|Year : 2021 | Volume
| Issue : 2 | Page : 78-82
Olfactory neuroblastoma of the nasal cavity managed with modified draf III and adjunctive radiotherapy: Case report and literature review
Abdulmohsin A Almehizia1, Sameer A Albahkaly2, Abdullatif Khan3, Osama A Altaleb1, Salem M Alshehri4
1 College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
2 Department of Otolaryngology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
3 Department of Pathology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
4 Department of Radiation Oncology, King Abdullah Specialized Children's Hospital, Riyadh, Saudi Arabia
|Date of Submission||06-Jul-2020|
|Date of Decision||19-Aug-2020|
|Date of Acceptance||23-Oct-2020|
|Date of Web Publication||10-Jun-2021|
Dr. Abdulmohsin A Almehizia
College of Medicine, King Saudi Bin Abdulaziz University for Health Sciences, Riyadh
Source of Support: None, Conflict of Interest: None
Olfactory neuroblastoma (ONB) is a rare neoplasm that grows in epithelial cells of olfactory tissue. It is related to many tumor oncogenes such as MYC. We present and discuss a rare case of ONB on the elderly with typical symptoms but different treatment approaches. It was treated with endoscopy and adjuvant radiotherapy.
Keywords: Elderly, esthesioneuroblastoma, olfactory neuroblastoma, otorhinolaryngology, radiotherapy
|How to cite this article:|
Almehizia AA, Albahkaly SA, Khan A, Altaleb OA, Alshehri SM. Olfactory neuroblastoma of the nasal cavity managed with modified draf III and adjunctive radiotherapy: Case report and literature review. Saudi J Otorhinolaryngol Head Neck Surg 2021;23:78-82
|How to cite this URL:|
Almehizia AA, Albahkaly SA, Khan A, Altaleb OA, Alshehri SM. Olfactory neuroblastoma of the nasal cavity managed with modified draf III and adjunctive radiotherapy: Case report and literature review. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2021 [cited 2021 Jul 23];23:78-82. Available from: https://www.sjohns.org/text.asp?2021/23/2/78/318131
| Introduction|| |
Esthesioneuroblastoma, also known as olfactory neuroblastoma (ONB), is a tumor that arises in olfactory neuroepithelial cells that are located in the roof of the nasal cavity., It comprises 2% of intranasal tumors with an incidence of 0.4 per million people, and it occurs mainly in the elderly population. ONB is a rare tumor, which was first described by Berger et al. in 1924; therefore, the information available remains scarce.
There are limited data regarding both genetics and ONB development. Researchers discovered that the sonic hedgehog pathway, vascular endothelial growth factor receptor 2, and MYC genes are involved in ONB development., Furthermore, Gallia et al. found that mutations in dystrophin and laminin alpha 2 are associated with ONB.
ONB occurs in a sensitive area and is highly liable to metastasize; therefore, it is challenging to treat. As a result, there is no standard treatment approach, and the treatment varies from minimally invasive to invasive surgeries with adjunctive modalities.
| Case Report|| |
A 71-year-old Caucasian male presented to the emergency department with headache, generalized fatigue, and nasal pain and swelling. He did not complain of ear pain. The patient could not stand or mobile, and he had occasional high blood pressure readings (over 200 mmHg) and shortness of breath with coughing. He was admitted. He is a known case of bronchial asthma, atrial fibrillation, and hypertension. Twenty years ago, he underwent left nephrectomy for suspicion of cancer.
On physical examination, the patient's weight and height were recorded as 104 kg and 175 cm, respectively. His vitals were stable, and he was afebrile. Otorhinolaryngology examination, nasal bleeding, and discharge were noted. There were signs of chronic infection. The throat was clear, with no signs of congestion and he did not feel pain in the ears. Endoscopic examination disclosed a polypoid mass in the right nasal cavity, and a biopsy was taken. The rest of the examinations were unremarkable, and the patient did not have a history of trauma.
Magnetic resonance imaging (MRI) revealed the presence of a large tumor on the right side of the nasal cavity that extended to the nasopharynx and ethmoid air cells as well as the medial wall of the right maxillary sinus and the antrum, which caused bulging into the right maxillary sinus [Figure 1]a and [Figure 1]b. It obstructed sinonasal flow and led to opacification and mucosal thickening; it was isointense on T1 to cerebral parenchymal and heterogenous on T2-weighted images. Both diffusion-weighted images and the apparent diffusion coefficient map revealed a hypocellularity pattern of the lesion. The tumor extended posteriorly and bulged into the sphenoid sinus. Moreover, abnormal signal intensity and enhancement were observed in both the frontal bone and olfactory groove bone.
|Figure 1: (a) Axial postcontrast T1. (b) Coronal postcontrast T1. (c) Sagittal view of CT brain|
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Computed tomography (CT) of the brain and sinuses showed a large lesion in the ethmoid cells and right nasal cavity [Figure 1]c, with the left ethmoid involved. The lesion extended to the nasopharynx and bulged into the right sphenoid and maxillary sinuses. After contrast injection, the lesion exhibited a strong enhancement in the CT imaging. Mucous retention was observed in the maxillary, sphenoid, and frontal sinuses on the right side. The intracranial area was spared.
Histological examination [Figure 2]a and [Figure 2]b showed tumor variable in size with small basaloid cells and eosinophilc fibrillary cytoplasm. No mitoses were seen, and there was no necrosis. The intervening stroma was highly vascularized. On immunohistochemistry analysis [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f, the lesional cells were observed to be positive for synaptophysin, CD56, and chromogranin. The lesional cells were negative for CD99, myogenin, pan-cytokeratin, and epithelial membrane antigen. The S100 highlighted sustentacular cells. The Ki-67 proliferation index was low.
|Figure 2: Nasal mucosa with an extended tumor, which is mostly arranged as variable-sized nests of tumor cells arrowed. (a) H and E, scanning magnification). Tumor cell nests were formed of fairly uniform cells showing hyperchromatic nuclei and slightly eosinophilic cytoplasm, and the intervening stroma is vascular. (b) H and E, ×40 magnification). (c) Upon immunohistochemistry, the tumor cells were strongly positive for synaptophysin. (d) Upon immunohistochemistry, the tumor cells were strongly positive for CD56. (e) Upon immunohistochemistry, the tumor cells were strongly positive for chromogranin. (f) Upon immunohistochemistry, the sustentacular cells are highlighted with S100|
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This case report is staged C according to the Kadish system [Table 1]. The patient has undergone endoscopic tumor resection with radiotherapy (RT). The approach was by utilizing the Draf III procedure, medial maxillectomy, bilateral ethmoidectomy, high septal resection, and navigation of sinuses. We started with the navigation of sinuses to debulk the mass and draw landmarks from the right olfactory fossa to the nasal cavity. Then, we widened the right maxillary for cleaning. Subsequently, we performed a medial maxillectomy and completely removed the inferior turbinate. In addition, we removed the medial wall of the right maxilla because the mass was engulfing the inferior turbinate as well as the nasolacrimal apparatus. We proceeded to remove the tumor from the anterior ethmoidal area and then penetrated the lamella to reach the posterior ethmoid to remove the tumor in the sphenoid sinus. Next, we performed a high septal resection to perform the Draf III procedure, which was used to clean the left frontal sinus. Then, we lowered down the septum and interseptal frontal sinuses. On the left side, we went through the maxilla to perform a maxillectomy to remove the tumor section present in the ethmoid. The patient was treated with 59.4 Gy in a total of 33 fractions and it is divided into Phases I and II. Phase I is 54 Gy in 30 fractions. Phase II is 5.4 Gy in 3 fractions. There was moderate blood loss (about 1200 ml), but no postoperative complications arose.
One-month postoperative, the patient did MRI [Figure 3]a and [Figure 3]b, which showed that the nasal cavity and nasopharynx tumor has been widely removed. However, it was observed that the nasal cavity, right maxillary sinus, and the ethmoid air cells were deformed, and the nasal septum was partially resected. The remaining ethmoid air cells were bilaterally filled with fluid/blood, and some thickening and enhancement of the mucosa were observed. Furthermore, there was also an enhancement in the nasal cavity and septum as well as nasopharynx along the surgical bed. These changes were probably due to postoperative changes, but the presence of an underlying residual tumor could not be excluded nor confirmed at this stage. The bilateral maxillary and sphenoid sinuses were filled with fluid and blood. There was a mucosal thickening in both maxillary sinuses. In contrast, the intracranial site was unchanged, and the only alterations observed were due to multiple old ischemic lesions. Six months later, the patient reported no watery discharge and absences of postoperative nasal obstruction.
|Figure 3: Postoperative CT scan and magnetic resonance imaging of the brain and sinuses. Coronal view. (a) It can be seen that the tumor was widely resected. Postoperative CT scan and magnetic resonance imaging of the brain and sinuses transverse T1 view. (b) It can be seen that the tumor was widely resected|
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| Discussion|| |
ONB manifestations are classified as nasal, neurological, oral, facial, and ophthalmological. The most common symptoms are unilateral nasal obstruction (70%) and epistaxis (46%)., Other symptoms are elevated cortisol in serum, blood glucose, and 24-h urinary cortisol levels as well as high adrenocorticotropic hormone levels. Unusual symptoms include severe proximal muscle weakness, lethargy, and lower extremity edema.
Kadish et al. established a clinical staging system in 1975 for ONB [Table 1]. At stage A, the tumor is confounded to the nasal cavity; at stage B, the tumor extends to the paranasal sinus; and at stage C, the tumor extends to the brain tissues. Moreover, Morita et al. added stage D to the system, to include a regional or distal metastasis. Thus, this staging system was extremely helpful to categorize ONB and is still widely used. In contrast, Hyams developed a pathological staging system to classify ONB, with up to four grades. Grade 1 is considered a mild disease, whereas Grade IV is considered a severe form of ONB. A total of 62% of ONB cases are classified as Grades I and II on the Hyams staging system,, and Malouf et al. noticed that patients who had a high-grade ONB also had larger tumor and lymph node involvement., Christopher et al. revised both staging systems: the Kadish and Hyams; they found that both advanced Kadish and Hyams staging predicted a poorer patient outcome and, thus, recommended aggressive approaches for treatment. A study recently published showed ONB metastasis to the submandibular area.
Another staging system was proposed by Dulguerov et al., and it is based on a tumor, lymph node, and metastasis approach and uses computed tomography (CT) and MRI. It can be used on patients before and after surgery.
Originally, lateral rhinotomy was the only available treatment for ONB, but later, a new treatment protocol was proposed, which consisted of craniofacial surgery, followed by RT. Nevertheless, the most suitable treatment is still under debate. Recently, new studies have suggested that a minimally invasive approach, such as endoscopic surgery, is better because it reduces surgery duration, complications, and hospital stay period., Folbe et al. conducted a large multicentered study evaluating the efficacy of an endoscopic approach of ONB; in comparison with traditional approaches, they found similar results only when the procedure was done in certain patients by an experienced hand. However, it also provides magnified multiangled views of the tumor and lowers the morbidity. Suriano et al. reported that endoscopic resection was related to a lower morbidity. Moreover, endoscopic surgery is a feasible treatment for advanced ONB (advanced according to the Kadish staging system).,
In contrast, Draf III, also known as modified endoscopic Lothrop procedure, was described in the mid-1990s; it provides access to frontal sinuses by median drainage and removal of inferior and superior structures on the frontal sinus as well as the removal of the inferior part of the interferential septum.
Domenico et al. assessed treatment characteristics and the outcomes of ONB in a tabular form, where they included several papers from Dulguerov and Calcateraa (1992) to Lapierre et al. (2016). Dulguerov and Calcaterra reported that craniofacial resection was favorable among patients. Furthermore, they found that 93% (of the 12 subjects) were disease-free when they combined surgery and radiation. John Hopkin's experience involved managing ONB with craniofacial resection with complete tumor resection and using a chemotherapy treatment with compounds such as cisplatin in advanced disease palliation. Alotaibi et al. studied single-center experiences on ONB and found that surgery with adjuvant radiation therapy is the best treatment modality.
Of late, there is a consensus that RT is important for eradicating tumors. However, the optimal RT volume is difficult to determine in most patients, and radiation dose varies according to literature. The reference doses of RT are 45 Gy preoperative and 50–60 Gy postoperative, and doses between 65 and 70 Gy should be given for definitive RT. Spaulding et al. advocated the use of preoperative RT of 50 Gy and craniofacial surgery for ONB in stages A and B tumors according to the Kadish staging system, whereas Broich and Urdaneta recommended using surgery and RT for all stages of Kadish's system.,
Survival of the patient is crucial, and it is one of the most important determiners when choosing a treatment. Eden et al. reported no significant difference in patient survival when using preoperative and postoperative RT, but they noticed an improvement in local tumor control. Nevertheless, there are clear data in the literature that confirms that surgery, followed by RT increases the survival rate. For example, Kim et al. found that patients who were treated with surgery, followed by RT exhibited a 46.7% 5-year progression-free survival (PFS) compared to the 5-year PFS of 19.4% of patients who were treated with surgery alone.
In the last 10 years, the role of chemotherapy in ONB has been studied. Bernold et al. used chemotherapy as adjunctive for severe cases. They reported that the survival rate and local occurrence were not significantly affected, but they consider that both the knowledge and use of chemotherapy in ONB will be expanded in the future.
In this case report, we carefully studied the patient history, considered different alternatives for the treatment, and finally opted for an aggressive approach with endoscopic surgery, followed by extensive RT. We think this approach need for further studies and reviews.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
We would like to extend our gratitude to the King Abdullah International Medical Research Centre, Pathology Department and Radiology Department in KAMC for guidance and cooperation.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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