Esthesioneuroblastoma is not a member of the primitive peripheral neuroectodermal tumour-Ewing's group.
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Esthesioneuroblastoma (ENB) is a rare, site-specific, locally aggressive neuronal malignancy so far thought to belong to primitive peripheral neuroectodermal tumour-Ewing's tumour (pPNETs-ETs). Its anatomical location, in addition to morphologic, immunophenotypic and ultrastructural features, suggests its origin in the neuronal or neuroendocrine cells of the olfactory epithelium. However, the cytogenetic and molecular data currently available appear controversial on the presence of the typical translocation t(11;22)(q24;q12) and of trisomy 8, chromosomal changes that characterize the tumours belonging to the pPNETs-ETs. Herein we have analysed five ENB tumour specimens for trisomy 8 by fluorescence in situ hybridization (FISH), for the presence of EWS gene rearrangements by FISH, reverse transcription polymerase chain reaction and Southern blot analyses, as well as for the expression of the Ewing sarcoma-associated MIC2 antigen by immunohistochemistry. Neither EWS/FLI-I, EWS/ERG and EWS/FEV fusion genes nor MIC2 expression were found in any tumour, whereas trisomy 8 was found in one case only. Moreover, DNA from three cases analysed by Southern blot did not show EWS gene rearrangements. Our results support the evidence that ENB is not a member of the pPNETs-ETs. (+info)
Esthesioneuroblastoma: case report.
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Esthesioneuroblatoma (ENB) is a rare tumor arising from the olfactory epithelium of the nasal vault which frequently invades the cranial base, cranial vault and orbit. ENB has a bimodal age distribution between 11 and 20 years and between 51 and 60 years. ENB accounts for approximately 1 to 5% of intranasal cancers and no consensus has been reached regarding treatment of this tumor. We report on a 66 year old female patient with a Kadish stage C tumor with frontal lobe invasion submitted a total craniofacial resection with a combined head neck and neurosurgeon team. The purpose of this study is to analyze the natural history, treatment and prognosis of this tumor, based on the literature review. (+info)
Treatment of intracranial metastatic esthesioneuroblastoma.
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BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon tumor that may metastasize to the central nervous system (CNS). To the authors' knowledge there is no current consensus regarding treatment. The current study was conducted to determine the toxicity and response rate of combined modality therapy in the treatment of patients with ENB metastatic to the brain. METHODS: Six patients (2 men and 4 women) ranging in age from 47-61 years (median age, 53.5 years) with ENB had clinical and neuroradiographic evidence of CNS metastases (brain parenchyma in 6 patients and leptomeningeal metastases in 3 patients). CNS-directed therapy included radiotherapy (to the brain in three patients and to the spine in two patients) and chemotherapy (systemic in six patients and regional in three patients). Systemic chemotherapy was comprised of carboplatin, lomustine, and vincristine administered every 2 months. Patients were evaluated by neuroradiographic and neurologic examination every other month. RESULTS: Between 1-6 cycles of systemic chemotherapy were administered (median, 4.5 cycles). and 6-19 cycles of regional chemotherapy were administered (median, 17 cycles). Toxicity included aseptic meningitis (three patients), radiation enteritis (one patient), and > or = Grade 3 (according to the National Cancer Institute Common toxicity Criteria) myelosuppression (four patients). Two patients required hospitalization for neutropenic fever and two patients required a transfusion (platelets in two patients and red blood cells in one patient). No treatment-related deaths were reported. A partial response was achieved in four patients, and two patients demonstrated progressive disease. The median duration of response was 9 months (range, 2-12 months). Overall survival ranged from 3-13 months (median, 10.5 months). The cause of death was progressive parenchymal brain disease in three patients, systemic disease in two patients, and leptomeningeal disease in one patient. CONCLUSIONS: In the small cohort of patients in the current study, combined modality therapy was found to have modest toxicity and palliative efficacy. (+info)
Aesthesioneuroblastoma in a woodworker.
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A case is described of aesthesioneuroblastoma in a woodworker who had been exposed to wood dust for 25 years, without any individual or environmental protection. The case described supports the contention that occupational exposure to wood dust may have caused the neoplasm. (+info)
Successful treatment of metastatic esthesioneuroblastoma.
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This case report describes a patient with a metastasised olfactorial esthesioneuroblastoma Hyams grade 4 who has been treated with debulking surgery and radiotherapy. After relapse in lymph node, lung and brain, he received additional irradiation and six cycles of carboplatin, vincristine and cyclophosphamide intravenously every three weeks. The patient has now been disease free for 7.8 years. Our data suggest that metastatic esthesioneuroblastoma is sensitive to platinum-based chemotherapy. This patient illustrates that this tumour is very sensitive to platinum-based chemotherapy and that durable complete response can be achieved, even in a metastatic ENB. (+info)
Malignant tumors of the anterior skull base.
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BACKGROUND: Cancers of the paranasal sinuses or nasal cavity are the most common malignant tumors of the anterior skull base. Several types of tumors occur in this location, including cancers of endodermal, mesodermal, and epidermal origins. Although anterior skull base surgery is a relatively recent approach in treating these tumors, widespread changes have already occurred in procedural methods and treatment goals. METHODS: We review the tumor types that occur in the anterior skull base and discuss the current treatment options, including multimodal therapy and the team approach to surgery. Surgical techniques are also described. RESULTS: Management of anterior skull base cancer is complex due to the anatomic detail of the region and the variety of cancers that occur in this area. Currently, the "gold standard" for surgery is the anterior craniofacial approach. Combined with adjuvant radiation therapy, 5-year disease-free survival rates have increased to 50%, with some tumors such as adenocarcinomas and esthesioneuroblastomas reaching up to 80% 5-year survival rates. Potential complications include cerebrospinal fluid leakage, meningitis, abscess formation, and pneumocephalus. CONCLUSIONS: Treatment of anterior skull base cancer is complex due to the significant anatomic detail of the region and the variety of cancers that occur in this area. Multimodal therapy through a team approach is the optimal management approach for these tumors. (+info)
Human achaete-scute homologue (hASH1) mRNA level as a diagnostic marker to distinguish esthesioneuroblastoma from poorly differentiated tumors arising in the sinonasal tract.
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Distinction of high-grade esthesioneuroblastomas from other poorly differentiated tumors arising in the nasal cavity is an important diagnostic challenge because it determines patient management and prognosis. The human achaete-scute homologue (hASH1) gene is critical in olfactory neuronal differentiation and is expressed in immature olfactory cells; therefore, it could have potential use as a diagnostic marker The aim of the present study was to determine the value of hASH1 messenger RNA (mRNA) levels in differentiating esthesioneuroblastoma from other poorly differentiated tumors. A real-time polymerase chain reaction assay was developed, permitting the comparative determination of hASH1 mRNA levels in triplicate in a double-blind pilot study including 24 frozen cases of esthesioneuroblastoma and poorly differentiated tumors. All 4 positive cases were esthesioneuroblastomas, and all 19 poorly differentiated tumors were negative. In addition, there was an inverse association between the grade of esthesioneuroblastomas and hASH1 mRNA levels. The hASH1 mRNA level might represent a useful tool for distinguishing esthesioneuroblastoma from poorly differentiated tumors of the sinonasal region. (+info)
Combination chemotherapy (cyclophosphamide, doxorubicin, and vincristine with continuous-infusion cisplatin and etoposide) and radiotherapy with stem cell support can be beneficial for adolescents and adults with estheisoneuroblastoma.
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BACKGROUND: Adolescent-onset and adult-onset esthesioneuroblastoma is a rare disease and is considered incurable. In many patients, local resection and radiation are chosen as clinical therapy with or without chemotherapy. It was reported previously that local resection and radiotherapy led to temporary remission and, in many patients, recurrent disease. Although combination with chemotherapy has been regarded as promising, an effective regimen has not been established. In the current study, the authors investigated the effect and tolerability of the combination of chemotherapy, radiotherapy, and peripheral blood stem cell transplantation (PBSCT). METHODS: The study population included 12 patients with adolescent-onset and adult-onset esthesioneuroblastoma classified as Kadish Stage A-D. The patients received two cycles of combination chemotherapy, which consisted of cyclophosphamide, doxorubicin, and vincristine (CAD) with continuous-infusion cisplatin and etoposide (CVP). This was combined with radiotherapy with or without PBSCT. RESULTS: Nine of 12 patients (75%) obtained more than a partial response after only 2 cycles of chemotherapy. After radiation with or without PBSCT, six patients obtained a complete remission (CR). The longest survival was > 3 years. All patients who underwent PBSCT obtained a CR. The most severe side effects were loss of sodium and potassium induced by cisplatin-related renal tubular distress. Those abnormalities were temporary, and all patients recovered. CONCLUSIONS: The chemotherapy regimen with CADO and CVP does not require continuation for a long time and is very effective and tolerable for patients with adolescent-onset and adult-onset esthesioneuroblastoma. The combination with radiotherapy and PBSCT may lead to a CR without facial disfigurement. In this report, the authors discuss the feasibility and efficacy of this multidisciplinary approach. (+info)