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Advances in Oto-rhino-laryngology 2020Olfactory neuroblastoma is a rare tumor. Nasal endoscopy typically identifies a soft mass arising from the olfactory cleft. Computer tomography and magnetic resonance... (Review)
Review
Olfactory neuroblastoma is a rare tumor. Nasal endoscopy typically identifies a soft mass arising from the olfactory cleft. Computer tomography and magnetic resonance imaging are mandatory for staging (in association with 18F-fluorodeoxyglucose positron emission tomography) in high-grade and/or high-stage tumors. Biopsy must be representative to confirm a diagnosis and for grading purposes. Two complementary classifications are described: one (Kadish) based on clinical-radiological analysis, and the other (Hyams) on histological criteria. Based on Hyams grading, studies have pointed out that grades III-IV entail significantly different behavior and prognosis. A multimodal approach, which may combine surgery, chemotherapy, and radiotherapy, is essential to manage these tumors. Treatment schedules which include surgery seem to be superior to others. Surgery classically consisted of anterior craniofacial resection to obtain good exposure. However, the role of transnasal endoscopic surgery has expanded because of its association with fewer complications, shorter hospital stays, and comparable oncologic results to the open surgical techniques. Unilateral endoscopic craniectomy can be performed for limited lesions to avoid definitive anosmia. Treatment that includes radio- and chemotherapy is recommended for advanced and high-grade tumors. The role of neoadjuvant chemotherapy in advanced-stage lesions is emerging. The main prognostic factors associated with poor patient outcome are Hyams grade III-IV, Kadish C-D, and positive surgical margins. Lifelong follow up is recommended.
Topics: Combined Modality Therapy; Endoscopy; Esthesioneuroblastoma, Olfactory; Female; Humans; Male; Nasal Cavity; Nose Neoplasms; Prognosis
PubMed: 32731231
DOI: 10.1159/000457935 -
Current Oncology Reports Feb 2018To provide a comprehensive review of the literature focusing on the recent advances in the diagnosis and management of olfactory neuroblastoma. (Review)
Review
PURPOSE OF REVIEW
To provide a comprehensive review of the literature focusing on the recent advances in the diagnosis and management of olfactory neuroblastoma.
RECENT FINDINGS
Multimodality treatment is usually recommended for the majority of ONB cases. Recent advances in surgical approaches include the evolving role of endonasal endoscopic surgical resection and reconstruction. The introduction of new conformal radiation techniques has improved the outcomes and reduced treatment-related toxicity to important structures such as the eye and the brain. The role of neoadjuvant and adjuvant chemotherapy is yet to be defined. In the last two decades, there have been advances in surgical techniques with endoscopic approaches, either alone or in combination with craniotomy, gradually replacing the open traditional approaches. Prolonged surveillance is recommended for ONB due to late recurrences associated with that tumor. The role of chemotherapy and elective neck irradiation is still controversial and needs more studies to investigate their optimal role.
Topics: Animals; Combined Modality Therapy; Esthesioneuroblastoma, Olfactory; Humans; Neuroblastoma; Nose Neoplasms
PubMed: 29411144
DOI: 10.1007/s11912-018-0661-6 -
Head and Neck Pathology Sep 2009Few neoplasms are unique to the sinonasal tract, but sinonasal undifferentiated carcinoma and olfactory neuroblastoma are malignant tumors which require unique... (Review)
Review
Few neoplasms are unique to the sinonasal tract, but sinonasal undifferentiated carcinoma and olfactory neuroblastoma are malignant tumors which require unique management. Due to the rarity of these tumors, practicing pathologists are not always aware of their distinctive clinical, radiographic, histologic, immunohistochemical, and molecular features. These cases are frequently submitted for consultation, further suggesting the diagnostic difficulties inherent to these tumors. Specifically, olfactory neuroblastoma is a neoplasm that can histologically mimic many tumors within the sinonasal tract, making recognition of this tumor important, as the management frequently requires a bicranial-facial surgical approach, a trephination procedure which can be quite technically difficult and challenging to achieve a good result. The management is therefore quite unique in comparison to other sinonasal tract malignancies, setting it apart diagnostically and managerially from other lesions.
Topics: Diagnosis, Differential; Esthesioneuroblastoma, Olfactory; Humans; Nasal Cavity; Nose Neoplasms
PubMed: 20596981
DOI: 10.1007/s12105-009-0125-2 -
Virchows Archiv : An International... Apr 2024Olfactory neuroblastomas are uncommon malignancies that arise from olfactory receptor cells located high in the nasal cavity. Accurate diagnosis plays a crucial role in... (Review)
Review
Olfactory neuroblastomas are uncommon malignancies that arise from olfactory receptor cells located high in the nasal cavity. Accurate diagnosis plays a crucial role in determining clinical results and guiding treatment decisions. Diagnosis can be a major challenge for pathologists, especially when dealing with tumours with poor differentiation. The discovery of several molecular and immunohistochemical markers would help to overcome classification difficulties. Due to the paucity of large-scale studies, standardisation of diagnosis, treatment and prediction of outcome remains a challenge. Surgical resection by endoscopic techniques with the addition of postoperative irradiation is the treatment of choice. In addition, it is advisable to consider elective neck irradiation to minimise the risk of nodal recurrence. Molecular characterisation will help not only to make more accurate diagnoses but also to identify specific molecular targets that can be used to develop personalised treatment options tailored to each patient. The present review aims to summarise the current state of knowledge on histopathological diagnosis, the molecular biology and management of this disease.
Topics: Humans; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Nasal Cavity; Biomarkers, Tumor
PubMed: 38386106
DOI: 10.1007/s00428-024-03758-z -
British Journal of Neurosurgery Apr 2023Olfactory neuroblastoma (ONB) is a rare tumour of the skull base, typically originating from the nasal cavity and around the cribriform plate. We present the rare case... (Review)
Review
Olfactory neuroblastoma (ONB) is a rare tumour of the skull base, typically originating from the nasal cavity and around the cribriform plate. We present the rare case of ONB originating from and limited to the sphenoid sinus in a 42-year old lady. Pre-operatively the lesion was thought to be a sinonasal polyp and underwent functional endoscopic sinus surgery (FESS) and total excision of the polypoid lesion. Review of histology unexpectedly revealed ONB. She underwent further surgery to ensure wide local excision was achieved with negative margins on histology, followed by radiotherapy. This is only the third reported case of ONB limited to the sphenoid sinus and the ninth reported case of primary sphenoid ONB in the literature. We review the literature pertaining with primary sphenoidal ONB here and suggest complete resection is indicated in ectopic ONB, not unlike classical ONB. There may be a role for adjuvant oncological treatments and lifelong follow up in a multidisciplinary approach is recommended.
Topics: Female; Humans; Adult; Esthesioneuroblastoma, Olfactory; Sphenoid Sinus; Nasal Cavity; Skull Base; Nose Neoplasms
PubMed: 35174758
DOI: 10.1080/02688697.2022.2038779 -
Current Oncology Reports Jan 2023The purpose of review is to provide a comprehensive review of the literature focusing on the recent advances in the diagnosis, molecular underpinning, and targeted... (Review)
Review
PURPOSE OF REVIEW
The purpose of review is to provide a comprehensive review of the literature focusing on the recent advances in the diagnosis, molecular underpinning, and targeted therapy of olfactory neuroblastoma (ONB).
RECENT FINDINGS
Studies focused on the molecular fingerprinting of ONB are critical to engage new promising treatment strategies. Molecular-based subtype classifications have been proposed (basal-like ONB and neural-like ONB) but are not widely used. The rationale for implementation of DNA methylation analysis and IDH2 sequencing in routine work-up for ONB is gaining recognition. Expression of somatostatin receptors (SSTR) in ONB open new avenues for both, diagnostic (especially metastatic disease) and new treatment protocols with somatostatin analogs. Olfactory carcinoma is proposed as a unifying diagnostic terminology pertinent to epithelial divergent differentiation in olfactory neuroblastoma. Molecular (genetic and epigenetic) efforts on olfactory neuroblastoma are promising; however further refinement is needed for employment of these biomarkers as clinical standard of care. Ongoing and future multi-institutional collaborative studies will contribute to further understanding of ONB biology and aid the development of targeted treatments for this disease.
Topics: Humans; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Nasal Cavity
PubMed: 36449116
DOI: 10.1007/s11912-022-01348-3 -
Veterinary Clinical Pathology Sep 2023Nasal tumors account for less than 10% of all feline neoplasms, with lymphoma, followed by adenocarcinoma, and squamous cell carcinoma, the most commonly reported. Nasal... (Review)
Review
Nasal tumors account for less than 10% of all feline neoplasms, with lymphoma, followed by adenocarcinoma, and squamous cell carcinoma, the most commonly reported. Nasal neuroectodermal tumors, including olfactory neuroblastoma (ONB), are scarcely described, and their tumorigenesis is largely unknown. Here we report the cytological, histological, and immunohistochemical features of a feline ONB. We also provide a pathological review of nasal neuroendocrine neoplasms in cats. A 7-year-old Burmese cat was evaluated for sneezing, occasional epistaxis, and upper respiratory noise for 8 months. Computed tomography (CT) imaging revealed a 7 × 5 × 3 mm irregular mass effacing and expanding the nasal cavity, which extended to the nasopharynx. Cytologically, neoplastic cells were round to polygonal and had a round nucleus with finely stippled chromatin, a single small nucleolus, and abundant pale blue cytoplasm, which contained abundant fine pale pink granules. They exhibited mild cellular atypia, anisocytosis, and mild to occasionally moderate anisokaryosis. Rhinoscopic biopsies revealed a densely cellular, malignant neuroepithelial neoplasm. Cells were arranged in densely packed trabeculae and formed Homer Wright and Flexner-Wintersteiner-like rosettes, with rare mitotic figures and scant supportive fibrovascular stroma. Immunohistochemically, neoplastic cells were positive for vimentin, cytokeratin AE1/AE3, COX-2, and beta-tubulin and negative for S-100, chromogranin A, CD117, and epithelial membrane antigen (EMA). An ONB was diagnosed based on histological and immunohistochemical findings. Interestingly, and similar to nasal carcinomas, neoplastic cells diffusely neo-expressed COX-2. To the authors' knowledge, there is no previous evidence of COX-2 in feline ONB. Histopathology and immunohistochemistry are required for a definitive diagnosis of ONB.
Topics: Cats; Animals; Esthesioneuroblastoma, Olfactory; Cyclooxygenase 2; Nose Neoplasms; Nasal Cavity; Carcinoma; Cat Diseases
PubMed: 37468966
DOI: 10.1111/vcp.13255 -
Journal of Neuro-oncology Dec 2020Sinonasal tumors that harbor neuroendocrine histologic features include olfactory neuroblastoma (previously known as esthesioneuroblastoma), sinonasal neuroendocrine... (Review)
Review
INTRODUCTION
Sinonasal tumors that harbor neuroendocrine histologic features include olfactory neuroblastoma (previously known as esthesioneuroblastoma), sinonasal neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. These tumors represent a diverse spectrum of clinical behavior and as such require histology-specific management. Herein, we review the management of these sinonasal tumors with neuroendocrine features and discuss fundamentals of multi-modality care for each histology. An emphasis is placed on olfactory neuroblastomas, given their relative frequency and skullbase origin.
METHODS
A comprehensive literature review on contemporary management of olfactory neuroblastoma, sinonasal neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma was performed.
RESULTS
Management of sinonasal tumors with neuroendocrine features can include surgical resection, radiation therapy, and/or chemotherapy. Due to their site of origin, these tumors can frequently involve the skullbase, which can require site-specific care. The optimal treatment modalities and the sequence in which they are performed are largely dependent on histology. In most cases, olfactory neuroblastoma is best managed with surgical resection followed by radiation therapy. Sinonasal neuroendocrine carcinomas represent a variety of histologic phenotypes (carcinoid, atypical carcinoid, small cell, and large cell), which determine the optimal treatment modality. Finally, sinonasal undifferentiated carcinoma is likely best managed by induction chemotherapy with subsequent therapy dictated by the initial response.
CONCLUSIONS
A team approach to multi-modality care is essential in the treatment of olfactory neuroblastoma, sinonasal neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. Early biopsy, histologic diagnosis, and comprehensive imaging are critical to determining the appropriate management paradigm.
Topics: Animals; Carcinoma; Carcinoma, Neuroendocrine; Combined Modality Therapy; Disease Management; Esthesioneuroblastoma, Olfactory; Humans; Maxillary Sinus Neoplasms; Nose Neoplasms; Skull Base Neoplasms
PubMed: 32424573
DOI: 10.1007/s11060-020-03537-1 -
Indian Journal of Pathology &... 2023Herein we present a case of a 62-year-old male patient who was admitted with the chief complaints of nasal obstruction. The histopathological and immunohistochemical... (Review)
Review
Herein we present a case of a 62-year-old male patient who was admitted with the chief complaints of nasal obstruction. The histopathological and immunohistochemical evaluation led to a diagnosis of olfactory neuroblastoma with rhabdomyoblasts. A review of the literature revealed that this is only the fourth case of olfactory neuroblastoma with rhabdomyoblasts. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and identify the best treatment to improve prognosis.
Topics: Male; Humans; Middle Aged; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Tomography, X-Ray Computed; Nasal Cavity
PubMed: 37077088
DOI: 10.4103/ijpm.ijpm_209_21 -
Cancer Research Communications Jun 2023Olfactory neuroblastoma is a rare tumor arising from the olfactory cleft region of the nasal cavity. Because of the low incidence of this tumor, as well as an absence of...
UNLABELLED
Olfactory neuroblastoma is a rare tumor arising from the olfactory cleft region of the nasal cavity. Because of the low incidence of this tumor, as well as an absence of established cell lines and murine models, understanding the mechanisms driving olfactory neuroblastoma pathobiology has been challenging. Here, we sought to apply advances from research on the human olfactory epithelial neurogenic niche, along with new biocomputational approaches, to better understand the cellular and molecular factors in low- and high-grade olfactory neuroblastoma and how specific transcriptomic markers may predict prognosis. We analyzed a total of 19 olfactory neuroblastoma samples with available bulk RNA-sequencing and survival data, along with 10 samples from normal olfactory epithelium. A bulk RNA-sequencing deconvolution model identified a significant increase in globose basal cell (GBC) and CD8 T-cell identities in high-grade tumors (GBC from ∼0% to 8%, CD8 T cell from 0.7% to 2.2%), and significant decreases in mature neuronal, Bowman's gland, and olfactory ensheathing programs, in high-grade tumors (mature neuronal from 3.7% to ∼0%, Bowman's gland from 18.6% to 10.5%, olfactory ensheathing from 3.4% to 1.1%). Trajectory analysis identified potential regulatory pathways in proliferative olfactory neuroblastoma cells, including PRC2, which was validated by immunofluorescence staining. Survival analysis guided by gene expression in bulk RNA-sequencing data identified favorable prognostic markers such as SOX9, S100B, and PLP1 expression.
SIGNIFICANCE
Our analyses provide a basis for additional research on olfactory neuroblastoma management, as well as identification of potential new prognostic markers.
Topics: Mice; Humans; Animals; Esthesioneuroblastoma, Olfactory; Olfactory Mucosa; Olfactory Pathways; Nose Neoplasms; RNA
PubMed: 37377616
DOI: 10.1158/2767-9764.CRC-23-0013