Olfactory neuroblastoma: a review and update

Area of Science:

• Pathology
• Oncology
• Surgical Pathology

Background:

• Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor originating in the nasal cavity.
• ONB exhibits characteristic nesting, stippled nuclei, neurofibrillary stroma, and pseudorosette formation.
• Distinctive immunoprofile includes keratin negativity, neuroendocrine marker positivity, and S100+ sustentacular cells.

Purpose of the Study:

• To review the typical clinicopathologic features of ONB.
• To highlight the diagnostic challenges posed by ONB's variability and mimicry.
• To discuss recent findings, including molecular genetics, and the differential diagnosis of ONB.

Main Methods:

• Review of clinicopathologic features of Olfactory neuroblastoma (ONB).
• Analysis of diagnostic variability, including immunophenotypic diversity and histological spectrum.
• Focus on differential diagnosis and comparison with mimicking tumors.
• Inclusion of recent findings and molecular genetics where applicable.

Main Results:

• ONB presents with characteristic but variable features, potentially causing diagnostic difficulty.
• Immunophenotypic diversity, patchy staining, and histological spectrum contribute to diagnostic challenges.
• Morphologic overlap with other tumors, including low- and high-grade neoplasms, complicates the differential diagnosis.
• Mimicking tumors also exhibit significant variability.

Conclusions:

• Accurate diagnosis of ONB is crucial due to treatment and prognostic implications.
• Understanding ONB's variability and potential mimics is essential for correct diagnosis.
• Further research into molecular genetics may aid in differentiating ONB and its mimics.