ABSTRACT
Herein we present a case of a 62-year-old male patient who was admitted with the chief complaints of nasal obstruction. The histopathological and immunohistochemical evaluation led to a diagnosis of olfactory neuroblastoma with rhabdomyoblasts. A review of the literature revealed that this is only the fourth case of olfactory neuroblastoma with rhabdomyoblasts. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and identify the best treatment to improve prognosis.
ABSTRACT
Resumen El neuroblastoma olfatorio (NBO) es un tumor maligno poco frecuente que se origina de las células neuroepiteliales olfativas. Su diagnóstico precoz es difícil debido a la poca especificidad de los síntomas que presentan los pacientes. Las pruebas de imagen juegan un papel importante en su diagnóstico y en la planificación quirúrgica, por lo que es importante que los radiólogos conozcan sus hallazgos y las diferentes clasificaciones que ayudarán a elegir el tratamiento más adecuado para cada tumor.
Abstract Olfactory neuroblastoma (ONB) is a rare malignant tumor that originates from olfactory neuroepithelial cells. Its early diagnosis is difficult due to the low specificity of the symptoms. Imaging tests play an important role in its diagnosis and surgical planning so it is important that radiologists know their findings and the different classifications that will help to choose the most appropriate treatment for each tumor.
Subject(s)
Humans , Male , Female , Esthesioneuroblastoma, Olfactory/classification , Esthesioneuroblastoma, Olfactory/diagnostic imaging , Magnetic Resonance Spectroscopy/methods , Tomography, X-Ray Computed/methods , Esthesioneuroblastoma, Olfactory/surgery , Esthesioneuroblastoma, Olfactory/therapyABSTRACT
Resumen El estesioneuroblastoma (ENB), también conocido como neuroblastoma olfatorio, es una neoplasia maligna rara de la bóveda nasal que se cree que surge de las células receptoras neurosensoriales en el epitelio olfatorio. El ENB representa el 3% de todos los tumores nasales. Los tratamientos para ENB incluyen cirugía, radioterapia y/o quimioterapia, aunque a veces, es difícil lograr un tratamiento radical utilizando estas estrategias, ya que la mayoría de los pacientes se diagnostican en una etapa avanzada. Presentamos el caso de un varón de 46 años a quien se le diagnosticó ENB en el año 2016. Fue tratado inicialmente con resección endoscópica nasal, seguido de una craneotomía bifrontal. Posteriormente, recibió radioterapia adyuvante. Actualmente, su seguimiento a 3 años no muestra evidencia de metástasis locales, regionales o distantes, ni recurrencia.
Abstract Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare malignant neoplasm of the nasal vault that is believed to arise from neurosensory receptor cells in the olfactory epithelium. ENB accounts for 3% of all nasal tumors. The treatments for ENB include surgery, radiotherapy and/or chemotherapy, though sometimes, it is difficult to achieve radical treatment using these strategies as most patients are diagnosed at a late stage. We present a case of a 46-year old male who had been diagnosed with ENB in 2016. He was initially treated with nasal endoscopic resection, followed by a bifrontal craniotomy. Afterwards, he received adjuvant radiotherapy. Currently, his 3-year follow up show no evidence of local, regional o distant metastasis, nor recurrence.
Subject(s)
Humans , Male , Middle Aged , Nose Neoplasms/diagnosis , Esthesioneuroblastoma, Olfactory , Nasal Cavity/abnormalities , Costa RicaABSTRACT
Presentamos el caso de una mujer de 56 años con diagnóstico de estesioneuroblastoma Kadish C/Hyams II tratado con resección quirúrgica mediante un abordaje endoscópico endonasal puro y radioterapia adyuvante. Describimos el caso y discutimos sus aspectos relevantes.
We report the case of a 56-year-old woman with diagnosis of esthesioneuroblastoma Kadish C/Hyams II treated with a purely endonasal endoscopic resection and adjuvant radiotherapy. We described the case and discuss its relevant aspects.
Subject(s)
Humans , Female , Middle Aged , Endoscopy/methods , Esthesioneuroblastoma, Olfactory/surgery , Nose Neoplasms/surgery , Treatment OutcomeABSTRACT
Estesioneuroblastoma o también llamado neuroblastoma olfatorio es un tumor maligno infrecuente derivado del neuroepitelio olfatorio. La historia clínica de un paciente con un neuroblastoma olfatorio es muy inespecífica. Durante la última década, los métodos endoscópicos se han aplicado gradualmente para el tratamiento de tumores de la base del cráneo. El tratamiento puramente endoscópico endonasal del estesioneuroblastoma ha mostrado excelentes resultados de supervivencia con disminución de las complicaciones. Presentamos un paciente afectado por un neuroblastoma olfatorio tratado solo con cirugía endoscópica endonasal.
Esthesioneuroblastoma or olfactory neuroblastoma is an uncommon malignant tumor arising from the olfactory neuroepithelium. The clinical symptoms of a patient with olfactory neuroblastoma are very inespecific. During the past decade, endoscopic approaches have been gradually applied in treating skull base tumors. The purely endoscopic endonasal surgery for esthesioneuroblastoma showed successful survival results with remarkably decreased complications. We reported a patient who suffered olfactory neuroblastoma treated with only endoscopic endonasal surgery.
Subject(s)
Humans , Male , Adult , Endoscopy/methods , Esthesioneuroblastoma, Olfactory/diagnosis , Esthesioneuroblastoma, Olfactory/surgery , Nose Neoplasms/diagnosis , Nose Neoplasms/surgery , Skull BaseABSTRACT
Olfactory neuroblastoma is a rare malignant tumor. Although multiple therapeutic modalities including surgery, radio-therapy, or chemotherapy could be used in patients with olfactory neuroblastoma, no standardized treatment has been achieved. This re-view introduces a case of adult olfactory neuroblastoma treated by a multiple disciplinary team in Tianjin Medical University Cancer In-stitute and Hospital. This review also aims to explore a complete set of diagnostic and treatment practices for the benefit of future pa-tients.
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Introduction: Malignant sinonasal tumors comprise less than 1% of all neoplasms. A wide variety of tumors occurring primarily in this site can present with an undifferentiated or poorly differentiated morphology. Among them are esthesioneuroblastomas, sinonasal undifferentiated carcinomas, and neuroendocrine carcinomas. Objectives: We will discuss diagnostic strategies, recent advances in immunohistochemistry and molecular diagnosis, and treatment strategies. Data Synthesis: These lesions are diagnostically challenging, and up to 30% of sinonasal malignancies referred to the University of Texas MD Anderson Cancer Center are given a different diagnosis on review of pathology. Correct classification is vital, as these tumors are significantly different in biological behavior and response to treatment. The past decade has witnessed advances in diagnosis and therapeutic modalities leading to improvements in survival. However, the optimal treatment for esthesioneuroblastoma, sinonasal undifferentiated carcinoma, and neuroendocrine carcinoma remain debated. We discuss advances in immunohistochemistry and molecular diagnosis, diagnostic strategies, and treatment selection. Conclusions There are significant differences in prognosis and treatment for esthesioneuroblastoma, neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. Recent advances have the potential to improve oncologic outcomes but further investigation in needed...
Subject(s)
Humans , Carcinoma, Neuroendocrine/therapy , Esthesioneuroblastoma, Olfactory/therapy , Nose Neoplasms , Review Literature as TopicABSTRACT
Objetivos: presentar un caso de estesioneuroblastoma que desarrolla hipopituitarismo como complicación grave de su tratamiento. Realizar una revisión de la literatura reciente sobre el tratamiento y pronóstico de estos tumores. Presentar una sistemática de pesquisa de hipipituitarismo en estos pacientes. Descripción: paciente masculino de 68 años que se presenta con metástasis submaxilar de estesioneuroblastoma. La RMN mostraba una masa neoplásica de gran tamaño centrada sobre el etmoides, que comprometía base de cráneo y corteza fronto-orbitaria izquierda. Intervención: se realizó tratamiento combinado de radio y quimioterapia preoperatorios, y cirugía por vía craniofacial. A los 20 meses ingresa con un cuadro convulsivo e hiponatremia grave. Se hace diagnóstico de panhipopituitarismo y el cuadro revierte con la administración de hidrocortisona de 10 mg vía oral. Conclusión: el estesioneuroblastoma es un tumor infrecuente cuyo tratamiento incluye cirugía, radioterapia y quimioterapia. Es necesario un alto índice de sospecha y el seguimiento de por vida para pesquisar la instalación del hipopituitarismo como complicación grave de su tratamiento.
Objectives. To report a case of esthesioneuroblastoma associated to hypopituitarism as a late complication of its treatment, to review the literature regarding treatment and prognosis and to resent a guideline for the diagnosis of hypopituitarism in these patients. Description. A 68-year-old male patient presented with a submaxilar metastasis of a very large esthesioneuroblastoma that involved both ethmoidal bones and invaded left cribiform plate and frontal lobe. Intervention. The patient underwent combined modality treatment with preoperative radio and chemotherapy, and craniofacial sugery of the tumor. Twenty months later he was admitted with a seizure and severe hyponatremia. A diagnosis of panhypopituitarism was made, and his sodium reverted to normal with de administration of 10 mg of hydrocortisone p.o. Conclusions Esthesioneuroblastomas are rare tumours that require multimodality treatment with surgery, radiotherapy and chemotherapy. Hypopituitarism is a late and potentially fatal complication that must be suspected and tested during the patients whole lifespan.
Subject(s)
Esthesioneuroblastoma, Olfactory , General Surgery , Hyponatremia , HypopituitarismABSTRACT
PURPOSE: To report on the changes in the patterns of care and survival over time for esthesioneuroblastoma. MATERIALS AND METHODS: We retrospectively analyzed 42 previously untreated and histologically confirmed esthesioneuroblastoma patients seen between March 1989 and June 2007. According to Kadish's classification, 3 patients (7%) were stage A, 6 (14%) at stage B, and 33 (79%) at stage C. Of the 33 Kadish C patients, 19 and 14 patients were treated from 1989 through 2000 and from 2001 through 2007, respectively. Treatment included surgical resection, radiotherapy, chemotherapy, or a combination of these methods. Chemotherapy was administered to 8 of 19 patients (42%) seen from 1989 through 2000, whereas all of the 14 patients seen from 2001 through 2007 received chemotherapy (p<0.001). No patient was treated by three-dimensional conformal radiotherapy (3D-CRT) from 1989 through 2000, however 8 of 14 patients (67%) seen from 2001 through 2007 underwent 3D-CRT (p<0.001). The median follow-up time for surviving patients was 6.5 years (range, 2.2~15.8 years). RESULTS: The 5-year overall survival (OS) and progression-free survival (PFS) rates for the entire cohort were 53% and 39%, respectively. The 5-year OS was 100% for Kadish stages A or B and 39% for stage C (p=0.007). For patients with stage C disease who were treated from 1989 to 2000 and from 2001 to 2007, the 5-year OS rate was 26% and 59% (p=0.029), respectively and the corresponding 5-year PFS rate was 16% and 46% (p=0.001), respectively. Intraorbital extension and treatment era (1989~2000 vs. 2001~2007) were found as independent factors for OS and PFS in a multivariate analyses. CONCLUSION: The results of this study suggest that treatment era, which features a distinction in treatment modality and technique with the introduction of 3D-CRT, may be the cause of improved OS and PFS in Kadish stage C patients. To achieve better outcomes for patients with Kadish stage C, combined chemoradiotherapy, especially 3D-CRT, is recommended in addition to surgery.
Subject(s)
Humans , Chemoradiotherapy , Cohort Studies , Disease-Free Survival , Esthesioneuroblastoma, Olfactory , Follow-Up Studies , Multivariate Analysis , Prognosis , Radiotherapy, Conformal , Retrospective StudiesABSTRACT
Olfactory neuroblastoma is a rare malignant tumor of the nasal cavity arising from the olfactory neuroepithelium. It usually present as a polypoid mass in the superior nasal cavity including the cribriform plate, superior turbinate, and superior portion of nasal septum. The development of olfactory neuroblastoma outside of the region, in which olfactory epithelium exists, is exceedingly rare. In this report, we present an interesting case of an isolated sphenoid sinus olfactory neuroblastoma with a brief review of the literature.
Subject(s)
Esthesioneuroblastoma, Olfactory , Ethmoid Bone , Nasal Cavity , Nasal Septum , Olfactory Mucosa , Sphenoid Sinus , TurbinatesABSTRACT
El neuroblastoma olfatorio es una neoplasia neuroectodérmica maligna rara que afecta a la bóveda nasal y que tiende a invadir las estructuras vecinas, entre estas a la cavidad intracraneal. Su asociación con aneurismas intracraneales nunca ha sido descrita. Presentamos a una paciente con la coexistencia de un aneurisma del segmento oftálmico de la arteria carótida interna y un neuroblastoma olfatorio con invasión intracraneal. La coexistencia entre neoplasias del sistema nervioso central y aneurismas intracraneales es infrecuente. En este artículo analizamos las posibles causas de la asociación entre neoplasias y aneurismas intracraneales.
The olfactory neuroblastoma is a rare malignant neuroectodermal neoplasia of the nasal roof. It has a highly tendency of invades near structures including intracranial compartment. Associations between olfactory neuroblastoma with intracranial aneurysms have been never described. We show a case of a female with coexistence of an internal carotid artery aneurysm and olfactory neuroblastoma. The coexistence between nervous system tumors with intracranial aneurysms is not common. In this paper we analyze the association between intracranial neoplasia and aneurysms.
Subject(s)
Humans , Female , Adult , Esthesioneuroblastoma, Olfactory , Esthesioneuroblastoma, Olfactory/surgery , Esthesioneuroblastoma, Olfactory/complications , Esthesioneuroblastoma, Olfactory/diagnosis , Esthesioneuroblastoma, Olfactory , Esthesioneuroblastoma, Olfactory/therapy , Intracranial Aneurysm , Ophthalmic Nerve , Head and Neck Neoplasms , Central Nervous System/pathologyABSTRACT
Olfactory neuroblastoma is a relatively uncommon malignant tumor originating from neuroectoderm and it usually arises from the superior portion of the nasal cavity, such as superior turbinate, superior part of septum, or cribriform plate. Cases of olfactory neuroblastoma from sphenoid sinus, in which olfactory epithelium rarely exists, have been rarely reported. Although nasal obstruction and epistaxis are the most common presenting symptoms of olfatory neroblastoma, our patient presented with headache and dizziness. The most common cause of isolated sphenoid lesion is inflammation, but the possibility of tumorous conditions should not be excluded without thorough evaluation. The authors experienced a rare case of olfactory neuroblastoma from sphenoid sinus that had been confused as isolated sphenoid sinusitis. So we report our unusual case with a brief review of literature.
Subject(s)
Humans , Dizziness , Epistaxis , Esthesioneuroblastoma, Olfactory , Ethmoid Bone , Headache , Inflammation , Nasal Cavity , Nasal Obstruction , Neural Plate , Olfactory Mucosa , Sphenoid Sinus , Sphenoid Sinusitis , TurbinatesABSTRACT
Olfactory neuroblastoma is a rare tumor of nasal cavity arising from the olfactory neuroepithelium. It is usually presented as a polypoid mass high in the nasal vault including cribriform plate, superior turbinate, and superior portion of nasal septum. Recently, we experienced a case of olfactory neuroblastoma occurred from very atypical location. An 89-year-old man visited our department with symptoms of occasional left nasal bleeding and obstruction. On the endoscopic examination, a polypoid mass with bleeding tendency was observed in the left inferior meatus, but other portions of nasal cavity showed normal appearance. Biopsy was performed and the final pathologic report turned out as olfactory neuroblastoma. We report this unusually presenting case of olfactory neuroblastoma with a review of the literature.
Subject(s)
Aged, 80 and over , Humans , Biopsy , Epistaxis , Esthesioneuroblastoma, Olfactory , Ethmoid Bone , Hemorrhage , Nasal Cavity , Nasal Septum , TurbinatesABSTRACT
Olfactory neuroblastoma is a rare, malignant neoplasm arising from the olfactory epithelium. It has an aggressive biological behavior that is characterized by local recurrence, atypical distant metastasis, and poor long-term prognosis. The incidence of cervical lymph node metastasis in olfactory neuroblastoma is variable, and treatment modalities are controversial. Moreover, few reports have been published concerning retropharyngeal lymph node metastasis from olfactory neuroblastoma. We present two cases of olfactory neuroblastoma with retropharyngeal lymph node metastasis. In addition, we provided a review of the current literature regarding olfactory neuroblastoma and retropharyngeal lymph node metastasis from olfactory neuroblastoma.
Subject(s)
Esthesioneuroblastoma, Olfactory , Incidence , Lymph Nodes , Neoplasm Metastasis , Olfactory Mucosa , Prognosis , RecurrenceABSTRACT
OBJECTIVE: The authors analyzed clinical features, long-term treatment outcome, and prognostic factors of the olfactory neuroblastoma. METHODS: Twenty-one cases of olfactory neuroblastomas, treated from 1979 to 2000, were retrospectively reviewed with medical records and radiological findings. Mean follow-up periods are 28.7 months(range 4-178). Extent of tumor was classified by UCLA staging system. Statistical analysis for survival was done using Kaplan Meier method and log-lank test. RESULTS: Mean age was 27 years(13-62), and most common group are second decades(8/21, 38%). Male to female ratio was 13: 8. Common symptoms are nasal obstruction, epistaxis, exopthalmos and headache. There were three cases of T1, five T2, six T3, and seven T4 according to UCLA staging system. The 5-year survival rate was 21.3% and average time was 28.9 months in surgical resection group(n=14) as primary modality have higher survival rate than radiation and chemotherapy group(n=7)[2-year survival rate: 39.2% vs 14.3%, 5-year survival rate: 19.6% vs 14.3%(p=0.0274)]. Early stage(T1, T2) groups showed better survival rate than advanced(T3, T4) groups(38.1% vs 9.1% p=0.0336). The local and regional recurrences were observed in 6(27%) and 2(9%) cases. Mean recurrence free time was 7.8 months(range 1-25). CONCLUSION: Early detection and extent of resection are the important prognostic factors. Regular follow up is mandatary for the detection of recurrence or metastasis.
Subject(s)
Female , Humans , Male , Drug Therapy , Epistaxis , Esthesioneuroblastoma, Olfactory , Follow-Up Studies , Headache , Medical Records , Nasal Obstruction , Neoplasm Metastasis , Recurrence , Retrospective Studies , Survival Rate , Treatment OutcomeABSTRACT
PURPOSE: Rarity of olfactory neuroblastoma makes it difficult for treating physician to practice with a consistent protocol. This study is performed to analyze our experience with various treatment modalities for patients with olfactory neuroblastoma. Discussion includes review of some recently published literatures. MATERIALS AND METHODS: Between June of 1979 and April of 1997, 20 patients were treated under the diagnosis of olfactory neuroblastoma at Seoul National University Hospital. There were 14 male and 6 female patients. Age at initial treatment ranged from 13 to 77 years with median of 24 years. Fifteen of 20 patients had Kadish stage C. They were treated with various combinations of surgery, radiation therapy and chemotherapy; surgery + postoperative radiation therapy + adjuvant chemotherapy for 2 patients, surgery + postoperative radiation therapy for 6, neoadjuvant chemotherapy + surgery for 1, surgery + adjuvant chemotherapy for 1, surgery only for 2, neoadjuvant chemotherapy + radiation therapy for 3, radiation therapy + adjuvant chemotherapy for 1, radiation therapy only for 3, and no treatment for 1 patient. RESULTS:Follow-up ranged from 2 month to 204 months with mean of 39.6 months. The overall 5- and 10-year survival rates are 20% and 10%, respectably. Four patients are alive at the time of data analysis. One of four living patients was treated with radical surgery, postoperative radiation therapy and adjuvant chemotherapy, two patients with radical surgery and postoperative radiation therapy, and one with radical surgery only. CONCLUSIONS: Multidisciplinary approach, including radical surgery, pre- or post-operative radiation therapy and chemotherapy, should be addressed at the initial time of diagnosis. Although limited by small number of the patients, this study suggests importance of local treatment modality, especially radical surgery in the treatment of olfactory neuroblastoma.
Subject(s)
Female , Humans , Male , Chemotherapy, Adjuvant , Diagnosis , Drug Therapy , Esthesioneuroblastoma, Olfactory , Seoul , Statistics as Topic , Survival RateABSTRACT
Purpose:To evaluate the effects of surgery plus irradiation for olfactory neuroblastoma Methods:From January 1975 to February 1998 fourteen patients with olfactory neuroblastoma (kadish dassification:One case in stage A, 8 in the Stage B, 5 in stage C) were resected and giver radiotherapy to an average dose of 6000 cGy to primary lesions.Results:Six of the 14 patients have been living for 2—24 years. 8 died (5 of recurrence and 3 of distant metastasis)in 6 months to 4 years after diagnosis.Conclusions: There is a high rate of pathologic misdiagnosis in olfactory neuroblastoma. Immunohistologic examination and electron microscopic study are benifitial for diagnosis. Surgery plus irradiation is a suitable method for olfactory neuroblastoma. Radiotherapy is able to reduce local recurrence after surgery and increase resectability rate as well as to control distant metastasis.
ABSTRACT
BACKGROUND AND OBJECTIVES: Several strategies have been reported for the treatment of olfactory neuroblastoma such as radiation therapy, chemotherapy, craniofacial resection, or their combination. However, a consensus regarding the optimal management has not been obtained. In this study, we examined the outcomes of different treatment modalities in five patients with olfactory neuroblastoma. MATERIAL AND METHOD: Five patients diagnosed as olfactory neuroblastoma during the past 10 years were retrospectively analyzed. RESULTS: Five patients were categorized by the modified Kadish's staging system. Three patients underwent surgery. Radiotherapy was given concurrently with chemotherapy for two patients. Distant metastases were observed in two cases, one in the heart and the other in the cervical lymph node. After a median follow-up of 34 (12-87) months, two patients who underwent craniofacial resection only and another two patients who received combined radiotherapy and chemotherapy are still alive. One patient who underwent Denker's operation with postoperative radiotherapy had died of heart failure as a result of cardiac metastasis. CONCLUSION: We suggest a complete surgical resection as a primary treatment modality, however, a multi-drug chemotherapy with radiotherapy could be the choice of treatment in cases of children or one with distant metastasis.
Subject(s)
Child , Humans , Consensus , Drug Therapy , Esthesioneuroblastoma, Olfactory , Follow-Up Studies , Heart , Heart Failure , Lymph Nodes , Neoplasm Metastasis , Radiotherapy , Retrospective Studies , Treatment OutcomeABSTRACT
PURPOSETo analyze the role of radiotherapy in the treatment of olfactory neuroblastoma. METHODS From 1974 to 1994. 11 patients with olfactory neuroblastoma were treated in Xinjiang Cancer Hospital. All patients were confirmed histologically. Six cases were stage B and 5, stage C. Five patients were treated with surgery and postoperative radiotherapy (4. stage B and 1, stage C), and 6. with radiotherapy alone. The tumor dose ranged from 45~68 Gy, 2. 0 Gy/Fx. Neck irradiation with total dose of 60~65 Gy was carried out for 3 patients with cervical lymph node metastases. RESULTS Three patients had local recurrences and 2 cases, distant metastases. The actuarial overall survivals at 3 and 5 year were 55% and 36%, respectively. Three and five year survivals were 33%and 17% for the patients treated with radiotherapy alone, and 80% and 60% for the patients treated with surgery and postoperative irradiation, respectively(P= 0. 2727). CONCLUSION For the patients with stage B and C olfactory neuroblastoma, combined modality of surgery and radiotherapy should be the treatment of choice.
ABSTRACT
Purpose:To analyze retrospectively the clinical manifestation, treatment and prognosis. Methods:From 1978 to 1996,16 patients with olfactory neuroblastoma were treated.4 cases were stage A,6 cases were stage B and 6 cases were stage C. Six patients were treated with radiotherapy alone or combined with chemotherapy, 9, with surgery and postoperative radiotherapy and 1,with operative alone. The radiation dosage ranged from 40～70Gy with 2 Gy per day. Results:The 1 , 3 and 5 year survival rates were 75%, 75% and 69%.Conclusions:Olfactory neuroblastoma is sensitivity for radiotherapy, for the patients with stage A, radiotherapy alone is effective for stage B and C olfactory neuroblastoma , combined modality of surgery and postoperative radiotherapy should be the treatment of choice.