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Esthesioneuroblastoma is a rare sinonasal malignancy presenting with nonspecific sinonasal complaints.
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Diagnosis is confirmed histopathologically, with characteristic small, round, blue cells in a neurofibrillary stroma with prominent microvascularity and lobular architecture.
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Higher histologic grade (Hyams) portends worse prognosis.
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Preoperative assessment and imaging are essential to guide surgical approach.
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Endoscopic endonasal resection is feasible in select cases with the goal of obtaining
Endoscopic Management of Esthesioneuroblastoma
Section snippets
Key points
Treatment goals and planned outcomes
Like other malignant sinonasal tumors, treatment goals and expected outcomes depend on extent of disease and tumor grade at presentation. Overall 5- and 10-year survival rates based on Surveillance, Epidemiology, and End Results tumor registry data were 62.1% and 45.6%.15 In the original report by Kadish and coworkers3 of 17 patients with ENB, 100% of patients with group A disease (seven of seven), 80% (four of five) with group B disease, and 40% (two of five) with group C disease survived 3 or
Preoperative planning and preparation
Preoperative planning begins with comprehensive history and physical by the neurosurgeon and the otolaryngologist–head and neck surgeon. Special emphasis must be placed on any factors that could increase the difficulty of the case, such as a history of chronic rhinosinusitis and previous functional endoscopic sinus surgery. As in all surgical patients, preoperative clearance must be performed by the appropriate internist and/or anesthesiologist to ensure that the patient can safely undergo
Patient positioning
The patient is placed in the supine position and the head secured using a Mayfield three-point head fixator. The neck is placed in extension with slight rotation toward the surgeons. The video monitor is placed on the patient’s left, across from the surgeons and monitors for both intraoperative CT and MRI navigation are placed adjacent to the video monitor. Care is taken to secure essential equipment including the nasal endoscope, suction tubing, suction monopolar electrocautery, pistol-grip
Procedural approach
The nasal cavity is first examined with the 0-degree endoscope and the tumor is debulked using the microdebrider, taking care to avoid injury to native mucosa. Once adequate debulking is performed, the nasal septum is assessed and endoscopic septoplasty is performed for access if needed. If the posterior septum is limiting access, a back-biting forceps may be used to perform a posterior septectomy, taking care to preserve the mucosa unilaterally in the event that a nasoseptal flap is needed for
Postoperative care
All patients are monitored postoperatively in the neurologic intensive care unit. A spiral head CT is performed to evaluate for any postoperative bleeding or pneumocephalus, and to assess the positioning of nasal packing. Transfer to the surgical floor generally occurs on postoperative Day 1, and an MRI scan is performed within 2 days of the surgery to assess extent of resection. Lumbar drainage is routinely continued for 36 to 48 hours postoperatively. Intravenous antibiotics are continued
Complications
Complications of expanded endonasal resection of ENB are categorized into immediate and delayed postoperative complications. Immediate complications are vascular, orbital, intracranial, neurologic, or systemic. Vascular complications include epistaxis, intracranial hemorrhage, and cerebrovascular accident. Intracranial hemorrhage is extremely rare and most commonly associated with injury to the orbitofrontal branches of the anterior cerebral artery. Cerebrovascular accident is considered less
Rehabilitation and recovery
The endoscopic endonasal approach to ENB described here is generally well tolerated. Inpatient hospital recovery varies and is typically less than 1 week. There are no acute or subacute rehabilitation needs postoperatively. Early postoperative management typically requires strict cerebrospinal fluid leak precautions including no nose blowing, and no strenuous activity for 1 month after surgery. Patients are asked to sleep with the head of bed elevated at least 30° and are provided with stool
Outcomes
Although ENB is a rare entity and data remain limited, there is growing evidence that expanded endoscopic approaches to resection of this tumor are feasible, effective, and safe. There have been several other small case series describing outcomes in treatment with expanded endonasal approaches. The largest series are described here. A comprehensive review of all studies evaluating endoscopic resection of ENB is provided in Table 3.
To date, the largest series of endoscopic ENB resection includes
Summary
ENB is an uncommon malignant tumor of the nasal cavity and paranasal sinuses, with frequent extension to the skull base and surrounding structures at presentation. Although the standard of care for these tumors was previously craniofacial resection, there is a growing body of evidence to suggest that an expanded endonasal approach to resection in selected cases may be a feasible alternative allowing for gross total resection with negative histopathologic margins and acceptable rates of
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Cited by (13)
Esthesioneuroblastoma in children, adolescents and young adults
2020, Bulletin du CancerCitation Excerpt :This method enables good visualization of the tumor and its extension, an en bloc resection of the mass and reconstruction facilitated by a vascularized pericranial flap [67]. However, endoscopic approaches have been developed and become the standard of care for ENB surgery enabling a good local control and a decrease in post-surgical morbidity compared to cranial-facial resection surgery [9,68–71]. Moreover, endoscopic endonasal approach (EEA) is now used in the treatment of more advanced tumors allowing for a good rate of complete resections without the morbidity related to the necessity of a facial incision or retraction of the frontal lobe in case of a craniotomy [72,73].
Expression of Programmed Cell Death Ligand 1 and Associated Lymphocyte Infiltration in Olfactory Neuroblastoma
2020, World NeurosurgeryCitation Excerpt :Numerous clinical trials are currently underway to investigate the efficacy of PD-1 and PD-L1 blockade in patients with malignant gliomas.19,20 Olfactory neuroblastoma (ONB) (also known as esthesioneuroblastoma) is a rare malignant neoplasm of the nasal cavity and anterior skull base.21 Although the cell of origin for this tumor is controversial, it is generally accepted to arise from the basal progenitor cells of the olfactory epithelium.22
Endoscopic management of Esthesioneuroblastoma: Our experience and review of the literature
2018, Journal of Clinical NeuroscienceThe Unusual Presentation of a Myxoma Within the Sphenoid Sinus: Case Report and Review of the Literature
2017, World NeurosurgeryCitation Excerpt :Esthesioneuroblastomas are sinonasal malignancies with low survival rates.47 The surgical goal when treating these patients is complete resection with negative margins.48 In the largest series of endoscopic esthesioneuroblastoma resection, there was no recurrence in 22 of 23 cases, and CSF leakage occurring in only 4 cases.49
Management of Cavernous Sinus Involvement in Sinonasal and Ventral Skull Base Malignancies
2017, Otolaryngologic Clinics of North AmericaCitation Excerpt :Since the seminal articles by Ketcham and colleagues9,10 in 1963 and 1966 detailing the role of surgery in intracranial involvement of head and neck malignancy, innovation of radical surgical resection procedures for the eradication of malignant ventral skull base tumors is arguably one of the most important advancements in the treatment of head and neck malignancy in the past half century. Besides the use of conventional open transcranial and transfacial approaches to achieve the surgical goal of oncologic resection, minimally invasive endoscopic approaches have been applied more recently to selected patients with reasonable outcomes.11–13 It was not uncommon in the past that malignant skull base tumors were considered inoperable.
The authors have no relevant financial conflicts of interest to disclose.