116

Nasopharnyx, defined as nasal part of the phar-ynx extending from the base of the skull to theupper surface of the soft palate and in frontcommunicates through choanae with nasal si-nuses is a rare site for the occurrence of neuro-blastomas. Usually most of the lesions involv-ing the nasal cavity are epidermoid carcinomasbut neuroblastomas are rare. Olfactory neuro-blastoma arises from sensory epithelium inupper nasal fossa at the level of cribriform plate.It constitutes about 5 % of malignant tumoursof nasal cavity. It is highly prevalent in Chi-nese population with a reported incidence of1 in 200,000 population.1 The incidence of car-cinoma of nasopharnyx in United States is 0.8/100,000 for males and 0.03/100,000 for fe-males. It is most common in the second andfifth decades of life but can be seen at any ageand occurs equally in males and females.Pathologically 90% of malignant lesions aris-ing from the nasopharynx are epidermoid orundifferentiated carcinomas, the other 10% aremainly lymphomas, plasmacytomas, melano-mas, and rhabdomyosarcoma and chordomas.The literature search revealed involvement ofnasopharnyx by esthesioneuroblastoma is veryrare when compared to other tumours.A 22-year-old male presented with complaintsof blockage of left nasal cavity and anosmia of2 months duration and epistaxis (10 episodesper day) not associated with foul smelling dis-charge. He also complained of headache whichwas holocranial and intermittent, that was ag-gravated on exertion and relieved spontane-ously. This was not associated with either vom-iting or blurring of vision. Giddiness on chang-ing the posture from standing to sitting was evi-dent. There was deviation of left eye ball later-ally and difficulty in movements of left eyepresent. There was no history suggestive ofdiplopia, blurring of vision, loss of conscious-

ness, seizures, earache or discharge from ear,deafness, gait disturbances or weakness, sen-sory deficits, urine or bowel habit disturbances,fever or trauma to nose. The patient was mod-erately built and nourished and was anaemic.Examination of the nasal cavity revealed thepresence of a friable mass extending into leftnasal cavity without the presence of either de-viated nasal septum or hypertrophied turbi-nates. Pre-operative computed tomographyshowed the presence of a mass extending intoleft nasal cavity and involving bilateral maxil-lary sinuses, left frontal, left sphenoid and leftethmoid sinuses. Mass was also extending intoextraconal compartment of left orbit involvingleft superior rectus and eroding the cribriformplate (Figure 1). The patient underwent exci-sion of mass under general anaesthesia by lat-eral rhinotomy through Moore's approach. Amass was found to be arising from thenasopharnyx and extending onto nasal cavityand bilateral nasal sinuses. Wide local excisionwas done and the excised material was sub-jected for histopathological examination whichrevealed the tumour cells seen predominantlyarranged in large lobules, separated by fibrovas-cular stroma with few areas of solid nests andcribriform patterns (Figure 2). Occasional tu-mour lobules showed Homer - Wright rosettes(Figure 3).The cells were small to intermedi-ate sized having vesicular nucleus with singlenucleolus and scanty acidophilic cytoplasm(Figure 3). Atypical mitotic figures (2-3/10high power fields) was noted. No neurofibril-lary background was seen. The stroma showedextensive areas of haemorrhage with mild lym-phocytic infiltration and the case was diagnosedas esthesioneuroblastoma. Post-operative pe-riod was uneventful. The patient was dis-charged and referred for radiotherapy.

Correspondence:Ectopic olfactory neuroblastoma

Ectopic olfactory neuroblastoma Gopala Kesari et al

117

Esthesioneuroblastoma is an uncommon ma-lignant neoplasm, representing 2%-3% ofsinonasal tract tumors with an incidence of ap-proximately 6%.2 These tumours arise from theolfactory neuroepithelium, which extends fromthe roof of the nose to the area of the superiorturbinate and a portion of the nasal septum. Thedevelopment of olfactory neuroblastoma out-side the region where olfactory neuroepitheliumdoes not normally exist is extremely rare. It has

been rarely reported in the literature from theregions such as the maxillary and sphenoidalsinuses, the petrous apex, nasopharnyx and thepituitary gland.3

To conclude, ectopic olfactory neuroblastomais a rare entity and with the invention of newmodalities of treatment like intensity-modu-lated radiation therapy and Image-guide radia-tion therapy a better prognosis can be expected.The Radiation Therapy Oncology Group re-ported that combined radio and chemotherapy

Figure 2: Photomicrograph showing the tumour cells seenpredominantly arranged in lobules, separated by fibrovas-cular stroma with few areas of solid nests and cribriformpatterns (Haematoxylin and eosin, 400)

Figure1: Coronal CT showing mass extending from left nasal cavity and involving bilateral maxillary sinuses, leftfrontal, left sphenoid and left ethmoid sinus

Figure 3: Photomicrograph showing tumour lobules withHomer - Wright rosettes. The cells were small to inter-mediate sized having vesicular nucleus with singlenucleolus and scanty acidophilic cytoplasm(Haematoxylin and eosin, 400)

Ectopic olfactory neuroblastoma Gopala Kesari et al

118

C.M. Gopala Kesari,1

B. Sreenivasa Rao,1

K.V.L. Anusha,1

K. Radhika,2

V. Chandrasekar,3

G. Sreenivas,3

D.R. Anand.4

Departments of 1Radiotherapy,2Pathology,

Sri Venkateswara Institute of Medical Sciences, Tirupati; andDepartments of 3E.N.T.Diseases,

4Radiology,Sri Venkateswara Medical College, Tirupati

Received : 24 August, 2012.

Gopala Kesari CM, Sreenivasa Rao B, Anusha KVL, Radhika K, Chandrasekhar V, Sreenivas G, Anand DR. Ectopic olfactoryneuroblastoma-report of a rare case. J Clin Sci Res 2013;2:116-8.

will give long term survival rates. In recentsystematic review of published series spanning40 years, a steady improvement of overall sur-vival of patients was noted.4

REFERENCES1. Zhang M, Zhou L, Wang DH, Huang WT, Wang SY.

Diagnosis and management ofesthesioneuroblastoma. ORL J OtorhinolaryngolRelat Spec 2010;72:113-8.

2. Theiland SA, Buchwald C, Ingeholm P, KornumLarsen S, Eriksen JG, Sand Hansen H.Esthesioneuroblastoma: a Danish demographic studyof 40 patients registered between 1978 and 2000.Acta Otolaryngol 2003;123:433-9.

3. Wormald R, Lennon P, O'Dwyer TP. Ectopic olfac-tory neuroblastoma: report of four cases and reviewof literature. Eur Arch Otorhinolaryngol2011;208:555-60.

4. Morita A, Ebersold MJ, Oslen KD, Foote RL, LewisJE,Quast LM. Esthesionuroblastoma: prognosis andmanagement. Neurosurgery 1993;32:706-14.

Ectopic olfactory neuroblastoma Gopala Kesari et al