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

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

The authors report a case of esthesioneuroblastoma with intracranial extension treated by craniofacial resection. The tumor was resected by transbasal approach and repaired the dural defect using pericranial flap. The defect of floor of anterior cranial fossa was repaired with splitted calvarium and pericranial flap. Otorhinolaryngologist removed the residual tumor mass located at paranasal sinuses using lateral rhinotomy. Using cranifacial resection, the authors could remove the mass completely. The patient was referred to hemato-oncologist for chemotherapy.
Cranial Fossa, Anterior ; Drug Therapy ; Esthesioneuroblastoma, Olfactory* ; Humans ; Neoplasm, Residual ; Paranasal Sinuses ; Skull

Objective: To summarize the preliminary experience in the treatment of esthesioneuroblastoma (ENB) and to explore the effect of age, chemotherapy, modified Kadish stage and pathological grade on the prognosis of ENB. Methods: The clinical data of 87 ENB patients from the First Affiliated Hospital of Sun Yat-sen University and Sun Yat-sen University Cancer Center between June 2002 and November 2017 were retrospectively analyzed. The modified Kadish stage was used to evaluate the extent of the lesions, and the Hyams grading system was used for pathological grading. The patients were followed up regularly to evaluate the recurrence and metastasis of the tumor. Cox proportional hazard model was used for univariate and multivariate analyses. Prognostic factors with P<0.05 in univariate analysis were included in multivariate analysis. After controlling the confounding factors, the model coefficients were used to calculate the hazard ratio (HR) and 95% confidence interval (CI). Results: The median follow-up time of ENB patients was 29 months, and the 5-year overall survival rate was 39.3%. In univariate analysis, age, chemotherapy, modified Kadish stage and pathology grade were independent predictors of overall survival, while gender, radiotherapy and surgery were not prognostic factors. Multivariate analysis showed that modified Kadish stage and pathology grade were independent predictors of overall survival rate after excluding confounding factors. Conclusions: Age, chemotherapy, modified Kadish stage and pathological grade are taking important role in the overall survival rate of patients with ENB. Modified Kadish stage and pathological grade are independent predictors of overall survival rate.
Esthesioneuroblastoma, Olfactory/therapy* ; Humans ; Nasal Cavity/pathology* ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Nose Neoplasms/therapy* ; Prognosis ; Retrospective Studies

OBJECTIVETo discuss the clinical characteristics, diagnosis and treatment of esthesioneuroblastoma (ENB).

METHODSThe clinical data of 7 patients with ENB were analyzed retrospectively, and the clinical characteristics, diagnosis, surgical approaches and prognosis of the disease were discussed.

RESULTSThe 7 patients received surgical treatment combined with radiotherapy and chemotherapy. Tumor relapse occurred within one year in two cases, which were treated with a second operation combined with radiotherapy, and one patient died and one survived with tumor after a one-year follow-up.

CONCLUSIONEarly diagnosis of ENB can be difficult. Transnasal-frontal approach with nasal endoscope is effective approach to ENB treatment, and the patients may benefit from postoperative radiotherapy and chemotherapy to improve the prognosis.

Adult ; Esthesioneuroblastoma, Olfactory ; diagnosis ; therapy ; Female ; Humans ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; therapy ; Paranasal Sinus Neoplasms ; diagnosis ; therapy ; Retrospective Studies ; Young Adult

OBJECTIVETo investigate the optimal treatment and prognostic factors of esthesioneuroblastoma (ENB).

METHODForty-nine cases of ENB, received therapies in Chinese people liberation army general hospital from October 2004 to June 2013, were reviewed retrospectively. The therapeutical strategies and prognostic factors were analyzed using the Kaplan-Meier method. Efficacy of different therapeutical strategies was compared.

RESULTThe 5-year overall survival (OS) in the group was 63.8%. The 5-year OS of Kadish stage A, B, C were 90.0%, 77.1%, 32.6%, respectively, with significant difference (χ(2) = 7.379, P = 0.03). The 5-year OS of the patients treated by surgery and radiotherapy, chemoradiotherapy, surgery with chemoradiotherapy were 68.6%, 0, 92.3%, respectively, with significant difference (χ(2) = 8.140, P = 0.02). The 5-year OS of the patients with transnasal endoscopic resection (or combined with transcranial resection) and lateral rhinotomy ( or combined with transcranial resection) were 63.7%, 50.0%, respectively, which had no significant difference (χ(2) = 3.818, P = 0.05).

CONCLUSIONSENB has a relatively good prognosis in head and neck malignancies, the prognosis is associated with clinical stages and treatment approaches. Transnasal endoscopic surgery based multimodality is the preferable choice.

Adolescent ; Adult ; Aged ; Combined Modality Therapy ; Esthesioneuroblastoma, Olfactory ; diagnosis ; therapy ; Female ; Humans ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; therapy ; Prognosis ; Retrospective Studies ; Young Adult

A case of dento-maxillofacial abnormality involving a 10-year-old male patient with a history of esthesioneuroblastoma is presented. This patient had been treated with 54 Gy (60)Co-gamma-radiation to the nasal cavity for 6 weeks and 6 cycles of combination chemotherapy of Cyclophosphamide, Cisplatin, Adriamycin, VM-26 (Teniposide), and DTIC (Dacarbazine) when he was 16 months of age. Five years after cessation of cancer therapy, he was disease free and transferred for extensive dental care to Kyung Hee University Dental Hospital. A clinical and radiologic follow-up over last 4 years showed root stunting, premature closure of the root apices, microdontia, developmental arrest, small crowns, and partial anodontia. Maxillofacial morphology evaluated by cephalometric analysis showed deficiency of maxillary development.
Anodontia ; Child ; Cisplatin ; Crowns ; Cyclophosphamide ; Dacarbazine ; Dental Care ; Doxorubicin ; Drug Therapy* ; Drug Therapy, Combination ; Esthesioneuroblastoma, Olfactory ; Follow-Up Studies ; Humans ; Male ; Nasal Cavity ; Radiotherapy* ; Teniposide

OBJECTIVE: To explore the diagnosis and treatment and prognosis of esthesioneuroblastoma. METHOD: The clinical data of 6 cases with esthesioneuroblastoma were analyzed retrospectively. One case was treated with radiotherapy alone; Four cases were treated with surgery combined with radiotherapy; One case was treated with surgery combined with radiotherapy and chemotherapy. RESULT: All cases have been followed up for one to ten years and the 1-year, 3-year and 5-year survival rate were 100.0% (6/6), 83.3% (5/6) and 66.7% (4/6) respectively. One case has survived without tumor for 8 years. CONCLUSION It was the best way to control local recurrence combined with radical surgery and postoperative radiotherapy, and chemotherapy contributed to the prevention of distance metastasis. The key to improving the survival rate of the disease is early accurate diagnosis and combined treatment.
Adolescent ; Adult ; Combined Modality Therapy ; Esthesioneuroblastoma, Olfactory ; diagnosis ; therapy ; Female ; Humans ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Young Adult

BACKGROUND AND OBJECTIVES: Olfactory neuroblastoma is a rare tumor arising from the olfactory neuroepithelium, for which surgery is the mainstay of treatment; but there are no generally accepted treatment approaches, according to tumor stage. In this study, we present our experiences with this tumor and the results of treatment to consider the clinical analysis and treatment modality. SUBJECTS AND METHOD: All of the patients, who had been treated for olfactory neuroblastoma at Severance hospital during the past ten years, were analyzed retrospectively and each of their diagnosis of olfactory neuroblastoma was histologically confirmed. RESULTS: Eleven patients aged 8 to 70 years were assessed, and 5 year disease-free survival rate were 60%. Sixty-four percent of those patients had received surgical treatment. Craniofacial resection was used mainly as an initial surgical treatment and followed by postoperative radiotherapy in 57% of the patients. If only cervical lymph node metastasis had developed at the initial diagnosis, elective neck treatment including bilateral neck dissection was performed. Chemotherapy was used either as postoperative adjuvant therapy, initial therapy with radiotherapy or salvage therapy, but not as an initial single treatment modality. Forty-five percent of the patients had recurrence and metastasis developed after the initial treatment. In the patients with cervical lymph node metastasis (n=1) and nodal recurrence (n=2), neck dissection was effective in nodal control. CONCLUSION: Complete surgical resection and postoperative radiotherapy were the main treatment modalities of olfactory neuroblastoma. Especially, postoperative radiotherapy is an essential treatment to both early- and advanced-stage olfactory neuroblastoma. Craniofacial resection is a main surgical modality, but in early stage, limited external intervention including endoscopic mass excision can be considered. If the patient has cervical lymph node metastasis or nodal recurrence, radical neck dissection should be performed. Chemotherapy seems to be considered as an adjuvant therapy, not single treatment modality.
Diagnosis ; Disease-Free Survival ; Drug Therapy ; Esthesioneuroblastoma, Olfactory* ; Humans ; Lymph Nodes ; Neck ; Neck Dissection ; Neoplasm Metastasis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Salvage Therapy ; Treatment Outcome*