de Queiroz, LS

Reis, F

Taneja, AK

Zanardi, VD

Arquivos de Neuro-Psiquiatria

Repositorio da Producao Cientifica e Intelectual da Unicamp

Arq Neuropsiquiatr 2009;67(3-A):704-706
704
Clinical / Scientific note
ESTHESIONEUROBLASTOMA
Atul Kumar Taneja1, Fabiano Reis2, Luciano Souza de Queiroz3, Verônica de Araújo Zanardi2
ESTESIONEUROBLASTOMA
Clinics Hospital (HC), Medical Sciences College (FCM), State University of Campinas (UNICAMP) Campinas SP, Brazil: 1Resident, Department of Radiol-
ogy; 2Professor, Department of Radiology; 3Professor, Department of Pathology.
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: atultaneja@gmail.com
Esthesioneuroblastoma (ENB) is a rare tumor that usu-
ally occurs in young men, with a second peak at the age of 
50-60 years1. Characteristic imaging features include ori-
gin 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 resec-
tion of an esthesioneuroblastoma with extension to eth-
moidal air cells.
CASE
This 40-year-old male, a former smoker, complained of bi-
lateral 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 pres-
sure in the right eye with lachrymation. A polypoid mass was 
found which occupied the right nasal vestibule, shifted the na-
sal septum contralaterally and was covered with reddish mu-
coid exudate. 
Computerized tomography (CT) and magnetic resonance im-
aging (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 en-
hanced 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 en-
hancing material interpreted as of inflammatory nature. 
A biopsy taken from the lesion yielded the diagnosis of es-
thesioneuroblastoma by conventional hematoxylin-eosin (HE) 
stained sections, immunohistochemistry and electron micros-
copy (Fig 2). 
The tumor was completely excised by transnasal approach 
and the patient referred to radiotherapy. 
DISCUSSION
Olfactory neuroblastoma (also known as esthesioneu-
roblastoma), first described by Berger and Luc in 19244, is 
rare, accounting for 3% of all intranasal tumors1. It is usu-
ally 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 neuro-
blastomas. 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 involve-
ment 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 bas-
al olfactory epithelial stem cells. This hypothesis is sup-
ported by their characteristic histology which includes 
Homer Wright pseudorosettes, and immunohistochem-
istry positive for cells of neuronal lineage. The most use-
ful 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 eno-
lase (NSE) and CD56. Neurofilament protein, though high-
ly 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 car-
cinoma, another small round blue cell malignancy com-
mon at this location. S-100 protein is helpful as it iden-
tifies sustentacular cells in better differentiated tumors 
with paragangliomatous features. The Hyams grading sys-
tem is based on preservation of lobular architecture, mi-
totic index, nuclear polymorphism, fibrillary matrix, ro-
settes 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 ex-
cellent. Tumor extension to orbits or through the cribri-
Arq Neuropsiquiatr 2009;67(3-A)
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Estesioneuroblastoma
Taneja et al.
form plate has significant prognostic implications2. Mori-
ta9 proposed a revised Kadish staging, with stage C con-
sisting of local disease extending beyond the paranasal 
sinuses and stage D consisting of cervical or distant me-
tastases. Both Biller10 and Dulguerov11 have proposed sys-
tems 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 eth-
moidal air cells, therefore carrying a good prognosis.
The radiologic appearance of ENB has been well de-
scribed. 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 hy-
pointense on T1-weighted images and iso- to hyperintense 
on proton density and T2-weighted images. Contrast en-
hancement 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 slight-
ly 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 intrac-
ranial 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 ho-
mogenously. CT is most useful in demonstrating bone de-
struction. 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 be-
tween postobstructive secretion and tumor tissue5. In the 
case here reported, postobstructive secretions showed a 
markedly hyperintense signal on T2WI, thus differentiat-
ing them from the isointense tumor in the same images. 
Thus, olfactory neuroblastomas can be detected, delin-
eated and its characteristics suspected by CT and MRI, but 
definite diagnosis however is still based on histopathology2.
Multi-modality treatment involving surgery, chemo-
Fig 1. [A] (T1 - Weighted MRI, Axial) and [B] (T2-WI) - Expransive solid lesion filling the nasal cavity and right maxillary sinus, isointense in T1 WI 
and T2WI. The left maxillary sinus is lined by T2 hipermintense material suggestive of inflammatory sinusopathy. [C] (T1 - Weighted MRI, Cor-
onal) e [D] (after Contrast) - Lesion shows irregular contrast enhancement and extends superiorly into the ethmoidal cells. [E] (T1 - Weighted 
MRI, Sagittal with contrast) - Lesion occupies nasal cavity but appears to have little if any intracranial extension. Frontal and sphenoidal sinus-
es display mucosal thickening and exudate.
Arq Neuropsiquiatr 2009;67(3-A)
706
Estesioneuroblastoma
Taneja et al.
therapy and radiotherapy appears highly effective in pre-
venting relapse in advanced ENB. The preferred surgical 
approach is cranio-facial resection, which was chosen in 
the patient we report, followed by radiotherapy. Large tu-
mors 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 consid-
ered 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 in-
dentify extranasal tumor invasion, which is fundamental 
to operative planning. 
REFERENCES
 1. Schiro BJ, Escott EJ, McHugh JB, Carrau RL. Bone invasion by an es-
thesioneuroblastoma mimicking fibrous dysplasia. Eur J Radiol 2008; 
65:69-72. 
 2. Pickuth D, Heywang-Kobrunner SH, Spielmann RP. Computed tomog-
raphy and magnetic resonance imaging features of olfactory neuroblas-
toma: an analysis of 22 cases. Clin Otolaryngol 1999;24:346-350.
 3. Som PM, Lidov M, Brandwein M, Catalano P, Biller HF. Sinonasal es-
thesioneuroblastoma with intracranial extension: marginal tumor cysts 
as a diagnostic MR finding. Am J Neuroradiol 1994;15:1259-1262. 
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sphenoid esthesioneuroblastoma studied with MR. Clin Imaging 2003; 
27:38-40. 
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a practical guide for differential diagnosis. Radiographics 2008;28:205-223. 
 6. Capelle L, Krawitz H. Esthesioneuroblastoma: a case report of diffuse 
subdural recurrence and review of recently published studies. J Med 
Imaging Radiat Oncol 2008;52:85-90. 
 7. Kadish S, GoodmanM, Wang CC. Olfatory neuroblastoma: a clinical 
analysis of 17 cases. Cancer 1976;37:1571-1576.
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view of sinonasal tumours. Cancer Imaging 2005;5:167-177.
 9. Morita A,Ebersold MJ, Olsen KD, Foote RL, Lewis JE, Quast LM. Esthesion-
euroblastoma prognosis and management. Neurosurgery 1993;32:706-714. 
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gical treatment without radiation. Laryngoscope 1990;100:1199-1201. 
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Fig 2. [A] (Surgical specimen) Elongated mass with smooth extemal surface, showing on section heterogeneous whitish nodules in a brownish 
background. Yellow calcified sports are visible near center. [B] (Hematoxylin and Eosin stained paraffin section): sheets of monotonous-look-
ing cells, featuring rounded nuclei with little atypia and scant cytoplasm. Pink fibrillary areas known as Homer Wrigth rosettes punctuated the 
tumor. [C, D, E] ( Immunohisto-chemistry): rosettes were characteristically positive for Synaptophysin [C], indicating neuronal lineage of neo-
plastic cells. Some cells were labeled for neuron specific enolase [D]. Several cells at the adge of lobules showed nuclear and cytoplasmic pos-
itivity for S-100 protein [E], an appearance similar to sustentacular cells in paragangliomas. [F] (Electron microscopy) disclosed tightly knit cell 
processes reminicent of neuropil, some profiles containing dense core vesicles of secretory type. 


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