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

Humans ; Consensus ; East Asian People ; Esthesioneuroblastoma, Olfactory/pathology* ; Nasal Cavity ; Nose Neoplasms/pathology*

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

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

Esthesioneuroblastoma as a source of ectopic Cushing's syndrome is rare, and to the best of our knowledge, only 20 cases have been reported worldwide. A 46-year-old healthy man visited a local clinic for general weakness and hyposmia, and underwent examination with serial endocrinological workup and brain imaging. ⁶⁸Gallium-DOTA-TOC positron emission tomography scan was helpful where diagnosis of sellar MRI and inferior petrosal sinus sampling were discordant. Combined transcranial and endoscopic endonasal approach surgery was performed, and a diagnosis of esthesioneuroblastoma was given.
Cushing Syndrome ; Diagnosis ; Esthesioneuroblastoma, Olfactory ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neuroimaging ; Petrosal Sinus Sampling ; Positron-Emission Tomography

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*

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

OBJECTIVE: To assess the long-term clinical efficacy of endoscopic surgery of primary sinonasal malignant neoplasms and find out the potential postoperative prognostic factors. METHOD: Forty-three clinical cases of primary sinonasal malignant neoplasms treated under endoscopy were analyzed retrospectively. RESULT: Fifteen patients died. The 1-year, 2-year, 3-year and 5-year survival rates were 83. 7% (36/43), 74. 4% (32/43), 69. 8% (30/43) and 65. 1% (28/43), respectively. Kaplan-Meier single-variable analysis showed that gender, T grade, TNM stage and pathological types of olfactory neuroblastoma were statistically significant prognostic factors (P< 0. 05). COX Pro-Portional hazard models showed that TNM stage was an independent prognostic factors. CONCLUSION Endoscopic surgery for primary sinonasal malignant neoplasms is a safe and effective minimally invasive surgical treatment, and it is an important supplement to the traditional surgery. Gender, T grade, TNM stage and pathological types of olfactory neuroblastoma might be significant prognostic factors.
Endoscopy ; Esthesioneuroblastoma, Olfactory ; surgery ; Humans ; Nasal Cavity ; pathology ; surgery ; Nose Neoplasms ; surgery ; Proportional Hazards Models ; Retrospective Studies ; Survival Rate ; Treatment Outcome

Clinical records of a patient with olfactory neuroblastoma presented with hyponatremia as initial symptoms were analyzed and the literatures were reviewed. At initial onset, the patient presented with hyponatremia. After pathological examination, the diagnosis was olfactory neuroblastoma. The blood sodium has been normal after operation and radiotherapy. The incidence rate of olfactory neuroblastoma is low, and it is easily misdiagnosed. Its diagnosis relies on pathological examination. We should pay more attention to the unspecific symptoms of patients with hyponatremia, which can help to improve early diagnosis and the prognosis.
Esthesioneuroblastoma, Olfactory ; complications ; pathology ; Humans ; Hyponatremia ; etiology ; Nasal Cavity ; pathology ; Nose Neoplasms ; complications ; pathology ; Prognosis

OBJECTIVE: To study the clinicopathologic features, immunophenotype, diagnosis and differential diagnosis of Sinonasal teratocarcinosarcoma (SNTCS). METHOD: The clinical findings, morphologic features and immunohistochemical markers in one case of SNTCS were studied, and the relevant literatures were reviewed. RESULT: The Tumor tissue is composed of three layers, with mature and immature squamous epithelium nests, neural epithelial cells and olfactory neuroblastoma-like cells derived of ectoderm; Sarcomatoid components and bone tissue derived of mesoderm; The glandular and tubular structures part of which is adenocarcinoma and respiratory epithelium derived of endoderm; The fetal clear cell squamous epithelium is typical. In addition, diffuse large cytoplasm-with high light and cytoplasm with dark light has no obviously boundery. Immunohistochemical staining showed immune markers of different germ layers corresponding, squamous epithelium, glandular epithelium and respiratory epithelium were positive for CK and EMA, neural epithelial cells and olfactory neuroblastoma-like cells were positive for S-100, NSE and Syn, sarcomatoid area was positive for Vim, light dye area was positive for Vim, CD99 and CK, dark area was positive for NSE and GFAP. CONCLUSION SNTCS is a rare malignant tumor with the features of teratoma and carcinosarcoma, its histopathological and immunohistochemical features were typical, should be more drawn and sliced to avoid misdiagnosis and missed diagnosis.
Adenocarcinoma ; Carcinosarcoma ; diagnosis ; immunology ; pathology ; Diagnosis, Differential ; Esthesioneuroblastoma, Olfactory ; diagnosis ; immunology ; pathology ; Humans ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; immunology ; pathology ; Teratoma ; diagnosis ; immunology ; pathology