Esthesioneuroblastoma

Estesioneuroblastoma

CLINICAL/SCIENTIFIC NOTE

Esthesioneuroblastoma

Estesioneuroblastoma

Atul Kumar Taneja^I; Fabiano Reis^II; Luciano Souza de Queiroz^III; Verônica de Araújo Zanardi^II

^IResident, Department of Radiology, Clinics Hospital (HC), Medical Sciences College (FCM), State University of Campinas (UNICAMP) Campinas SP, Brazil

^IIProfessor, Department of Radiology, Clinics Hospital (HC), Medical Sciences College (FCM), State University of Campinas (UNICAMP) Campinas SP, Brazil

^IIIProfessor, Department of Pathology, Clinics Hospital (HC), Medical Sciences College (FCM), State University of Campinas (UNICAMP) Campinas SP, Brazil

Esthesioneuroblastoma (ENB) is a rare tumor that usually occurs in young men, with a second peak at the age of 50-60 years¹. Characteristic imaging features include origin from olfactory epithelium high in the nasal cavity and contrast enhancement either by computed tomography (CT) or magnetic resonance imaging (MRI)^2,3.

We report a patient with persistent nasal obstruction and epistaxis, submitted to biopsy and endoscopic resection of an esthesioneuroblastoma with extension to ethmoidal air cells.

CASE

This 40-year-old male, a former smoker, complained of bilateral nasal obstruction worse on the right side for about two years. This was accompanied by a clear, but sometimes bloody discharge, decreased sense of smell, pain and a feeling of pressure in the right eye with lachrymation. A polypoid mass was found which occupied the right nasal vestibule, shifted the nasal septum contralaterally and was covered with reddish mucoid exudate.

Computerized tomography (CT) and magnetic resonance imaging (MRI) disclosed a solid mass filling the right maxillary sinus and right nasal cavity, with areas suggestive of bone erosion and proptosis of the right eye. On MRI, the tumor measured 9.5 × 4.5 × 5.4 cm was isointense on T1 and T2 weighted images and enhanced diffusely and heterogeneously after contrast; a superior extension of the mass occupied part of the ethmoidal air cells (Fig 1). Other paranasal sinuses contained hydrated, contrast enhancing material interpreted as of inflammatory nature.

A biopsy taken from the lesion yielded the diagnosis of esthesioneuroblastoma by conventional hematoxylin-eosin (HE) stained sections, immunohistochemistry and electron microscopy (Fig 2).

The tumor was completely excised by transnasal approach and the patient referred to radiotherapy.

DISCUSSION

Olfactory neuroblastoma (also known as esthesioneuroblastoma), first described by Berger and Luc in 1924⁴, is rare, accounting for 3% of all intranasal tumors¹. It is usually found in young men, with a secondary peak at the age of 50-60 years⁵. Our patient was 40 years old.

ENB should be suspected in patients with persistent unilateral nasal obstruction and recurrent epistaxis. The latter reflects the marked vascularity of olfactory neuroblastomas. Penetration into the cribriform plate can cause anosmia. Orbital extension may induce pain, proptosis and excessive lachrymation. Almost all of these symptoms, which are non-specific, were found in our case. Ear pain and otitis media can result from the tumor obstructing the eustachian tube. Frontal headache suggests involvement of the frontal sinus. Other cranial nerves may also be affected. Patients tend to present with advanced stage disease because of the nonspecificity of symptoms and signs².

ENB arise in the upper nasal cavity, possibly from basal olfactory epithelial stem cells. This hypothesis is supported by their characteristic histology which includes Homer Wright pseudorosettes, and immunohistochemistry positive for cells of neuronal lineage. The most useful of these is synaptophysin, a protein found in synaptic vesicles, which is considered more specific, although less sensitive than other markers such as neuron specific enolase (NSE) and CD56. Neurofilament protein, though highly specific, often fails to react, as it is usually found only in well differentiated neurons. The panel should include antibodies to cytokeratin (AE1AE3, CK7, 34βE12, 35βH11) to help in the differential diagnosis with nasopharyngeal carcinoma, another small round blue cell malignancy common at this location. S-100 protein is helpful as it identifies sustentacular cells in better differentiated tumors with paragangliomatous features. The Hyams grading system is based on preservation of lobular architecture, mitotic index, nuclear polymorphism, fibrillary matrix, rosettes and necrosis⁶. Almost all these features were found in our specimen.

The first clinical staging system was proposed by Kadish et al. in 1976⁷, and still is an important prognosis predictor⁸. Survival for stage A and B tumors is usually excellent. Tumor extension to orbits or through the cribriform plate has significant prognostic implications². Morita⁹ proposed a revised Kadish staging, with stage C consisting of local disease extending beyond the paranasal sinuses and stage D consisting of cervical or distant metastases. Both Biller¹⁰ and Dulguerov¹¹ have proposed systems based on TNM staging, but neither has been widely accepted, as there is no evidence that they provide better prognostic differentiation than the Kadish system⁶. Our case fits into the Kadish B stage, with extension to ethmoidal air cells, therefore carrying a good prognosis.

The radiologic appearance of ENB has been well described. CT and MRI are complementary examinations used for initial diagnosis, staging and follow-up. The MRI appearance is often that of a large, soft tissue, dumbbell-shaped mass centered within the superior nasal cavity and extending intracranially. The tumor is generally hypointense on T1-weighted images and iso- to hyperintense on proton density and T2-weighted images. Contrast enhancement is usually mild to marked and can be uniform or mildly heterogenous. These MR findings are however relatively non-specific. The present case showed a slightly different situation, with the mass being isointense on T1 despite the more usual finding of a hypointense tumor. Som et al. showed that cystic components at the intracranial margins of the tumor may be highly suggestive of ENB³. MRI is also important for staging, as it exhibits the extent of soft tissue invasion with involvement of local structures, such as orbits and sinuses. On CT, the tumor is iso- to slightly hyperdense to muscle and enhances homogenously. CT is most useful in demonstrating bone destruction. In addition, calcifications within the mass are reported to be a relatively specific diagnostic indicator of esthesioneuroblastoma. However, with erosion of the surrounding bone structures by the mass, it is difficult to distinguish true tumoral calcification from residual bone¹. The use of both modalities is critical for discriminating between postobstructive secretion and tumor tissue⁵. In the case here reported, postobstructive secretions showed a markedly hyperintense signal on T2WI, thus differentiating them from the isointense tumor in the same images.

Thus, olfactory neuroblastomas can be detected, delineated and its characteristics suspected by CT and MRI, but definite diagnosis however is still based on histopathology².

Multi-modality treatment involving surgery, chemotherapy and radiotherapy appears highly effective in preventing relapse in advanced ENB. The preferred surgical approach is cranio-facial resection, which was chosen in the patient we report, followed by radiotherapy. Large tumors are considered for pre-operative chemotherapy and post-operative radiotherapy⁸.

In summary, olfactory neuroblastoma is an uncommon tumor of the superior nasal cavity that should be considered as a diagnostic possibility of a mass in this location demonstrating both expansible and destructive growth proprieties. Both CT and MRI are useful, each with its own characteristics, to stage tumor extent accurately and indentify extranasal tumor invasion, which is fundamental to operative planning.

Received 10 November 2008, received in final form 28 April 2009. Accepted 4 June 2009.

Dr. Atul Kumar Taneja - Departamento de Radiologia FCM/UNICAMP - Caixa Postal 6111 - 13083-970 Campinas SP - Brasil. E-mail: [e-mail]

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Publication Dates

• Publication in this collection
  21 Aug 2009
• Date of issue
  Sept 2009