Lymph node metastasis from olfactory neuroblastoma at presentation and as disease relapse: A systematic review and proportion meta-analysis of prevalence data and variables influencing regional control.

BACKGROUND: Aim of this study is to investigate the prevalence of cervical nodal metastasis at presentation and as disease relapse in primary, treatment-naive olfactory neuroblastoma (ONB), and to review treatment modalities, risk factors for regional failure and survival outcomes according to nodal status. METHODS: A systematic review and proportion meta-analysis were conducted following PRISMA guidelines based on PubMed, Web of Science, and Scopus. RESULTS: Eighteen articles were examined. The pooled proportion of patients with nodal metastasis at presentation (11.5%) was comparable to that of cN0 patients not receiving elective neck treatment developing nodal metastasis during follow-up (12.3%). Of the latter, most were Kadish stage C tumors (85.5%). CONCLUSIONS: Cervical involvement is common both at presentation and during follow-up of cN0 ONB. The highest risk of developing late nodal metastasis is seen in cN0 patients with Kadish stage C tumors not receiving elective neck treatment. Elective cN0 neck treatment should be encouraged in selected patients to increase regional control.

Presentation and Outcomes of Non-Squamous Cell Carcinoma Sinonasal Malignancies: A National Perspective.

BACKGROUND: Non-squamous cell carcinoma sinonasal malignancies (NSCCSM) are relatively rare. Neoadjuvant radiotherapy and/or chemotherapy (NTx) have been proposed to improve outcomes compared to surgery alone. In this study, we aim to examine the prevalence of NTx utilization and associated outcomes. METHODS: A retrospective study utilizing the National Cancer Database, 2004 to 2015. The study population included adult patients diagnosed with primary NSCCSM. RESULTS: A total of 574 patients were included. The mean age of the study population was 61.7 ± 16.5 years. The median follow-up time was 40.4 months (interquartile range: 15.3-81.3 months). The histopathological diagnoses identified included: (i) 37.0% adenocarcinoma, (ii) 22.8% adenoid cystic carcinoma, (iii) 20.0% mucosal melanoma, (iv) 11.9% esthesioneuroblastoma, and (v) 8.2% sinonasal undifferentiated carcinoma (SNUC). NTx was utilized in 70 (12.20%) of the study population. Patients who received NTx were more likely to have SNUC or esthesioneuroblastoma (P < .01 each) and to have stage III or IV disease (P < .01 each). NTx was most likely to be administrated in a high-volume center [OR: 3.94, 95%CI: (1.47, 10.53), P = .006]. Patients who received NTx had a significantly lower prevalence of positive margin postoperatively [OR: 0.48, 95%CI: (0.26, 0.87), P = .016]. In patients with NSCCSM, negative margin was associated with improved overall survival [HR: 0.55, 95%CI: (0.36, 0.82), P = .004]. CONCLUSIONS: This study provides an epidemiological perspective regarding NSCCSM and related practice patterns and survival outcomes. Neoadjuvant radiotherapy and/or chemotherapy is likely to decrease the risk of positive margin which ultimately could improve survival in this population.

Short-Term Morbidity and Predictors of Adverse Events Following Esthesioneuroblastoma Surgery.

INTRODUCTION: The short-term adverse events and predictors of morbidity in surgical resection of esthesioneuroblastoma (ENB) are largely unknown, and investigating these variables can help direct planning for at-risk patients. METHODS: The 2005-2017 National Surgical Quality Improvement Program database was queried to identify patients with a diagnosis of ENB undergoing skull base surgery for tumor resection. Information regarding demographics, patient morbidity score, pre-operative and intra-operative data, and post-operative outcomes were extracted. Cox proportional hazard analysis was utilized to assess complication and readmission/reoperation rates. RESULTS: A total of 95 patients undergoing skull base surgery for resection of ENB were included. Mean age, BMI, operation time, and post-operative length of stay (LOS) of the cohort were 53.6 ± 16.2 years, 29.1 ± 6.5, 392.0 ± 204.6 minutes, and 5.8 ± 4.6 days, respectively. In total, 31 patients (32.6%) experienced at least one 30-day adverse event, which included blood transfusion intra-operatively or within 72 hours from the operation (22.1%), readmission (10.7%), intubation >48 hours (7.4%), reintubation (4.2%), organ or space infection (4.2%), reoperation (4.0%), superficial or deep surgical site infection (2.1%), sepsis (2.1%), pulmonary embolism (1.1%), and myocardial infarction (1.1%). Patients who experienced at least one adverse event had significantly higher operation time (486.8 ± 230.4 vs. 347.5 ± 176.2 minutes, p = 0.002), LOS (9.2 ± 5.6 days vs. 4.2 ± 3.0, p < 0.001), and lower hematocrit (37.3 ± 5.9 vs. 41.2 ± 3.8, p < 0.001) and albumin levels (3.8 ± 0.6 vs. 4.2 ± 0.3, p = 0.009). Patients with a higher American Society of Anesthesiologists (ASA) score (HR = 2.39; p = 0.047) or longer operation time (HR = 1.004; p = 0.001) had a significantly higher risk for experiencing adverse events. Obesity was not associated with different intra- or post-operative outcomes, but older patients had shorter operations (p = 0.002) and LOS (p = 0.0014). CONCLUSION: Longer operation time and lower pre-operative hematocrit and albumin levels may all increase complication rates in ENB resection. Patients with high ASA score or more advanced age may have different short-term outcomes.

Consenso español para el tratamiento de los tumores nasosinusales / Spanish consensus for the management of sinonasal tumors

Los tumores nasosinusales son neoplasias poco frecuentes. Su epidemiología, histopatología y características clínicas son diferentes a las del resto de neoplasias malignas de cabeza y cuello. El diagnóstico y tratamiento de estos tumores plantea diversos desafíos debido a su escasa incidencia, su diversidad histológica, la producción de sintomatología inespecífica en los estadios precoces y por tener un pronóstico variable en función de su histología, lugar de origen y estadificación. Su localización centrofacial y la proximidad de estructuras como la órbita y la base del cráneo hacen que su tratamiento sea difícil y complejo, conllevando una elevada morbimortalidad. La cirugía seguida de radioterapia es el tratamiento de elección en la mayor parte de los casos. Para conseguir unos buenos resultados se requiere de equipos multidisciplinares altamente especializados. En este artículo se expone un protocolo de consenso para el tratamiento de los tumores nasosinusales realizado por la Sociedad Española de Otorrinolaringología en colaboración con la Sociedad Española de Oncología Médica y la Sociedad Española de Oncología Radioterápica (AU)

Sinonasal tumors are rare neoplasms with distinctive clinical, aetiological and pathological features. The diagnosis and treatment of these tumours is challenging because of their low incidence, histological diversity and production of non-specific symptoms in the early stages. They have a variable prognosis depending on their histology, origin and staging. Their location, close to neurocritical structures, which are of special relevance to surgery and postoperative treatment, makes their treatment difficult and complex, leading to high morbidity and mortality. Surgery followed by radiotherapy is the mainstay of treatment. To provide the best possible care, patients with sinonasal cancer should be treated in clinical referral centres specializing in skull-base pathologies. Such centres should include a multidisciplinary team led by otolaryngologist surgeons. This article outlines a consensus protocol for the management of these tumours devised by the Spanish Society of Otolaryngology in collaboration with the Spanish Society of Medical Oncology and the Spanish Society for Radiation Oncology (AU)

Genealogical databases as a tool for extending follow-up in clinical reviews.

BACKGROUND: Long-term follow-up in clinical reviews often presents significant difficulty with conventional medical records alone. Publicly accessible genealogical databases such as Ancestry.com provide another avenue for obtaining extended follow-up and added outcome information. No previous studies have described the use of genealogical databases in the follow-up of individual patients. METHODS: Ancestry.com, the largest genealogical database in the United States, houses extensive demographic data on an increasing number of Americans. In a recent retrospective review of esthesioneuroblastoma patients treated at our institution, we used this resource to ascertain the outcomes of patients otherwise lost to follow-up. Additional information such as quality of life and supplemental treatments the patient may have received at home was obtained through direct contact with living relatives. RESULTS: The use of Ancestry.com resulted in a 25% increase (20 months) in follow-up duration as well as incorporation of an additional 7 patients in our study (18%) who would otherwise not have had adequate hospital chart data for inclusion. Many patients within this subset had more advanced disease or were remotely located from our institution. As such, exclusion of these outliers can impact the quality of subsequent outcome analysis. CONCLUSION: Online genealogical databases provide a unique resource of public information that is acceptable to institutional review boards for patient follow-up in clinical reviews. Utilization of Ancestry.com data led to significant improvement in follow-up duration and increased the number of patients with sufficient data that could be included in our retrospective study.

Olfactory Neuroblastoma: Fate of the Neck--A Long-term Multicenter Retrospective Study.

OBJECTIVE: Olfactory neuroblastoma and the management of neck disease has posed considerable challenges to the treating physician. The aims of the study were to determine the incidence and factors influencing neck disease and to identify at-risk patients with cervical node-negative disease at presentation. STUDY DESIGN: Multicenter case series with retrospective chart review. SETTING AND SUBJECTS: In sum, 113 patients with a histopathologic diagnosis of olfactory neuroblastoma across 6 tertiary hospitals in Australia and the United States. METHODS: Treatment modalities for the primary site and neck included surgery, radiotherapy, and combined therapy. Treatment outcomes were measured in relation to date of primary treatment, and long-term follow-up was recorded. Disease-free survival was calculated as time for patients to develop delayed neck disease following primary treatment. RESULTS: A total of 113 patients (46 females, 49.7 ± 13.2 years) were identified with a median follow-up of 41.5 months (interquartile range, 58.2); 7.1% of patients presented with primary neck disease, while 8.8% of patients presented with delayed neck disease. Neck disease was present in patients with Hyams grade II (22.2%), III (55.6%), and IV (22.2%) lesions (χ(2) = 5.66, P = .13). Histologic grade was higher in patients with primary neck disease (χ(2) = 16.22, P = .001). Positive surgical margins were associated with a higher risk of delayed neck disease as compared with clear surgical margin (17.9% vs 5%, P = .034). CONCLUSION: Neck metastasis is an important clinical consideration for olfactory neuroblastoma at presentation and in surveillance. Primary treatment of the neck could be considered in select patients. Long-term surveillance of the neck and primary site is essential.

Is sex an independent prognostic factor in esthesioneuroblastoma?

OBJECTIVE: To determine if sex independently affects presentation and disease-specific survival (DSS) in patients with esthesioneuroblastoma (ENB). STUDY DESIGN: A case-control study from the Surveillance Epidemiology and End Results (SEER) data base. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Neuroblastoma olfatório ectópico: estudo de caso e revisão da literatura / Ectopic olfactory neuroblastoma: case study and literature review

O neuroblastoma olfatório, também conhecido como estesioneuroblastoma (ENB), é tumor maligno de crescimento lento, com origem principalmente na neuroectoderme da cavidade nasal. O pico de incidência do ENB é aos 53 anos, com a maioria dos casos ocorrendo entre os 40 e 70 anos, principalmente em homens. Há poucos relatos de ENB nos seios etmoidal, maxilar e esfenoidal, hipófise e nasofaringe. Esse tumor geralmenteapresenta-se como doença invasiva e tendência a recorrer tardiamente. A disseminação mais frequente do ENB é para os linfonodos cervicais e, se presente, significa pior prognóstico. Seus sintomas são inespecíficos, sendo a obstrução nasal o mais comum. Os exames de imagem são importantes no diagnóstico do ENB, juntamente com o histopatológico e o imuno-histoquímico, que também auxiliam no diagnóstico diferencial. O tratamento cirúrgico de ressecção craniofacial associado à radioterapia apresenta os melhores resultados na sobrevida. Descreve-se seguidamente um caso clínico ilustrativo dessa doença em paciente masculino de oito meses, faixa etária incomum da ENB, com queixa familiar inicial de obstrução nasal e coriza hialina com rajas de sangue...

Olfactory neuroblastoma, also known as esthesioneuroblastoma (ENB), is a slow-growing malignant tumor of that originates mainly in the neuroectodermal cells in the nasal cavity. The peak incidence of ENB is at age 53, with the majority of cases occurring between 40 and 70 years, and mostly among men. There are a few reports of ENB in the ethmoid, maxillary, and sphenoid sinuses, or in the pituitary and nasopharynx. This tumor usually presents as an invasive disease with a tendency for later recurrence. The most frequent dissemination of ENB is to the cervical lymph nodes, which represents a worse prognosis. Its symptoms are nonspecific and nasal obstruction is the most common complaint. Imaging tests are important for the diagnosis of ENB, along with histopathological and immunohistochemical tests to help in the differential diagnosis. Surgical treatment of craniofacial resection associated with radiotherapycan achieve the best results for survival. We present a discussion of an illustrative clinical case of the disease in a male patient aged eight months, an unusual age for ENB, brought to attention by an initial family complaint of nasal obstruction and bloodstained hyaline rhinorrhea...

Esthesioneuroblastoma.

Esthesioneuroblastoma is a rare malignant neoplasm in the olfactory region of the nasal cavity and anterior skull base. Diagnosis and staging require anatomic imaging and careful pathologic assessment. Standard treatment is anterior craniofacial resection with postoperative irradiation. The role for chemotherapy is not defined, but is generally for the most advanced cases and used in the neoadjuvant setting and/or postoperatively with irradiation. Prognosis is favorable; however, metastasis rates remain relatively high. Regional and distant metastasis portends a poor outcome. Intensity-modulated radiation treatment and endoscopic surgery have reduced morbidity, but outcomes with these techniques must be fully evaluated.

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 51 years (31-70 years) with a median follow-up of 41.4 months (1.3-175 months). 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.

Esthesioneuroblastoma: a contemporary review of diagnosis and management.

Esthesioneuroblastoma (ENB) is a rare malignancy, representing only 3% to 6% of all sinonasal malignancies. A wide array of treatment options for ENB have been described in the literature, but prospective clinical trials are absent given the tumor's rarity and natural history. Delay in diagnosis leading to an initial advanced stage of presentation is common secondary to the clinically hidden primary site at the anterior skull base. This article presents data from the current body of literature and reviews the advocated roles for surgery, radiation therapy and chemotherapy.

Estudio retrospectivo de 11 casos de estesioneuroblastomas tratados en el Hospital Santa Creu i Sant Pau entre los años 2000 y 2008 más revisión de la literatura / A retrospective study about 11 cases of Esthesioneuroblastomas treated in Hospital Santa Creu i Sant Pau between 2000 and 2008 and literature review

Introducción. El estesioneuroblastoma (ENB) es un tumor maligno de origen neuroectodérmico poco habitual que afecta principalmente a la fosa craneal anterior y cavidad nasal. Objetivos. Revisar nuestra experiencia en el manejo de los ENB, evaluar la validez del diagnóstico anatomopatológico, el tipo de tratamiento y factores pronósticos de la enfermedad comparando nuestros hallazgos con los descritos en la literatura. Material y métodos. Estudio retrospectivo de 11 casos de ENB tratados en nuestro hospital en el periodo comprendido entre 2000 y 2008. Se realiza análisis estadístico sobre la existencia de factores pronósticos. Se revisa la bibliografía publicada sobre el ENB entre 1990 y 2009.Resultados. Se trataron 3 mujeres y 8 hombres, cuya edad media fue 42 años (rango 20-71 años). El síntoma inicial fue la obstrucción nasal (81%), epistaxis (27%), disminución de agudeza visual (18%), cefalea y otros. Según la clasificación de Kadish, 2 son estadío B y 9 estadío C; según la clasificación de Dulguerov, 2 son T2, 3 son T3 y 6 son T4. El resultado histológico según la clasificación de Hyams fue de 2 casos en grado I, 4 en grado II, 3 en grado III y 2 en grado IV. Dos de los casos inicialmente catalogados en grado IV cambiaron de diagnóstico en la segunda (..) (AU)

Introduction. Esthesioneuroblastoma (ENB) is a very uncommon malignant tumor with a neuroectodermal origin that usually involves the anterior cranial fossa and nasal cavity. Objectives. To review our experience in the management of ENB and assess the validity of the histopathological diagnosis, modality of treatment and prognostic factors of the disease comparing our findings with the literature. Methods. A retrospective study of 11 cases with the diagnosis of esthesioneuroblastoma treated in our hospital between 2000 and 2008. Statistical analysis was performed in search for prognostic factors. The bibliography about ENB published between 1990 and 2009 (..) (AU)

Screening-level population risk assessment of nasal tumors in the US due to naphthalene exposure.

Several naphthalene Unit Risk Factors (URFs) were proposed by the US Environmental Protection Agency in 2004 using data on the development of olfactory epithelial neuroblastomas and nasal respiratory epithelial adenomas in rats, but these URFs may be inappropriate and unnecessarily conservative for estimating human cancer risks. The purpose of the present exercise was to perform a screening-level population risk assessment of the US population to compare the observed number of naphthalene-induced nasal tumors in the US to the number that would be predicted if the URFs for naphthalene were as proposed. Nine scenarios were evaluated to represent the range of exposures individuals have typically experienced. Results indicate that the total predicted burden of naphthalene-induced nasal tumors per year in the US (65,905 rare nasal tumors, of which 29,121 are olfactory epithelial neuroblastomas) is much greater than the number of these tumors actually observed per year (910 total nasal tumors, of which 66 are olfactory neuroblastomas) over the period 1973-2006. This suggests that using rat nasal tumor data to derive a naphthalene URF for humans should be re-evaluated.

Cancer of the nasal cavity in the pediatric population.

OBJECTIVE: The purpose of this work was to investigate the clinical manifestations and diagnostic range of malignant entities presenting as a nasal mass in the pediatric population. PATIENTS AND METHODS: A retrospective cohort analysis was conducted at a specialty hospital and a tertiary care university hospital. Patients aged between birth and 18 years and diagnosed with a malignancy that arose within the nasal cavity between the years 1991 and 2006 were included. This institution-specific patient group was compared with a similar cohort of patients extracted from the national Surveillance Epidemiology and End Results database. The main outcome measures were the incidence, presentation, and diagnoses of nasal cancer presenting in this population. RESULTS: Sixteen patients with nasal malignancies presented institutionally in the defined pediatric age group. Patient age at the time of diagnosis ranged from 7 months to 17 years, with a slight male predominance. The main presenting symptoms were unilateral nasal congestion and ophthalmologic complaints. The median time from presentation to diagnosis was 7 weeks; patients who presented with nonspecific complaints, such as nasal obstruction, headache, and fatigue, were given a diagnosis, on average, later than those who presented with focal manifestations. Nationwide, 47 patients were identified from the Surveillance Epidemiology and End Results database. In both subject groups, the most common diagnoses were rhabdomyosarcoma (37.5% institutionally and 23% in the Surveillance Epidemiology and End Results group) and esthesioneuroblastoma (25% institutionally and 28% Surveillance Epidemiology and End Results). In the Surveillance Epidemiology and End Results cohort, the overall mean survival rate was 188 months. CONCLUSIONS: Nasal cancer in the pediatric population often presents with nonspecific signs and symptoms, and a high index of suspicion is necessary for a timely diagnosis. Soft tissue sarcomas are expectedly common. The relative high frequency of esthesioneuroblastoma is particularly noteworthy.

Olfactory neuroblastoma: long-term clinical outcome at a single institute between 1979 and 2003.

CONCLUSIONS: The progression of olfactory neuroblastoma showed a biphasic pattern. As well as Hyams' histopathological grading and neck metastasis at presentation, early phase recurrence should be regarded as an important prognosticator. A high local failure rate suggests that craniofacial resection followed by postoperative radiotherapy should still be the standard treatment for olfactory neuroblastoma. OBJECTIVE: The aim of this study was to evaluate factors associated with survival and local control of olfactory neuroblastoma in the long run and to estimate treatment strategies. PATIENTS AND METHODS: Twelve patients (seven men and five women) who had undergone initial curative treatment for olfactory neuroblastoma were retrospectively analyzed. RESULTS: Cause-specific 10-year survival was 64.8%, while disease-free 10-year survival remained 28.6%. Local failure was found in half of the patients. All of the three patients who did not receive radiotherapy developed local failure. A biphasic pattern of recurrence was observed. The early phase recurrence group showed a significantly poorer survival than the late phase recurrence group. Hyams' histopathological grading and neck metastasis at presentation were also correlated with survival.

Tratamiento del estesioneuroblastoma. Revisión de la literatura / Esthesioneuroblastoma treatment

Introducción: El estesioneuroblastoma es un tumor maligno infrecuente originado en el epitelio olfatorio. No existe acuerdo sobre el mejor sistema de tratamiento. Son objetivos principales de este trabajo analizar qué características de estaneoplasia pueden influir en la elección de su tratamiento, cuáles la estrategia terapéutica más utilizada y qué tasas de supervivencia se alcanzan. Material y Métodos: Se realiza una revisión de las publicaciones aparecidas entre 1990 y 2004 útiles al fin de esta investigación. Resultados y conclusiones: El dato con más base de información estudia la tasa de supervivencia a 5años, que es de 51,2%; esta cifra se obtiene por la constatación de 39 trabajos que incluyen 713 casos clínicos. Por el análisis de 583 pacientes extraídos de 34 publicaciones conocemos quela cirugía, sola o combinada, es el medio terapéutico más utilizado (78%) y que la estrategia más habitual es la combinación de cirugía y radioterapia (47% de los casos) (AU)

Introduction: Esthesioneuroblastoma is an uncommon malignancy of the olfactory neuroepithelium. The best treatment has yet to be defined. The purpose of this study is to analyze the tumors´s behaviour to choose the ideal treatment, the therapeutic strategy and the patterns of failure. Materials and Methods: We carry out a revision of the series published between 1994 to 2004. In these series, we found 39 papers with713 patients. Discussion. Conclusions: In this review the 5-yearssurvival rate is 51.2%. Through the analysis of 583 patients found in 34 publications, surgery (alone or combined) is the treatment most used (78%). The commonly management is surgical in combination with radiotherapy (47%) (AU)

Resultados a largo plazo del tratamiento del estesioneuroblastoma. Experiencia en Aragón (1981- / Long term results of the esthesioneuroblastoma treatment. Experience in Aragón (1981-2003)

Propósito: el estesioneuroblastoma o neuroblastoma olfatorio es un tumor infrecuente. Se han publicado sólo un millar de casos en la literatura médica. La edad media de presentación es a los 50 años, no teniendo predilección por ningún sexo. Es un tumor de agresividad local con recidivas locales tardías. Se han descrito metástasis a distancia, frecuentemente pulmón y hueso. Presentamos nuestra experiencia en el manejo y tratamiento de este tipo de tumor.- Material y métodos: entre 1981 y 2003, 8 casos de estesioneuroblastoma fueron diagnosticados en el Hospital Clínico Universitario de Zaragoza, un hospital terciario con 882 camas, que es referencia para radioterapia de alta energía en todo Aragón, por lo que con toda probabilidad, este número de casos corresponde al de diagnósticos en la Comunidad Autónoma de Aragón en ese período. Cinco varones y 3 mujeres con una mediana de edad de 60 años (rango 49-82). Los síntomas más frecuentes a la presentación incluyeron: obstrucción nasal, epistaxis, anosmia, exoftalmus, edema palpebral y tumefacción local. El estadio de Kadish al diagnóstico fue: 3 pacientes estadio B, 2 estadio C y 3 estadio D. Cirugía, radioterapia y quimioterapia fueron frecuentemente combinadas. En dos pacientes el tratamiento fue cirugía, sola en un paciente y en otro con radioterapia radical. Tres pacientes recibieron quimioterapia, sola en dos pacientes y combinada con radioterapia en el otro. Tres pacientes fueron tratados con radioterapia sólo.- Resultados: dos pacientes están vivos sin enfermedad tras 87 y 108 meses del diagnóstico y uno más está actualmente en tratamiento. Cuatro pacientes murieron con progresión a los 6, 8, 38 y 63 meses del diagnóstico. Un paciente falleció por un segundo tumor a los 36 meses del diagnóstico.- Conclusión: el control local es un requisito esencial para obtener supervivencias a largo plazo en el estesioneuroblastoma (AU)

[Advances in the diagnosis of ENT tumors in childhood]. / Avances en el diagnóstico de los tumores otorrinolaringológicos.

In the present study we review ENT tumor pathology in childhood. Only the most salient aspects are emphasized and the variety of entities reviewed was restricted. Molecular biology techniques reveal infection by human papilloma virus (types 6 and 11) in 50 % of papillomas, while immunohistochemical techniques are less effective in papilloma virus detection. The myofibroblastic nature of nasal angiofibroma has been demonstrated and its incidence is 25 times more frequent in patients with familial polyposis of the colon. Overexpression of p53 occurs in the initial stages of nasopharyngeal carcinoma, while overexpression of c-myc is correlated with an unfavorable prognosis. Recently, olfactory neuroblastoma has been shown not to express the protein product of the MIC-2 gene (antibody 12E7), thus the hypothesis that it could be a member of the Ewing tumor family (neuroectodermal peripheral tumors) has not been confirmed, although it is a primitive neural tumor. The head and neck rhabdomyosarcoma with the best prognosis is that located in the orbit, and cytogenetic studies have shown chromosomic translocation t(2;13) in 50 % of these childhood tumors when they are of the alveolar-type, while trisomy of chromosome 2 or 20 is more characteristic of the embryonic-type. Currently, any classifying features of ENT lymphomas must be based on the Revised European-American Classification of Lymphoid Neoplasms (REAL). Papillary and medullary carcinomas are the most common histological types of thyroid carcinoma in childhood. Alterations in ret/PTC play a significant role in the pathogenesis of both.

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.