|Year : 2014 | Volume
| Issue : 4 | Page : 1101-1103
Sphenoidal esthesioneuroblastoma treated with sequential chemo radiotherapy: A case report and review of the literature
Omar Abdel-Rahman, Khaled Kamal
Department of Clinical Oncology, Faculty of Medicine, Ain Shams University, Cairo, Egypt
|Date of Web Publication||9-Jan-2015|
Department to Clinical Oncology, Faculty of Medicine, Ain Shams University, Lotfy Elsayed Street, Cairo, Postal Code: 11665
Source of Support: None, Conflict of Interest: None
Esthesioneuroblastoma (ENB)-also known as olfactory neuroblastoma-originates primarily from the olfactory epithelium in the roof of the nasal cavity. We present here the 7 th reported case of ENB arising from the sphenoidal sinus. We undertook a review of the case notes from the time of initial presentation and literature review of this topic. A 55-year-old male presented with a 3-year history of unilateral progressive nasal obstruction, epistaxis, difficult nasal-breathing, facial pain, frontal headache and blood-tinged discharge. Nasal examination revealed a glistening mass in right nasal cavity. Endoscopy-assisted transnasal excision was performed. Histologically, tumor was identified as small round cell tumor and confirmed by immunohistochemistry to be olfactory neuroblastoma (negative leucocyte common antigen, positive neurone specific enolase). Sphenoidal ENB is rare in the literature, and this presentation is the 7 th reported case; in addition, surgical treatment is very challenging, and non-surgical treatment is used most commonly.
Keywords: Chemotherapy, esthesioneuroblastoma, paranasal sinuses, radiotherapy, sphenoidal tumors
|How to cite this article:|
Abdel-Rahman O, Kamal K. Sphenoidal esthesioneuroblastoma treated with sequential chemo radiotherapy: A case report and review of the literature. J Can Res Ther 2014;10:1101-3
|How to cite this URL:|
Abdel-Rahman O, Kamal K. Sphenoidal esthesioneuroblastoma treated with sequential chemo radiotherapy: A case report and review of the literature. J Can Res Ther [serial online] 2014 [cited 2020 Jul 13];10:1101-3. Available from: http://www.cancerjournal.net/text.asp?2014/10/4/1101/146110
| > Introduction|| |
Esthesioneuroblastoma (ENB)-Also known as olfactory neuroblastoma-originates primarily from the olfactory epithelium in the roof of the nasal cavity. 
The incidence is estimated to be 3-5% of nasal malignant tumors. The sex distribution is roughly equal, and it is generally a disease of white individuals. No etiologic basis has been determined. In addition, it has bimodal age distribution (from 10 to 20 years) and (from 40 to 60 years). 
We are here presenting the 7 th reported case of ENB arising from the sphenoidal sinus in a 55-year-old male.
| > Case report|| |
A 55-year-old male presented with 3-year history of unilateral progressive nasal obstruction, epistaxis, difficult nasal-breathing, facial pain, frontal headache and blood-tinged discharge. Nasal examination revealed a glistening mass in right nasal cavity. On probing the mass, it was insensitive to touch, but it bled on handling. Nasal septum was deviated to the left, nasal-patency test reduced bilaterally, and olfactory test revealed relative hyposmia on the right side. Vision and rest of the head-neck examination were normal.
Computed tomography (CT) revealed soft tissue density areas extending through the right sphenoidal sinus with obliteration of the right sphenoethmoidal recess [Figure 1] and [Figure 2].
|Figure 1: coronal computed tomography images showing the anatomical extent of the lesion|
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|Figure 2: Axial computed tomography images showing the anatomical extent of the lesion|
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Endoscopy-assisted transnasal excision was performed. Histologically, tumor was identified as small round cell tumor and confirmed by immunohistochemistry to be olfactory neuroblastoma (negative leukocyte common antigen, positive neurone specific enolase) [Figure 3] and [Figure 4].
|Figure 3: Immunohistochemical study showing positivity for neurone specific enolase and negativity for leukocyte common antigen in the tumor cells|
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The case has been considered surgically unresectable by our surgical team. Thus it has been referred for definitive sequential chemoradiotherapy (3 cycles of cisplatin/5-FU chemotherapy to be followed by definitive radiotherapy).
| > Discussion|| |
Esthesioneuroblastoma is an uncommon malignant neoplasm of the nasal vault, believed to arise from the olfactory epithelium. The tumor was first described in 1924 by Berger et al., and given the name esthésioneuroépithéliome olfactif. Uncertainty about the precise histological origin has led to the use of various names for this tumor, but the only two terms used in recent publications are ENB and olfactory neuroblastoma. 
A staging classification for ENB was first proposed by Kadish et al., consisting of three categories. Group A is limited to tumors of the nasal fossa; in group B extension is to the paranasal sinuses; group C is defined as an extension beyond the paranasal sinuses.  However, this most commonly used system did not describe an isolated sphenoidal origin of this tumor.
The occurrence of ENB in a site outside of where olfactory epithelium exists is exceedingly rare. Isolated ENB has been described in the ethmoid sinus, maxillary sinus, nasopharynx, and pituitary gland. Only six cases of ENB isolated to the sphenoid sinuses have been described in the literature to date. ,,] One case had an unusual metastasis to the skin of the sternal region. ,
Several theories exist as to the origin of ENB in locations where olfactory neuroepithelium is not thought to exist. One theory is that ectopic cell nests are deposited in these areas (maxillary sinuses, ethmoid sinuses, pituitary gland, and sphenoid sinuses) during embryologic development. , A second possible theory was first suggested by Jakumeit in 1971. ,, This theory is based on the development and degeneration of the accessory olfactory systems during fetal life. The cells of these accessory systems eventually degenerate in fetal life. Persistence of these cells may be the origin of ENB occurring in these sites.
As it is a rare disease, so most studies are small, single institution and/or retrospective, however, surgery is frequently considered the primary modality. In addition, there is some evidence that postoperative RT improves local control, particularly for more advanced disease. With some additional evidence that concurrent chemo-RT may be beneficial. Moreover, the impact of neoadjuvant and adjuvant chemotherapy remains unknown. Sohrabi et al. reported the use of cisplatin (60 mg/m 2 ) with etoposide (120 mg/m2) with concomitant radiotherapy of 50 Gy for the preoperative treatment of Kadish stage C, and led in 2 patients to complete pathologic responses. This approach needs, off course, further investigation. ,
However, what is unique in our case report is that with gross disease in the sphenoid, resectability is a very challenging consideration because of the proximity to the optic nerves, carotid arteries and the clivus and brainstem posteriorly. This makes this tumor more challenging to treat, and one might argue in favor of non-surgical treatment (definitive chemoradiotherapy in various combinations and schedules) as opposed to surgical treatment. Additionally, the CT scans of our case show disease in the ethmoids/nasal cavity in addition to the sphenoidal disease, which raises the possibility of this being a garden variety ENB with sphenoid extension and this may complicate the treatment decisions further in such a case.
| > Conclusions|| |
Esthesioneuroblastoma is a rare tumor and needs extensive IHC analysis for diagnosis. The standard of care in such disease is to offer patient surgery, followed by chemo radiation. However in advanced disease chemotherapy, followed by local treatment is a suitable therapy. The disease resection has to be optimum for better loco regional control. Chemotherapy sensitivity is questionable in some patients as the disease may not respond in all patients.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]