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CORRESPONDENCE
Year : 2014  |  Volume : 10  |  Issue : 4  |  Page : 1101-1103

Sphenoidal esthesioneuroblastoma treated with sequential chemo radiotherapy: A case report and review of the literature


Department of Clinical Oncology, Faculty of Medicine, Ain Shams University, Cairo, Egypt

Date of Web Publication9-Jan-2015

Correspondence Address:
Omar Abdel-Rahman
Department to Clinical Oncology, Faculty of Medicine, Ain Shams University, Lotfy Elsayed Street, Cairo, Postal Code: 11665
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.146110

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 > Abstract 

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.

 > Abstract in Chinese 

序贯放化疗治疗蝶窦嗅神经母细胞瘤:1例报告及文献复习

摘要


嗅神经母细胞瘤(ENB)也被称为嗅成神经母细胞瘤,主要来源于在鼻腔顶的嗅上皮。在此我们报道第7例ENB,源于蝶窦,以及有关的文献回顾。一位55岁的男性,有3年病史的单侧渐进性鼻塞,鼻出血,鼻呼吸困难,面部疼痛,头痛和血涕。鼻腔检查显示右鼻腔肿块。内镜下经鼻切除。组织学上,肿瘤被确定为小圆细胞肿瘤,经免疫组化确诊是嗅神经母细胞瘤(白细胞共同抗原阴性,神经元特异性烯醇化酶阳性)。蝶ENB在文献中是罕见的,这例报道是第7例;此外,手术治疗是非常具有挑战性的,非手术治疗也是最很常用的。


关键词:嗅神经母细胞瘤,化疗,放疗,鼻窦,蝶窦肿瘤


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 2019 Nov 12];10:1101-3. Available from: http://www.cancerjournal.net/text.asp?2014/10/4/1101/146110


 > Introduction Top


Esthesioneuroblastoma (ENB)-Also known as olfactory neuroblastoma-originates primarily from the olfactory epithelium in the roof of the nasal cavity. [1]

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). [2]

We are here presenting the 7 th reported case of ENB arising from the sphenoidal sinus in a 55-year-old male.


 > Case report Top


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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Figure 4: Histopathological views of the lesion

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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 Top


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. [3]

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. [4] 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. [5],[6],[7]] One case had an unusual metastasis to the skin of the sternal region. [8],[9]

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. [10],[11] A second possible theory was first suggested by Jakumeit in 1971. [12],[13],[14] 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. [13],[15]

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 Top


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.

 
 > References Top

1.
Elkon D, Hightower SI, Lim ML, Cantrell RW, Constable WC. Esthesioneuroblastoma. Cancer 1979;44:1087-94.  Back to cited text no. 1
    
2.
Broich G, Pagliari A, Ottaviani F. Esthesioneuroblastoma: A general review of the cases published since the discovery of the tumour in 1924. Anticancer Res 1997;17:2683-706.  Back to cited text no. 2
    
3.
Dulguerov P, Allal AS, Calcaterra TC. Esthesioneuroblastoma: A meta-analysis and review. Lancet Oncol 2001;2:683-90.  Back to cited text no. 3
    
4.
Kadish S, Goodman M, Wang CC. Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer 1976;37:1571-6.  Back to cited text no. 4
    
5.
Morris L, Govindaraj S, Genden EM. Primary sphenoid sinus esthesioneuroblastoma. Am J Otolaryngol 2004;25:350-3.  Back to cited text no. 5
    
6.
Chirico G, Pergolizzi S, Mazziotti S, Santacaterina A, Ascenti G. Primary sphenoid esthesioneuroblastoma studied with MR. Clin Imaging 2003;27:38-40.  Back to cited text no. 6
    
7.
Sharma SC, Reddy CE, Srinivasan SS, Rawal A, Singh DP. Isolated esthesioneuroblastoma of sphenoid sinus. Am J Otolaryngol 2002;23:287-9.  Back to cited text no. 7
    
8.
Akinfolarin J, Jazaerly T, Jones K, Abu-Hamdan M, Lonardo F, Folbe A, et al. Fine needle aspiration cytology of primary sphenoid sinus esthesioneuroblastoma metastatic to the skin. Avicenna J Med 2012;2:15-8.  Back to cited text no. 8
[PUBMED]  Medknow Journal  
9.
Abdel-Rahman O. Systemic therapy for hepatocellular carcinoma (HCC): From bench to bedside. J Egypt Natl Canc Inst 2013;25:165-71.  Back to cited text no. 9
    
10.
Abdel-Rahman O, Elsayed ZA. Combination trans arterial chemoembolization (TACE) plus sorafenib for the management of unresectable hepatocellular carcinoma: A systematic review of the literature. Dig Dis Sci 2013;58:3389-96.  Back to cited text no. 10
    
11.
Zappia JJ, Bradford CR, Winter PH, McClatchey KD. Olfactory neuroblastoma associated with Kallman′s syndrome. J Otolaryngol 1992;21:16-9.  Back to cited text no. 11
    
12.
Jakumeit HD. Neuroblastoma of the olfactory nerve. Acta Neurochir (Wien) 1971;25:99-108.  Back to cited text no. 12
    
13.
Sohrabi S, Drabick JJ, Crist H, Goldenberg D, Sheehan JM, Mackley HB. Neoadjuvant concurrent chemoradiation for advanced esthesioneuroblastoma: A case series and review of the literature. J Clin Oncol 2011;29:e358-61.  Back to cited text no. 13
    
14.
Abdel-Rahman O, Fouad M. Risk of selected gastrointestinal toxicities in breast cancer patients treated with regimens containing lapatinib; a pooled analysis of randomized controlled studies. Expert Rev Anticancer Ther 2014;14:1229-42.  Back to cited text no. 14
    
15.
Abdel-Rahman O, Fouad M. Correlation of cetuximab-induced skin rash and outcomes of solid tumor patients treated with cetuximab: A systematic review and meta-analysis. Crit Rev Oncol Hematol 2014.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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