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Year : 2002  |  Volume : 12  |  Issue : 2  |  Page : 205-206
Case report : Marginal cysts-a diagnostic criterion in intracranial extension of esthesioneuroblastoma

Department of Radiodiagnosis, Jipmer, Pondicherry-605006, India

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Keywords: Esthesioneuroblastoma (ENB), marginal cysts

How to cite this article:
Elangovan S, Bhuvaneswari V. Case report : Marginal cysts-a diagnostic criterion in intracranial extension of esthesioneuroblastoma. Indian J Radiol Imaging 2002;12:205-6

How to cite this URL:
Elangovan S, Bhuvaneswari V. Case report : Marginal cysts-a diagnostic criterion in intracranial extension of esthesioneuroblastoma. Indian J Radiol Imaging [serial online] 2002 [cited 2020 May 30];12:205-6. Available from:

   Introduction Top

Esthesioneuroblastoma (ENB) is a specific type of malignant neuroectodermal tumour that arises from the bipolar sensory receptor cells [1] in the olfactory mucosa and rarely in the region of the middle turbinates and adjacent paranasal sinuses [2]. Berger ad co-workers first described ENB in 1924[3]. To date, barely 200 cases have been recorded in the World literature. We present a case of esthesioneuroblastoma with intracranial extension and marginal cysts. These can be considered a diagnostic criterion.

   Case Report Top

A 52 year old man with left nasal obstruction and proptosis of the left eye attended our Institute. There was a history of one episode of bleeding per nostril. The patient also complained of occasional headache. On clinical examination there was an irregular large mass in the nostrils completely obstructing the nasal cavity. The mass was partially necrotic and insensitive to touch. Ophthalmic examination revealed proptosis of left eye with total loss of vision. A computed tomography (CT) examination was performed; axial and coronal 5mm section images of the paranasal sinuses were obtained using a Somatom-Plus machine (Siemens, Germany).

A large mass occupying the paranasal sinuses and nasal cavity extending into the anterior cranial fossa, was demonstrated. The tumour had eroded into the frontal and ethmoid sinuses which were obstructed. Similarly the sphenoid sinus was also destroyed by the tumour. There was extension of the tumour into both orbits.

The intracranial component of the mass was seen on either side of falx cerebri with multiple large peripheral cysts. Fluid-fluid levels were also seen in these cysts. The radiological diagnosis of ENB, based on the presence of these marginal cysts, was confirmed by biopsy

   Discussion Top

There is a constant desire on the part of both radiologists and clinicians to predict a histologic diagnosis based on sectional imaging findings. This is especially true with regard to malignancies [4],[5]. Very few tumours have unique radiologic features from which a prediction of the histologic diagnosis can be made with confidence.

Esthesioneuroblastomas generally demonstrate a fairly consistent pattern in CT. They are usually iso to hyperdense with moderate contrast enhancement[8]. Calcification has been reported both histologically[9] and radiologically[10]. Bony destruction with extension of the tumour beyond the paranasal sinuses, particularly involving the orbits and the anterior cranial fossa through the cribriform place, is quite common[8]. All the features are not unique to esthesio-neuroblastoma. They can be seen in many of the undifferentiated small cells tumours, for example anaplastic carcinoma, lymphoma, extra medullary plasmacytoma and amelanotic melanoma of the paranasal sinuses. But none of these tumours has been reported to have marginal cysts. Tumoral cysts at the margins of intracranial extension has been reported only in esthesioneuroblastoma [7]. The tissue composing the walls of these marginal cysts are arachnoid and tumor[7]. An arachnoid cyst associated with an acoustic neuroma has been observed[11]. The precise reason for the association of arachnoid cyst adjacent to an extra axial mass is not known. Marginal cyst may be identified with other Multiple tumours; however the only known sinonasal tumour to be associated with such cysts is esthesio-neuroblastoma[7]. Thus the presumptive diagnosis of esthesio-neuroblastoma can be made based on this imaging criterion, with a very high degree of reliability.

   References Top

1.Micheau, C. A new histochemical and biochemical approach to olfactory esthesioneuroma. A nasal tumor of neural crest origin. Cancer 1977, 40:314-318.  Back to cited text no. 1    
2.Shah J. P. Feghali J. Esthesioneuroblastoma. Cancer J. Clin, 1983, 33:154-159.  Back to cited text no. 2    
3.Berger L, Luc, R. L'esthesioneuroepithelioma olifactif. Bull. Assoc. Franc Etude Cancer, 1924, 13: 410-421.  Back to cited text no. 3    
4.Som, P. M, Shapiro, M.D, Biller, H.F. et al . Sinonasal Tumours and Infalmmatory Diseases: Differentiation with M.R. Radiology, 1988, 167: 803-808.  Back to cited text no. 4    
5.Yousem, D.M,Fellows, D.W, Kennedy, D.W. et al . Inverted Papilloma: Evaluation with M.R. imaging. Radiology, 1992, 185: 501-5 05.  Back to cited text no. 5    
6.Peter M. Som, Hugh D. Curtin. The paranasal sinuses Head and Neck Imaging 1996, Mosby, 210-211.  Back to cited text no. 6    
7.Som, P.M. Lidov, M, Brandwin, M. et al . Sinonasal esthesioneuroblastoma with intracranial extension: marginal Tumour cysts as a Diagnostic MR finding. American Journal of Neuroradiology 1994, 15: 1259-1262.  Back to cited text no. 7    
8.Hurst, R. W, Erickson, S, Cair, W.S. et al . Computed tomographic features of esthesioneuroblastoma. Neuro-radiology 1989, 31: 253 - 257.  Back to cited text no. 8    
9.berman, H.A, Rice, D.H, Olfactory neuroblastomas: a clinicopathologic study. Cancer 1976, 38: 2494-2502.  Back to cited text no. 9    
10.Manelfe C, Bonafe A, Fabre P, Pessey J. J. Computed tomography in olfactory neuroblastoma. One case of esthesioneuroepithelioma and four cases of esthesio-neuroblastoma. J. Comput. Assist. Tomogr. 1978, 2:412-420.  Back to cited text no. 10    
11.Hasso A. N. Hinshaw D. B, Kief-Cracia M.L. Neoplasms of the cranial nerves ad skull base In: Stark D. D, Bradley W.G.Jr,eds. Magnetic Resonance Imaging, Vol. I, 2nd ed St. Louis Mosby Year Book 1992, 860 - 863.  Back to cited text no. 11    

Correspondence Address:
S Elangovan
Department of Radiodiagnosis, Jipmer, Pondicherry-605006
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Source of Support: None, Conflict of Interest: None

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