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Esthesioneuroblastoma

  • Author: Michael Somenek, MD; Chief Editor: Jules E Harris, MD, FACP, FRCPC  more...
 
Updated: Apr 14, 2015
 

Background

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.

Images of esthesioneuroblastomas are shown below.

Esthesioneuroblastoma. Coronal CT scan of the orbi Esthesioneuroblastoma. Coronal CT scan of the orbits and sinuses shows a large, enhancing, and expansile mass occupying the ethmoid air cells that is invading the cribriform plate and breaking through to the left anterior cranial fossa. Image courtesy of Michael Lev, MD.
Esthesioneuroblastoma. A 39-year-old man presented Esthesioneuroblastoma. A 39-year-old man presented with 1 month of decreased vision, left facial numbness, and swelling. Physical examination demonstrated left-sided exophthalmos and blindness. He had also lost his sense of smell. Contrast-enhanced T1-weighted MRI demonstrated a large lesion that originated in the paranasal sinuses and extended through the cribriform plate into the anterior cranial fossa. He underwent a bifrontal craniotomy for resection of this tumor.

The prognosis depends on the magnitude of the disease on initial diagnosis. It should be noted that precise histologic diagnosis is difficult because ENBs are often confused with other small round cell neoplasms of the nasal cavity. Despite the difficulties associated with the treatment of ENB, evolving treatment modalities, including surgery, radiation, and adjuvant chemotherapy, have contributed to the better management and increased survival of ENB patients. This article provides an updated summary of ENB, including ongoing research, current thought on pathophysiology, staging, and an update on new surgical techniques used to treat these tumors.[2]

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Pathophysiology

Esthesioneuroblastomas (ENBs) are undifferentiated tumors of neuroectodermal origin derived from the olfactory epithelium.[3] 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 ENBs, and this ambiguity can confound clinical and prognostic decisions. In essence, ENBs contain variable arrangements of their small cells. Additionally, there exists a variable presence (or absence) of true rosettes and neurofibrillary material.

To date, no certain genetic factor has been identified that can accurately assist in the diagnosis or predict prognosis. This is partially due to the ability to analyze cancer genomes on a whole genome basis. Recently, a tool called array comparative genomic hybridization was applied to the analysis of ENBs.[4] Although many alterations were identified in this study, chromosomal gains in 7q11 and 20q and deletions in 2q, 5q, 6p, 6q, and 18q have been confirmed by at least 2 other studies. Interestingly, 20q is a region that has been implicated in other cancers, including breast, ovarian, and squamous cell carcinoma. Still, further experimentation will be required to determine the role of these genomic regions in ENB.

The demonstration of human achaete-scute homologue (HASH1) gene expression, although still investigational, could become the diagnostic procedure of choice.[5] The HASH1 gene is involved in olfactory neuronal differentiation and is expressed in immature olfactory cells[6] ; therefore, it could be useful in distinguishing ENB from other poorly differentiated small blue cell tumors.

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Epidemiology

Frequency

United States

The incidence of esthesioneuroblastoma in the United States follows trends observed worldwide.

International

Based on the reports in the literature, approximately 1,200 cases of esthesioneuroblastoma (ENB) have been identified since 1924. Interestingly, 80% of these have been identified in the last 25 years. However, the current data set cannot distinguish between a rising incidence and better recognition of the disease. ENB has an estimated incidence of 4 cases per 10 million individuals and accounts for approximately 5% of all sinonasal tumors. Similar incidence rates have been obtained through epidemiologic studies performed in Denmark.[7] To our knowledge, no studies suggest a geographic deviation.

Race

Esthesioneuroblastoma (ENB) does not show a predilection toward any individual race. ENB does not show familial prevalence and has been reported in all races and on all continents.

Sex

Esthesioneuroblastoma (ENB) affects males and females with similar frequency.

Age

Esthesioneuroblastoma (ENB) occurs in a wide range of age groups (3-90 y). There exists a bimodal peak of occurrence in the third and sixth decades of life.

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Contributor Information and Disclosures
Author

Michael Somenek, MD Physician, Facial Plastic and Reconstructive Surgery, Ruff Plastic Surgery

Michael Somenek, MD is a member of the following medical societies: Alpha Omega Alpha, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Guy J Petruzzelli, MD, PhD, MBA, FACS Physician-in-Chief and Vice President of Oncology Programs, Curtis and Elizabeth Anderson Cancer Institute at Memorial University Medical Center; Professor of Surgery-Head, Neck, and Endocrine Surgery, Mercer University School of Medicine-Savannah Campus

Guy J Petruzzelli, MD, PhD, MBA, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Association for the Advancement of Science, American College of Surgeons, American Head and Neck Society, American Medical Association, Chicago Medical Society, North American Skull Base Society, Society of Surgical Oncology, Society of University Otolaryngologists-Head and Neck Surgeons, SWOG, American Association of Clinical Anatomists, American Society of Clinical Oncology, International Academy of Oral Oncology, International Head and Neck Scientific Group, Georgia Society of Otolaryngology-Head and Neck Surgery

Disclosure: Nothing to disclose.

Nicholas C Shera, PhD Freelance Medical/Scientific Writer

Disclosure: Nothing to disclose.

Thomas C Origitano, MD, PhD, FACS Professor and Chair, Department of Neurological Surgery, Medical Director, Neuroscience Service Line, Co-Director, Center for Cranial Base Surgery, Loyola University Medical Center

Thomas C Origitano, MD, PhD, FACS is a member of the following medical societies: American Association of Neurological Surgeons, American College of Surgeons, North American Skull Base Society, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Jules E Harris, MD, FACP, FRCPC Clinical Professor of Medicine, Section of Hematology/Oncology, University of Arizona College of Medicine, Arizona Cancer Center

Jules E Harris, MD, FACP, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Society of Hematology, Central Society for Clinical and Translational Research, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Additional Contributors

Robert C Shepard, MD, FACP Associate Professor of Medicine in Hematology and Oncology at University of North Carolina at Chapel Hill; Vice President of Scientific Affairs, Therapeutic Expertise, Oncology, at PRA International

Robert C Shepard, MD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for Physician Leadership, European Society for Medical Oncology, Association of Clinical Research Professionals, American Federation for Clinical Research, Eastern Cooperative Oncology Group, Society for Immunotherapy of Cancer, American Medical Informatics Association, American College of Physicians, American Federation for Medical Research, American Medical Association, American Society of Hematology, Massachusetts Medical Society

Disclosure: Nothing to disclose.

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Esthesioneuroblastoma. Coronal CT scan of the orbits and sinuses shows a large, enhancing, and expansile mass occupying the ethmoid air cells that is invading the cribriform plate and breaking through to the left anterior cranial fossa. Image courtesy of Michael Lev, MD.
Esthesioneuroblastoma. A 39-year-old man presented with 1 month of decreased vision, left facial numbness, and swelling. Physical examination demonstrated left-sided exophthalmos and blindness. He had also lost his sense of smell. Contrast-enhanced T1-weighted MRI demonstrated a large lesion that originated in the paranasal sinuses and extended through the cribriform plate into the anterior cranial fossa. He underwent a bifrontal craniotomy for resection of this tumor.
Table. Histopathologic Grading According to Hyams [14]
Grade Lobular Architecture Preservation Mitotic Index Nuclear Polymorphism Fibrillary Matrix Rosettes Necrosis
I + Zero None Prominent HW rosettes None
II + Low Low Present HW rosettes None
III +/- Moderate Moderate Low FW rosettes Rare
IV +/- High High Absent None Frequent
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