Esthesioneuroblastoma: A rare Presentations with ...

Esthesioneuroblastoma: A rare Presentations with Concha bullosa tumor and seizure 1 1 2 Mohammad Ebrahim Yarmohammadi , Poopak Izadi , Siamak Afshin Majd Iranian Journal of Neurology, Vol.9, No.29-30, Spring & Summer 2010, 686-692

Abstract Introduction: Esthesioneuroblastoma or olfactory neuroblastoma is a rare cancer arising from the neuroepithelium of the olfactory epithelium of the nasal cavity. Esthesioneuroblastoma occurs in all age group with a peak incidence in the age group of 11 to20 years old and again between 51 to 60 years old .Computed tomography (CT) and magnetic resonance imaging (MRI) usually show a homogenous soft tissue mass in the nasal cavity producing some erosion of the lamina papyracea , cribriform plat and fovea ethmoeidalis . The purpose of this paper is to present a 41 year old woman case of a Esthesioneuroblastoma filling the cavity of concha bullosa mimicking mucocele of middle turbinate, treated with endoscopic surgery. The chif complain of this patient was seizure that is very rare manifestation of this tumor. Key words: Esthesioneuroblastoma, Concha bullosa, seizure

1 2

Department of Ear, Nose and Throat, School of Medicine, Shahed University, Tehran, Iran. Department of neurology School of Medicine, Shahed University, Tehran,

687/ Iran. J. Neurol. Vol.9; No. 29-30, Spring & Summer2010

Introduction Esthesioneuroblastoma or olfactory neuroblastoma is a rare cancer arising from the neuroepithelium of the olfactory epithelium of the nasal cavity.(1) Sinusal, orbital and intracranial expansions are common. The most common presenting symptoms are nasal obstruction, epistaxis, and persistent nasal discharge; less common presenting symptoms include sinus pain, visual changes, headache, proptosis, diplopia, hyposmia, anosmia, facial pain, facial swelling, and syncope. Symptoms may be present for years before the patient seeks medical advice. An esthesioneuroblastoma very rarely manifests with acute neurological deterioration.(2,3) They are often discovered incidentally during septoplasty or polypectomy.(4) Esthesioneuroblastoma occurs in all age group with a peak incidence in the age group of 11 to20 years old and again between 51 to 60 years old.(5)Computed tomography (CT) and magnetic resonance imaging (MRI) usually show a homogenous soft tissue mass in the nasal cavity producing some erosion of the lamina papyracea , cribriform plat and fovea ethmoeidalis.(6) Treatment will generally associate debulking or curative surgery (with combined rhinoneurosurgical accesses) and conformal stereo-tactic radiotherapy.(3) Over the last two decades there has been increasing evidence that proton beam

radiation may offer superior results to standard external beam radiation for Esthesioneuroblastoma of the skull base(7,8) In experienced hands and carefully selected patients, endoscopic resection of esthesioneuroblastoma respects the principles of oncologic surgery, providing an adequate exposure for margin assessment as well as reliable reconstruction of the anterior skull defect with a relatively low morbidity.(9,10) The purpose of this paper is to present a case of a Esthesioneuroblastoma filling the cavity of concha bullosa mimicking mucocell of middle turbinate and The chif complain of this patient was seizure that is very rarely manifestion of this tumor. Case report A 41 year old woman was admitted to our hospital in neurologic service with symptom of true seizure . She had history of nasal obstruction and nasal stuffiness from 10 years ago without any symptoms of nasal bleeding or drug consumption. Family history was negative for seizure. The seizure was controlled with Carbamazpine200 mg three times daily. Right middle turbinate was hyperthrophic In the anterior rhinoscopy with normal anterior surface mucosa. Brian parenchyma was normal in CTscan and MRI. Right middle turbinate mass mimikig the mucocel of

688/ Esthesioneuroblastoma: A rare Presentations with Concha bullosa tumor and seizure

concha bullosa was seen in both brain CT scan and MRI.Erosion of lamina papyracea ,cribriform plate, fovea

etmoeidalis and interacranial extention were not seen (graphy1,2).

Graphy 1 preoperation CTscan


Graphy 2 preoperation CTscan

Endoscopic surgery was performed .After incision of vertical and horizontal attachment of middle turbinate the mass was removed. During endoscopic surgery CSF leakage was seen in the site of vertical attachment of middle turbinate. CSF leakage site repair with four layers (bone, fat, muscles and surgecel) endoscopically.

On immunohistochemistry analysis the tumor was identified as an Esthesioneuroblastoma. postoperative radiothrapy added to treatment.The patient have not any sign of relapse in MRI and endoscopic biopsy after two years(graphy3,4).


Graphy 3 post operation CTscan


Graphy 4 post operative MRI

Discussion The most common symptoms of, Esthesioneuroblastoma are nasal obstruction, epistaxis and headache .Because most of this symptom are similar to those of benign nasal disease ,Esthesioneuroblastoma is often missed during early stages.(2) Olfactory neuroblastomas usually arise in the cribriform plate and superior turbinate. However, the origin and isolation of olfactory neuroblastomas to the sphenoid sinus is exceedingly rare. Only four cases of olfactory

neuroblastoma isolated in the osseous margine of esphenoid sinus have been described in literature . In esthesioneuroblastoma frequency of presenting symptoms with cranial neuropathies and acute neurological deterioration is rare.(11) The chief complain of my patient was seizure and tumor mimicking mucocel of middle turbinate .The Esthesioneuroblastoma in this reported case was located in bullosa portion and surrounding with bone of middle turbinate .


Ectopic ACTH syndrome due to esthesioneuroblastoma is extremely uncommon with only five other cases being discussed in the literature.(12) Esthesioneuroblastoma is a rare malignant tumour and its presentation with ophthalmologic symptoms and signs is very infrequent.(13) In animals Neoplasia of the nasal cavity should be considered in the differential diagnosis of seizure.(14) We recommended that nasal cavity tumor must be considered in the differential diagnosis of patients with seizure .

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