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Setting the Stage for Olfactory Neuroblastoma | Neuro-oncology | JAMA Otolaryngology–Head & Neck Surgery | ÌÇÐÄvlog

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Invited Commentary
August 3, 2023

Setting the Stage for Olfactory Neuroblastoma

Author Affiliations
  • 1Department of Otolaryngology–Head and Neck Surgery, Walter Reed National Military Medical Center, Bethesda, Maryland
  • 2Department of Surgery, Uniformed Services University of the Health Sciences, Bethesda, Maryland
  • 3Department of Otolaryngology–Head and Neck Surgery, McGill University, Centre Intégré de Santé et des Services Sociaux de l’Outaouais, Gatineau, Quebec, Canada
  • 4Department of Otolaryngology–Head and Neck Surgery, Columbia University Medical Center, New York Presbyterian Hospital, New York, New York
JAMA Otolaryngol Head Neck Surg. 2023;149(9):845-846. doi:10.1001/jamaoto.2023.1943

In this issue of JAMA Otolaryngology–Head and Neck Surgery, Choby et al1 present a novel approach to staging olfactory neuroblastoma (ONB) that may well lead to a paradigm shift in the approach to this complex disorder. As the authors expound, current staging systems fall short not only in prognosticating patient outcomes and survival but also in guiding appropriate surgical approach and adjuvant therapy recommendations. The Kadish system and its modifications, the Dulguerov system, and the American Joint Committee on Cancer (AJCC) system all poorly estimate overall 10-year survival.2 Hyams grading of tumor histopathology, on the other hand, has consistently been associated with survival outcomes.3 By examining tumor architecture, cellular pleomorphism, presence of a neurofibrillary matrix and rosettes, mitotic activity, and presence of calcifications or necrosis, Hyams grading captures the intrinsic biological behavior of the tumor. Choby et al performed a retrospective, multicenter study of patients with ONB in order to develop and explore a creative model that incorporates Hyams grading, dichotomized as either low Hyams grade (1-2) or high (3-4), into traditional staging systems, which ultimately showed improved ability to estimate recurrence and disease-specific survival. The potential outcomes of a more accurately predictive ONB staging system are profound for both clinicians and patients. This multi-institutional study also identifies other clinical factors associated with survival and disease recurrence.

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