Abstract
Objectives: Olfactory neuroblastoma (ONB), also known as esthesioneuroblastoma, is a rare malignant
neoplasm of the nasal vault and anterior skull base. The results of treatment for
ONB are relatively good; however, regional and distant metastases can develop several
years after definitive treatment. This study aimed to validate the treatment modality
of ONB for oncological outcomes, especially for regional recurrence.
Methods: We retrospectively reviewed the medical records of 22 patients diagnosed with ONB
at Kyoto University Hospital between 2009 and 2020. Descriptive statistics were calculated,
and Kaplan–Meier curves were used.
Results: The median follow-up time was 58.2 months. One (4.5%) patient was clinically node
positive, (cN+) and the remaining 21 (95.5%) were clinically node negative (cN0) at
presentation. Eighteen patients underwent an endoscopic endonasal approach (EEA) for
primary resection, and the remaining four patients underwent a combined EEA and transcranial
approach. Elective neck dissection was not performed for 21 patients with cN0 ONB,
whereas unilateral neck dissection with removal of ipsilateral lateral retropharyngeal
node was performed for one patient with cN+ ONB. Postoperative radiotherapy without
concurrent chemotherapy was performed only at the primary tumor bed for 21 patients
with cN0 ONB, and at the primary tumor bed and bilateral neck for one patient with
cN+ ONB. The 5-year overall, disease-specific, and disease-free survival rates were
94.1%, 100%, and 69.6%, respectively. No patients developed local recurrence, but
6 (27.2%) patients experienced recurrence with a median time to recurrence of 36.4
months, including four and two patients who initially developed regional recurrences
and bone metastases, respectively. Five (22.7%) patients had delayed neck recurrence.
The salvage rate was only 60.0% in the five patients who had delayed neck recurrence.
Regarding the level of delayed neck recurrence, 4 (18.2%) patients had lateral retropharyngeal
lymph node metastases.
Conclusion: Patients with ONB have excellent survival outcomes after endoscopic surgical resection
of the primary lesion with postoperative radiotherapy only to the primary tumor bed.
Despite excellent survival, delayed neck recurrence, including the lateral retropharyngeal
lymph node, remains high. Because salvage surgery for lateral retropharyngeal lymph
node recurrence is sometimes technically difficult, it may be better to extend the
field of postoperative radiotherapy from the primary tumor bed only to include bilateral
lateral retropharyngeal lymph node regions in patients with clinically N0 ONB. Further
prospective studies with a large number of patients are needed to determine the extent
of postoperative radiotherapy.
Keywords
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Article info
Publication history
Published online: December 30, 2022
Accepted:
December 9,
2022
Received:
September 17,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2022 Japanese Society of Otorhinolaryngology-Head and Neck Surgery, Inc. Published by Elsevier B.V. All rights reserved.
