Endoscopic view of an esthesioneuroblastoma that resembles a benign polyp.

A 62-year-old man presented with a 1-year history of left nasal obstruction and intermittent anosmia and bloody discharge. Nasal examination revealed that several large vascular polypoid masses had filled the left nasal airway (figure, A). Some edema was also noted medial to the left eye. Computed tomography (CT) of the sinuses demonstrated extensive opacification of the left frontal, ethmoid, and maxillary sinuses (figure, B).

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The patient underwent left endoscopic sinusotomies and biopsy with a microdebrider, and all gross polypoid tissue was completely removed. Two weeks postoperatively, the middle meatus, ethmoid sinus, and frontal recess appeared to be free of tumor (figure, C). However, the final pathologic diagnosis 2 weeks later indicated that the polypoid mass was an esthesioneuroblastoma (figure, D). The patient was referred to a tertiary care center for further evaluation and treatment. He was found to have an intracranial tumor invasion of several millimeters. The patient elected to undergo craniofacial resection and radiation therapy. At the 1-year follow-up, he was doing well.

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Esthesioneuroblastoma is an uncommon upper nasal cavity malignant tumor that arises from the olfactory neuroepithelium. It was first described in 1924 by Frenchmen Berger and Luc, who called it esthesioneuroepitheliome olfactif. (1) Esthesioneuroblastoma, also known as olfactory neuroblastoma, (2-4) has been confused with other tumors, such as inverting papilloma or benign nasal polyps.

Kadish et al suggested that the tumor could be staged on clinical grounds. (4) Their suggested staging system:

* Group A--tumor is confined to the nasal cavity.

* Group B--tumor involves the nasal cavity and paranasal sinuses.

* Group C--tumor has spread beyond the nasal cavity and sinuses.

In retrospect, the tumor in our patient fell into group C.

The diagnosis of esthesioneuroblastoma is made by histopathologic examination. This malignancy is still considered rare, although as of 1997, more that 1,400 cases had been reported in the literature. (5,6)

Clinically, patients often present with symptoms of unilateral nasal obstruction, anosmia, and epistaxis. (2-4) Local invasion can lead to orbital signs and symptoms such as proptosis or double vision. Sinusitis frequently occurs secondary to nasal obstruction. CT usually shows a unilateral intranasal and paranasal soft-tissue mass with or without erosion of the bone of the ethmoid roof (the cribriform plate), the orbit, and the antral walls. (4) Intranasal examination usually detects an obstructive vascular soft-tissue

mass that is often assumed to be polypoid tissue. Because most polypectomies today are performed with a microdebrider, we might forget to take a biopsy. This is unfortunate, because it is extremely important to obtain a tissue diagnosis, especially in patients with unilateral soft-tissue disease. Endoscopic biopsy and tissue evaluation confirm the diagnosis.

Patients with esthesioneuroblastoma can be treated with various combinations of surgery, radiotherapy, and chemotherapy; treatment philosophies vary among institutions. Newer craniofacial surgical techniques combined with radiotherapy have significantly improved disease-free long-term survival rates. (3,5,7,8)

We cannot presume that all polypoid masses in the nasal cavity or sinuses are benign. We must always entertain the possibility of an esthesioneuroblastoma.

References

(1.) Berger L, Luc R. L'esthesioneuroepitheliome olfactif. Bull de l'Assoc Franc pour l'Etude Cancer (Paris) 1924;13:410-20.

(2.) Harrison DF. Unusual rumor. In: Myers EN, Suen IY, eds. Cancer of the Head and Neck. 2nd ed. New York: Churchill Livingston, 1989.

(3.) Lund V J, Howard D, Wei W, Spittle M. Olfactory neuroblastoma: Past, present and future? Laryngoscope 2003;113:502-7.

(4.) Kadish S, Goodman M, Wang CC. Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer 1976;37:1571-6.

(5.) Broich G, Pagliari A, Ottaviani F. Esthesioneuroblastoma: A general review of the cases published since the discovery of the tumour in 1924. Anticancer Res 1997;17:2683-2706.

(6.) Argiris A, Dutra J, Tseke P, Haines K. Esthesioneuroblastoma: The Northwestern University experience. Laryngoscope 2003; 113: 155-60.

(7.) Levine PA, Gallagher R, Cantrell RW. Esthesioneuroblastoma: Reflections of a 21-year experience. Laryngoscope 1999;109: 1539-43.

(8.) Constantinidis J, Steinhart H, Koch M, et al. Olfactory neuroblastoma: The University of Erlangen-Nuremberg experience 1975-2000. Otolaryngol Head Neck Surg 2004;130:567-74.

From the Department of Otolaryngology, University of South Florida College of Medicine, Tampa, and the Halifax Medical Center, Daytona Beach, Fla. (Dr. Mirante and Dr. Christmas); and the Southern New England Ear, Nose, Throat, and Facial Plastic Surgery Group; the Section of Otolaryngology, Hospital of St. Raphael; and the Section of Otolaryngology, Yale University School of Medicine, New Haven, Conn. (Dr. Yanagisawa).