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Case 91

Esthesioneuroblastoma

MRI of a man with nasal obstruction and anosmia

Ozgur Kilickesmez

A 50 year old man was admitted to the otolaryngology service with 1 year history of headaches and 4 month history of nasal obstruction and anosmia. He also had a history of occasional epistaxis. The patient subsequently underwent MRI of the head.

Findings

MRI of the cranium demonstrated obstruction of the nasal cavity, sphenoid and frontal sinuses with a mass. The mass is hypointense on T1 WI, hyperintense on T2 WI and enhanced strongly after contrast injection. There is no evidence of destruction of cribriform plate and extension into cranium. On the last image also strongly enhancing subcortical metastatic nodules in the parietal and occipital lobes are seen, a very rare finding.

Diagnosis

Esthesioneuroblastoma

Differential

Squamous Cell Carcinoma
Undifferentiated Sinonasal Carcinoma
Lymphoma
Malignant Meningioma
Metastasis
Sarcoma

Discussion

Esthesioneuroblastoma (ENB) is an uncommon malignant neoplasm of neural crest origin arising from the olfactory sensory epithelium in the roof of the nasal fossa. Once arisen, ENB tends to spread submucosally in all directions to involve the paranasal sinuses, nasal cavity, and surrounding structures. The tumor is locally aggressive, and local recurrence strikes in up to 68% of cases regardless of initial treatment protocol. ENB also metastasizes widely by both hematogenous and lymphatic routes, with the neck being the most common site. Symptoms are typically present for months, and sometimes for years before presentation. Patients may have had multiple procedures for removal of "polyps". Another common symptom is recurrent epistaxis. Other symptoms include anosmia, facial pain, headache, proptosis, diplopia, syncope, and lethargy. The most common physical sign is a high nasal mass.

References

1. Girod D, Hanna E, Marentette L. Esthesioneuroblastoma. Head Neck. 2001 Jun;23(6):500-5. No abstract available.
2. Simon JH, Zhen W, McCulloch TM, Hoffman HT, Paulino AC, Mayr NA, Buatti JM. Esthesioneuroblastoma: the University of Iowa experience 1978-1998.Laryngoscope. 2001 Mar;111(3):488-93.
3. Derdeyn CP, Moran CJ, Wippold FJ, Chason DP, Koby MB and Rodriquez F. MRI of Esthesioneuroblastoma. J Computed Assisted Tomography 18(1): 16-21.
4. Schuster JJ, Phillips D, Levine PA. MR of Esthesioneuroblastoma (Olfactory Neuroblastoma) and Appearance after Craniofacial Resection. AJNR 15: 1169-1177, Jun 1994.

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