handed woman with a history of hypertension and hypercholesterolemia presented with acute vision loss. She noticed visual decline over several months; however, over the past 24 hours her vision deteriorated to the point in which she only had light perception in both eyes. She was taken to the emergency room, and imaging revealed a large skull base lesion ( Fig. 65.1 ).

Medications : Hydrochlorothiazide, simvastatin.
Allergies : No known drug allergies.
Past medical and surgical history : Hypertension, hypercholesterolemia.
Family history : No history of intracranial malignancies.
Social history: Retired school teacher, no smoking or alcohol.
Physical examination : Awake, alert, oriented to person, place, time; Cranial nerves II to XI; Moves all extremities with good strength.
Chest/abdomen/pelvis computed tomography (CT): No evidence of systemic disease.

	William T. Curry, MD, Massachusetts General Hospital, Boston, MA, United States	José Hinojosa Mena-Bernal, MD, PhD, Sant Joan de Deu, Barcelona, Spain	Shaan M. Raza, MD, MD Anderson Cancer Center, Houston, TX, United States	Henry W. S. Schroeder, MD, PhD, University of Greifswald, Greifswald, M-V, Germany
Preoperative
Additional tests requested	CT maxillofacial	Ophthalmology evaluation (visual fields, OCT) Endocrinology evaluation CT chest, abdomen, pelvis Anosmia evaluation	Medical oncology evaluation Radiation oncology evaluation Ophthalmology evaluation MRI face/neck/orbits with fat saturation Biopsy of mass, drainage of sphenoid Based on esthesioneuroblastoma pathology, potential urgent platinum based chemotherapy for Hyams III/IV or urgent curative surgery for Hyams I/II	Ophthalmology evaluation (visual fields) Endocrinology evaluation Anesthesia evaluation CT maxillofacial
Surgical approach selected	Bifrontal craniotomy and endoscopic endonasal	Endoscopic endonasal +/– bifrontal craniotomy	Craniotomy and endoscopic endonasal	Bifrontal craniotomy and endoscopic endonasal
Other teams involved during surgery	ENT	ENT	ENT	ENT
Anatomic corridor	Bifrontal and craniofacial	Transcribriform/transplanum, bifrontal if needed	Bifrontal and endonasal	Bifrontal and endonasal
Goal of surgery	Restoration of vision, total resection, skull base reconstruction	Radical tumor resection with negative margins	For Hyams I/II, gross total resection without orbitectomy. For Hyams III/IV, induction chemotherapy	Radical tumor resection
Perioperative
Positioning	Supine neutral	Supine neutral	Supine neutral with slight extension	Supine with right rotation, left tilt
Surgical equipment	Surgical microscope Endoscope	Surgical navigation Endoscopes Ultrasonic aspirator Ultrasonic bone cutter Surgical microscope Microdoppler	Surgical microscope Ultrasonic aspirator Endoscope	Surgical navigation Surgical microscope Endoscopes Ultrasonic aspirator
Medications	Mannitol Diuretics Steroids Antiepileptics	Steroids	Mannitol Steroids	Steroids
Anatomic considerations	Optic nerves, ICA, ACA	Optic nerves and chiasm, ICA, ACA, ACOM	Olfactory bulbs, orbital roofs, planum sphenoidale, frontal/sphenoid/ethmoid/maxillary sinuses, nasal septum, right orbital apex, anterior/posterior ethmoidal arteries, sphenopalatine arteries, right orbital apex (periorbita, medial/superior rectus, superior division of CN III)	ICA, optic nerves
Complications feared with approach chosen	Visual decline, lack of negative margins	CSF leak, epiphora, facial scarring, venous thrombosis, vascular injury	Brain and orbital manipulation, positive margins	Injury to critical neurovascular structures
Intraoperative
Anesthesia	General	General	General	General
Skin incision	Bicoronal	Bicoronal if needed	Bicoronal	Bicoronal
Bone opening	Bifrontal, anterior skull base	Cribriform plate, planum, bifrontal if needed	Bifrontal, anterior skull base	Bifrontal, anterior skull base
Brain exposure	Bilateral frontal lobes, anterior skull base	Anterior skull base and bilateral frontal lobes if needed	Bilateral frontal lobes	Bilateral frontal lobes
Method of resection	Harvest pericranial flap, bifrontal craniotomy, open dura bilaterally with ligation of superior sagittal sinus, divide falx posterior to tumor, dissect tumor off of frontal lobes and mobilize ACA, remove tumor down to skull base, amputate cribriform, dissect tumor off of optic nerves, watertight dura closure with synthetic material or pericranium, ENT to resect sinonasal component, drainage of mucocele, dissect tumor from lamina papyracea/sphenoid sinus/maxillary sinus, palate, drill through craniotomy site to define posterior margin and remove from orbit, remove from frontal sinus, sectioning of anterior and posterior nasal septum, check margins, tack down pericranial flap	Endonasal first, middle and superior turbinates removed, middle meatal antrostomy, tumor debulking with suction and ultrasonic aspiration, sphenoidotomy, sphenoethmoidectomy, expose periorbita on both sides, coagulate anterior and posterior ethmoidal arteries, remove cribriform plate and roof of ethmoidal sinuses and posterior nasal septum, identify olfactory nerves and spare if not invaded, Draf III frontal sinusotomy approach, open dura, transect falx, deliver tumor along with underlying dura paying attention to separation ACA and ACOM, multilayer reconstruction with fascia lata and abdominal fat graft with nasoseptal flap, if any of the tumor cannot be reached, then an anterior bifrontal craniotomy is done with pericranial graft, dura is opened and SSS is ligated, retraction of frontal lobe, remove any intracranial extension, ultrasonic bone scalpel into ethmoid to establish negative margins, en bloc removal of cribriform plate, reconstruction with vascularized pericranium, watertight dural closure	Bicoronal incision with two-layer scalp dissection (scalp/pericranium), subfascial dissection laterally, low bifrontal craniotomy to level of nasofrontal suture in midline after bilateral McCarty burr holes, unify frontal sinuses with removal of posterior table and opening of nasofrontal ostia widely and mucosal margins taken, resect crista galli along the medial aspect of orbital roofs, coagulate and cut anterior/posterior ethmoidal arteries in orbit, open dura over both frontal lobes and ligate and cut SSS and falx above foramen cecum, intracranial portion of tumor debulked internally and capsule dissected from basal surface of both frontal lobes, cuff of brain is resected as margin, dissect olfactory bulbs posteriorly beyond gross disease and obtain margin, anterior skull base dura is incised circumferentially around gross disease where posterior limit of dural incision is just anterior to optic canals, resect lamina papyracea bilaterally and drilling planum sphenoidale to access sphenoid sinus with margins obtained, endoscopic approach next, septectomy with septal margins, bilateral maxillary antrostomies with mucosal margins and resection of superior/middle/inferior turbinates, SPA identified and sacrificed, inspect right nasolacrimal duct and extend medial maxillectomy and transect duct if necessary, resect remnants of medial orbital walls and biopsy left medial periorbita if necessary for margin, right periorbita incised anteriorly/superiorly/inferiorly, resect all gross disease from orbital apex after identifying superior rectus, dura reconstruction with pericranial flap that is rotated and sutured to planum sphenoidale	Harvest pericranial flap, bifrontal craniotomy, resection of intradural portion until skull base is reached, decompress bilateral optic nerves, resection of infiltrated dura, continue resection into paranasal sinuses with removal of tumor in ethmoid air cells and sphenoid sinus, parallel ENT access from below, resection of intraorbital portion of tumor on right, repair defect with pericranial flap and augmented with free pericranium fixed with sutures and fibrin glue, nasal pledgets, lumbar drain if CSF leak occurs
Complication avoidance	Harvest pericranium, large craniotomy, intradural portion first and then closure, communicate endonasal with transcranial component, check margins, reconstruction	Endonasal first, wide bony opening of anterior skull base, careful dissection of ACA and ACOM, multilayer closure, bifrontal craniotomy with wide bony opening if necessary, pericranial flap	Harvest pericranium, craniotomy first with sequential margins, devascularize skull base with ethmoidal arteries, devascularize nasal cavity with SPA, leave right periorbita for last, vascularized pericranium for dura closure	Harvest pericranium for reconstruction, multilayered closure, concomitant surgery with ENT, lumbar drain if CSF leak
Postoperative
Admission	ICU	ICU	Intermediate care	Intermediate care
Postoperative complications feared	CSF leak	CSF leak, visual deterioration, endocrine dysfunction, cognitive decline, anosmia	CSF leak, pericranial flap breakdown, frontal lobe injury, orbital injury (muscular or cranial nerve)	CSF leak, visual deterioration
Follow-up testing	MRI within 72 hours after surgery	MRI within 24 hours after surgery PET for systemic disease	MRI within 48 hours after surgery Adjuvant therapy 4 weeks after surgery	MRI within 24 hours after surgery Lumbar drain for 5 days if CSF leak
Follow-up visits	14 days after surgery with neurosurgery 14 days after surgery with oncology and radiation oncology	10–15 days after surgery	10 days after surgery	Continual follow-up with ENT 3 months after surgery with neurosurgery
Adjuvant therapies recommended	Surgery or radiation therapy depending on resectability	Second look surgery if possible, radiation for modified Kadish stage 3 without prophylactic neck radiation	Hyams I/II: adjuvant radiation therapy Hyams III/IV: adjuvant chemoradiation therapy	Surgery for remnant, radiation therapy after surgery