Olfactory neuroblastoma (esthesioneuroblastoma) was first described by Berger and Luc in 1924. It is considered to be an uncommon malignancy of the nasal cavity. The tumor arises from the specialized sensory epithelial olfactory cells, normally situated at the upper part of the nasal cavity, including the superior nasal concha, the roof of the nose and the cribriform plate. The imaging modalities of choice are computed tomography (CT) and magnetic resonance imaging (MRI). Combination of surgery and radiotherapy (either conventional radiotherapy or stereotactic radiosurgery), with or without chemotherapy is considered to be the standard of care for primary site disease by the majority of researchers. Combined transfacial and neurosurgical conventional approaches are also adopted in many reported cases, mainly due to the endocranial extension and the close anatomic relationship of esthesioneuroblastomas with the ethmoid roof and cribriform plate. Recent literature supports that endoscopic resection correlates with similar oncologic control rates compared with conventional open surgery, provided that basic oncologic surgical principles with clearance of margins and intradural dissection (when required) are completely maintained.
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Int Forum Allergy Rhinol
January 2025
Department of Otolaryngology-Head and Neck Surgery, University of California, Irvine, Orange, California, USA.
Background: Olfactory neuroblastoma (ONB) is a rare sinonasal malignancy primarily treated with surgery. For tumors arising from the olfactory area, traditional treatment involves transcribriform resection of the anterior cranial fossa. Surgery can be performed with unilateral or bilateral resection depending on extent of involvement; however, there are currently no studies comparing outcomes between the two.
View Article and Find Full Text PDFCancers (Basel)
December 2024
Department of Otolaryngology, Head and Neck Surgery, Helios HSK Wiesbaden, 65199 Wiesbaden, Germany.
: Sinonasal malignancies are rare and highly diverse cancers that pose significant diagnostic challenges due to their variable histological features and complex anatomical locations. Accurate diagnosis is critical for guiding treatment, yet conventional methods often require multiple biopsies. This study aimed to evaluate the potential of confocal laser endomicroscopy (CLE) for real-time imaging of sinonasal tumors to characterize specific features of different entities and improve diagnostic precision.
View Article and Find Full Text PDFCancers (Basel)
November 2024
Center for Immuno-Oncology, National Cancer Institute, National Institute of Health, Bethesda, MD 20894, USA.
Olfactory neuroblastoma (ONB), sinonasal undifferentiated carcinoma (SNUC), and sinonasal neuroendocrine carcinoma (SNEC) are rare malignancies arising from the sinonasal tract with limited therapeutic options. The expression of the somatostatin receptor 2 gene (), which is expressed in other neuroendocrine neoplasms and is therapeutically actionable, has been reported in these tumors. Here, we analyzed gene expression and its associations with genomic features, established biomarkers predicting of immune response, and the tumor immune microenvironment in a cohort of ONB, SNUC, and SNEC tumor samples (26, 13, and 8 samples, respectively) from a real-world database.
View Article and Find Full Text PDFNeurocirugia (Astur : Engl Ed)
December 2024
Departamento de Neurocirugía, Fundación Oftalmológica de Santander - Clínica Ardila Lülle, Floridablanca, Colombia. Electronic address:
The olfactory neuroblastoma is a rare malignant neoplasm derived from the olfactory neuroepithelium. It can metastasize to cervical lymph node chains and distant organs through hematogenous or lymphatic routes. Two clinical cases are presented: the first, a 56-year-old man with no pathological history, exhibited symptoms evolving over 2 months, characterized by persistent rhinorrhea with frequent epistaxis, ipsilateral proptosis, left hemicranial pain, anosmia, and dysgeusia.
View Article and Find Full Text PDFInt Forum Allergy Rhinol
January 2025
Section of Otorhinolaryngology-Head and Neck Surgery, Department of Neuroscience, University of Padova, Padova, Italy.
Introduction: Olfactory neuroblastoma (ONB) is a rare malignant tumor originating from the olfactory neuroepithelium, typically within the sinonasal cavity. Cases of ONB originating outside of the olfactory cleft area are extremely rare and are referred to as "ectopic" (eONB), in contrast to "orthotopic" tumors (oONB). ONB has been associated with paraneoplastic syndromes (PNSs), including the syndrome of inappropriate antidiuretic hormone secretion (SIADH).
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