Esthesioneuroblastoma other diagnostic studies: Difference between revisions

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'''For immunohistochemistry, click [[Esthesioneuroblastoma differential diagnosis|here]].'''  
'''For immunohistochemistry, click [[Esthesioneuroblastoma differential diagnosis|here]].'''  
'''For histopathology, click [[Esthesioneuroblastoma pathophysiology|here]].'''
'''For histopathology, click [[Esthesioneuroblastoma pathophysiology|here]].'''
Clinical presentation is usually secondary to nasal stuffiness and rhinorrhoea or epistaxis. Presentation is often delayed and symptoms may have been present for many months. Patients often present late with larger tumours which can extend into the intracranial compartment (25-30% at diagnosis) and usually result in anosmia. radio
Because many of the symptoms associated with ENB are similar to those of inflammatory disease and other benign conditions of the sinonasal cavity, there is often a delay in the diagnosis. In most studies, the mean time from onset of symptoms to diagnosis of ENB ranges between 6–12 months. For this reason, many patients have advanced stage at the time of diagnosis. A high index of suspicion is therefore critical to achieve an accurate and timely diagnosis.
Many patients undergo sinus surgery, only to have the diagnosis established as an unexpected pathologic finding. Therefore, sending all the tissue removed during sinus surgery for pathologic examination is important for diagnosis of esthesioneuroblastoma, as is the vigilance of the pathologist in examining the tissue.
For the most part, malignancy is not considered until secondary symptoms such as facial pain and deformity or cranial nerve impairment are observed. However, early referral for an intranasal biopsy is essential to early diagnosis.[8] A patient with a unilateral nasal obstruction and/or recurrent epistaxis lasting longer than 1-2 months should undergo a thorough nasal evaluation by an otolaryngologist, although the cost-effectiveness of this approach has not been evaluated
CNS metastasis is usually noted 0–10 years after the initial diagnosis and reported as having a survival expectancy of 2 years or less [13]. Spinal metastasis of ENB is rare with only 28 documented cases involving the spine, spinal cord, or leptomeninges. We report a case of ENB with multiple drop metastasis and significant progression of the tumor despite multiple aggressive surgical resections in conjunction with chemotherapy and radiation therapy.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Revision as of 03:34, 27 January 2016

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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

Biopsy may be helpful in the diagnosis of esthesioneuroblastoma. Findings on biopsy diagnostic of esthesioneuroblastoma include small, round-to-oval cells with coarsely granular chromatin, prominent nuclear membranes, multiple small nucleoli, and scant cytoplasm with pseudorosette or occasional Homer-Wright rosettes separated by fibrous septa. Calcification and necrosis are sometimes seen, and mitotic figures are rare.

Other Diagnostic Studies

  • Biopsy and endoscopy should be performed under general anesthesia. The specimen should be sent for regular staining, as well as for immunohistochemistry and possibly electron microscopy.

For immunohistochemistry, click here. For histopathology, click here.


Clinical presentation is usually secondary to nasal stuffiness and rhinorrhoea or epistaxis. Presentation is often delayed and symptoms may have been present for many months. Patients often present late with larger tumours which can extend into the intracranial compartment (25-30% at diagnosis) and usually result in anosmia. radio

Because many of the symptoms associated with ENB are similar to those of inflammatory disease and other benign conditions of the sinonasal cavity, there is often a delay in the diagnosis. In most studies, the mean time from onset of symptoms to diagnosis of ENB ranges between 6–12 months. For this reason, many patients have advanced stage at the time of diagnosis. A high index of suspicion is therefore critical to achieve an accurate and timely diagnosis.

Many patients undergo sinus surgery, only to have the diagnosis established as an unexpected pathologic finding. Therefore, sending all the tissue removed during sinus surgery for pathologic examination is important for diagnosis of esthesioneuroblastoma, as is the vigilance of the pathologist in examining the tissue.

For the most part, malignancy is not considered until secondary symptoms such as facial pain and deformity or cranial nerve impairment are observed. However, early referral for an intranasal biopsy is essential to early diagnosis.[8] A patient with a unilateral nasal obstruction and/or recurrent epistaxis lasting longer than 1-2 months should undergo a thorough nasal evaluation by an otolaryngologist, although the cost-effectiveness of this approach has not been evaluated CNS metastasis is usually noted 0–10 years after the initial diagnosis and reported as having a survival expectancy of 2 years or less [13]. Spinal metastasis of ENB is rare with only 28 documented cases involving the spine, spinal cord, or leptomeninges. We report a case of ENB with multiple drop metastasis and significant progression of the tumor despite multiple aggressive surgical resections in conjunction with chemotherapy and radiation therapy.


References

Template:Central nervous system tumors

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