Kadish C期嗅神经母细胞瘤行鼻内镜手术切除及综合治疗的临床分析

目的回顾性分析Kadish C期嗅神经母细胞瘤行鼻内镜手术切除及综合治疗的效果,探讨手术适应证、方法及围术期管理经验。方法收集2004年12月—2018年9月首都医科大学附属北京同仁医院接受鼻内镜手术并证实为Kadish C期嗅神经母细胞瘤患者17例的临床资料。其中男13例,女4例;年龄26～67岁,平均45.4岁。所有患者术前均行鼻窦CT和 MRI、颈部超声及胸部CT检查。采用Kaplan Meier法进行生存分析、计算总体生存率和无瘤生存率。结果17例患者中初次手术11例,术后复发再次手术6例。所有患者均行鼻内镜手术切除,１２例患者行颅底重建,其中1０患者切除硬脑膜和嗅球。术后病理确诊为嗅神经母细胞瘤,病理采用Hyams评分系统进行病理分级,Ⅱ级9例,Ⅲ级8例。１６例辅助放疗;８例辅助化疗,其中４例接受术前诱导化疗。无严重手术并发症;随访8~109个月,平均随访44.2个月,失访1例,复发和/或转移4例。死亡3例,均死于肿瘤脑转移。1年和5年总体生存率为94.1%和80.7%,1年和5年无瘤生存率为76.5%。结论Kadish C嗅神经母细胞瘤采用鼻内镜切除加术后辅助综合治疗可以获得满意的5年生存率;术者经验和技术及多层颅底重建修复是手术成功的关键。     

嗅神经母细胞瘤

嗅神经母细胞瘤是一种罕见的，多发于鼻腔、鼻窦及颅底的肿瘤。目前，还没有公认的预后评价和标准治疗方案。章根据Kadish分期法对诊断和治疗的26例嗅神经母细胞瘤患，采取跨学科的多形式治疗，并对晚期肿瘤患进行了针对性的挽救治疗，明显提高长期生存率。根据Hyams分类法，发现肿瘤分期和组织病理学分级是决定生存率和预后的重要因素。     

嗅神经母细胞瘤误诊原因分析

目的：探讨嗅神经母细胞瘤的误诊原因。方法：回顾分析1993年1月至2004年12月收治的24例嗅神经母细胞瘤的临床资料，主要包括临床诊断、影像学术前诊断、术前术后病理学诊断。结果：24例中男14例、女10例，4．70岁，平均37．92岁，病程10d-19个月，平均6个月。Kadish分期：A期4例，B期7例，C期13例。临床误诊13例（54．16％），分别为鼻息肉5例（20．83％），鼻窦炎3例（12．50％）、淋巴瘤2例（8．33％）、视神经萎缩1例（4．17％）、血管球瘤1例（4．17％）。术前影像与实际不符16例（66．67％）。病理误诊6例（25．00％），分别为胚胎性横纹肌肉瘤2例（8．33％）、原始神经外胚叶肿瘤2例（8．33％）、浆细胞肉瘤1例（4．17％）。病理诊断不明确为小细胞恶性肿瘤1例（4．17％）。结论：对有鼻塞或鼻出血并伴有鼻腔顶部可疑新生物者要警惕本病；CT及MRI显示中心位于鼻腔顶部和筛窦，密度较均匀的大片状组织肿块，侵犯邻近结构（眼眶、颅内），筛板及眼眶内侧壁被侵蚀性破坏，应首先考虑嗅神经母细胞瘤；对临床可疑而初次病理诊断为非本病的病例，应反复取活检并结合形态学特点及免疫组化结果尽早确诊。     

成人嗅神经母细胞瘤20例综合治疗结果分析

目的 回顾性分析与比较20例经综合治疗的嗅神经母细胞瘤患者的生存差异,探讨最佳治疗策略.方法 1998年1月至2005年1月中山大学肿瘤防治中心采用综合疗法治疗了≥14岁的嗅神经母细胞瘤患者20例,6例为Kadish B期,14例为Kadish C期.12例采用诱导化疗联合放疗,8例为手术联合放疗.以Kaplan-Meier法统计患者的生存率和无瘤生存率,采用Log-rank法比较两种治疗方法 的生存率差异,Fisher精确概率法检验其组间均衡性.结果 自治疗开始之日随访至2008年3月31日,20例综合治疗患者3年生存率50.0%.诱导化疗联合放疗组12例的3年生存率和3年无瘤生存率分别为25.0%和16.7%;手术联合放疗组8例的3年生存率和3年无瘤生存率分别为87.5%和75.0%.手术联合放疗组的生存率和无瘤生存率均优于诱导化疗联合放疗组(X2值分别为6.81和7.33,P值分别为0.0091和0.0068).结论 对于中晚期成人嗅神经母细胞瘤,手术联合放疗的疗效可能优于诱导化疗联合放疗,积极手术联合放疗及化疗的综合治疗策略有望进一步改善其生存率.     

11例Kadish C期嗅神经母细胞瘤临床分析

目的 探讨Kadish C期嗅神经母细胞瘤的治疗及预后.方法 回顾性分析我科1993年1月～2003年12月收治的晚期嗅神经母细胞瘤患者的临床资料,总结治疗经验.结果 11例患者中,男9例,女2例,平均年龄30.91岁;平均病程6.46月.1例因胸腰骶椎转移放弃治疗,其余10例均采用综合治疗,其中7例为手术 放疗,2例为手术 放疗 化疗,1例为手术 化疗.手术方式包括鼻侧切开3例,颅面切除2例,颅鼻内镜联合2例,冠状开颅2例,鼻内镜1例.全组病例中5例复发,复发时间平均33.4月.结论 Kadish C期嗅神经母细胞瘤复发率高,随访时间应长,首次治疗宜采取综合治疗,挽救治疗有效.     

嗅神经母细胞瘤

嗅神经母细胞瘤是一种罕见的,多发于鼻腔、鼻窦及颅底的肿瘤.目前,还没有公认的预后评价和标准治疗方案.文章根据Kadish分期法对诊断和治疗的26例嗅神经母细胞瘤患者,采取跨学科的多形式治疗,并对晚期肿瘤患者进行了针对性的挽救治疗,明显提高长期生存率.根据Hyams分类法,发现肿瘤分期和组织病理学分级是决定生存率和预后的重要因素.     

嗅神经母细胞瘤12例

目的　总结嗅神经母细胞瘤临床资料，探讨临床特点及合理治疗方法。方法　回顾性分析1999年8月～2016年6月间在北京大学第一医院收治的12例嗅神经母细胞瘤患者的临床资料并结合文献分析其临床特征、治疗方式及其预后。男女各6例，年龄30～62岁，中位年龄53.0岁。Kadish分期A、B期各1例，C期10例。治疗方式：手术及放化疗5例，单纯手术治疗7例。结果　随访1～12年，中位随访时间3.5年，1年生存率75.0%（9/12），3年生存率63.6%（7/11）。随访期间死亡6例，Kadish分期均为C期。结论　成人嗅神经母细胞瘤多以头痛、鼻堵及涕中带血为首发症状；Kadish临床分期与预后有关。颈淋巴结转移是最常见的转移途径，对于嗅神经母细胞瘤应重视长期随访。     

经鼻内镜切除嗅神经母细胞瘤疗效观察

目的探讨经鼻内镜切除嗅神经母细胞瘤的疗效及辅助放射治疗的意义。方法对2001年8月至2005年9月经鼻内镜手术治疗的6例鼻腔鼻窦嗅神经母细胞瘤患者的临床特征、治疗效果及失败的原因进行回顾性分析。其中男4例，女2例；1例儿童，5例成人，年龄9—68岁；KadishB期2例，C期4例。所有病例随访14—63个月。结果6例鼻腔鼻窦嗅神经母细胞瘤患者，5例肿瘤完全切除，包括1例先开颅手术，1例颅内侵犯经鼻内镜切除肿瘤并进行颅底修复，1例颈淋巴清扫术，1例次全切除。5例首次术后辅助放疗，均无瘤生存，1例未加放疗手术后复发带瘤生存至31个月死亡，中位生存期44．5个月。结论内镜手术具有手术进路简便，视野清晰，损伤小，术后恢复快，面部不留瘢痕，易为患者接受的优点。初步结果显示该术式是治疗鼻腔鼻窦嗅神经母细胞瘤的有效方法，但术后必须结合放射治疗方可获得满意效果。     

嗅神经母细胞瘤：8例临床分析

我院在1982～1994年间共收治嗅神经母细胞瘤8例。其主要症状为血涕（7/8）和鼻堵（5/8），无颈部淋巴结或其他远处转移，按照Kadish分期：A期1例，B期4例，C期3例。1例（C期）单纯放疗，3个月后死于颅内广泛受浸；7例行鼻侧切开径路种物切除术加术后放疗，现生存1～12年，继续随访。本文就该病的临床表现、病理诊断、治疗和预后进行讨论。     

嗅神经母细胞瘤12例CSCD

目的总结嗅神经母细胞瘤临床资料,探讨临床特点及合理治疗方法。方法回顾性分析1999年8月～2016年6月间在北京大学第一医院收治的12例嗅神经母细胞瘤患者的临床资料并结合文献分析其临床特征、治疗方式及其预后。男女各6例,年龄30～62岁,中位年龄53.0岁。Kadish分期A、B期各1例,C期10例。治疗方式:手术及放化疗5例,单纯手术治疗7例。结果随访1～12年,中位随访时间3.5年,1年生存率75.0%(9/12),3年生存率63.6%(7/11)。随访期间死亡6例,Kadish分期均为C期。结论成人嗅神经母细胞瘤多以头痛、鼻堵及涕中带血为首发症状;Kadish临床分期与预后有关。颈淋巴结转移是最常见的转移途径,对于嗅神经母细胞瘤应重视长期随访。     

Esthesioneuroblastoma: endoscopic nasal and anterior craniotomy resection

OBJECTIVES/HYPOTHESIS: The objective was to illustrate the use of endoscopic techniques as an evolving surgical modality in excision of esthesioneuroblastoma. The authors advocate this method with excision with anterior craniotomy for removal of cribriform plate or anterior cranial fossa tumor extension. STUDY DESIGN: A retrospective chart review of patients with esthesioneuroblastoma treated surgically at a tertiary care institution from 1991 to 2002 using this surgical paradigm. METHODS: Patients were excluded for nonsurgical treatment or nasal and sinus tumor excision by nonendoscopic techniques. Patient demographics, tumor histological findings, presenting signs and symptoms, staging (Kadish and Dulguerov), postoperative complications, adjunct therapy, and recurrence were examined. RESULTS: Seven of 13 patients with esthesioneuroblastoma met all inclusion criteria. The average patient age was 47 years, with a male-to-female distribution of 4 to 3. Patients were grouped by tumor stage using Kadish (stages A, B, and C included three, one, and three patients, respectively) and Dulguerov (stages T1, T2, and T4 included three, two, and two patients, respectively) methods. Average follow-up was 62.3 months. All patients had an endoscopic excision of the nasal and sinus component with anterior craniotomy. The most common postoperative complication was temporary mental status change, which was seen in two patients. There were no postoperative deaths. All patients received radiation therapy, and one also received chemotherapy. Two of the seven patients had recurrences. At last follow-up, six patients had no evidence of disease and one was alive with disease. CONCLUSION: Endoscopic excision of the nasal and sinus component with anterior craniotomy for cribriform or anterior cranial fossa extension is an effective treatment of esthesioneuroblastoma.     

Long-term experiences in the therapy of esthesioneuroblastoma

BACKGROUND: Esthesioneuroblastoma is a rare and clinically variable tumor of nasal sinus and skull base and challenging for modern multidisciplinary therapy. There are no generally known prognostic factors or generally accepted standard therapy regimens. PATIENTS AND METHODS: Between 1975 and 2001 26 patients were treated after the diagnosis of esthesioneuroblastoma was established. The cases were evaluated retrospectively. According to the classification of Kadish 1 patient (4 %) had stage A, 16 patients (53 %) stage B and 11 cases (43 %) had stage C tumors. Hyams grading could be obtained in 22 cases (81 %). Tumors were in 12 patients (52 %) graded I or II and in 10 cases tumors had grade III or IV (48 %). Operative therapy was performed in 23 patients (88.5 %), being in 5 patients a single mode therapy. In 18 cases combined therapy was performed (surgery and radiotherapy or radiotherapy and chemotherapy). RESULTS: Actuarial survival was 61.5 % (16/26). Disease specific 10- and 15-year-survival according to the estimation of Kaplan-Meier was 76.2 %. Patients with small tumors (Kadish A/B) had a 15-year-survival of 86.7 % compared to 63.6 % in cases with advanced tumors (Kadish C). In 7 cases (26.9 %) recurrences developed. Salvage therapy was performed in 5 cases (71.4 %) with a success rate and a 15-year survival each of 60 %. CONCLUSIONS: Therapy of esthesioneuroblastoma is challenging because of rarity and biologic variability of the tumor and and because of lack of a standard therapy. An interdisciplinary multimodal therapeutic approach is necessary especially in case of advanced tumors with promising results. Histopathological grading according to Hyams and tumor stage are important factors for survival and prognosis. Although recurrence can occur with high frequency even after prolonged time interval, long time survival can be improved after aggressive salvage therapy. Therefore longterm follow up is mandatory.     

Esthesioneuroblastoma and sinonasal undifferentiated carcinoma: impact of histological grading and clinical staging on survival and prognosis

OBJECTIVES: Hyams proposed a histological grading system for esthesioneuroblastoma in which grade I tumors have an excellent prognosis and grade IV tumors are uniformly fatal. The Hyams grading system predated advanced craniofacial techniques, extensive use of immunohistochemistry, and the recognition of sinonasal undifferentiated carcinoma (SNUC) as a distinct entity. Therefore we aimed to determine whether Hyams classification is useful in predicting outcome for esthesioneuroblastoma and SNUC. STUDY DESIGN: A retrospective review of cases from 1970 to 1999. METHODS: Twenty-six patients (12 with esthesioneuroblastoma and 14 with SNUC) were reviewed. The Kadish clinical stage was determined, and histopathological slides were reviewed and graded using the Hyams system. RESULTS: Kadish staging was available for 26 patients (2 patients with stage A tumors; 7 with stage B; and 17 with stage C). Of the 8 evaluable patients with Kadish stage A or B tumors, 6 remained disease free for more than 2 years compared with only 5 of the 17 Kadish stage C tumors. Slides were available for Hyams grading in 21 patients (2 patients with grade I tumors; 4 with grade II; 4 with grade III; and 11 with grade IV). Of the 6 patients with Hyams grade I or II tumors, 4 remained disease free for more than 2 years compared with only 4 of the 15 patients with Hyams grade III or IV tumors. Of note, three patients with Kadish stage C tumors (two with esthesioneuroblastoma, one with SNUC) and two patients with Hyams grade IV tumors (one with esthesioneuroblastoma and one with SNUC) survived for more than 5 years. CONCLUSIONS: Both the Hyams grading system and the Kadish staging system can be used as independent predictors of outcome. Although limited by small numbers, the results of this study demonstrate that patients with either advanced clinical stage or pathological grade of esthesioneuroblastoma or SNUC have poor prognosis, but that long-term survival is possible in these patients if aggressive treatment is used.     

Endoscopic craniofacial resection for pediatric esthesioneuroblastoma

Pediatric esthesioneuroblastomas are rare tumors with only about 100 reported cases. Standard surgical treatment is the open craniofacial resection. Pre-treatment imaging is critical for planning, and surgery remains the primary treatment modality followed by adjuvant therapy. We report a 12-year-old female who underwent endoscopic-assisted resection of an extensively calcified Kadish C and Dulguerov–Calcaterra T3N0M0 esthesioneuroblastoma. Following radiation therapy, she is disease-free 18 months post-treatment. Although there are few reported cases in pediatric patients, our case supports the use of endoscopic endonasal approaches for select pediatric skull base malignancies.     

Esthesioneuroblastoma: continued follow-up of a single institution's experience

OBJECTIVES: To analyze outcomes and to provide follow-up for our increasing patient cohort with esthesioneuroblastoma. DESIGN: Retrospective cohort analysis. SETTING: Patients were examined from September 1, 1976, to May 30, 2004, in a tertiary care academic hospital. PATIENTS: Fifty consecutive patients diagnosed as having esthesioneuroblastoma were treated with a standardized protocol during a 28-year period. Patients with tumors staged Kadish A or B received preoperative radiotherapy followed by craniofacial resection, while patients with Kadish stage C disease were treated with preoperative sequential chemotherapy and radiotherapy followed by a craniofacial resection. The mean follow-up is 93 months (range, 1-330 months). RESULTS: The disease-free survival was 86.5% and 82.6% at 5 and 15 years, respectively. There were 17 patients (34%) who developed recurrent disease, most of which was locoregional (12 patients [71%]). There was a long interval to relapse (mean, 6 years), with the longest time to regional recurrence being 10 years. Distant relapses occurred sooner, with poorer outcomes. Of these 17 patients, 7 (41%) underwent successful salvage surgery, while 3 remain alive with disease. CONCLUSIONS: Excellent outcomes for esthesioneuroblastoma are achievable. Long-term follow-up is necessary because of the extended interval for recurrent disease; unlike most sinonasal malignancies, surgical salvage is possible.     

Esthesioneuroblastoma: a population-based analysis of survival and prognostic factors

OBJECTIVES: To investigate the characteristics associated with survival in esthesioneuroblastoma and to determine whether the modified Kadish staging system can predict outcome. DESIGN: Retrospective population-based cohort study. SUBJECTS: All patients in the Surveillance, Epidemiology, and End Results tumor registry diagnosed as having esthesioneuroblastoma (1973-2002). MAIN OUTCOME MEASURES: The modified Kadish stage and the overall and disease-specific survival rates were determined. RESULTS: The cohort included 311 patients with a mean age of 53 years and a unimodal age distribution. The overall 5- and 10-year survival rates were 62.1% and 45.6%, respectively. The modified Kadish staging system was applied to 261 patients. Kaplan-Meier analysis showed the overall and disease-specific survival rates at 10 years to be 83.4% and 90%, respectively, for patients with stage A disease; 49% and 68.3% for patients with stage B disease; 38.6% and 66.7% for patients with stage C disease; and 13.3% and 35.6% for patients with stage D disease. Log-rank test comparisons found Kadish stage (P<.01), treatment modality (P<.002), lymph node status (P<.01), and age at diagnosis (P<.001) to be significant predictors of survival. Cox regression analysis confirmed that Kadish stage remained a significant predictor of disease-specific survival. CONCLUSION: The modified Kadish staging system, lymph node status, treatment modality, and age are useful predictors of survival in patients who present with esthesioneuroblastoma.     

Esthesioneuroblastoma: a Danish demographic study of 40 patients registered between 1978 and 2000

OBJECTIVE: A retrospective review of all diagnosed cases of esthesioneuroblastoma registered in Denmark between 1978 and 2000 was carried out in order to obtain epidemiological data and optimize national treatment guidelines. MATERIAL AND METHODS: Forty cases were verified histologically and included in the analysis Epidemiological and histopathological data were evaluated in relation to the clinical outcome. RESULTS: The 40 cases represent an incidence rate of 0.4 cases/million inhabitants per year. Eight (20%) patients were classified as Kadish stage A, 13 (32.5%) as stage B and 19 (47.5%) as stage C. The histopathological findings were classified according to the grading system of Hyams The median follow-up time was 2.3 years (range 0.3-11.1 years). The 5-year crude survival rate was 61%, with a median survival of 3.1 years (range 0.3-19.2 years). The 5-year disease-free survival rate was 50%, with a median survival of 1.7 years (range 0-19.2 years). Only 3 (7%) patients had positive cervical lymph nodes at presentation. A nationwide consensus regarding treatment was seen in patients classified as Kadish stages A and B. The longest duration before the first recurrence of esthesioneuroblastoma was 5(1/2) years. CONCLUSIONS: The following therapeutic guidelines are suggested: Kadish stage A patients, surgical tumour resection and radiotherapy; Kadish stage B, surgical tumour resection and radiotherapy; Kadish stage C, surgical tumour resection via a craniofacial resection and radiotherapy combined with chemotherapy. Long-term follow-up of esthesioneuroblastoma patients is mandatory.     

Esthesioneuroblastoma: the Northwestern University experience

OBJECTIVE: To review our experience with esthesioneuroblastoma, a rare malignancy of the head and neck. STUDY DESIGN: Retrospective review of Tumor Registry data. METHODS: We performed a computerized search of the Northwestern Memorial Hospital Tumor Registry database from 1981 to 2000. RESULTS: Sixteen patients with esthesioneuroblastoma were identified and analyzed. Their mean age was 42 years. Eleven of 16 patients (69%) had Kadish stage C; 8 patients (50%) had brain involvement at presentation. Craniofacial resection was performed in 13 patients (81%). Fourteen patients received either preoperative or postoperative therapy; radiation therapy was employed in 11 cases and chemotherapy in 4. The actuarial 5-year survival was 60%, and the actuarial 5-year disease-free survival was 33%, with a median follow-up of 4.3 years. Recurrences occurred at a median time of 11 months after diagnosis (2.5 mo-18 y). The first site of failure was locoregional alone in 10 of 12 patients who progressed, and in 6 patients involved the brain or the meninges. Two patients were successfully salvaged. Patients with high-grade tumors had a trend toward work survival. CONCLUSIONS: Esthesioneuroblastoma is a rare tumor that is potentially curable by surgical resection and radiation therapy. However, the rate of local failure is high, and late recurrences are not uncommon. The role of chemotherapy warrants further investigation.     

Esthesioneuroblastoma: a Danish Demographic Study of 40 Patients Registered Between 1978 and 2000

Objective A retrospective review of all diagnosed cases of esthesioneuroblastoma registered in Denmark between 1978 and 2000 was carried out in order to obtain epidemiological data and optimize national treatment guidelines.

Material and Methods Forty cases were verified histologically and included in the analysis Epidemiological and histopathological data were evaluated in relation to the clinical outcome.

Results The 40 cases represent an incidence rate of 0.4 cases/million inhabitants per year. Eight (20%) patients were classified as Kadish stage A, 13 (32.5%) as stage B and 19 (47.5%) as stage C. The histopathological findings were classified according to the grading system of Hyams The median follow-up time was 2.3 years (range 0.3–11.1 years). The 5-year crude survival rate was 61%, with a median survival of 3.1 years (range 0.3–19.2 years). The 5-year disease-free survival rate was 50%, with a median survival of 1.7 years (range 0–19.2 years). Only 3 (7%) patients had positive cervical lymph nodes at presentation. A nationwide consensus regarding treatment was seen in patients classified as Kadish stages A and B. The longest duration before the first recurrence of esthesioneuroblastoma was 5(½) years.

Conclusion The following therapeutic guidelines are suggested: Kadish stage A patients, surgical tumour resection and radiotherapy; Kadish stage B, surgical tumour resection and radiotherapy; Kadish stage C, surgical tumour resection via a craniofacial resection and radiotherapy combined with chemotherapy. Long-term follow-up of esthesioneuroblastoma patients is mandatory.