HEAD AND NECK
Year : 2006 | Volume
: 16 | Issue : 4 | Page : 669--672
S Turakhia, K Patel
Assistamt Professor, B.J. Medical College, India
4, Nanduprasad Mahadeviya Society, Near Sanghavi High School, Vijaynagar Road, Naranpura, Ahmedabad - 380013
|How to cite this article:|
Turakhia S, Patel K. Esthesioneuroblastoma.Indian J Radiol Imaging 2006;16:669-672
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Turakhia S, Patel K. Esthesioneuroblastoma. Indian J Radiol Imaging [serial online] 2006 [cited 2020 Jan 19 ];16:669-672
Available from: http://www.ijri.org/text.asp?2006/16/4/669/32295
Esthesioneuroblastoma or olfactory neuroblastoma is an uncommon tumour that originates from neural creast cells and arises from the olfactory mucosa. The incidence curve for this disease has a bimodal shape with the first peak in the 2nd decade and 2nd peak in 6th decade. . The clinical course is characterized by local aggressiveness and less commonly distant metastasis.
We would like to report a patient-diagnosed to have esthesioneuroblastoma in nasal cavity-operated for the same and with recurrence with cervical lymph nodes metastasis.
A 20 years old female patient presented with difficulty in breathing and right proptosis. Patient was operated for nasal mass before 3 months.
X-ray PNS shows destruction of right lateral wall of nose with soft tissue opacity in nasal region and right orbital region.[Figure 1]
CT axial and coronal scan were performed and sagittal reconstruction was done. Large well-defined soft tissue density mass lesion was seen in both nasal cavities with central necrosis. Loss of medial wall of right maxillary sinus, right lateral nasal wall and extension into right maxillary sinus. There was also destruction of medial wall of right orbit with extension of soft tissue within right orbit displacing the eyeball anteriorly and inferiorly. Ballooning of left lateral nasal wall.[Figure 2] There was also intracranial extension.[Figure 3] Post contrast study showed marked enhancement of lesion[Figure 4]. Intracranial part showed cyst formation.[Figure 5] Multiple enlarged lymph nodes were seen in right cervical region and in post auricular region.[Figure 6]
Histopathologic study shows respiratory epithelium, nasal bony tissue, fibrous tissue, necrotic and inflammatory material and increased vascularity. Deeper tissue shows malignant tumour with uniform small round cells with scanty cytoplasm and salt pepper nuclei. No definate rosette seen. The cells arranged in nesting neurofibrillary material seen. Biopsy from cervical lymph node shows small round cells. Findings were suggestive of olfactory neuroblastoma with metastasis.
Esthesioneuroblastoma is an uncommon malignant tumour that arises from bipolar sensory receptor cells in the olfactory mucosa. These cells originate in the neural crest and differentiate into the olfactory sensory elements. Esthesioneuroblastomas are histologically similar to adrenal or sympathetic ganglionic neuroblastomas and retinoblastomas. 
It was originally descried by Bergen et al. in 1924 as 'esthesioneuroepithelioma olfactif'. It was introduced into North American literature by Schall and Lineback in 1951. 
Incidence peaks once in 11-20 years of age group (16.8% of all tumors) and again in 50-60 years age group (22.8% of all tumors); however, age of these patients ranges from 3-88 years. It occurs equally in men and women. Synonyms include olfactory esthesioneuroma, neuroesthesioma and olfactory neurocystoma .
The main symptoms are nasal occlusion, proptosis, epistaxis, headache, excessive lacrimation, anosmia and visual disturbances. ,. Because sensory nerves of smell originates in olfactory bulb and pass through cribriform plate to olfactory area of nasal mucosa which is located in the most superior part of both nasal fosse, usual primary sites of occurrence include superior nasal cavity or nasal septum, the turbinates, the ethmoid, or the cribriform plate, although an extra nasal site of origin has been suggested. Even if the lesion can not be demonstrated radiographically above the cribriform plate, involvement of both sided of the plate is presumed and surgical approaches include removal of this area. . This polypoid tumour may be of variable consistency and may bleed profusely on biopsy.  It is almost always unilateral and only in highly neglected cases, they appear as a bilateral nasal fosse mass.  The mass may be relatively slow growing for malignant tumour and cause some expansion and remodeling of bony structures. 'Hyperostosis' may be an inflammatory reaction related o obstructed neighboring air cells. This neoplasm is locally aggressive and cause metastasis by lymphatic and hematogenous routes.  Local recurrence has been reported in upto 57% of patients. A metastatic rate of 20% to 60% is reported with the most common site being the cervical lymph node. Other sites include the parotid glands, skin, lungs, bone, liver, orbit, spinal cord and spinal canal. 
Metastasis to the central nervous system is infrequent and usually identified only on post-mortem examination. In the spinal cord 80% of metastasis is in the cauda equine.  There are isolated reports of metastasis to the scalp, aorta, spleen, adrenal glands and ovary. 
Histologically, esthesioneuroblastoma has a characteristic appearance, containing discrete nests, trabeculae and nodules of tumour cells, fibrillary (or neurofibrillary) material, and prominent collections of stromal blood vessels. The uniform tumour cells have small, dark round or oval nuclei, lack nucleoli and show little cytoplasm. Mitotic activity is variable. The collections of tumour cells are well-defined and more an 'organoid' arrangement, usually lying around a knot of blood vessels. Some examples contain pseudorossets, tumour cells arranged in circular fashion around central areas of eosinophilic fibrillary material. The vascularity of tumour may be as prominent as to suggest as blood vessel neoplasm. The eosinophilic fibrillary material consists of fine, pale-pink fibrils resembling neural tissues may show immunohistochemical staining with antibody to S 100 protein. The only histological feature of prognostic importance is the presence or absence of tumour necrosis with cytological atypia and mitotic rate showing little relationship to long term outcome.  It can be histologically classified as esthesioneuroblastoma with pseudorossets (20% Cases), esthesioneuroblastoma with true rosettes (40% of cases) and esthesioneuroblastoma with sheets of cells without rosettes (40% of cases). The last variety is most often confused with anaplastic carcinoma, large cell lymphoma, melanoma, extramedullary plasmacytoma and embryonal rhabdomyosarcoma. However, electron microscopy and immunohistochemical tests can differentiate these lesions. 
The diagnosis and evaluation of staging of esthesioneuroblastoma can be done by CT which provides the best information about the tumour invasion into bony structures. The protocols for CT scanning include axial and coronal scans of 1 to 5 mm thick slices with IV contrast. The tumour is presented as homogenous density mass, equal to or greater than surrounding soft tissues. Contrast enhancement is usually moderate and homogenous. They commonly extends to ipsilateral ethmoid and maxillary sinuses and only rarely involve sphenoid sinuses. When large, they can extend to involve both sides of nasal cavity and the paranasal sinuses. Intralesional calcification and presence of cysts along the intracranial margins in case of intracranial extension yield a definite diagnosis. ,
Obstruction of sinus draining ostia results in an accumulation of nasal secretions which tend to difficult to differentiate from tumour tissue when viewed by a CT scan. Bone erosion is frequent and usually accompanied by modeling of bone. Staging, determined by extension and critical for treatment decisions, is well evaluated by CT. (13)
On T1W MR Images, esthesioneuroblastoma presents as homogenously enhancing tumour with intermediate signal intensity, where as T2W images, the original intensity is increased. MRI can delineate intraorbital and intracerebral extension. The tumour appears hypo to gray matter onT1WI and iso or hyper to gray matter on T2WI. (14) Gadolinium enhanced MR images help to differentiate tumour from obstructed secretions in paranasal sinuses, determining meningeal and extradural spread and to detect perineural spread.  Angiography demonstrates tumour blush in majority of cases. 
The staging system of Kadish, Goodman and Win-
A Disease confined to nasal cavity
B Disease in nasal cavity and one or more paranasal sinuses
C Disease extending beyond the nasal cavity and paranasal sinuses.
Using this system 30% of patients are of stage A, 42% patients of stage B and 28% patients are of stage C and respective 5 years survival rates are 75%, 68% and 41.2%. 
The main drawback of Kadish classification is the language of defining stage C is too broad. In 1992, Dulgerov and Calcaterra proposed modified TNM staging based on CT and/or MR findings.
Modified TNM staging: 
T1 Tumour involving the nasal cavity and paranasal sinuses ( excluding sphenoid )
T2 Tumour involving the nasal cavity and paranasal sinuses ( including the sphenoid) with extension to or erosion of cribriform plate
T3 Tumour extending into the orbit or protruding into the anterior cranial fossa without dural invasion
T4 Tumour involving the brain
N0 No cervical lymph node metastasis
N1 Any form of cervical lymph node metastasis
M0 No metastasis
M1 Distant metastasis
Craniofacial resection performed on all patients may cure more than 90% of cases. This approach takes into account the microscopic presence of intracranial tumour that can occur with an intact and normal appearing cribriform plate. This is in contrast to performing an extended lateral rhinotomy for stage A and stage B patients, who has a recurrence rate of nearly 50%. The 5 year survival rate for all patients is 69%.
Adjuvant chemotherapy is recommended for patients with high grade tumors or positive surgical resection margins.
Others recommend adjuvant radiotherapy for all patients because combined surgery and irradiation resulted in 92% of disease free status.
Loco regional and distant metastasis occurs in upto 38% of patients. Late recurrences or metastatic disease can occur upto two decades after initial presentation.
Negative prognostic factors include female sex, age over 50 years at presentation, tumour recurrence, metastasis, high tumour grade and Kadish stage C at presentation.
The role of imaging in these patients is to map the tumour so as to precisely anticipate surgical boundaries. 
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