Journal of Indian Academy of Oral Medicine and Radiology

: 2008  |  Volume : 20  |  Issue : 4  |  Page : 157--161


Anil Kumar Bhoweer1, AP Chitre2,  
1 Department of Oral Medicine and Radiology, I.T.S. Centre for Dental Studies and Research, Delhi - Meerut Road, Murad Nagar, Ghaziabad-201 206, India
2 Honorary Consulting Oral and Maxillofacial Surgeon, Bombay Hospital and Harkisondas Hospital, Mumbai, India

Correspondence Address:
Anil Kumar Bhoweer
Department of Oral Medicine and Radiology, I.T.S. Centre for Dental Studies and Research, Delhi - Meerut Road, Murad Nagar, Ghaziabad-201 206


Pain in the dental arches is a very common cause for patients to seek the dentist«SQ»s advice. Persistence of pain following exclusion of dental causes by treatment and especially when the pain refers to the other areas of the jawbone makes the dentist think of neurologic causes. However, proper investigation and consideration of intracranial tumors as the cause of neuralgic pain around dental arches and jawbone is of utmost importance. A rare case of esthesioneuroblastoma (ENB) arising from naso-ethmoidal complex encroaching the posterior aspect of left maxilla causing neuralgic type of pain in the dental arches, and therefore primarily seeking advice of the dentist is described. It is common for these patients suffering from ENB to have classical symptoms with nasal cavity, maxillary sinus or vision. It is extremely rare to have dento-facial pain as the first clinical symptom seeking dental advice. Awareness of such rare cranial tumors causing dento-facial pain is important. Necessary investigations include radiographs, CT scan images, histological examination for accurate diagnosis and treatment modality.

How to cite this article:
Bhoweer AK, Chitre A P. Esthesioneuroblastoma.J Indian Acad Oral Med Radiol 2008;20:157-161

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Bhoweer AK, Chitre A P. Esthesioneuroblastoma. J Indian Acad Oral Med Radiol [serial online] 2008 [cited 2022 Jun 26 ];20:157-161
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"Esthesioneuroblastoma" (ENB) or "olfactory neuroblastoma" (ON) was first described in 1924, in French literature. The entity was then called "L'Esthesioneuro Epitheliome Olfactil". Later in 1926, Berger [1] and Coutard [2] and in 1928 Portmann et al . [3] found certain morphological variants in "esthesioneuroblastoma". Later in 1951, American literature published some reports on "olfactory neuroblastoma". [4],[5] Elkon et al . [6] in 1979 reported in their review that until 1980, nearly 200 cases of ON have been reported in literature. The increase in the number of cases over time could be attributed to the increasing awareness of the condition in the medical field.

Berges and co-workers described the histopathological feature in their case report as: a group of cylindrical cells arranged in rosette formation. They also quote another type in which sheets of undifferentiated cells similar in appearance to neuroblastoma are seen.

Robert et al . [7] classified this tumor into three different types considering its clinical and histopathological appearance:

Pattern I: A tumor with sheets of small, round cells separated by connective tissue septa; pseudorosettes consisting of a ring of nuclei around a space filled with fibrils are sometimes seen.

Pattern II: A tumor containing round to oval nuclei. Clear nuclear membranes, scanty cytoplasm and indistinct cell borders are the features. Rosettes consist of spaces lined by columnar cells with the nuclei oriented radially.

Pattern III: A tumor similar to neuroblastoma found elsewhere in the body.

However, the author admits that classifying the cellular pattern and relating histopathological appearance to malignant activity was unsuccessful.

Cantrell et al . describe characteristics of the "ON" as:

Plexiform intercellular fibrilsPoorly defined, almost nonexistent cytoplasmRound or oval nucleiDistinct and sharply defined chromatinCompartmentalization of sheets of neoplastic cells into lobules by slender vascular fibrous septaRosettes and pseudorosettes

Robert emphasized that finding rosettes or pseudorosette-formation may not be necessary and can even be misleading. He found in his study that only few cases showed rosettes or pseudorosettes, which he felt was consistent with the findings in the studies carried out by Schenck [8] and Ogura. Cantrell [7] et al . in their study report that the tumor arises from naso-ethmoidal complex and neural crest.

McGavran [10] described the presence of secretary granules similar to catecholamine granules in the cytoplasm of "ENB". Immunochemical and biochemical studies carried out by Michaw and co-workers [11] showed the presence of Dopamine-B hydroxy lamine and catecholamines. These findings are highly suggestive of "ENB" as being of asympathetic origin.

The cases published so far had the following symptoms: nasal obstruction, epistaxis, mass in the nasal cavity, obliteration of maxillary sinus, diplopia, proptosis, etc. Very few or solitary examples quote dental or facial pain as the primary symptom.

This particular case was unique in that the patient had neurological type of pain from the left maxillary molars radiating to the face and referred unilateral headaches. However, it is difficult to diagnose a case of "esthesioneuroblastoma" merely by clinical examination and radiological investigations. Histopathological diagnosis becomes mandatory to diagnose this rare entity.

 Case Report

A female patient, age 37 years, moderately built, apparently healthy, reported to dental OPD with a complaint of persistent pain in the left maxillary molar area for a period of over a month. Brief history revealed that she never visited her family physician, but visited the local dentist from the area for her pain in the left maxillary molar region. The dental surgeon, after examining her, decided to extract the left maxillary third molar.

Even after the extraction and usual antibiotic, analgesics and anti-inflammatory drugs, she continued to have pain in the region referred to the? cheek on the left side. Since there was no response to the usual drugs, dentists felt that she may be suffering from trigennial neuralgia. Hence, she was kept on carbemezapine drug which, however, could control her pain only temporarily.

She was then referred for a second opinion and for the necessary therapy. On clinical examination, there was no evidence of any deformity of the face. The left cheek areas were tender on palpation. There was no evidence of polypoid mass in the nasal cavity on the left side. Intra-orally the third molar socket had healed well. However, at the junction of the hard and soft palate on the left side behind the second molar region, a soft compressible mass was palpable which was partly tender. Regional cervical lymph nodes were not palpable.

Radiological investigations were called for: orthopantomogram and occlusal view of maxilla.

Orthopantomogram revealed destruction of the left tuberosity and the pterygoid plates [Figure 1].

Occlusal radiograph of the maxilla also revealed the bony destruction in the left tuberosity and the palate region, posterior to the second molar [Figure 2],[Figure 3].

These routine radiological investigations revealed unusual destruction of left maxillary tuberosity and the pterygoid plates and hard palate, which led us to suspect the condition to be of a malignant nature or probably chronic osteomyelitis of the region.

Waters' projection for para nasal sinuses was ordered which revealed opacity of the left maxillary sinus. However, the destruction of the walls of the sinus could not be appreciated [Figure 4].

These radiological features led us to a working diagnosis of malignancy in the left maxillary sinus invading posterior part of maxilla. It was therefore decided to explore the left maxillary sinus for biopsy. Blood and urine reports were normal. The left maxillary sinus was explored and the tissue was collected for histological examination.

Histological features: Predominant densely packed mass of cells with darkly stained round vesicular nucleus and poorly defined eosinophilic cytoplasm, typical of neuroblast cells with angiomatous connective tissue septae seen. Presence of typical rosettes and pseudorosette pattern of tumor cells with scanty connective tissue stroma-esthesioneuroblastoma [Figure 5] and [Figure 6].

The microscopic features reported by the experts in the field drew our attention, labeling it as "esthesioneuroblastoma" (a primary naso-ethmoidal malignant tumor). It was a great surprise that such a naso-ethmoidal tumor could first report to the dental OPD with neuralgic symptoms. It is a real rarity that such a patient may produce initial dento-facial symptoms. Awareness of such occurrence is of great importance.

Knowing its origin, it was decided to have a CT scan to know in detail the invasion of the tumor to other regions. There was, however, a gap of one month from the initial working diagnosis and the CT scan. The CT scans revealed the extension of the tumor mass in the maxillary sinus, ethmoidal complex, left nasal fossa, hard palate, left posterior area and even destruction of pterygoid plates on the left side encroaching the pterygoid fossa [Figure 7]. The post diagnostic CT scans revealed a more extensive and locally aggressive nature of the tumor into the other areas [Figure 8].

Radioisotope uptake studies revealed dense area on the (lt.) maxilla and naso-ethmoidal complex [Figure 9]. General skeletal bones did not reveal any area suggestive of metastasis. Radiography of the lungs [Figure 5],[Figure 6],[Figure 10] did not reveal any metastasis in the lung.

Even on clinical examination there was no evidence of metastasis in the regional lymph nodes. Considering the rapid extension of the tumor mass, the patient was referred to a neuro surgeon for opinion for a possibility of radical excision and if necessary post-surgical radiotherapy. The patient was however reluctant to undergo surgery and was considered for chemotherapy. However, the patient died after two months.

The patient presented with the symptoms related to dento-facial pain similar to neuralgias occurring in the region without any gross deformity of the face. It was unique to have such a patient of "ENB" to the dental OPD with only toothache or neuralgic pain as a primary symptom. No doubt such pain could have been due to local spread of the tumor in the region with involvement of branches of the maxillary nerve. More specific of the "ENB", unlike other malignancies which do not cause much pain, this tumor may give neuralgic symptoms. Dentists should be aware of such a possibility.

There were no detectable metastases in the cervical lymph nodes, lungs or bony skeleton as also stated in their reports by Elkon [6] in 1979 and Bailing and Burton [12] in 1975. However, 20% of the cases may have distant metastasis in the cervical lymph nodes and lungs which needs to be investigated. Routine radiological procedures are inadequate to diagnose the condition "ENB", but modern imaging techniques, viz. CT scan, are of great importance in delineation of such tumors. Since this tumor has greater tendency to local invasion, CT scan is very important to see its extension into the max sinuses, orbit and cranial cavity. In this patient, the tumor had extended into lt. maxillary sinus, the pterygoid fossa destroying pterygoid plates. However, the orbit was spared as the extension of the tumor occurred more in the posterior side into the maxilla.


The entity known as esthesioneuroblastoma (ENB) or olfactory neuroblastoma (ON) was first mentioned in French literature in 1924. Some reports were published later, but in 1980 a review of nearly 200 cases was published. The surge in the number of cases could be due to improved awareness over time.

Most of the reported cases were based on histopathological features resulting in the classification of the tumor.

The clinical symptoms in all the cases were mainly in relation with nasal fossae and in advanced cases the orbit was involved. Rarely it mentioned dental or facial pain as a primary symptom. However, in our case the primary symptom was only in dental arches (lt.). This could be due to the involvement of the maxillary nerve.

The patient was treated with antibiotics and anti-inflammatory analgesics, ultimately leading to extraction of a tooth without any relief. Later she was also treated with carbamezapine which helped to some extent.

There was no obvious dental etiology or any pathology seen in maxillofacial area except for a palpable mass at the junction of the hard and soft palate on intra-oral examination. There was no cervical lymph node involvement.

Various radiological views showed destruction of bone in posterior region of left maxilla and opaque (lt.) maxillary sinus leading to the tentative diagnosis of malignant lesion. The blood and urine reports were normal. The left maxillary sinus was then explored for biopsy.

The histopathological examination revealed a primary naso-ethmoidal malignant tumor - esthesioneuroblastoma (ENB).

The case was further investigated with CT scan and radioisotope studies to assess the extension of the lesion and to rule out metastasis.


In any patient presenting with neuralgic dento-facial pain without any obvious cause in the region, ENB should be considered as a possible differential diagnosis though rare. CT scan of head and face region is of importance in such cases.


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