Esthesioneuroblastoma: A Comprehensive Review of Diagnosis, Management, and Current Treatment Options

Fiani, Brian; Quadri, Syed A.; Cathel, Alessandra; Farooqui, Mudassir; Ramachandran, Anirudh; Siddiqi, Imran; Ghanchi, Hammad; Zafar, Atif; Berman, Blake; Siddiqi, Javed

doi:10.1016/j.wneu.2019.03.014

Cited by 81 publications

(85 citation statements)

References 132 publications

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“…17 No geographic, environmental or lifestyle risk factors are associated with these tumours. 19 The initial diagnostic work-up should include a thorough physical (including neurological) examination and a combination of sinus CT and MRI is usually required to assess the degree of local invasion. CT of the neck is also paramount to evaluate for nodal involvement.…”

Section: Discussionmentioning

confidence: 99%

Treatment outcomes of sinonasal tumours with neuroendocrine features

Valente

Farinha

Jacóme

et al. 2021

Int J Otorhinolaryngol Head Neck Surg

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Background: Sinonasal tumours with neuroendocrine immunophenotype include olfactory neuroblastoma (ONB), sinonasal neuroendocrine carcinoma (SNEC) and sinonasal undifferentiated carcinoma (SNUC). These neoplasms usually present in advanced stages and are associated with poor outcome. This study describes the clinical features of these tumours and analyzes treatment outcomes of patients with these malignancies.Methods: Retrospective chart review of all patients with sinonasal tumours diagnosed from 2009 to 2019, in a tertiary cancer centre. Clinical and histopathological prognostic factors were determined by univariate analysis. Overall survival was estimated using Kaplan-Meier method. Results: A total of 27 patients (77.8% male) with a mean age of 52.0±16.8 years were included in the study. ONB was diagnosed in 9 patients, SNEC in 10 patients and SNUC in 8 patients. TNM stage IV disease was found in 20 patients (74.0%) at presentation. According to staging and treatment results, curative therapy was attempted in 21 patients (77.8%), of whom sixteen (76.2%) received multimodality treatment. Overall mean survival was 49 months and 1-year, 3-years and 5-years overall survival rates were 70.5%, 47.3% and 37.8%, respectively. Patients with SNEC had worse overall survival (p=0.044). Regarding treatment options, patients with SNUC treated with surgery and adjuvant radiation therapy had improved overall survival (p=0.027), as well as patients with SNEC selected for endoscopic resection surgery (p=0.049).Conclusions: Accurate histologic diagnosis, grading, and clinical staging are essential for characterization and treatment selection in this heterogeneous group of sinonasal tumours. Consensus in the management of these tumours is lacking due to their rarity, difficulties in diagnosis and diverse current treatment approaches.

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Section: Discussionmentioning

confidence: 99%

Treatment outcomes of sinonasal tumours with neuroendocrine features

Valente

Farinha

Jacóme

et al. 2021

Int J Otorhinolaryngol Head Neck Surg

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“…The role of chemotherapy was not clear 6 . A recent review of 149 articles also suggested surgical resection followed by radiotherapy as the standard approach for higher‐grade tumors 7 …”

Section: Discussionmentioning

confidence: 99%

“…6 A recent review of 149 articles also suggested surgical resection followed by radiotherapy as the standard approach for higher-grade tumors. 7 A beneficial role of chemotherapy in ONB has been suggested owing to its biological behavior similar to other chemosensitive neural crest tumors like high-grade neuroendocrine tumors. 8 however, not well defined.…”

Section: Casementioning

confidence: 99%

Facial disfigurement due to olfactory neuroblastoma: beauty regained with chemotherapy

et al. 2020

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Background Olfactory neuroblastoma (ONB) is a sinonasal malignancy seldom seen in clinical practice. It is also known by various other names like esthesioneuroblastoma, esthesioneuroepithelioma, esthesioneurocytoma, and esthesioneuroma. Surgery and radiation therapy are considered as standard treatment modalities for ONB; however, the role of chemotherapy is not well established. Aims We aim to define the role of chemotherapy in the neoadjuvant setting in a case of ONB. Methods and Results We report a young female patient presenting with a naso‐facial swelling causing facial disfigurement, proptosis, decreased visual acuity, and poor performance status. She was diagnosed with advanced‐stage ONB. Prompt administration of chemotherapy led to the improvement in the symptoms and rapid regression of the tumor mass. Later on, the tumor mass was excised completely without any neurological deficit. Conclusion This report justifies the role of neoadjuvant chemotherapy in the management of ONB.

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“…Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is also a rare malignant neoplasm of the nasal cavity that arises from the olfactory neuroepithelium. ENB represents about 3%–6% of sinonasal tract tumors with an incidence of 0.4 cases per million and approximately 1200 cases have been reported to date 6,7 . ENB can be histologically difficult to distinguish from other small round cell malignant tumors of the sinonasal tract including SNEC, sinonasal undifferentiated carcinoma (SNUC), lymphoma, rhabdomyosarconoma and mucosal malignant melanoma 6,8 .…”

Section: Introductionmentioning

confidence: 99%

“…Distant metastasis is indicative of a poor prognosis 6,9 . Recommendations for diagnosis and management of ENB are currently limited and controversial given the rarity of pathology 7 …”

Section: Introductionmentioning

confidence: 99%

The value of ⁶⁸Gallium‐DOTATATE PET/CT in sinonasal neuroendocrine tumor management: A case series

et al. 2021

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Background 68Gallium‐DOTATATE (68Ga‐DOTATATE) is a somatostatin analog used as a PET tracer to successfully identify neuroendocrine tumors (NETs). Due to the rarity of sinonasal NETs, there are few recommendations for 68Ga‐DOTATATE imaging in these patients. Methods We discussed the impact of 68Ga‐DOTATATE imaging on the management of six sinonasal NET cases and reviewed existing literature. Results 68Ga‐DOTATATE PET/CT revealed an unknown primary in one case and identified metastatic disease in a primary sinonasal small cell neuroendocrine carcinoma (SNEC) patient missed on conventional imaging. In two esthesioneuroblastoma (ENB) patients, 68Ga‐DOTATATE detected abnormal radiotracer uptake not present on 18F‐FDG PET/CT and identified a patient for treatment with 177Lu‐DOTATATE. Conclusions This is the one of the first few reports, and the largest series to our knowledge, demonstrating the utility of 68Ga‐DOTATATE imaging for primary sinonasal SNEC and ENB. Further study is required to determine its role in sinonasal NET management.

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Esthesioneuroblastoma: A Comprehensive Review of Diagnosis, Management, and Current Treatment Options

Cited by 81 publications

References 132 publications

Treatment outcomes of sinonasal tumours with neuroendocrine features

Treatment outcomes of sinonasal tumours with neuroendocrine features

Facial disfigurement due to olfactory neuroblastoma: beauty regained with chemotherapy

The value of ⁶⁸Gallium‐DOTATATE PET/CT in sinonasal neuroendocrine tumor management: A case series

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Esthesioneuroblastoma: A Comprehensive Review of Diagnosis, Management, and Current Treatment Options

Cited by 81 publications

References 132 publications

Treatment outcomes of sinonasal tumours with neuroendocrine features

Treatment outcomes of sinonasal tumours with neuroendocrine features

Facial disfigurement due to olfactory neuroblastoma: beauty regained with chemotherapy

The value of 68Gallium‐DOTATATE PET/CT in sinonasal neuroendocrine tumor management: A case series

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The value of ⁶⁸Gallium‐DOTATATE PET/CT in sinonasal neuroendocrine tumor management: A case series