Introduction: Olfactory carcinoma (OC) is a rare tumor that is often misdiagnosed as olfactory neuroblastoma (ONB). Due to its rarity, there is no established standard treatment for OC, and there have been no reports of successful long-term follow-up treatment yet.
Case Presentation: Two male patients, aged 54 and 48 years, presented with similar symptoms of nasal obstruction, anosmia, facial swelling, and epistaxis. Imaging in both cases revealed large, locally advanced sinonasal neoplasms. Initial biopsies were concerning for ONB; however, final pathological analyses confirmed high-grade OC. Treatment comprised induction chemotherapy, extensive skull base tumor resection, adjuvant chemoradiotherapy, and reconstructive surgery in both cases. The 54-year-old remains disease-free at 7 years, while the 48-year-old demonstrates no evidence of disease at 8 months.
Conclusion: Accurate diagnostic differentiation is imperative to optimize a treatment approach. Although outcomes with OC tend to be worse than those of ONB, effective control can be achieved with judicious concurrent use of neoadjuvant, surgery, and adjuvant treatments. Ongoing surveillance, biobanking, and genomic analysis are crucial to gain a better understanding of this complex disease.
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http://dx.doi.org/10.1159/000541170 | DOI Listing |
Cancers (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 PDFMod Pathol
November 2024
Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, New York. Electronic address:
The diagnosis and treatment of sinonasal small round epithelial/neuroepithelial malignancies depend on the expression of conventional neuroendocrine markers (NEMs), such as synaptophysin, chromogranin A, INSM1, and CD56/NCAM1. However, these tumors remain diagnostically challenging because of overlapping histologic and immunohistochemical features. The transcriptional regulators ASCL1, NEUROD1, POU2F3, and YAP1 are novel NEM (nNEM) used for the subtyping of small-cell lung cancer (SCLC).
View Article and Find Full Text PDFZhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
November 2024
Department of Otolaryngology Head and Neck Surgery, Shanghai Jiao Tong University School of Medicine Affiliated Sixth People's Hospital, Shanghai200233, China.
To study the feasibility and efficacy of pericranial flaps for the repairs of large anterior skull base defects. The average length of the pericranial flaps needed for skull base repair was determined with computed tomography measurements in 20 adults and anatomical dissections in 5 cadaver specimen. A series of patients who underwent endoscopic skull base surgeries and subsequent reconstructions with pericranial flaps at the Department of Otolaryngology Head and Neck Surgery, Shanghai Jiao Tong University School of Medicine Affiliated Sixth People's Hospital from 2016 to 2022 were retrospectively reviewed.
View Article and Find Full Text PDFSurg Pathol Clin
December 2024
Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA; Department of Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address:
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