Kadish Stage Research Articles

Endonasal Endoscopic Resection and Radiotherapy in Skull Base Chordomas.

This study evaluates the impact combined endonasal endoscopic resection and radiotherapy for skull base chordomas. Thirty-two patients with skull base chordomas between July 2006 and June 2015 were divided into 2 groups: the surgery alone group and the surgery with radiation therapy group. Gross total resection was achieved in 9 (28.1%) patients with skull base chordoma, subtotal resection was achieved in 16 (50.0%) patients, and partial resection was achieved in 7 (21.9%) patients. The progression-free survival (PFS) rate at 3 and 5 years was 44.0% and 16.5%, respectively. The overall survival (OS) rate at 3 and 5 years was 79.4% and 69.5%, respectively. Kadish staging predicted PFS and OS with statistical significance when the extent of resection was categorized into gross total resection, subtotal resection, and partial resection (P = 0.035 and P = 0.003, respectively). There was a significant OS advantage for the surgery plus radiation group compared with the surgery alone group (P = 0.035). Gross total resection can achieve very good results for the treatment of skull base chordomas. Postoperative adjuvant radiation therapy is recommended for all skull base chordomas, as it offered a higher OS rate.

Esthésioneuroblastome : étude rétrospective et revue de la littérature

PurposeOlfactory neuroblastoma or esthesioneuroblastoma is a rare entity among head and neck neoplasms. In this paper, we report the experience of our institution and compare it with a comprehensive review of the literature. Patients and methodsWe retrospectively analysed clinical and treatment data of patients referred to the Lyon Sud University Hospital (France) for histologically proven olfactive esthesioneuroblastoma. ResultsTen patients treated between 1993 and 2015 have been analysed. Disease stage at diagnosis, according to the Kadish staging system, was C in 90% of cases. Median follow-up was 136 months. Ten-year overall survival was 90%. Five- and ten-year progression-free survival were 70% and 50%. Nine patients (90%) underwent surgical resection first. Seven of the nine patients who underwent resection (77%) received adjuvant three-dimensional (3D)-conformal radiotherapy (n=7), intensity-modulated radiotherapy (n=1), or volumetric arctherapy (n=1). The mean dose to the tumour volume was 61Gy. None of the patients received elective nodal irradiation. Two patients received concurrent chemotherapy. Five patients (50%) presented with disease recurrence, which was local (n=1), nodal (n=2) and cerebral (n=2). ConclusionOur results are consistent with the literature. Because of the lack of prospective study and the low number of cases in the literature, each institution's experience is of the utmost important to improve standardised management of these tumours.

Significance of Tumor Stage in Sinonasal Undifferentiated Carcinoma Survival: A Population-Based Analysis.

To describe the incidence and determinants of survival of patients with sinonasal undifferentiated carcinoma (SNUC) from 1973 to 2011 using the SEER database (Surveillance, Epidemiology, and End Results), with consideration of tumor stage based on the Kadish system. Retrospective database analysis. Academic medical center. The SEER registry was utilized to calculate survival trends for 328 patients with SNUC between 1973 and 2011. Patient data were then analyzed with respect to histopathology, age, sex, race, subsite, modified Kadish stage, tumor size, and treatments rendered. The cohort was composed of 61.9% males with median age of 60 years. The median overall survival (OS) was 1.9 years. Most tumors presented in the nasal cavity, maxillary sinus, and ethmoid sinus (29.3%, 27.4%, 21%, respectively); 43.7% of patients received both surgical and radiation therapy. OS at 2, 5, and 10 years was 43%, 30%, and 25%, respectively. On univariate analysis, age, Kadish stage, and tumor size were associated with worse OS and disease-specific survival (DSS), while surgery and radiation therapy were associated with improved OS and DSS (all P < .05). On multivariate analysis, radiation therapy and lower Kadish stage were associated with improved OS and DSS, while younger age was additionally associated with improved OS (all P < .05). SNUC is a rare but aggressive sinonasal malignancy. Tumor stage as determined by the Kadish system is associated with worse survival, with radiation therapy appearing to play a key role in therapeutic management.

Endoscopic Endonasal Management of Olfactory Neuroblastoma: A Retrospective Analysis of 10 Patients with Quality-of-Life Measures

Anterior craniofacial resection has served as the traditional surgical treatment of olfactory neuroblastoma (ON). With the development of extended endonasal approaches, the opportunity exists for using minimal access techniques for management of select tumors. This study assesses the impact of endoscopic resection on ON and patient outcomes and quality of life. A retrospective review identified 10 patients with ON (3 women, 7 men; mean age 49.1 years) who underwent endoscopic resection during the period 2010-2013. Modified Kadish staging divided the cohort into 3 stage B patients (30%), 5 stage C patients (50%), and 2 stage D patients (20%). Outcome measures included extent of resection, complications, recurrence, and preoperative and postoperative Sino-Nasal Outcome Test-20 scores. Gross total resection was achieved in all patients, with negative margins in 9 patients. One patient had negative frozen section pathology but was noted to have a positive posterior dural margin on final pathology. There was a 20% complication rate (pneumocephalus, ethmoid meningoencephalocele). Neoadjuvant chemotherapy and radiation were performed in 2 patients (Kadish stage C and D). Adjuvant chemotherapy and radiation were performed in 5 patients (4 Kadish stage C and 1 stage D). Postoperative radiation alone was administered in 3 patients (Kadish stage B). Analysis of postoperative Sino-Nasal Outcome Test-20 scores demonstrated no significant change relative to preoperative Sino-Nasal Outcome Test-20 scores. At the most recent follow-up examination, there was no evidence of recurrent disease in patients who underwent endoscopic resection. One patient (Kadish stage D) died during the follow-up period. Mean follow-up duration was 21.1 months. This series adds to the growing body of literature that suggests equivalent or improved outcomes of purely endonasal resection for select patients. Given the advanced Kadish stage of most of our patients, longer follow-up is required to determine the full applicability of purely endoscopic approaches to the treatment of ON.

Advantage of Extended Craniofacial Resection for Advanced Malignant Tumors of the Nasal Cavity and Paranasal Sinuses: Long-Term Outcome and Surgical Management

Craniofacial resection (CFR) for advanced sinonasal malignant tumors (SNMTs) is mandatory for radical resection. Surgeons must be aware of perioperative complications and long-term outcome because this procedure is extremely invasive, especially when the tumor involves the anterior skull base. Thirty-eight consecutive surgical patients with advanced SNMT of T4 stage or Kadish stage C (31 men and 7 women; mean age, 55 years; range: 19-76 years) treated with CFR in the past 28 years were followed up for 59.4 months. In cases of unilateral orbital extension, en-bloc resection was achieved using several neurosurgical techniques (extended CFR) from 2005 onwards. Herein, we evaluated the safety and effectiveness of surgery by comparing survival data between 2 time periods (first stage: 1984-2004, second stage: 2005-2012). Squamous cell carcinoma was the most common histological type observed (65.8%), followed by esthesioneuroblastoma (15.8%). Using a combination of adjuvant radiation therapy, the 5-year overall survival and the 5-year disease-specific survival rates were 55.5% and 59.4%, respectively. Sarcomatous histology was a poor prognostic factor. The 5-year disease-specific survival rate was 48.9% in the first stage and improved to 82.1% in the second stage (P= 0.057); this was related to improvements in local control rate. CFR and postoperative radiotherapy are safe and effective for treating advanced SNMTs. Extended CFR, including radical orbital exenteration, may contribute to good long-term outcomes. A diverse surgical team may help perform radical resection and reconstruction in patients with advanced tumors.

Patterns of regional and distant metastasis in esthesioneuroblastoma.

To define the incidence and risk factors of metastatic disease and the effectiveness of salvage therapy in esthesioneuroblastoma (ENB). Retrospective analysis of 57 patients presenting from 1979 through 2009. Cumulative incidence of neck failure, distant failure, and survival were assessed using the Kaplan-Meier method. Overall survival for all patients was 85% at 5 years and 75% at 10 years. Overall survival was negatively impacted by intracranial tumor extension (P < 0.001), positive resection margins (P = 0.05), and neck metastases (P = 0.017). Neck lymph nodes were not routinely electively irradiated during this time period. Nodal metastases developed in 17% of patients at a median time of 60 months. Kadish stage was not associated with a risk of nodal metastasis (P = 0.78). After treatment for nodal recurrence, locoregional control was achieved in 78% of patients. Of patients developing nodal recurrence, more than half developed distant metastases. The cumulative incidence of distant metastasis was 39% at a median time of 40 months. Patients who presented with Kadish stage C or D had a significantly increased risk of distant failure (P < 0.001). In patients developing nodal (P = 0.017) or distant metastasis (P = 0.001), the probability of survival was significantly decreased. Regional and distant metastases in patients with esthesioneuroblastoma occur in a delayed fashion and negatively impact survival. Neck nodal recurrence may be a harbinger of distant metastases. At the Memorial Sloan Kettering Cancer Center, New York, New York, we now treat the majority of ENB patients with elective nodal irradiation. However, the chief obstacle to long-term cure is distant metastases. 4. Laryngoscope, 126:1556-1561, 2016.

Olfactory neuroblastoma: A clinicopathological experience of a rare entity from Pakistan.

Objectives:To present the clinicopathological experience of Olfactory Neuroblastoma (ONB) with emphasis on histopathological and immunohistochemical features.Methods:A descriptive cross-sectional study was done on 36 cases of ONB, selected by non-probability purposive sampling. Theses cases of ONB were retrieved and reviewed from surgical pathology database of Aga Khan University Hospital reported between January 1993 and March 2015.Results:Tumor size and age of presentation was wide in range without any distinct bimodal distribution. Nasal cavity was most common site along with involvement of paranasal sinuses. More than 50% cases had Kadish stage A. Microscopically, most cases were Grade-1 and majority showed partial or complete lobular architecture. Neurofibrillary matrix was observed in 2/3rd of cases. Among immunohistochemical markers, Neuron Specific Enolase was most frequently expressed. Unusual positive expression of Cytokeratin AE1/AE3 and Cytokeratin CAM5.2 was also seen focally in few cases.Conclusion:The ONB has great variability of histological and clinical presentation, and immunohistochemical markers are useful to differentiate from more common small round blue cell tumours of nasal cavity.

Neck recurrence and mortality in esthesioneuroblastoma: Implications for management of the N0 neck.

To review the literature on neck recurrence in esthesioneuroblastoma. PubMed database. A PubMed database search was performed using keywords "esthesioneuroblastoma," "olfactory neuroblastoma," and "esthesioneuroblastoma neck metastasis." Articles written in English with greater than 10 subjects that had data regarding the association of neck recurrence and mortality and/or the association of neck recurrence with Kadish stage were included for analysis. Thirteen studies met inclusion criteria with information regarding the association of neck recurrence and mortality, and 15 studies had data associating neck recurrence and Kadish stage. The neck recurrence rate was 14.1% in studies analyzing mortality. Among those patients who developed regional metastases, mortality was 60%. Of patients without regional recurrence, the mortality rate from disease was 26% (P < 0.0001) and overall mortality was 32% (P < 0.0001). The rate of neck recurrence within each Kadish stage was 0%, 11%, 21%, and 18% for Kadish stages A, B, C, and D, respectively. The trend toward an increased incidence of neck recurrence from stage A to stage D is statistically significant, with P value 0.003. The rate of neck recurrence in esthesioneuroblastoma is close to 15%. There is a strong association of recurrence with Kadish stage B and C. Mortality from disease in patients with recurrence in cervical lymph nodes is significant when compared to those who never develop neck disease. Prospective studies are needed to evaluate a potential role for elective neck dissection versus elective neck radiation for patients with esthesioneuroblastoma. N/A. Laryngoscope, 126:1373-1379, 2016.

Multimodality Treatment of Pediatric Esthesioneuroblastoma.

Esthesioneuroblastoma (ENB) is a rare cancer of the nasal cavity in children. Radical surgery followed by postoperative radiation is considered the standard of care in adults. A similar approach in children can lead to significant long-term morbidity. A retrospective multi-institutional review of patients less than 21 years of age diagnosed with ENB between 1990 and 2014 was performed. Clinical features, treatment, and outcome were obtained from the medical records. Twenty-four patients were identified with a median age of 14 years (range 0.6-20 years) at diagnosis. The majority (75%) were females. Headache was the most common presenting symptom, followed by nasal obstruction and epistaxis. Eight patients had Kadish stage B tumors and 16 had Kadish stage C tumors. Nine patients had metastatic disease. Gross total resection was achieved at diagnosis in eight patients and after neoadjuvant chemotherapy in four patients. Twenty-one patients received radiation therapy (45-68.4 Gy). Thirteen patients received neoadjuvant chemotherapy with 84% objective response rate. Seven patients experienced disease progression or relapse-five in central nervous system, one local, and one in cervical lymph node. Fifteen patients were alive at the last follow-up. The 5-year disease-free survival and overall survival were 74% and 73%, respectively. Late effects were observed in 78% of long-term survivors. Four patients developed subsequent malignant neoplasms. Pediatric ENB is a chemosensitive disease. Preoperative chemotherapy-based multimodal approach should be used in patients with advanced stage disease. Radiation therapy is effective for local control, but lower doses should be considered in children.

An analysis of multimodal treatment outcomes and patterns of treatment failure in esthesioneuroblastoma

Objective To evaluate the multimodal treatment outcomes and patterns of treatment failure in esthesioneuroblastoma at a single institution. Methods One hundred and twelve patients who were newly diagnosed with esthesioneuroblastoma but no distant metastasis in our institution from 1979 to 2014 were retrospectively analyzed. The treatment modes, outcomes, and patterns of treatment failure in these patients were analyzed. According to the modified Kadish staging system, the numbers of patients with stage A, B, C, and D esthesioneuroblastoma were 1, 23, 60, and 28, respectively. Fifty-one patients received surgery and postoperative radiotherapy with or without chemotherapy; forty-six patients received radiotherapy with or without chemotherapy; eleven patients received preoperative radiotherapy and surgery with or without chemotherapy; three patients received surgery with or without chemotherapy; one patient received chemotherapy alone. The survival rates were calculated using the Kaplan-Meier method. Results In all patients, the 5-year sample size was 44, and the 5-year overall survival (OS) and disease-free survival (DFS) rates were 66.4% and 54.7%, respectively. The 5-year OS and DFS rates were 91% and 82% in patients who received preoperative radiotherapy and surgery with or without chemotherapy, 80% and 66% in patients who received surgery and postoperative radiotherapy with or without chemotherapy, and 46% and 37% in patients who received radiotherapy with or without chemotherapy. Three patients treated with surgery alone had relapse of the disease; one patient treated with palliative chemotherapy survived 6 months. Treatment failed in 47(42%) out of 112 patients. In patients with failed treatment, 53% had distant metastasis as the first pattern of treatment failure, 36% had locoregional relapse, and 11% had concurrent distant metastasis and locoregional relapse. Conclusions Surgery combined with radiotherapy is still the recommended multimodal treatment regimen for esthesioneuroblastoma. The multimodal treatment achieves satisfactory local-regional control rate and treatment outcomes in the treatment of esthesioneuroblastoma. The major pattern of treatment failure is distant metastasis. Key words: Eeshesineuroblastoma/radiotherapy; Treatment outcomes; Failure pattern

Is sex an independent prognostic factor in esthesioneuroblastoma?

To determine if sex independently affects presentation and disease-specific survival (DSS) in patients with esthesioneuroblastoma (ENB). A case-control study from the Surveillance Epidemiology and End Results (SEER) data base. The assessment identified 611 patients in the SEER data base who were diagnosed with ENB from 1988 to 2010. Data on race, ethnicity, age at diagnosis, sex, histologic grade, radiation treatment status, and surgical treatment status of patients with ENB from 1988 to 2010 were extracted. By using tumor extension data, the modified Kadish stage of each case was determined. The modified Kadish system was able to successfully classify 547 of 611 tumors from 1988 to 2010. Histologic grade, modified Kadish stage and DSS of male patients was compared with the DSS of female patients. Demographic data showed that male patients presented with a significantly higher grade (p < 0.05) and a trend toward a higher stage (p = 0.08). With unmatched data, male patients had significantly worse DSS than female patients (p < 0.05). After case-matching, the difference between the DSS for male versus female patients was no longer significant. Male patients with ENB seemed to have significantly worse DSS at 10 years than female patients. This disparity seems to be due to higher grade and stage in male patients at presentation. After accounting for these two factors, the prognosis of male patients was not found to be significantly different from that of female patients.

Endoscopic endonasal resection of esthesioneuroblastoma: A single center experience of 24 patients

ObjectiveEsthesioneuroblastoma (ENB) is an uncommon malignant tumor. During the past decade, endoscopic approaches have been gradually applied in treating skull base tumors. However, the experience in using this approach to treat ENB is still limited. Kadish staging and Dulguerov staging are common methods used for ENB staging, but it remains unclear as to which method is better. In this study, we reviewed our experiences with endoscopic surgeries for ENB and analyzed the prognostic roles of the two staging methods. MethodsA total of 24 patients with ENB treated with only endoscopic endonasal surgery between January 2001 and March 2012 were included. Overall survival (OS) and disease-free survival (DFS) were analyzed using the Kaplan–Meier method and early and advanced stages were compared using the log-rank test. The prognostic roles of the two staging methods were also analyzed. ResultsAmongst the 24 patients, 19 patients presented with newly diagnosed ENB, and 5 patients presented with recurrent disease. The three-year OS and DFS rates were 82% and 70.8%, respectively. Four patients (16.6%) died from recurrence of the tumor. Dulguerov staging predicted OS with significant differences (P=0.042), whereas Kadish staging predicted DFS with significant differences (P=0.020) between the early and advanced stages. ConclusionsThe present study showed experiences that purely endoscopic endonasal surgery for ENB showed successful survival results with remarkably decreased complications. Dulguerov staging and Kadish staging play different prognostic roles in patients treated with purely endoscopic endonasal resection based on various end points.

Esthesioneuroblastoma of the Nasal Cavity

Esthesioneuroblastoma is an uncommon cancer of the nasal cavity. We describe the outcomes for 26 patients treated with curative intent with photon radiotherapy (RT) at the University of Florida. Between May 1972 and June 2007, 26 patients received RT for previously untreated esthesioneuroblastoma of the nasal cavity. Sixteen patients were males and 10 were females with a median age of 55 years (range, 3 to 82 y). The modified Kadish stage distribution was: B, 7 patients; C, 17 patients; and D, 2 patients. Treatment modalities included the following: definitive RT, 5 patients; preoperative RT, 2 patients; and postoperative RT after resection, 19 patients. Elective neck irradiation (ENI) was performed in 17 (71%) of 24 N0 patients. Rates of local control, cause-specific survival, and absolute overall survival at 5 years were 79%, 72%, and 69%, respectively. Overall survival among patients treated with definitive RT was 20% at 5 years, compared with 81% among those who underwent surgery and adjuvant RT (P=0.01). One (6%) of 17 patients who received ENI developed a recurrence in the neck and was successfully salvaged. Ultimate neck control was 100% at 5 years for patients who received ENI versus 69% among those not receiving ENI (P=0.0173). Resection combined with adjuvant RT is more effective than surgery or RT alone in the treatment of esthesioneuroblastoma. ENI reduces the risk of regional relapse in patients with Kadish stage B and C cancers.

Olfactory neuroblastoma: the long-term outcome and late toxicity of multimodal therapy including radiotherapy based on treatment planning using computed tomography.

BackgroundOlfactory neuroblastoma (ONB) is a rare tumor originating from olfactory epithelium. Here we retrospectively analyzed the long-term treatment outcomes and toxicity of radiotherapy for ONB patients for whom computed tomography (CT) and three-dimensional treatment planning was conducted to reappraise the role of radiotherapy in the light of recent advanced technology and chemotherapy.MethodsSeventeen patients with ONB treated between July 1992 and June 2013 were included. Three patients were Kadish stage B and 14 were stage C. All patients were treated with radiotherapy with or without surgery or chemotherapy. The radiation dose was distributed from 50 Gy to 66 Gy except for one patient who received 40 Gy preoperatively.ResultsThe median follow-up time was 95 months (range 8–173 months). The 5-year overall survival (OS) and relapse-free survival (RFS) rates were estimated at 88% and 74%, respectively. Five patients with stage C disease had recurrence with the median time to recurrence of 59 months (range 7–115 months). Late adverse events equal to or above Grade 2 in CTCAE v4.03 were observed in three patients.ConclusionMultimodal therapy including radiotherapy with precise treatment planning based on CT simulation achieved an excellent local control rate with acceptable toxicity and reasonable overall survival for patients with ONB.

Multidisciplinary treatment of olfactory neuroblastoma: Patterns of failure and management of recurrence

PurposeEsthesioneuroblastoma is an uncommon malignancy of the head and neck for which there is no defined treatment protocol. The purpose of this study is to report our experience with the treatment and patterns of failure of this disease. Methods and materialsFrom 1994 to 2012, 37 previously unreported patients with esthesioneuroblastoma were evaluated, and 32 eventually treated for cure at 2 academic medical centers. All patients were staged with Kadish criteria. The mean and median follow-ups were 96.1 and 76.5months respectively (range 6–240months). ResultsThe Kadish stage was A in 6 patients, B in 13 patients, and C in 13 patients. Four patients were initially treated with concurrent chemo-radiation therapy. Twenty-eight patients were treated with primary surgery. Two (2) underwent open medial maxillectomy and 26 underwent craniofacial resection (open – 17, endoscopic – 9). Three patients received curative surgical resection only. Seven patients failed either within the cranial axis or distantly, 6 of the 7 are dead of disease, 10–194months following initial treatment. Six patients had isolated neck recurrences, 4/6 were salvaged with neck dissection and additional chemo-radiation and remain alive 30–194months following initial treatment. Estimated overall survival rate at 10years was 78% based on Kadish and T stages. ConclusionIn this retrospective analysis of 32 patients, Kadish stage C and stage T3/T4 tumors were associated with worse outcome. Total radiation dose of 60Gy, margin status, patient age, were not found to have significant prognostic value.

An endoscopic endonasal approach for early-stage olfactory neuroblastoma: an evaluation of 2 cases with minireview of literature.

We describe the clinical findings in two patients with pathologically diagnosed olfactory neuroblastoma (ONB) of the sinonasal area and the surgical methods used for its treatment. Using an endoscopic endonasal approach (EEA) without dura resection, along with radiotherapy, we successfully treated ONB at the Kadish stage A. One of our patients, however, experienced tumor recurrence 24 years after open surgery with radiotherapy that was conducted at another hospital. This patient was no longer eligible for radiotherapy, and the tumor was therefore resected with dura resection using an EEA combined with duraplasty. The dura resection with duraplasty using fascia lata and a pedicled nasal septal flap was minimally invasive. As with surgery without duraplasty, a postoperative computed tomography (CT) examination revealed that EEA with duraplasty led to quick improvement of the postoperative inflammatory response as well as pneumocranium. Here, we investigated whether to modify the method of surgery depending upon the primary site of early-stage ONB. We suggest that, in early-stage ONB, an endoscopic endonasal approach is an effective and less invasive method. It is also advisable to perform dura mater resection of the lesion site despite the absence of obvious intracranial invasions in image findings.

Importance of tumor grade in esthesioneuroblastoma survival: a population-based analysis.

There is a need for larger studies characterizing the effect of tumor grade on survival for patients with esthesioneuroblastoma. To investigate prognostic factors for survival in patients diagnosed with esthesioneuroblastoma, including emphasis on tumor grade. Retrospective, population-based cohort study of patients in the Surveillance, Epidemiology, and End Results (SEER) tumor registry who were diagnosed with esthesioneuroblastoma from January 1, 1973, to January 1, 2010. The last date of survival follow-up was 2013. Overall and disease-specific survival. The cohort included 281 patients with a mean age of 52 years. There were 154 males (54.8%) and 127 females (45.2%). Kaplan-Meier analysis demonstrated an overall and disease-specific survival rate of 61% and 70% at 5 years and 50% and 64% at 10 years, respectively. Multivariable Cox regression analysis showed that advanced tumor grade and modified Kadish stage (hazard ratio, 4.930; 95% CI, 2.635-9.223; P = .001) portended worse disease-specific survival, and radiation therapy (hazard ratio, 0.499; 95% CI, 0.272-0.916; P = .03) improved disease-specific survival. Patients with low-grade tumors (grades I and II) demonstrated an overall and disease-specific survival rate of 84% and 92% at 5 years and 67% and 87% at 10 years, respectively. Multivariable analysis of low-grade tumors only revealed receiving surgery (P = .004) as an independent positive predictor of disease-specific survival. High-grade tumors (grades III and IV) demonstrated overall and disease-specific survival of 40% and 50% at 5 years and 34% and 43% at 10 years, respectively. Multivariable analysis of high-grade tumors showed modified Kadish stage (hazard ratio, 2.025; 95% CI, 1.430-2.866; P < .001) predicted worse disease-specific survival, and radiation therapy (hazard ratio, 0.433; 95% CI, 0.228-0.864; P = .02) independently predicted improved disease-specific survival. Here, to our knowledge, we report the largest study investigating prognostic factors for survival, with the inclusion of tumor grade, in patients diagnosed with esthesioneuroblastoma. Patients with high-grade tumors had substantially worse survival rates than patients with low-grade tumors. Multivariable analysis revealed only receiving surgery as an independent predictor of disease-specific survival for patients with low-grade tumors, while modified Kadish stage and postoperative radiation therapy were significant factors in predicting disease-specific survival in patients with high-grade tumors. This study highlights the growing evidence that tumor grade should be a key factor in predicting survival in patients with esthesioneuroblastoma, and that adjuvant radiation therapy improves survival rates among patients with high-grade, but not low-grade, tumors.

Esthesioneuroblastoma: an update on the UCLA experience, 2002-2013.

Objectives To profile the clinical presentation and treatment results of esthesioneuroblastomas at the University of California, Los Angeles (UCLA), from 2002 to 2013. Design Retrospective review. Setting Tertiary academic institution. Participants Forty-one patients with esthesioneuroblastomas treated at UCLA. Main Outcome Measures Overall survival (OS) and recurrence-free survival (RFS). Results Thirty-six patients were included with a mean age of 50.1 years and a median duration of follow-up of 33 months. The 5-year RFS and OS were 54% and 82%, respectively. Modified Kadish stage was the only factor identified to affect OS. Multivariate analysis demonstrated that tumor grade was the only factor that had an independent impact on RFS. There was no statistical difference in survival among the surgical approaches chosen. Conclusions The updated data on the UCLA experience reveals that all three surgical approaches chosen provide comparable survival, although longer follow-up will be needed to ascertain if these findings hold true. The endoscopic approach had a statistically significant decrease in length of hospital stay and a trend toward reduced blood loss, intensive care unit admission, and complications. The modified Kadish staging was the only factor identified to predict OS. Multivariate analysis revealed that tumor grade was an independent predictor of recurrence; therefore, its importance should be emphasized in future staging systems.

Treatment of esthesioneuroblastomas

ObjectiveTo analyze the clinical features, treatment outcomes, pattern of failures, and course of the disease of a cohort of patients treated for esthesioneuroblastoma (ENB) with craniofacial resection (CFR) at a single institution during a 12-year period. Material and methodsRetrospective analysis of 11 patients with ENB treated with CFR in a tertiary care academic medical center from 1998 to 2009. ResultsMedian age at diagnosis was 51years (range 41–67years). The most common presenting symptom was nasal obstruction (91%). Four patients (36%) presented with Kadish stage B, six patients (55%) with Kadish stage C, and one patient (9%) with Kadish stage D. The initial treatment was craniofacial resection (CFR) alone for three patients (23%), CFR followed by postoperative radiation therapy (RT) in seven patients (64%), while one patient (9%) received both neoadjuvant and adjuvant RT in addition to surgery. The mean and median follow-up times were 66 and 58months, respectively (range 23–158months). Seven patients are currently alive with no evidence of disease (64%), while two patients are alive with disease (18%). Overall survival was 100% at one year postoperatively and 80% five years after the primary treatment. The progression free survival was calculated to 73% at one year and 64% at five years. ConclusionsENB is an uncommon diagnosis with an incidence of 0.037/100,000 persons/year in the catchment area of our institution. Treatment can be challenging, especially with advanced disease. CFR with RT offers good oncologic disease control with minimal morbidity.

Prognostic utility of Hyams histological grading and Kadish-Morita staging systems for esthesioneuroblastoma outcomes.

Esthesioneuroblastoma (ENB) is derived from the specialized olfactory neuroepithelium. Hyams grading and Kadish staging have been used to prognosticate and to guide treatment decisions. In this study, we sought to validate the prognostic utility of these systems in a large ENB cohort. We retrospectively analyzed the records of patients with ENB who had been evaluated and treated at our institution. The association of grade and stage with prognostic outcome was assessed; the Kaplan-Meier estimator was used to generate 5-year OS and DFS curves. Out of 124 cases we identified, 121 were assessed for grading and 109 for staging. Review of the tissue samples revealed that 62 % of tumors were low grade (I/II) and 21 % were high grade (III/IV); 17 % of tumors were metastasis. The OS rate was 75 % at 5 years. The DFS was 60 % at 5 years. The OS was significantly worse for metastatic ENB (low-grade ENB vs metastatic ENB p = 0.01598); the DFS was significantly worse for high grade versus low grade ENB. Of the 109 cases that had been staged, 16 % were stage A, 33 % stage B, 43 % stage C, and 8 % stage D. In the A, B, and C groups, there were no significant differences between recurrence, distant metastasis, or 5-year survival rates. Statistical significance was not reached with the T, N, M and overall staging system. Age cutoff of 65 years reliably predicted OS. High grade of ENB was significantly associated with poor outcome, while advanced stage was not associated with poor outcome in this large cohort. Grading should certainly be considered in prognostication and treatment decisions for ENB.