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Clinical Problem Solving: Pathology
July 2005

Pathology Quiz Case 2: Diagnosis

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2005;131(7):644-645. doi:10.1001/archotol.131.7.644-b

Diagnosis: Esthesioneuroblastoma (ENB), grade III/IV

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare, malignant, neuroepithelial tumor that arises from the olfactory epithelium lining the cribriform plate and upper third of nasal cavity. Esthesioneuroblastoma, which was first described by Berger et al1 in 1924, accounts for 3% of malignant neoplasms of the nasal cavity.1 It has a bimodal age distribution, with an initial peak between the ages of 11 and 20 years and a later peak between the ages of 51 and 60 years,2 and a slight male predominance.3 The tumor can spread submucosally within the sinonasal cavity, cross the cribriform plate, and invade the meninges and brain. It can metastasize by blood, lymph, and cerebrospinal fluid. Metastasis occurs in 10% to 30% of cases.4 Cervical lymph nodes are the most common site of metastatic spread. Other less common sites include the lungs and pleura, brain, bone, spinal cord, breasts, and abdominal viscera.