Esthesioneuroblastoma natural history, complications and prognosis
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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
Natural History
Esthesioneuroblastoma tumors display varying clinical behaviors ranging from indolent growth to highly aggressive invasion.[1] The average delay between the appearance of the initial symptom and the diagnosis is six months, however diagnosis may be delayed for years in some cases. The delay is predictable because initial symptoms tend to be subtle and are frequently common, occurring also in nasal diseases, including allergic polypoid sinus disease or long-term rhinosinusitis.[2] Despite aggressive therapy, esthesioneuroblastoma has been noted to have a high local recurrence rate of approximately 50–60% with 10–62% presenting as metastatic cases and 20–30% of those cases involving the CNS. Once esthesioneuroblastoma cells invade the cribriform plate, they may spread to the anterior skull base, extend to the leptomeninges or brain parenchyma and can lead to drop metastasis anywhere in central neural axis.[3]
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.
Complications
Prognosis
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.
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Treatment usually involves combined chemotherapy and/or radiotherapy with surgical excision. Prognosis is significantly affected by presence of distant metastases.
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References
- ↑ Kane, Ari J., et al. "Posttreatment prognosis of patients with esthesioneuroblastoma: clinical article." Journal of neurosurgery 113.2 (2010): 340-351.
- ↑ Zhang M, Zhou L, Wang DH, Huang WT, Wang SY (2010). "Diagnosis and management of esthesioneuroblastoma". ORL J Otorhinolaryngol Relat Spec. 72 (2): 113–8. doi:10.1159/000278255. PMID 20453548.
- ↑ Shirzadi, Ali S.; Drazin, Doniel G.; Strickland, Allison S.; Bannykh, Serguei I.; Johnson, J. Patrick (2013). "Vertebral Column Metastases from an Esthesioneuroblastoma: Chemotherapy, Radiation, and Resection for Recurrence with 15-Year Followup". Case Reports in Surgery. 2013: 1–8. doi:10.1155/2013/107315. ISSN 2090-6900.