Esthesioneuroblastoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
Esthesioneuroblastoma is a rare form of cancer involving nasal cavity and believed to arise from the olfactory epithelium. It can cause loss of vision, and taste. It is often considered synonymous with "olfactory neuroblastoma", but the tissue of origin is not yet well characterized. Esthesioneuroblastomas are undifferentiated tumors of neuroectodermal origin derived from the olfactory epithelium. The tumor cells are mitotically active and are the precursor cells that develop into sustentacular and neuronal cells. Inconsistent histologic presentations initially led to controversy surrounding the exact histologic origin of esthesioneuroblastomas, and this ambiguity can confound clinical and prognostic decisions. In essence, esthesioneuroblastomas contain variable arrangements of their small cells. Additionally, there exists a variable presence (or absence) of true rosettes and neurofibrillary material. They usually present as a soft tissue mass in the superior olfactory recess involving the anterior and middle ethmoid air-cells on one side and extending through the cribriform plate into the anterior cranial fossa. Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare neoplasm originating from olfactory neuroepithelium. Approximately 1,000 cases have been identified since Berger and Luc described the first case in 1924.[1] Due to the rare and complex nature of ENB, multiple opinions exist regarding the etiology, optimal staging system, and treatment modalities. These tumors often display varying biologic activity ranging from indolent growth, with patient survival exceeding 20 years, to a highly aggressive neoplasm capable of rapid widespread metastasis, with survival limited to a few months. Olfactory neuroblastomas, also known as esthesioneuroblastomas, are tumours arising from the basal layer of the olfactory epithelium in the superior recess of the nasal cavity. They usually present as a soft tissue mass in the superior olfactory recess involving the anterior and middle ethmoid air-cells on one side and extending through the cribriform plate into the anterior cranial fossa. Contrast enhancement is often marked in both CT and MRI studies. Olfactory neuroblastoma (ONB), also referred to as esthesioneuroblastoma, is a rare malignant tumor of neuroectodermal origin. Olfactory neuroblastomas are thought to arise from the olfactory epithelium.[1]
Historical Perspective
Classification
Pathophysiology
Causes
Differential Diagnosis
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications, and Prognosis
Staging
History and Symptoms
Physical Examination
Laboratory Findings
CT
MRI
Other Imaging Studies
Other Diagnostic Studies
Medical Therapy
Primary Prevention
Secondary Prevention
Esthesioneuroblastoma is a rare form of cancer involving nasal cavity and believed to arise from the olfactory epithelium. The tissue of origin is not yet well characterized.
References
- ↑ Barnes, Leon. Pathology and genetics of head and neck tumours. Lyon: IARC Press, 2005. Print.