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Esthesioneuroblastoma: Unraveling the Enigma and Emerging Paradigms With a Retrospective Cohort and Review of Literature.

作者信息

Gokulanathan Narendhar, Ikkurthi Vedamanasa, Sundaramoorthy Senthamizhan, Raj Madhan

机构信息

Medical Oncology, Apollo Hospitals, Bangalore, IND.

Radiation Oncology, Homi Bhabha Cancer Hospital and Research Centre (Tata Memorial Center), Chandigarh, IND.

出版信息

Cureus. 2025 Jul 15;17(7):e87985. doi: 10.7759/cureus.87985. eCollection 2025 Jul.


DOI:10.7759/cureus.87985
PMID:40821301
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12354911/
Abstract

Background Esthesioneuroblastoma (ENB), or olfactory neuroblastoma, is a rare malignancy arising from the olfactory epithelium. Standardized treatment protocols are lacking, and existing knowledge is largely derived from retrospective case series.  Methods  This study was undertaken to evaluate treatment outcomes and toxicities associated with multimodal therapy in a rare malignancy. We retrospectively reviewed the clinical, pathological, radiological, and dosimetric data of patients diagnosed with ENB and treated them at the Regional Cancer Centre, Jawaharlal Institute of Postgraduate Medical Education & Research (JIPMER), between 2015 and 2022. All patients underwent baseline imaging, including contrast-enhanced CT of the head, neck, thorax, and abdomen and MRI of the head and neck. Treatment included combinations of neoadjuvant chemotherapy, craniofacial resection, and intensity-modulated radiotherapy (IMRT). Radiological response assessment and clinical follow-up were performed periodically, with the last follow-up in March 2023.  Results  Around eight patients aged 17-68 years (median: 53 years) were included. Most patients presented with Kadish stage C (n=5), followed by stage D (n=3). T3 disease was predominant (7/8), with nodal involvement in four patients. Histologically, most tumors were Hyams grade 3 (n=7), neuron-specific enolase-positive, with mitotic indices of 7-17/10 high-power field. All patients received IMRT (66-70 Gy); five underwent R0 surgery and adjuvant radiotherapy, while three received radical chemoradiation. Neoadjuvant chemotherapy (cisplatin and etoposide) was administered in seven patients and adjuvant in one. At a median follow-up of 51.5 months, four patients were alive and disease-free, one had locoregional recurrence, and three had died (one due to recurrence, one due to a second malignancy, and one unrelated). Treatment-related toxicities were manageable and generally resolved with supportive care.  Conclusion This case series highlights the feasibility and efficacy of multimodal therapy incorporating surgery, radiotherapy, and chemotherapy in advanced-stage ENB. With careful staging, aggressive local therapy, systemic treatment, and long-term disease control is achievable. However, larger multicentric studies and molecular diagnostics are necessary to formulate management strategies and refine prognostication in this rare tumor.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9268/12354911/d87d8303e82d/cureus-0017-00000087985-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9268/12354911/a03a9dc066d0/cureus-0017-00000087985-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9268/12354911/8b3dc9445fe1/cureus-0017-00000087985-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9268/12354911/d87d8303e82d/cureus-0017-00000087985-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9268/12354911/a03a9dc066d0/cureus-0017-00000087985-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9268/12354911/8b3dc9445fe1/cureus-0017-00000087985-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9268/12354911/d87d8303e82d/cureus-0017-00000087985-i03.jpg

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本文引用的文献

[1]
A Review on Esthesioneuroblastoma.

Indian J Otolaryngol Head Neck Surg. 2022-10

[2]
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Front Oncol. 2020-7-17

[3]
Hyams grading as a predictor of metastasis and overall survival in esthesioneuroblastoma: a meta-analysis.

Int Forum Allergy Rhinol. 2019-6-28

[4]
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World Neurosurg. 2019-3-9

[5]
Comparing Kadish, TNM, and the modified Dulguerov staging systems for esthesioneuroblastoma.

J Surg Oncol. 2019-1

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Cancers (Basel). 2018-11-20

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Comprehensive Genomic Profiling of Esthesioneuroblastoma Reveals Additional Treatment Options.

Oncologist. 2017-7

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The Eighth Edition AJCC Cancer Staging Manual: Continuing to build a bridge from a population-based to a more "personalized" approach to cancer staging.

CA Cancer J Clin. 2017-1-17

[9]
Multimodal treatment and long-term outcome of patients with esthesioneuroblastoma.

Oral Oncol. 2013-6-5

[10]
Stereotactic body radiation therapy for locally recurrent, previously irradiated nonsquamous cell cancers of the head and neck.

Head Neck. 2011-11-11

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