Background: Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a rare malignant tumor that presents as a locally aggressive disease. It accounts for an incidence of 0.4 per million population affecting men and women equally in all ages. As olfactory neuroblastoma is rare, having an intracranial extension is even more unusual. Only a few cases have been reported in literature, hence, there is no widely accepted standard of care. Case Presentation: This is a case of a 33-year-old female who presented with a 4-month history of nasal congestion which progressed to a rapidly growing nasal mass and bilateral proptosis. She soon became bedridden and exhibited signs of increased intracranial pressure. Imaging revealed a heterogeneously enhancing nasal cavity mass with intracranial extension. Biopsy and immunohistochemistry confirmed the olfactory neuroblastoma. She subsequently underwent chemotherapy and radiotherapy since the tumor was unresectable. In a span of three months, after only three cycles of chemotherapy with cisplatin and etoposide and thirty fractions of radiation therapy, the patient significantly improved from being completely bedridden to an ambulatory individual capable of self-care. We completed eight cycles of chemotherapy and referred to surgical specialists for possible resectability. However, the patient did not consent for surgery and opted to have palliative care. Conclusion Most cases of olfactory neuroblastoma are treated through combination therapy. The patient significantly improved from the administration of chemotherapy with cisplatin and etoposide plus radiotherapy. This case report shows the significance of chemotherapy with radiotherapy as the treatment of choice for late-stage olfactory neuroblastoma in which surgery is not amenable.
Esthesioneuroblastoma, Olfactory ; Drug Therapy ; Radiotherapy

Irradiation of the posterior segment of the eye resulting from radiotherapy either of local lesions or of extraocular lesions of the adjacent regions(carcinoma of the antrum and nose) has been known to produce delayed retinal vascular changes which develop many months or years after actual irradiation, and may cause disturbances of vision. The authors experienced a case of radiation retinopathy OS in a 37 year old male who received radiotherapy for olfactory neuroblastoma about 9 months ago.
Adult ; Esthesioneuroblastoma, Olfactory ; Humans ; Male ; Radiotherapy ; Retinaldehyde

Aged ; Diabetes Insipidus ; etiology ; Esthesioneuroblastoma, Olfactory ; complications ; Female ; Humans

This 57 year-old woman presented with a seizure. She had a history of attending the ENT and neurosurgical departments for more than a decade. At the time of her initial presentation many years prior, her main complaint was of nasal congestion. A nasopharyngeal biopsy confirmed an olfactory neuroblastoma. Olfactory neuroblastoma is an uncommon slow growing tumour of the nasal cavity with no established etiological basis. With a neuroectodermal origin, it arises from the olfactory epithelium of the upper nasal cavity.1 Most cases arise from the cribriform plate, upper third of the nasal septum, superior turbinates or anterior ethmoidal air cells. However, it typically presents late when multiple structures are involved, which may include the orbits and intracranial compartments.2 Accounting for approximately 2% of sinonasal tumors, although often late to present, ironically only a minority of patients experience anosmia.3 The commonest complaint at initial presentation is nasal blockage accounting for nearly a quarter of cases, with headache and epistaxis the next most frequent symptoms.1 Multi-modality imaging is essential in that the most recognized management of this infrequent tumor is a combination of craniofacial surgery and radiotherapy. The imaging pathway in this case was typical, with CT and MRI complementing each other in maximizing tumor delineation. Computed Tomography has superior definition is reviewing bony involvement which is a typical finding, whereas MRI has superiority in evaluating the extent of soft tissue invasion and establishing tumor boundaries against post obstruction fluid in the paranasal sinuses.3 In this case the CT illustrates the gross destruction of the skull base, orbital and sinus margins. (Figure 1-4) The MRI outlines the extension of disease involving the pituitary fossa, brainstem and frontal sinus invasion. (Figures 5 and 6)
Human ; Female ; Middle Aged ; Neuroblastoma ; Women ; Nasal Cavity ; Esthesioneuroblastoma, Olfactory

Olfactory neuroblastoma is a rare malignant tumor of the nasal cavity arising from the olfactory neuroepithelium. It usually present as a polypoid mass in the superior nasal cavity including the cribriform plate, superior turbinate, and superior portion of nasal septum. The development of olfactory neuroblastoma outside of the region, in which olfactory epithelium exists, is exceedingly rare. In this report, we present an interesting case of an isolated sphenoid sinus olfactory neuroblastoma with a brief review of the literature.
Esthesioneuroblastoma, Olfactory ; Ethmoid Bone ; Nasal Cavity ; Nasal Septum ; Olfactory Mucosa ; Sphenoid Sinus ; Turbinates

Olfactory neuroblastoma is a rare, malignant neoplasm arising from the olfactory epithelium. It has an aggressive biological behavior that is characterized by local recurrence, atypical distant metastasis, and poor long-term prognosis. The incidence of cervical lymph node metastasis in olfactory neuroblastoma is variable, and treatment modalities are controversial. Moreover, few reports have been published concerning retropharyngeal lymph node metastasis from olfactory neuroblastoma. We present two cases of olfactory neuroblastoma with retropharyngeal lymph node metastasis. In addition, we provided a review of the current literature regarding olfactory neuroblastoma and retropharyngeal lymph node metastasis from olfactory neuroblastoma.
Esthesioneuroblastoma, Olfactory* ; Incidence ; Lymph Nodes* ; Neoplasm Metastasis* ; Olfactory Mucosa ; Prognosis ; Recurrence

Olfactory neuroblastoma is an uncommon malignant tumor(just over 300 cases have been reported in the medical literature by 1996) arising within the nasal cavity. This tumor has a neuroectodermal cell origin and is believed to specifically arise from the olfactory epithelium. The tumor was first described in the French medical literature by Berger and associates in 1924 by the name esthesioneuroepitheliome olfactif. The english version of this term is esthesioneuroblastoma, and current medical literature uses this name interchangeably with olfactory neuroblastoma. We experienced a case of olfactory neuroblastoma in the maxillary sinus. We treated this patient with tumor resection, chmotherapy and radiotherapy. And now, the patient has been following up for 2years 6month, but olfactory neuroblastoma is very rare. So we report this case with a review of literature.]]>
Esthesioneuroblastoma, Olfactory ; Humans ; Maxillary Sinus ; Nasal Cavity ; Neural Plate ; Olfactory Mucosa ; Radiotherapy

Schwannoma is a benign, slowly growing nerve sheath tumor which can arise from any peripheral nerve containing schwann cells. The olfactory and optic nerves, the extentions of the central nerve system, are encased by glial cells and they lack of schwann cells. They do not give rise to schwannomas in the head and neck area. Therefore, it is generally known that schwannomas seldom occur in the nasal cavity occupied by the olfactory nerve. We have experienced a tumor that developed in the upper nasal cavity including the olfactory cleft and ethmoid sinus. Based on the location of tumor development, we initially diagnosed it as an olfactory neuroblastoma, and its biopsy results reported the tumor to be a schwannoma in the nasal cavity. The tumor was completely removed through craniofacial resection. We herein report a case of an olfactory schwannoma with a brief review of the literature in order to alert other clinicians to this rare tumor to expedite appropriate management.
Biopsy ; Esthesioneuroblastoma, Olfactory* ; Ethmoid Sinus ; Head ; Nasal Cavity* ; Neck ; Neurilemmoma* ; Neuroglia ; Olfactory Nerve ; Optic Nerve ; Peripheral Nerves ; Schwann Cells

PURPOSE: Rarity of olfactory neuroblastoma makes it difficult for treating physician to practice with a consistent protocol. This study is performed to analyze our experience with various treatment modalities for patients with olfactory neuroblastoma. Discussion includes review of some recently published literatures. MATERIALS AND METHODS: Between June of 1979 and April of 1997, 20 patients were treated under the diagnosis of olfactory neuroblastoma at Seoul National University Hospital. There were 14 male and 6 female patients. Age at initial treatment ranged from 13 to 77 years with median of 24 years. Fifteen of 20 patients had Kadish stage C. They were treated with various combinations of surgery, radiation therapy and chemotherapy; surgery + postoperative radiation therapy + adjuvant chemotherapy for 2 patients, surgery + postoperative radiation therapy for 6, neoadjuvant chemotherapy + surgery for 1, surgery + adjuvant chemotherapy for 1, surgery only for 2, neoadjuvant chemotherapy + radiation therapy for 3, radiation therapy + adjuvant chemotherapy for 1, radiation therapy only for 3, and no treatment for 1 patient. RESULTS:Follow-up ranged from 2 month to 204 months with mean of 39.6 months. The overall 5- and 10-year survival rates are 20% and 10%, respectably. Four patients are alive at the time of data analysis. One of four living patients was treated with radical surgery, postoperative radiation therapy and adjuvant chemotherapy, two patients with radical surgery and postoperative radiation therapy, and one with radical surgery only. CONCLUSIONS: Multidisciplinary approach, including radical surgery, pre- or post-operative radiation therapy and chemotherapy, should be addressed at the initial time of diagnosis. Although limited by small number of the patients, this study suggests importance of local treatment modality, especially radical surgery in the treatment of olfactory neuroblastoma.
Chemotherapy, Adjuvant ; Diagnosis ; Drug Therapy ; Esthesioneuroblastoma, Olfactory* ; Female ; Humans ; Male ; Seoul ; Statistics as Topic ; Survival Rate

We report a case of esthesioneuroblastoma with intracranial extension treated with surgical resection and chemotherapy. A 5-year-old child presented with visual disturbance, and was subsequently found to have a huge sized mass in the anterior skull base, suprasellar region and lateral ventricle. A bifrontal craniotomy with excision of the tumor through subfrontal and interhemispheric approach was performed. The patient received postoperative chemotherapy according to Tandem protocol. A follow-up magnetic resonance(MR) image showed that the tumor showed partial regression, however, there was no change in tumor size in lateral ventricle. Second operation with excision of remnant mass through a transcallosal approach was performed. MR image, 9 months after first operation showed the postoperative change in frontal area without enhancing mass. By employing staged operation and chemotherapy, the authors obtained good results in the treatment of esthesioneuroblastoma with intracranial extension.
Child ; Child, Preschool ; Craniotomy ; Drug Therapy ; Esthesioneuroblastoma, Olfactory* ; Follow-Up Studies ; Humans ; Lateral Ventricles ; Skull Base