Background: The optimal chemotherapy regimen for the treatment of olfactory neuroblastoma has not been clearly defined. The purpose of the current study was to evaluate the efficacy of neoadjuvant chemotherapy with the combination of etoposide, ifosfamide, and cisplatin (VIP) for patients with olfactory neuroblastoma.
Methods: Eleven consecutive patients with newly diagnosed olfactory neuroblastoma were treated with etoposide (75 mg/m2), ifosfamide (1000 mg/m2), and cisplatin (20 mg/m2) all administered intravenously on Days 1-5. Cycles were repeated every 21 days. Patients were excluded from analysis if they had previously received surgery or radiotherapy.
Results: Nine patients achieved objective responses (objective response rate, 82%; 95% confidence interval, 52-95%), which included 2 complete responses and 7 partial responses. The major side effect was hematologic toxicity, with Grade 3/4 neutropenia observed after the receipt of 37% of all cycles and febrile neutropenia observed after the receipt of 2 cycles. All toxic events were reversible, and no chemotherapy-related deaths were documented. The median survival period was 18 months (range, 3-45 months).
Conclusions: Neoadjuvant VIP chemotherapy was active in the treatment of olfactory neuroblastoma.
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http://dx.doi.org/10.1002/cncr.20648 | DOI Listing |
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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