Esthesioneuroblastoma: the University of Iowa experience 1978-1998

OBJECTIVE: Esthesioneuroblastoma is rare and the best treatment has yet to be defined. The purpose of this study is to analyze the natural history, treatment, and patterns of failure of esthesioneuroblastoma treated at one institution. METHODS: Between 1978 and 1998, 13 patients with esthesioneuroblastoma were identified using the University of Iowa Tumor Registry. All patients were staged according to Kadish criteria. Mean follow-up was 6.3 years. Six patients had 5 or more years of follow-up and four had follow-up exceeding 9.5 years. One patient was lost to follow-up at 36 months. RESULTS: No patients had Kadish stage A disease, five were stage B, and eight stage C. Overall actuarial 5- and 10-year survival rate was 61% and 24%, respectively. Disease-free survival rate at 5 and 10 years was 56% and 42%, respectively. Seven patients have died, three of intercurrent disease and three of disease progression, one with an unknown disease status. Six patients remain alive, three without evidence of disease and three have experienced a local or regional recurrence. Five patients who were initially controlled developed recurrence, three local only, one locoregional, and one regional and distant. Median time to failure was 96 months. All patients with follow-up exceeding 12 years have experienced either a local or regional recurrence. Survival after salvage therapy in these patients ranged from 3 to 12 years. CONCLUSION: Esthesioneuroblastoma has a long natural history characterized by frequent local or regional recurrence after conventional treatment. Successful retreatment can lead to prolonged survival.     

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: 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.     

High incidence of head and neck squamous cell carcinoma in patients with Fanconi anemia

BACKGROUND: Fanconi anemia (FA) is a rare autosomal recessive disorder characterized by a high degree of genomic instability and predisposition to cancer development. Recent evidence suggests that the incidence of head and neck squamous cell carcinoma (HNSCC) may be increased in patients with FA. OBJECTIVE: To determine the cumulative incidence, tumor distribution, and outcome of HNSCC in patients with FA. DESIGN AND SETTING: We analyzed data from 754 subjects from the International Fanconi Anemia Registry, a prospectively collected database of patients with FA. MAIN OUTCOME MEASURES: Cumulative incidence of HNSCC and 2-year overall, relapse-free and disease-specific survival. RESULTS: Of the 754 patients in the International Fanconi Anemia Registry, 19 (3%) had HNSCC. This is a significantly higher incidence of HNSCC compared with that observed in the general population (standardized incidence ratio, 500; 95% confidence interval, 300-781) (P<.001). The patients' age ranged from 15 to 49 years (median, 31 years), and there was a 2:1 female predominance. Surgical treatment was well tolerated (n = 17); however, radiation therapy and chemotherapy were associated with significant morbidity and mortality. Of the 19 patients, 10 (53%) developed locoregional recurrences within a median of 16 months from diagnosis. The median follow-up was 29 months. The 2-year disease-specific, overall, and relapse-free survival rates were 49%, 49%, and 42%, respectively. The cumulative incidence of relapse by the age of 40 years was 50%. CONCLUSIONS: In patients with FA, there is a high incidence of aggressive HNSCC at a young age. Surgery remains the mainstay of treatment because patients with FA tolerate radiation therapy and chemotherapy poorly, with significant morbidity. An increased understanding of FA-associated malignancies is not only important in the clinical management of patients with FA but can also elucidate the role of chromosomal instability in the development of HNSCC in general.     

嗅神经母细胞瘤34例临床治疗经验

目的 探讨嗅神经母细胞瘤的诊断、治疗及预后。方法 回顾分析1958～1998年收治的34例嗅神经母细胞瘤患者的临床资料。根据Kadish分期：A期1例，B期9例，C期24例。治疗方法：单纯手术3例，单纯放射治疗9例，综合治疗22例。综合治疗包括手术结合放射治疗15例，放射治疗结合化学治疗4例，手术结合放射、化学治疗3例。结果 5年生存率为47．1％(16／34)，其中A期100％(1／1)，B期88．9％(8／9)，C期29．2％(7／24)；单纯手术生存率为33．3％(1／3)，单纯放射治疗生存率为33．3％(3／9)，综合治疗生存率为54．5％(12／22)。局部控制率和远处转移率分别为61．8％(21／34)和32．4％(11／34)。10例死于远处转移，5例死于局部复发。早期(A、B期)患者的生存率好于晚期患者(C期)(χ^2=8．174，P=0．004)，年轻患者(≤30岁)的远处转移率较高(χ^2=3．865，P=0．049)，预后较差(χ^2=4．194，P=0．041)。结论 早期发现和综合治疗对改善生存率有重要意义。术前放射治疗加手术有助于提高嗅神经母细胞瘤的局部控制率。远处转移是影响预后的主要因素。     

Factors affecting survival of patients with tumors of the anterior skull base

Fifty-four patients with primary neoplasms of the anterior skull base were treated by craniofacial resection with curative intent. The most common tumor was esthesioneuroblastoma (24), followed by squamous cell carcinoma (10). The overall 2-year and 5-year survivals were 75% and 49%, respectively. High-grade tumors, grades 3 and 4, had a poorer prognosis. Tumor size, dural involvement, sphenoid sinus involvement, age, and sex had no significant influence on survival when examined by multivariate survival analysis. Sixteen complications were noted in the postoperative period. Cerebrospinal fluid leakage occurred in 2 patients and loss of frontal bone occurred in 4. There were no operative or perioperative deaths. Craniofacial resection permits surgical resection of the majority of anterior skull base tumors with acceptable morbidity.     

Intra-arterial cisplatin and concomitant radiation therapy followed by surgery for advanced paranasal sinus cancer

OBJECTIVE: To report the long-term efficacy of a combined regimen of intra-arterial high-dose cisplatin chemotherapy and concomitant radiation therapy followed by organ-sparing surgery when possible in the treatment of advanced paranasal sinus cancer. DESIGN: Review of prospectively collected data. SETTING: Academic referral center.Patients Nineteen patients with advanced paranasal sinus malignancies with a minimum follow-up of 2 years. Malignancies included 14 squamous cell carcinomas (74%), 2 adenocarcinomas (10%), 2 adenoid cystic carcinomas (10%), and 1 undifferentiated carcinoma (5%). Sixteen patients (84%) had T4 disease.Intervention Treatment consisted of preoperative radiation therapy (2.0 Gy/fraction per day; total dose, 50 Gy in 5 weeks) given concomitantly with 3 to 4 weekly infusions of intra-arterial cisplatin (150 mg/m(2) per week) and systemic sodium thiosulfate neutralization. The regimen included planned surgery performed approximately 8 weeks after completion of radiation therapy. Ten patients underwent a transcranial anterior craniofacial resection; 1, a medial maxillectomy; and 1, an endoscopic restaging only. RESULTS: After a median follow-up of 53 months, actuarial overall survival at 2 and 5 years was 68% and 53%, respectively. One patient died of myocardial infarction during treatment. No other treatment-limiting toxic effect was noted. Although 3 patients had persistence of disease, delayed local failure occurred only in 2 and distant metastasis in 3. Except for cataract in 2 patients, no visual loss developed. CONCLUSION: Despite the advanced stage and unfavorable nature of cancer in this cohort, our results indicate that this regimen holds promise and merits further study.     

Nasopharyngectomy for recurrent nasopharyngeal carcinoma: a review of 53 patients and prognostic factors

CONCLUSIONS: Salvage surgery is a justified treatment for primary recurrence of nasopharyngeal carcinoma (NPC). Skull base surgery can play a role in rescuing patients with more advanced local recurrence of NPC. OBJECTIVES: The purpose of this study was to report the local control and overall survival outcome of patients with (NPC) with local failure who received salvage nasopharyngectomy and to identify prognostic factors. PATIENTS AND METHODS: Fifty-three consecutive patients who had primary recurrence of NPC and underwent salvage surgery with curative intention from July 1993 to December 2006 were retrospectively reviewed. The follow-up time ranged from 5.1 to 142.2 months. The numbers of cases of recurrent NPC stage were as follows: stage I, 26; stage II, 9; stage III, 10 and stage IV, 8. Fifty patients had one course of radiation therapy while 3 had two courses of radiation therapy before the salvage surgery. For the nasopharyngectomy, 2 patients underwent endoscopic approach and 33 underwent facial translocation, while 18 had craniofacial resection. Postoperative adjuvant treatment included radiation therapy, 4 cases; radiosurgery, 8 cases; concurrent chemoradiation therapy, 7 cases; and chemotherapy, 2 cases. RESULTS: The 5-year local control rates were T1, 58.3%; T2, 27.8%; T3, 53.3%; T4, 75.0%; and all stages, 53.6%. The 5-year overall survival rates were stage I, 64.8%; stage II, 38.1%; stage III, 25.9%; stage IV, 46.9%; and all stages, 48.7%. Multivariate analysis revealed that gender, margin status, adjuvant treatment type and parapharyngeal space involvement were significant impact factors of local control, whereas dura or brain involvement, local recurrence and adjuvant treatment type were significant impact factors of survival.     

Determinants of survival in parotid gland carcinoma: a population-based study

PURPOSE: To determine survival and factors influencing survival for parotid gland cancer. METHODS: Cases of parotid gland malignancy were extracted from the SEER database for 1988 to 1998. Kaplan-Meier survival analysis was conducted for the most common tumor histologies. Cox proportional hazards modeling was conducted to determine the influence of age, gender, histopathology, grade, size, regional modal status, extraglandular extension, and radiation therapy on survival. Subset analysis was conducted for mucoepidermoid carcinoma according to grade. RESULTS: Nine hundred three patients were identified with a mean age of 59.2 years. Mean follow-up was 51.8 months. Mean 5-year and 10-year actuarial survivals for the entire cohort were 87.8 months, 66.6% and 49.7%, respectively. Mean tumor size at diagnosis was 2.7 cm; 38.0% of patients had extraglandular extension of the tumor, 26.8% of patients had positive nodal disease, and 59.4% of patients received radiation therapy. Tumor histology did predict survival, with squamous cell carcinoma and acinar cell carcinoma exhibiting the poorest and best survivals, respectively. Stratified Cox proportional hazards modeling revealed that increasing age, tumor size, grade, extraglandular extension, and nodal positivity significantly negatively influenced survival (all P相似文献   

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.     

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。     

Analysis of treatment results for oral tongue cancer

OBJECTIVE: The study reports the results of treatment of oral tongue cancer with five different treatment modalities with long-term follow-up. STUDY DESIGN: Retrospective study of 332 patients with oral tongue cancer treated in the Departments of Otolaryngology-Head and Neck Surgery and Radiation Therapy at Washington University School of Medicine (St. Louis, MO) from 1957 to 1996. METHODS: Patients with biopsy-proven squamous cell carcinoma of the oral tongue who were previously untreated and were treated with curative intent by one of five modalities and who were eligible for 5-year follow-up were included. The treatment modalities included local resection alone, composite resection alone (with neck dissection), radiation therapy alone, local resection with radiation therapy, and composite resection with radiation therapy. Multiple diagnostic, treatment, and follow-up parameters were studied using standard statistical analysis to determine statistical significance. RESULTS: The overall 5-year disease-specific survival rate (DSS) was 57% with death due to tumor in 43%. The 5-year cumulative disease-specific survival probability (CDSS) was 0.61 (Kaplan-Meier) with a mean of 17.5 years and a median of 30.1 years. The DSS by treatment modality included local resection (73%), composite resection (61%), radiation therapy (46%), local resection and radiation therapy (65%), and composite resection with radiation therapy (CR/RT) (44%). Overall, local resection had a significantly improved DSS and CR/RT had a decreased DSS that was related to the stage of disease being treated. In treating stage IV disease, CR/RT produced a more significantly improved CDSS than the other treatment modalities. Recurrence at the primary site was as common as recurrence in the neck. Eighty-nine percent of recurrences occurred within the first 60 months. Recurrence significantly decreased survival. DSS was significantly improved in patients with clear margins of resection. Metastasis to a distant site occurred in 9.6% of patients. Twenty-one percent of patients had second primary cancers, and 54% of these patients died of their second primary cancer. CONCLUSIONS: Significant improvement in DSS was seen in patients with clear margins, early stage grouping and clinical (pretreatment) tumor stage, and negative nodes. Significant decrease in DSS was seen in patients with close or involved margins, advanced stage grouping and clinical (pretreatment) tumor staging, positive clinical (pretreatment) node staging, and tumor recurrence. Obtaining clear margins of resection is crucial because it significantly affects survival. A minimum of 5 years of close monitoring is recommended because of the high incidence of second primary cancers.     

Management of stage IV glottic carcinoma: therapeutic outcomes

OBJECTIVES/HYPOTHESIS: The best therapeutic approach for the treatment of stage IV glottic carcinoma is controversial. STUDY DESIGN: A retrospective study. METHODS: A retrospective study of Tumor Research Project data was performed using patients with stage IV glottic squamous cell carcinoma treated with curative intent by five different treatment modalities from 1955 to 1998 at Washington University School of Medicine and Barnes-Jewish Hospital (St. Louis, MO). RESULTS: Ninety-six patients with stage IV glottic carcinoma were treated by five modalities: total laryngectomy (TL) (n = 13), total laryngectomy with neck dissection (TL/ND) (n = 18), radiation therapy alone (RT) (n = 7) (median dose, 69.5 Gy), total laryngectomy combined with radiation therapy (TL/RT) (n = 10), and total laryngectomy and neck dissection combined with radiation therapy (TL/ND/RT) (n = 48). The overall 5-year observed survival (OS) rate was 39%, and the 5-year disease-specific survival (DSS) rate was 45%. The 5-year DSS rates for the individual treatment modalities included the following: TL, 58.3%; TL/ND, 42.9%; RT, 50.0%; TL/RT, 30.0%; and TL/ND/RT, 43.9%. There was no significant difference in DSS for any individual treatment modality (P =.759). The overall locoregional control rate was 69% (66 of 96). The overall recurrence rate was 39% with recurrence at the primary site and in the neck at 19% and 17%, respectively. Recurrence was not related to treatment modality. The 5-year DSS after treatment of locally recurrent cancer (salvage rate) was 30% (3 of 10) and for recurrent neck disease (28 of 67) was 42%. The incidence of delayed regional metastases was 28%; of distant metastasis, 12%; and of second primary cancers, 9%. There was no statistically significant difference in survival between node-negative (N0) necks initially treated (5-y DSS, 31%) versus N0 necks observed and later treated if necessary (5-y DSS, 44%) (P =.685). CONCLUSION: The five treatment modalities had statistically similar survival, recurrence, and complication rates. The overall 5-year DSS for patients with stage IV glottic carcinoma was 45%, and the OS was 39%. The cumulative disease-specific survival (CDSS) was 0.4770 with a mean survival of 10.1 years and a median survival of 3.9 years. Patients younger than age 55 years had better survival (DSS) than patients 56 years of age or older (P =.0002). Patients with early T stage had better survival than patients with more advanced T stage (P =.04). Tumor recurrence at the primary site (P =.0001) and in the neck (P =.014) and distant metastasis (P =.0001) had a deleterious effect on survival. Tumor recurrence was not related to treatment modality. Patients with clear margins of resection had a statistically significant improved survival (DSS and CDSS) compared with patients with close or involved margins (P =.0001). Post-treatment quality of life was not significantly related to treatment modality. Patients whose N0 neck was treated with observation and appropriate treatment for subsequent neck disease had statistically similar survival compared with patients whose N0 neck was treated prophylactically at the time of treatment of the primary. A minimum of 7 years of follow-up is recommended for early identification of recurrent disease, second primary tumors, and distant metastasis. None of the standard treatment modalities currently employed has a statistical advantage regarding survival, recurrence, complications, or quality of life.     

Long-term carcinologic results of advanced esthesioneuroblastoma: a systematic review

Surgical resection followed by radiotherapy can be considered like the optimal treatment modality for limited esthesioneuroblastoma. However, therapeutic management of locally advanced tumors remains a challenge. The aim of our study was to access and compare the oncologic results of the different treatment modalities in advanced esthesioneuroblastoma. We performed a systematic review using the Medline, and Cochrane database in accordance with PRISMA criteria and included all the cases of advanced esthesioneuroblastoma published between 2000 and 2013. We also retrospectively included 15 patients with an advanced esthesioneuroblastoma managed at our tertiary care medical center. Long-term survival rates defined as the time from diagnosis or randomization to the date of death or last follow-up were evaluated for each treatment with Kaplan–Meier survival curve analyses. 283 patients have been included. The mean follow-up was 78 months. Five-year highest survival rates were obtained in patients treated by surgery associated with radiotherapy. Ten-year highest survival rates were obtained in patients treated by the association of surgery, radiotherapy and chemotherapy (p = 0.0008). Within the surgical group, 5-year highest survival rates were obtained in patients treated by endoscopic resection (p = 0.003). Surgical resection combined with radiotherapy offers the gold standard of care. Adjuvant chemotherapy seems to improve the long-term survival in patients with locally advanced esthesioneuroblastoma. Endoscopic resection in advanced tumors should be discussed on a case-by-case basis.     

Classification and prognosis of esthesioneuroblastoma based on 7 treated cases

BACKGROUND: The esthesioneuroblastoma is a rare tumour of neuroectodermal origin, which arises usually in the area of the olfactory epithelium and invades the paranasal sinuses, the orbit and the brain. The low incidence of this disease makes a development of standardised clinical and histological classification difficult. Up to now this tumour is considered to be slow progressive but strained by a high rate of local recurrences. Metastasis are usually seen late at an advanced stage. PATIENTS: In the last 18 years 7 patients with an esthesioneuroblastoma were treated in our department. This relatively large number of patients allows a retrospective evaluation of the different already existing classifications concerning treatment and prognosis. RESULTS AND CONCLUSIONS: In all cases of a disease limited on the paranasal sinuses the patients were successfully treated either by a combination of resection and radiation (4 cases) or resection alone (1 case). No patient underwent a chemotherapy. Two cases with lethal outcome showed an extremely aggressive tumour progression. In such cases of extensive disease an additional chemotherapy has always to be taken into account. Our experiences and the analysis of the literature gives some indications that middle-aged patients have a worse prognosis than young or old patients.     

Treatment of salivary gland neoplasms with fast neutron radiotherapy

OBJECTIVE: To evaluate the efficacy of fast neutron radiotherapy for the treatment of salivary gland neoplasms. DESIGN: Retrospective analysis. SETTING: University of Washington Cancer Center, Neutron Facility, Seattle. PATIENTS: The medical records of 279 patients treated with curative intent using fast neutron radiotherapy at the University of Washington Cancer Center were reviewed. Of the 279 patients, 263 had evidence of gross residual disease at the time of treatment (16 had no evidence of gross residual disease), 141 had tumors of a major salivary gland, and 138 had tumors of minor salivary glands. The median follow-up period was 36 months (range, 1-142 months). MAIN OUTCOME MEASURES: Local-regional control, cause-specific survival, and freedom from metastasis. RESULTS: The 6-year actuarial cause-specific survival rate was 67%. Multivariate analysis revealed that low group stage (I-II) disease, minor salivary sites, lack of skull base invasion, and primary disease were associated with a statistically significant improvement in cause-specific survival. The 6-year actuarial local-regional control rate was 59%. Multivariate analysis revealed size 4 cm or smaller, lack of base of skull invasion, prior surgical resection, and no previous radiotherapy to have a statistically significant improved local-regional control. Sixteen patients without evidence of gross residual disease had a 100% 6-year actuarial local-regional control. The 6-year actuarial freedom from metastasis rate was 64%. Factors associated with decreased development of systemic metastases included negative lymph nodes at the time of treatment and lack of base of skull involvement. The 6-year actuarial rate of development of grade 3 or 4 long-term toxicity (using the Radiation Therapy Oncology Group and European Organization for Research on the Treatment of Cancer criteria) was 10%. No patient experienced grade 5 toxic effects. CONCLUSIONS: Neuron radiotherapy is an effective treatment for patients with salivary gland neoplasms who have gross residual disease and achieves excellent local-regional control in patients without evidence of gross disease.     

Factors predicting survival for cancer of the ethmoid sinus

OBJECTIVE: The aim of this study was to determine survival and prognostic factors for ethmoid sinus cancer. METHODS: From the Surveillance, Epidemiology, and End Results database for the time period 1988-1998, all cases of ethmoid sinus malignancy were extracted. Demographic, staging, treatment, and survival data were computed. Survival analysis was conducted with the Kaplan-Meier method. Clinical factors influencing survival were determined with the Cox proportional hazards model. RESULTS: After excluding patients with metastatic disease on presentation (8.9%) and patients with missing data for T stage, a total of 180 cases were identified. Average age was 60.2 years. Squamous cell carcinoma was the most common tumor (27.8%), followed by adenocarcinoma (12.8%). Overall mean survival was 57 months (median, 38 months) with a 5-year survival of 40.3%. The percentage of patients presenting with T4 lesions was 45.6%, which had a notably lower mean survival of 38 months (median, 18 months). Only 2.3% of patients had positive nodal disease. Increasing age, T stage, and absence of radiation therapy predicted poorer survival in the multivariate model Adenocarcinoma, adenoid cystic carcinoma, esthesioneuroblastoma, and melanoma showed more favorable survival than other tumor types. CONCLUSIONS: T stage and tumor histology are the most important prognostic factors in ethmoid sinus carcinoma. Survival for T4 lesions is markedly worse than survivalfor T1-T3 lesions. Radiation therapy offers a survival benefit in ethmoid sinus malignancy.     

Analysis of treatment results for base of tongue cancer

OBJECTIVE: The study reported the results of treatment for base of tongue cancer with five different treatment modalities with long-term follow-up. STUDY DESIGN: This was a retrospective study of 262 patients with base of tongue cancer treated in the Departments of Otolaryngology-Head and Neck Surgery and Radiation Therapy at Washington University School of Medicine (St. Louis, MO) from July 1955 to January 1998. METHODS: The study population included previously untreated patients with biopsy-proven squamous cell carcinoma of the base of tongue who were treated with curative intent by one of five modalities and were all eligible for 5-year follow-up. The treatment modalities included local resection alone, composite resection alone, radiation therapy alone, local resection with radiation therapy, and composite resection with radiation therapy. Multiple diagnostic, treatment, and follow-up parameters were studied using standard statistical analysis to determine statistical significance. RESULTS: The overall 5-year disease-specific survival (DSS) was 49.6% with death due to tumor in 50.4%. The 5-year cumulative disease-specific survival probability (CDSS) was 0.526 (Kaplan-Meier) with a mean of 7.8 years and a median of 5.6 years. Patients with early disease had significantly improved DSS compared with patients with more advanced disease (stages I and II; TN stages T1N0, T2N0, and T2N1; and T stages T1 and T2.). Patients with N0 had better DSS than patients with positive lymph nodes (P =.010). The DSS for all stages by treatment modality included local resection (70.0%), composite resection (47.6%), radiation therapy (40.4%), local resection and radiation therapy (50.0%), and composite resection with radiation therapy (51.5%). Overall and within the stages there was no significant difference in either DSS or CDSS by treatment modality. Local-regional recurrence occurred in 26% of patients, and overall salvage was 10.5%. Patients with clear resection margins did better than patients with close or involved margins (DSS and CDSS). Patients treated with radiation therapy alone had improved capacity to swallow (P =.001), speak (P =.01), and work (P =.001) compared with patients treated with the other modalities. CONCLUSIONS: Cancer of the base of tongue is a lethal disease, and its treatment results in significant disability. No treatment produced a significantly improved survival advantage. Focus on improving local-regional control might improve overall survival. All treatment modalities were associated with major treatment-related complications. Radiation alone produced significantly improved post-treatment function and quality of life compared with the other modalities. Because of the recurrence rates at the primary and neck sites and the high rates of development of distant metastasis and second primary cancers, patients should be monitored for a minimum of at least 4 years.     

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.     

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.