Objective: To evaluate the efficacy of neoadjuvant chemotherapy (NACT) in the treatment of locally advanced olfactory neuroblastoma (ONB), and to explore the factors related to the efficacy of NACT. Methods: A total of 25 patients with ONB who underwent NACT in Beijing TongRen Hospital from April 2017 to July 2022 were retrospectively analyzed. There were 16 males and 9 females, with an average age of 44.9 years (ranged 26-72 years). There were 22 cases of Kadish stage C and 3 cases of stage D. After multiple disciplinary team(MDT) discussion, all patients were treated sequentially with NACT-surgery-radiotherapy. Among them, 17 cases were treated with taxol, cis-platinum and etoposide (TEP), 4 cases with taxol, nedaplatin and ifosfamide (TPI), 3 cases with TP, while 1 case with EP. SPSS 25.0 software was used for statistical analysis, and survival analyses were calculated based on the Kaplan-Meier method. Results: The overall response rate of NACT was 32% (8/25). Subsequently, 21 patients underwent extended endoscopic surgery and 4 patients underwent combined cranial-nasal approach. Three patients with stage D disease underwent cervical lymph node dissection. All patients received postoperative radiotherapy. The mean follow-up time was 44.2 months (ranged 6-67 months). The 5-year overall survival rate was 100.0%, and the 5-year disease-free survival rates was 94.4%. Before NACT, Ki-67 index was 60% (50%, 90%), while Ki-67 index was 20% (3%, 30%) after chemotherapy [M (Q₁, Q₃)]. The change of Ki-67 before and after NACT was statistically significant (Z=-24.24, P<0.05). The effects of age, gender, history of surgery, Hyams grade, Ki-67 index and chemotherapy regimen to NACT were analyzed. Ki-67 index≥25% and high Hyams grade were related to the efficacy of NACT (all P<0.05). Conclusions: NACT could reduce Ki-67 index in ONBs. High Ki-67 index and Hyams grade are clinical indicators sensitive to the efficacy of NACT. NACT-surgery-radiotherapy is effective for patients with locally advanced ONB.
Male ; Female ; Humans ; Adult ; Middle Aged ; Aged ; Neoadjuvant Therapy/methods* ; Retrospective Studies ; Esthesioneuroblastoma, Olfactory/etiology* ; Ki-67 Antigen ; Paclitaxel ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Nasal Cavity ; Nose Neoplasms/therapy* ; Neoplasm Staging

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

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

Neuroblastomas are sometimes associated with abnormal constitutional karyotypes, but the XYY karyotype has been rarely described in neuroblastomas. Here, we report a case of an esthesioneuroblastoma in a boy with a 47, XYY karyotype. A 6-year-old boy was admitted to our hospital because of nasal obstruction and palpable cervical lymph node, which he first noticed several days previously. A polypoid mass in the right nasal cavity was detected through sinuscopy. Biopsy of the right nasal polyp was performed. Based on the result, the patient was diagnosed with a high-grade esthesioneuroblastoma. Nuclear imaging revealed increased uptake in both the right posterior nasal cavity and the right cervical IB-II space, suggesting metastatic lymph nodes. Cytogenetic analysis revealed a 47, XYY karyotype. Twelve courses of concurrent chemotherapy were administered. Three years after the completion of chemotherapy, the patient had had no disease recurrence. He manifested behavioral violence and temper tantrums, so we started methylphenidate for correction of the behavior.
Biopsy ; Child ; Chromosome Aberrations ; Cytogenetic Analysis ; Drug Therapy ; Esthesioneuroblastoma, Olfactory* ; Humans ; Karyotype ; Lymph Nodes ; Male* ; Methylphenidate ; Nasal Cavity ; Nasal Obstruction ; Nasal Polyps ; Neuroblastoma ; Recurrence ; Violence ; XYY Karyotype*

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 review the diagnosis, treatment and prognosis of olfactory neuroblastoma.

METHODSClinical data were retrospectively reviewed in 10 cases of olfactory neuroblastoma admitted from 1998 to 2002, including data of transmission electron microscopic (TEM) observation in 4 cases. According to Kadish's classification, 2 cases were in stage A, 4 in stage B and 4 in stage C. Three patients were treated with surgery alone, 7 with combined surgery and radiation.

RESULTAmong 10 cases, the overall 5-year survival rate was 60 %(6/10); 3 patients died from local recurrence, 1 lost follow-up. TEM demonstrated granules in the cytoplasm of 3 patients.

CONCLUSIONThe combined surgery and radiation can achieve excellent local control. Transmission electron microscope is important for its diagnosis.

Adult ; Aged ; Combined Modality Therapy ; Esthesioneuroblastoma, Olfactory ; diagnosis ; pathology ; radiotherapy ; surgery ; Female ; Humans ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; pathology ; radiotherapy ; surgery ; Retrospective Studies

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*

OBJECTIVETo investigate the clinical feature, prognostic factors and the appropriate treatment modality of esthesioneuroblastoma (ENB).

METHODSThe data of 49 patients with ENB treated from Dec. 1978 to Dec. 2001 were retrospectively reviewed and analyzed. In this series, 3 patients had modified Kadish stage A disease, 15 stage B, 22 stage C, and 9 stage D lesion. The treatment modalities included surgery alone in 4 patients, and radiotherapy alone in 11, surgery plus radiotherapy in 19, radiotherapy plus chemotherapy in 8, surgery plus radiotherapy plus chemotherapy in 7. Statistic analysis was performed using software SPSS 10.0. Overall survival (OS) and disease free survival (DFS) were calculated using Kaplan-Meier method. Differences between survival curves were tested by Log rank method.

RESULTSThe 5-year OS and DFS of the whole group was 60.5% and 41.9%, respectively. The 5-year OS of patients with modified Kadish stage A or B disease and those with stage C or D was 78.4% and 49.7% (chi(2) = 2.10, P = 0.15), and the 5-year DFS was 47.1% and 38.4% (chi(2) = 0.08, P = 0.78), respectively. The 5-year OS of patients with or without neck lymph nodal metastasis was 17.8% and 70.8% (chi(2) = 2.32, P = 0.13), and the 5-year DFS was 0 and 53.4% (chi(2) = 11.67, P < 0.01), respectively. For patients with kfs > or = 80 and those with kfs < 80, the 5-year OS was 69.0% and 30.1% (chi(2) = 7.01, P < 0.01), and 5-year DFS was 46.7% and 24.9% (chi(2) = 6.37, P = 0.01), respectively. As regard to the treatment modality, The 5-year OS was 69.7% for the patients treated with combined modalities and 46.3% for those with surgery alone or radiotherapy alone (chi(2) = 3.49, P = 0.06), and the 5-year DFS were 52.2% and 21.8% (chi(2) = 7.03, P < 0.01), respectively. The 5-year OS was 71.1% for patients who received surgical treatment and 44.6% for those without it (chi(2) = 7.99, P < 0.01), and 5-year DFS was 54.0% and 24.1% (chi(2) = 6.41, P = 0.01), respectively. The 5-year OS and DFS of 11 patients who received radiotherapy alone were 47.7% and 30.7%, respectively. For 19 patients treated by radiotherapy with radical purpose (including patients who received combined modality with radiotherapy plus chemotherapy), the 5-year OS were 33.9% for < 70 Gy patients and 48.0% for > or = 70 Gy (chi(2) = 0.89, P = 0.35), and the 5-year DFS was 13.3% and 33.3% (chi (2) = 4.48, P = 0.03), respectively. For those who received chemotherapy or not, the 5-year OS was 50.0% and 64.9% (chi(2) = 0.91, P = 0.34), and the 5-year DFS was 38.9% and 43.1% (chi(2) = 0.01, P = 0.91), respectively.

CONCLUSIONEsthesioneuroblastoma is more prevalent in the young male adults than female, usually with locally advanced stage lesion when first diagnosed. Performance status and neck lymph node metastasis are significantly correlated with the prognosis. Combined treatment modality consisting of surgery may help to gain more favorable result. Radiotherapy plays an important role in the management of the disease.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Combined Modality Therapy ; Esthesioneuroblastoma, Olfactory ; radiotherapy ; surgery ; Female ; Humans ; Lymphatic Metastasis ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; radiotherapy ; surgery ; Prognosis ; Treatment Outcome

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