Kadish Stage Research Articles

Diagnosis and treatment of esthesioneuroblastoma

Esthesioneuroblastoma is one of the most uncommon nasal malignancies which is characterized by low incidence and high misdiagnosis rate.The only prognosis factor is the metastasis on lymph node of neck.Its clinical symptoms are associated with tumor infiltrating extent.Pathological diagnosis is the “gold standard” on esthesioneuroblastoma,and the Kadish staging based on the image is the most important staging standard.The traditional operation therapy mode has been replaced by the combined method of operation and radiotherapy,with the adjunctive therapy of chemotherapy. Key words: Olfactory nerve; Neuroblastoma; Diagnosis; Therapy

Clinical analysis of 49 cases of esthesioneuroblastoma

To investigate the optimal treatment and prognostic factors of esthesioneuroblastoma (ENB). Forty-nine cases of ENB, received therapies in Chinese people liberation army general hospital from October 2004 to June 2013, were reviewed retrospectively. The therapeutical strategies and prognostic factors were analyzed using the Kaplan-Meier method. Efficacy of different therapeutical strategies was compared. The 5-year overall survival (OS) in the group was 63.8%. The 5-year OS of Kadish stage A, B, C were 90.0%, 77.1%, 32.6%, respectively, with significant difference (χ(2) = 7.379, P = 0.03). The 5-year OS of the patients treated by surgery and radiotherapy, chemoradiotherapy, surgery with chemoradiotherapy were 68.6%, 0, 92.3%, respectively, with significant difference (χ(2) = 8.140, P = 0.02). The 5-year OS of the patients with transnasal endoscopic resection (or combined with transcranial resection) and lateral rhinotomy ( or combined with transcranial resection) were 63.7%, 50.0%, respectively, which had no significant difference (χ(2) = 3.818, P = 0.05). ENB has a relatively good prognosis in head and neck malignancies, the prognosis is associated with clinical stages and treatment approaches. Transnasal endoscopic surgery based multimodality is the preferable choice.

Endoscopic Endonasal Management of Esthesioneuroblastoma

Esthesioneuroblastoma is a rare sinonasal malignancy arising from the olfactory epithelium. Classically, craniofacial resection represented the gold standard for management of these tumors. With the advent and evolution of extended endoscopic endonasal techniques, sole endoscopic management of esthesioneuroblastoma has become a viable option. We retrospectively analyzed patients with esthesioneuroblastoma who underwent sole endoscopic resection at the University of Texas Southwestern between 2010 and 2013. We identified 10 patients with esthesioneuroblastoma, seven males and three females. Nine patients were treated for primary disease and one patient was treated following subtotal resection at an outside facility. Kadish staging revealed three patients (30%) with stage B, five patients (50%) with stage C and two patients (20%) with stage D disease. All patients had a complete resection with negative intraoperative margins. On review of the final pathology, one patient was noted to have a positive posterior dural margin and subsequently underwent adjuvant chemotherapy and radiation. One patient had a CSF leak evidenced by symptomatic, worsening pneumocephalus and required an endoscopic revision. Two patients received neoadjuvant chemotherapy and radiation. Five patients received adjuvant chemotherapy and radiation. Three patients (Kadish stage B) received post-operative radiation alone with one patient electing to halt radiation treatment following administration of 2150 cGy via intensity modulated radiation therapy. One patient died (Kadish stage D) during the follow-up period. We have a mean follow-up period of 14.8 months with no evidence of recurrent disease including a Kadish stage D patient 33 months out from surgery and adjuvant chemotherapy and radiation. There is a growing body of literature suggesting outcomes from a purely endonasal approach is equivalent with the traditional craniofacial approach and outcomes may be better in patients with smaller tumors via an extended endonasal approach. As the majority of the patients in our series are Kadish stage C and D, a longer follow-up period will be valuable and may further influence the shift toward pure endoscopic management of esthesioneuroblastoma.

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

<h3>Background</h3> 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. <h3>Methods</h3> 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. <h3>Results</h3> 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, <i>p</i>=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 neither reached with T, N, M and overall staging system. Age cutoff of 65 years reliably predicted OS. <h3>Conclusions</h3> 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.

Treatment outcome and failure pattern of olfactory neuroblastoma: a clinical analysis of 14 cases and meta-analysis of Japanese cases

The olfactory neuroblastoma, first described in 1924, is a rare tumor arising from the olfactory epithelium. Because of its rarity, it is difficult to accrue a large individual series. To elucidate the characteristics of olfactory neuroblastomas in Japan, we report herein on our institutional experience of 14 cases and reviewed 104 cases reported from Japan. In our cases, one out of nine surgically treated patients died during treatment and the remaining 8 patients are alive without disease. Among the five non-surgically treated patients, four patients experienced local treatment failure and the other one patient died of metastasis. In the 104 Japanese cases, 54 patients were treated with multimodality treatment including surgery and radiation. The 3-year overall survival rates for surgically treated patients and non-surgically treated patients were 85% and 73%, respectively. The prognostic factors for survival were modified Kadish stage, Hyams' grade and surgical treatment. Further investigation is required for the validation of endoscopic resection.

Esthesioneuroblastoma: An Update on the Massachusetts Eye and Ear Infirmary and Massachusetts General Hospital Experience with Craniofacial Resection, Proton Beam Radiation, and Chemotherapy

Objectives To update the Massachusetts General Hospital (MGH) and Massachusetts Eye and Ear Infirmary (MEEI) experience in the management of esthesioneuroblastoma (ENB) with multimodality therapy and to reassess treatment outcomes and complications in a larger cohort with longer follow-up times. Design A retrospective chart review. Setting A tertiary referral center. Participants All patients presenting with ENB and managed at the MGH and MEEI from 1997 to 2013. Main Outcome Measures Disease-free and overall survival. Results Twenty-two patients were identified with an average follow-up of 73 months. Ten patients presented with Kadish stage B disease and 12 with stage C disease. A total of six patients (27%) developed regional metastases. Treatment for all patients included craniofacial resection (CFR) followed by proton beam irradiation with or without chemotherapy. The 5-year disease-free and overall survival rates were 86.4% and 95.2%, respectively, by Kaplan-Meier analysis. Negative margins were a significant factor in disease-free survival. One patient experienced severe late-radiation toxicity. Conclusions ENB is safely and effectively treated with CFR followed by proton beam irradiation. The high incidence of regional metastases warrants strong consideration for elective neck irradiation. Proton beam radiation is associated with lower rates of severe late-radiation toxicity than conventional radiotherapy.

Optimization of long-term outcomes for patients with esthesioneuroblastoma.

Esthesioneuroblastoma is a rare cancer of the anterior cranial base that arises in the region of the olfactory rootlets. The purpose of this study was to review the long-term outcomes of patients diagnosed with esthesioneuroblastoma (ENB) treated at a single institution to determine factors associated with improved disease control and survival. A retrospective review of 70 patients with ENB treated at the University of Texas MD Anderson Cancer Center between 1992 and 2007 was undertaken. Survival and recurrence was analyzed and compared using the Kaplan-Meier method and log-rank statistics. Seventy patients were reviewed. The majority (77%) had T3 or T4 disease at presentation, 38% identified as modified Kadish stage C or D. Ninety percent of patients received surgical resection as part of their treatment, and 66% received postoperative radiation or chemoradiation. The median follow-up was 91.4 months (7.6 years). Forty-eight percent of patients developed recurrent disease and the median time to recurrence was 6.9 years. Overall and disease-specific median survival was 10.5 and 11.6 years, respectively. Patients who were treated with surgery alone had a median disease-specific survival of 87.9 months, whereas those who were treated with surgery and postoperative radiation had a median disease-specific survival of 218.5 months (p = .047). Patients with ENB can achieve favorable long-term survival, even if disease is locally advanced. Survival is improved considerably when surgical resection is followed by postoperative radiation. However, recurrence rates and mortality remain high, and therefore long-term observation in these patients is warranted.

Multimodal treatment and long-term outcome of patients with esthesioneuroblastoma

To analyze the clinical features, treatment modalities and outcome of patients treated for a localized esthesioneuroblastoma (ENB). Forty-three consecutive patients with biopsy proven ENB treated at two referral cancer centers between 1998 and 2010 were retrospectively reviewed. Overall, 5 patients had stage A disease, 13 stage B, 16 stage C and 9 stage D according to the modified Kadish classification. Neo-adjuvant chemotherapy was performed in 23 patients leading to a 74 % response rate. Thirty-one patients were treated by surgery. Thirty-nine patients (90.6%) underwent radiation therapy. Twelve patients received bilateral cervical lymph node irradiation (LNI). After a median follow-up of 77 months, the 5-year overall and progression free survival were 65% and 57%. Twelve patients (28%) had a locoregional relapse leading to 10 ENB-related deaths. The major prognostic factor was the modified Kadish stage with a 3-year survival for stage A-B, C and D of 100%, 48% and 22% respectively (p < 0.0001). Two (9%) isolated cervical lymph node relapses occurred among staged B and C patients treated without elective LNI and none after elective or adjuvant LNI. The high risk of locoregional failure in ENB justifies the use of multimodal therapy. Induction chemotherapy leads to a high response rate. Elective LNI might prevent regional failure in locally advanced disease.

Esthesioneuroblastoma: Mansoura University Hospitals’ experience with multimodality therapy in 10 years

BackgroundEsthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare and aggressive malignant tumour of the nasal cavity and paranasal sinuses. This study was conducted to evaluate and analyse the outcomes of the combined modality regimen including surgery, radiotherapy and chemotherapy. MethodsFrom January 2001 to January 2012; in a retrospective analysis; 10 patients with ENB were treated in Mansoura University Hospitals at the Otolaryngology Department. Four patients were of Kadish stage B and six were of stage C. In four cases of stage C, invasion of the anterior cranial fossa was detected. No patient had lymph nodes or distant metastases. All patients received a combined multimodality treatment including surgery; craniofacial or endoscopic resections; radiotherapy and combined vepesid and cisplatin chemotherapy. ResultsNine patients, over an average follow-up which ranged from 5 to 8years (mean 6.2; SD 3.01), are actually alive with no evidence of disease in eight patients and locoregional recurrence in one patient who underwent salvage therapy. However, the overall survival of the nine patients was 5.3–8.75years (mean 6.4; SD 3.04). Local recurrence and distant metastasis were detected in one patient with an average survival of 3years. ConclusionsENB can be a highly curable sinonasal malignancy. Combined modality treatment is considered an integral part of management protocol.

Esthesioneuroblastoma arising from the middle meatus

A 35-year-old female presented with 13-year history of unilateral recurrent nasal mass, epistaxis and facial pain. Nasal examination revealed a pale glistening mass in the right nasal cavity. On probing, mass was insensitive to touch and bled on handling. Computed tomographic scan showed a mass filling the right nasal cavity, ipsilateral maxillary and ethmoid sinuses. Diagnosis of pansinusitis polyposis was made.Transnasal endoscopy-assisted excision of the mass was done, and the diagnosis of olfactory neuroblastoma was established by histopathology and confirmed by immunohistochemistry. The mass was classified as a Kadish stage B tumor. Further intervention including medial maxillectomyand ethmoidectomy, and complete endoscopic-resection of the tumor from cribriform plate was done via lateral rhinotomy approach. The tumor was found adhered to the lateral wall-the middle meatus and was easily peeled away from the cribriform plate and ethmoids. Patient was referred for radiotherapy. No evidence of loco-regional recurrence or systemic metastasis observed at 10-month follow-up.

The prognostic implications of Hyam’s subtype for patients with Kadish stage C esthesioneuroblastoma

Esthesioneuroblastoma (EN) is a rare sinonasal tumor with varied aggressiveness and potential for intracranial invasion. EN is staged anatomically with radiographic evaluation using the Kadish staging system (stages A, B, and C) and histologically by using Hyam’s criteria (grades 1–4). Here we show that despite radiographic evidence of aggressive features, the prognosis of patients with Kadish stage C EN is best predicted by tumor histology using Hyam’s criteria. We retrospectively analyzed patients with EN with Kadish stage C who were evaluated and treated at our institution between 1995 and 2009. Clinical information was collected using patient medical records, imaging, and review of pathological specimens. Twenty patients with Kadish stage C EN were identified with mean age of 51years (31–70years) with a median follow-up of 41.4months (1.3–175months). Upon pathological review, 44.4% of patients had low-grade (1/2) and 55.6% had high-grade (3/4) histology. About 37.5% of patients with low-grade EN had undergone gross total resection (GTR) and the remaining 62.5% had GTR and adjuvant radiation, whereas 50% of patients with high-grade ER had undergone GTR, 20% had undergone GTR and adjuvant radiation, and 30% had been treated with a subtotal resection (STR) and adjuvant radiation. The 5-year and 10-year survival in patients with low-grade EN was 86% in comparison to 56% and 28% with high-grade EN, respectively. In patients with low-grade EN, the 2-year progression free survival (PFS) was 86% and the 5-year PFS was 65% in comparison to 73% and 49% in patients with high-grade EN, respectively. The patient’s tumor histology (Hyam’s criteria) appeared to be the best way of predicting the prognosis and for selecting patients for adjuvant radiotherapy.

Stereotactic Radiosurgical Salvage Treatment for Locally Recurrent Esthesioneuroblastoma

Esthesioneuroblastoma (ENB) is a rare malignant neuroendocrine tumor considered to be radiation sensitive. Local recurrence may be treated in a variety of ways, including stereotactic radiosurgery (SRS); however, little information on its effectiveness is available. To determine whether SRS is effective in providing local control for recurrent ENB. This was a retrospective single-institution experience including 109 patients with ENB treated at the Mayo Clinic (1962-2009). Sixty-three patients presented with Kadish stage C disease, and 21 patients developed local recurrence. Of these 21 patients, 7 patients underwent SRS at our institution and an additional patient underwent SRS after transnasal biopsy. Therefore, a total of 8 patients are reported. The median age at time of local recurrence was 50 years. All patients had Kadish C disease at initial diagnosis. Six of 8 patients were found to have Hyams grade 3 disease; the remaining 2 patients had grade 2 disease. The median treatment volume was 8.4 cm (mean, 18.9 cm; range, 1.4-76.3 cm), and the median dose to the tumor margin was 15 Gy (mean, 14.4 ± 2.2 Gy; range, 10-18 Gy). Of the 16 treatments, 13 had adequate follow-up to assess treatment response, with 92% achieving local control over a median follow-up of 42 months from the time of SRS. Five lesions decreased in size, 7 lesions stabilized, and only 1 lesion had in-field progression. There were no documented complications secondary to SRS. SRS appears to be a reasonable and safe option for treatment of intracranial recurrence of ENB.

Survival and failure outcomes in locally advanced esthesioneuroblastoma: a single centre experience of 15 patients

Esthesioneuroblastoma (ENB) constitutes 3 % of all malignant intranasal tumors. As the tumor is very rare, the number of patients of ENB treated in individual departments is small. We present our institute's experience in combined modality management of 15 successive patients of ENB treated from 2006 to 2010. Clinical characteristics and treatment modality in form of surgery, radiotherapy and chemotherapy were noted. Kadish stage C was the most common stage (12 patients) followed by stage B (3 patients). Fourteen patients underwent primary surgery, of which nine had total excision and five had subtotal excision. One patient was treated with combination of chemotherapy (CT) and radiotherapy (RT). Median RT dose delivered was 54 Gy. Twelve patients received CT with cisplatin and etoposide. Overall, eight patients had complete response, five had partial response, while one had static disease and progressive disease each. Two patients had distant metastases. Four-year loco-regional control (LRC) was 25 % and 4-year overall survival (OS) was 45 %. Most common presentation in our series was locally advanced tumors. Most of these patients require adjuvant RT, which helps in significant LRC. Systemic CT benefits in inoperable, advanced and high risk tumors. Risk-adapted and multimodality approach is the need of hour to achieve good control rates while minimizing treatment related toxicity.

Esthesioneuroblastoma of the Nasal Cavity

OBJECTIVES 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. METHODS 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. RESULTS 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). CONCLUSIONS 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.

Endoscopic resection of esthesioneuroblastoma

Esthesioneuroblastoma, or olfactory neuroblastoma, is an uncommon malignant tumor arising in the upper nasal cavity. Surgical approaches to this and other sinonasal malignancies involving the anterior skull base have traditionally involved craniofacial resections. Over the past 10years to 15years, there have been advances in endoscopic approaches to skull base pathologies, including malignant tumors. In this study, we review our experience with purely endoscopic approaches to esthesioneuroblastomas. Between January 2005 and February 2012, 11 patients (seven men and four women, average age 53.3years) with esthesioneuroblastoma were treated endoscopically. Nine patients presented with newly diagnosed disease and two were treated for tumor recurrence. The modified Kadish staging was: A, two patients (18.2%); B, two patients (18.2%); C, five patients (45.5%); and D, two patients (18.2%). All patients had a complete resection with negative intraoperative margins. Three patients had 2-deoxy-2-(18F)fluoro-d-glucose avid neck nodes on their preoperative positron emission tomography–CT scan. These patients underwent neck dissections; two had positive neck nodes. Perioperative complications included an intraoperative hypertensive urgency and pneumocephalus in two different patients. Mean follow-up was over 28months and all patients were free of disease. This series adds to the growing experience of purely endoscopic surgical approaches in the treatment of skull base tumors including esthesioneuroblastoma. Longer follow-up on larger numbers of patients is required to clarify the utility of purely endoscopic approaches in the management of this malignant tumor.

Gamma Knife® radiosurgery for recurrent intracranial olfactory neuroblastoma (esthesioneuroblastoma): a case report

BackgroundWe report the use of salvage radiosurgery to manage an aggressive olfactory neuroblastoma (esthesioneuroblastoma) with multiple recurrences and intracranial extension.Case presentationA 43-year-old Caucasian woman presented 11 years ago with progressive nasal blockage and headaches. A necrotic polyp originating in her left middle meatus and extending to the ethmoid air cells and cribriform plate (Kadish stage C) was radically resected via a craniofacial approach. Four years later, a local recurrence extending into her left cavernous sinus was identified and deemed inoperable. She received vincristine, ifosfamide, doxorubicin and etoposide chemotherapy (with minimal benefit) and external beam radiotherapy (60Gy in 30 fractions) to her skull base. Two years later, tumour extension in her left neck was treated with radical radiotherapy. She developed visual disturbances in her left eye, which progressed to blindness in the next two years. Having exhausted chemoradiotherapy, the left cavernous sinus esthesioneuroblastoma was treated with Gamma Knife® radiosurgery 2 years ago (20Gy at 50% isodose, tumour volume 7.5cm3). At one year, there was dramatic reduction in the tumour and no new symptoms; however, there were new tumour foci (in her left frontal lobe and above her right orbital apex). These were again treated with radiosurgery (20Gy at 50% isodose, total tumour volume 0.67cm3). Repeat imaging at six months showed no further disease progression.ConclusionWhilst rare, olfactory neuroblastoma (esthesioneuroblastoma) can present management challenges and Gamma Knife® radiosurgery may prove a useful strategy in controlling intracranial spread.

Long-Term Outcome of Esthesioneuroblastoma: Hyams Grade Predicts Patient Survival

Object Esthesioneuroblastoma (ENB) is a rare malignant neuroendocrine tumor originating from the olfactory neuroepithelium in the cribriform plate. Controversy still exists regarding the role of pathologic grading (Hyams grade) in prognostication. This study was undertaken to describe our experience with ENB and assess the role of pathologic grading in patient outcome. Methods This was a retrospective, single-institution experience, including 109 patients with ENB treated at our institution from 1962 to 2009. Multivariate analysis was performed utilizing Cox regression analysis models utilizing age, gender, modified Kadish stage, and Hyams grade. Results Mean age was 49 ± 16 (median 50) years at presentation (range 12 to 90 years). Median follow up was 5.1 years. All-cause mortality was significantly influenced by Hyams grading in univariate (p = 0.04) and multivariate (p = 0.02) analysis, in addition to proven prognostic factors, Kadish staging, lymph node metastasis, and age. Median survival was 9.8 years compared with 6.9 years with low (grade 1 to 2) versus high (grade 3 to 4) Hyams grade. Median overall survival was 7.2 ± 0.7 years. Conclusion ENB has a variable outcome, which is primarily prognosticated by the extent of involvement at presentation (Kadish stage and lymph node metastasis) and higher Hyams pathologic grade.

Craniofacial Surgery for Esthesioneuroblastoma: Report of an International Collaborative Study

Introduction Impact of treatment and prognostic indicators of outcome are relatively ill-defined in esthesioneuroblastomas (ENB) because of the rarity of these tumors. This study was undertaken to assess the impact of craniofacial resection (CFR) on outcome of ENB. Patients and Methods Data on 151 patients who underwent CFR for ENB were collected from 17 institutions that participated in an international collaborative study. Patient, tumor, treatment, and outcome data were collected by questionnaires and variables were analyzed for prognostic impact on overall, disease-specific and recurrence-free survival. The majority of tumors were staged Kadish stage C (116 or 77%). Overall, 90 patients (60%) had received treatment before CFR, radiation therapy in 51 (34%), and chemotherapy in 23 (15%). The margins of surgical resection were reported positive in 23 (15%) patients. Adjuvant postoperative radiation therapy was used in 51 (34%) and chemotherapy in 9 (6%) patients. Results Treatment-related complications were reported in 49 (32%) patients. With a median follow-up of 56 months, the 5-year overall, disease-specific, and recurrence-free survival rates were 78, 83, and 64%, respectively. Intracranial extension of the disease and positive surgical margins were independent predictors of worse overall, disease-specific, and recurrence-free survival on multivariate analysis. Conclusion This collaborative study of patients treated at various institutions across the world demonstrates the efficacy of CFR for ENB. Intracranial extension of disease and complete surgical excision were independent prognostic predictors of outcome.

Recurrent pure subdural extension of esthesioneuroblastoma

Esthesioneuroblastoma is a rare neoplasm arising from the olfactory epithelium, and shows intracranial extension in the advanced disease. We report here a 40-year-old female, who had initially been diagnosed to have a Kadish stage C esthesioneuroblastoma. Recurrence presented about 10 months after the first operation, radiotherapy and chemotherapy, with pure bilateral subdural extension but without local signs. Although recurrence is common in advanced esthesioneuroblastoma, such a pattern of presentation is rare, with only three similar cases reported previously. We discuss the possible pathophysiology of such an event and propose possible means of prevention.

Meta‐analysis of Treatment Outcomes for Sinonasal Undifferentiated Carcinoma

This study reviews the published outcomes regarding sinonasal undifferentiated carcinoma (SNUC) since the initial description in 1986. This article attempts to (1) understand and better describe the benefit and survival advantages associated with using radiation, chemotherapy, and surgical treatment and (2) support the recommendations of a treatment regimen with current available data in the literature. Published English-language literature. A PubMed search for articles related to SNUC, along with the bibliographies of those articles to avoid missing articles. All articles were examined for an independent patient data meta-analysis. Thirty studies with 167 cases from 1986 to October 2009 were identified. Demographics, disease extent, treatment, follow-up, and survival were analyzed. Patient cohorts mirroring Kadish staging were created. Kaplan-Meier curves were constructed. Follow-up range was 1 to 195 months (mean 23.4 months, median 15 months). At last follow-up, 26.3% of patients were alive with no evidence of disease, 21.0% were alive with disease, and 52.7% were dead of disease. The use of surgery was found to be the best single modality, but chemotherapy and radiation were important as adjuncts in extensive and aggressive disease. The presence of neck metastases was a poor prognostic sign. This study, containing the largest pool of SNUC patients, confirms a poor overall prognosis. The data suggest that the optimal treatment should include surgery, with radiation and/or chemotherapy as adjunct treatments. Neck disease in advanced local disease is a poor prognostic sign and merits aggressive treatment with multimodality therapy.