|Year : 2013 | Volume
| Issue : 1 | Page : 99-101
Esthesioneuroblastoma arising from the middle meatus
Ashok Kumar1, Bhawna Sethi2, Yogesh Kumar3, Jai Prakash Mishra2
1 Department of ENT, V.C.S.G. Govt. Medical Sciences and Research Institute, Srinagar, Uttarakhand, India
2 Department of Pathology, V.C.S.G. Govt. Medical Sciences and Research Institute, Srinagar, Uttarakhand, India
3 Department of Ophthalmology, V.C.S.G. Govt. Medical Sciences and Research Institute, Srinagar, Uttarakhand, India
|Date of Web Publication||10-Apr-2013|
Department of Pathology, V.C.S.G. Govt. Medical Sciences & Research Institute, Srinagar, Pauri Garhwal, Uttarakhand
Source of Support: None, Conflict of Interest: None
A 35-year-old female presented with 13-year history of unilateral recurrent nasal mass, epistaxis and facial pain. Nasal examination revealed a pale glistening mass in the right nasal cavity. On probing, mass was insensitive to touch and bled on handling. Computed tomographic scan showed a mass filling the right nasal cavity, ipsilateral maxillary and ethmoid sinuses. Diagnosis of pansinusitis polyposis was made.Transnasal endoscopy-assisted excision of the mass was done, and the diagnosis of olfactory neuroblastoma was established by histopathology and confirmed by immunohistochemistry. The mass was classified as a Kadish stage B tumor. Further intervention including medial maxillectomyand ethmoidectomy, and complete endoscopic-resection of the tumor from cribriform plate was done via lateral rhinotomy approach. The tumor was found adhered to the lateral wall-the middle meatus and was easily peeled away from the cribriform plate and ethmoids. Patient was referred for radiotherapy. No evidence of loco-regional recurrence or systemic metastasis observed at 10-month follow-up.
Keywords: Epistaxis, esthesioneuroblastoma, olfactory neuroblastoma, polyposis
|How to cite this article:|
Kumar A, Sethi B, Kumar Y, Mishra JP. Esthesioneuroblastoma arising from the middle meatus
. J Can Res Ther 2013;9:99-101
| > Introduction|| |
Esthesioneuroblastoma/olfactory neuroblastoma (ONB), first described by Berger and Luc,  represents 2-3% of sinonasal tract tumors,  and remains neoplasm with controversies regarding origin, biological activity, staging and management. It exhibits varying biological activity, ranging from an indolent growth to a highly aggressive neoplasm capable of widespread metastasis. Histologically, it is confused with other undifferentiated neoplasms of nasal cavity. Treatment ranges minimally invasive approachto craniofacial resection with radiotherapy.
| > Case Report|| |
A 35-year-old female presented with 13-year history of unilateral recurrent nasal mass, progressive nasal obstruction, epistaxis, difficult nasal-breathing, facial pain and blood-tinged discharge. Nasal examination revealed a pale glistening mass in right nasal cavity. On probing, mass was insensitive to touch, bled on handling. Septum deviated to left, nasal-patency test reduced bilateraly, andolfactory test (subjective qualitative clinical examination based upon comparative analysis using known odors, e.g., clove oil, camphor, coffee powder) revealedrelative hyposmia on right side. Visual acuity/field of vision/eye movements and rest of the head-neck examination were normal.
Computed tomography (CT) revealed mass in right nasal cavity with extensions into ipsilateral maxillary sinus, anterior and posterior ethmoids, and raised suspicion of origin from the lateral wall. Intracranial/intraorbital/nasopharygeal extensions and erosions into wall of sinuses or cribriform plate were not seen [Figure 1].
|Figure 1: (a) Polpoid glistening mass in right middle meatus on endoscopy, S denotes septum, MT denotes middle turbinate, MM denotes middle meatus and M denotes Mass. (b) CT Scan depicting soft tissue density in right nasal cavity, maxillary and ethmoid sinus|
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Patient underwent five transnasal-excisions elsewhere, but no records are available except one with diagnosis of benign nasal polyp. Recurrence was seen 5.5-12 months (mean recurrence time being 7.4 months) after each surgery, because of incomplete removal of mass and lack of postoperative radiotherapy, or simultaneous presence of polyp and tumor.
Endoscopy-assisted transnasal excision was performed. Histologically, tumor was identified as olfactory neuroblastoma and confirmed by immunohistochemistry [Figure 2]. Further intervention including medial maxillectomy, ethmoidectomy,and the complete endoscopic-resection of the tumor from cribriform plate was done via lateral rhinotomy approach. Tumor was found adhered to lateral wall-the middle meatus. The postoperative period was uneventful except for facial swelling, recurrent epistaxis and blood-tinged discharge for about 2 weeks. Postoperative nasal endoscopy and CT scan revealed complete removal of the mass. Three weeks after surgery, patient received radiotherapy without significant side effects. At 10-month follow-up, there was no evidence of loco-regional recurrence or systemic metastases, and has been advised regular follow-ups.
|Figure 2: (a) lobules of tumor cells (H&E, × 40). (b) Tumor cells with focal neurofibrillary material and rosettes (H&E, ×100). (c) Slightly pleomorphic cells arranged in the form of rosettes (H&E, × 400). (d) positiveimmunostaining for NSE (++)|
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| > Discussion|| |
Sinonasal neoplasms comprise less than 1% of all malignancies. Despite their infrequency, they represent a diagnostic challenge because the presenting signs symptoms may be indistinguishable from benign or inflammatory disorders. Although certain findings (cranial neuropathies/facial swelling/epistaxis/evidence of metastasis) favor malignancy, the presentation may be nonspecific, as in this case. Endoscopy-assisted transnasal resection was done, and mass was removed in fragments. Grossly, it measured 5.5 × 4 × 3.5 cm, was polypoid, multilobulated, soft to firm, glistening, pinktopale, with reddish brown-pale cut surface, and areas of hemorrhage and necrosis.
Typical histopathological findings include small uniform cells separated into nests/components by fibrovascularseptae, neurofibrillary intercellular matrices, and rosettes, but findings may vary according to the differentiation of tumor cells. This mass revealed benign polypous changes with a large portion in the submucosa revealing features of olfactory neurogenic tumor [Figure 2]: cellular neoplasm, composed of small-intermediate, slightly pleomorphic cells with round to few ovoid/spindled vesicular nuclei, inconspicous-to-single nucleoli, increased mitosis, scant-moderate acidophilic cytoplasm, and neurofibrillary cytoplasmic material present focally. Tumor-cells arranged in lobules, intervened by scanty fibrovascularstroma with marked proliferation of capillaries. Scattered Homer-Wright rosettes, areas of hemorrhage, necrosis, and specks of calcifications noticed. Tumor graded grade-III (Hyams system). 
To confirm and rule out the differentials; lymphoma, malignant melanoma, neuroendocrine carcinoma, undifferentiated sinonasal carcinoma, and metastatic carcinoma, immunohistochemistry was done.
The neoplastic cells were found immunoreactive for: neuron-specific enolase(2+), synaptophysin(1+), neurofilament(1+), and cytokeratin(1+);score being lower due to higher tumor grade. S 100, LCA and desmin were negative.
Esthesioneuroblastoma usually arises from the olfactory neuroepithelium. Incidence peaks bimodally in 11-20 and 50-60 years; however, age may range 3-88 years with no sex predilection/slight female-predominance. Usually unilateral, may be bilateral in neglected cases. Local recurrence (up to 57%) and metastasis (20-60%) reported. Distant metastasis occurs to cervical-lymph node (commonest), parotid glands, skin, lungs, bone, liver, orbit and spinal cord.  This female had unilateral, recurrent nasal mass, noticed at the age of 22, reappearing 5.5 months−a year after excisions.
Main symptoms include nasal occlusion, proptosis, epistaxis, headache, excessive lacrimation, anosmia and visual disturbances. In this case, there was no intraorbital/intracranial extension of the mass, hence patient was not complaining of orbital/visual disturbances/headache.
The tumor was staged Kadish stage-B.  Keeping in mind the extensions of mass and probable site of origin, medial maxillectomy, ethmoidectomy, and complete endoscopic-resection of the tumor from cribriform plate and posterior ethmoids was done via lateral rhinotomy. The tumor was adhered to lateral wall-the middle meatus, and was easily peeled away from cribriform plate and ethmoids. Histopathological examination revealed benign poplypous changes from portions of mass within maxillary sinus and roof-area, while features of ONB in remaining portions. Soft and bony margins were free from tumor infiltration.
Primary sites include superior nasal cavity/nasal septum, turbinates, ethmoids, or cribriform plate, although cases arising from nasopharynx, inferior meatus, maxillary sinus, and extra-nasal site have been reported.  Cell of origin is controversial; vomeronasal organ of Jackobson, sphenopalatine ganglion, ectodermal olfactory placode, autonomic ganglia, and the olfactory epithelium.  In our case, peroperatively and histopathologically, the mass was found originating from middle meatus. Several hypotheses can be postulated for this; origin from ectopic cell-rests of vomeronasal nerve, persistent terminal ganglion cells in middle meatus, sympathetic/parasympathetic autonomic ganglion in mucosa, or undiscovered primary tumor with multicentric ONB. However, tumor cells were not discernible except the middle meatus' fragments and, even if the primary tumor had existed in other regions, it cannot be removed completely or regress without proper surgery and/or radiotherapy. All this suggest that ONB in this case is the primary tumor originating from the middle meatus.
Clinical stage, histological differentiation and safe margins are important prognostic predictors. This case, staged B, has intermediate 5-year prognosis (70.9%),  but poorer prognosis (25%) is expected due to high histological grade (Hyams grade-III). 
Although, chemotherapy has been introduced, surgical resection combined with postoperative radiotherapy is still considered gold standard. Results of transnasal endoscopic-resection with radiation are comparable to those of craniofacial resection.  Recent series have focused on the role of endoscopic resections.
Patient was referred for radiotherapy and has been called for regular follow-ups due to chances of recurrence even after aggressive therapy. No loco-regional recurrence/systemic metastases have been observed at 10-months follow-up.
| > Conclusions|| |
ONB is difficult to diagnose, recurrent, malignant, slow-growing tumor which can mimic benign polyp. So, even routine polypectomy requires histological examination. This report has a value as the first case of primary ONB from the middle meatus.
| > References|| |
|1.||Berger L, Luc H, Richard RL. Esthesioneuroepitheliomeolfactif. Bull Cancer (Paris)1924;13:1410-21. |
|2.||Wenig BM. Tumors of the upper repiratorytract : Nasal cavity, Paranasal sinuses and Nasopharynx. In: Fletcher CD, editor. Diagnostic histopathology of tumors. 3 rd ed. Churchill livingstone, Elsevier; 2007.p. 112-8. |
|3.||Hyams VJ. Tumors of the upper respiratory Tract and ear. In: Hyams VJ, Batsakis JG, Michaels L, editors. Atlas of tumor pathology. 2 nd series. fascicle 25. Washington, D C: Armed Forces Institute of pathology; 1988. p. 240-8. |
|4.||TurakhiaS, Patel K. Esthesioneuroblastoma. Head Neck 2006;16:669-72. |
|5.||LeeJ Y, Kim HK. Primary olfactory neuroblastoma originating from the inferior meatus of nasal cavity.Am J Otolaryngol 2007;28:196-200. |
|6.||Kadish S, Goodman M, Wang CC. Olfactory neuroblatoma. A clinical analysis of 17 cases.Cancer 1976;37:1571-6. |
|7.||Casiano RR, Numa WA, Falquez AM. Endoscopic resection of Esthesioneuroblastoma. Am J Rhinol 2001;15:271-9. |
[Figure 1], [Figure 2]