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Frontiers in Surgery 2021Our purpose was to estimate the safety and effectiveness of the endoscopic endonasal approach (EEA) in olfactory neuroblastoma (ONB) and determine whether preservation...
OBJECTIVE
Our purpose was to estimate the safety and effectiveness of the endoscopic endonasal approach (EEA) in olfactory neuroblastoma (ONB) and determine whether preservation of the dura and olfactory bulb could be considered in selected patients.
METHODS
We retrospectively reviewed patients diagnosed with ONBs between July 2010 and June 2021 at our institution, and collected data on demographic, disease stage, surgical approach, overall survival (OS), disease-free survival (DFS), and postoperative complications.
RESULTS
The study sample included 42 patients (8 treated for recurrence and 34 initial cases), 28 of which were men and 14 were women with a median age of 47.19 years. The mean duration from the beginning of treatment and follow-up time was 8.91 and 51 months, respectively. Among the 42 patients, 32 had unilateral lesions, and the rest had bilateral lesions. Patient symptoms were predominantly nasal, and four patients presented without any symptoms. The modified Kadish staging was A in three patients, B in 14 patients, C in 17 patients, and D in 8 patients. According to the preoperative examinations, five patients had cervical lymph node metastasis, and no patients had distant metastases. EEA was used in 38 patients, cranioendoscopic approach in 3, and open craniofacial approach in 1. The 5-year OS and DFS rates were 89.1 and 79.2%, respectively. The 2-year OS and DFS rates were both 89.1%. The overall surgical complication incidence was 9.52% (one cerebrospinal fluid rhinorrhea, one cervical hematoma, and two epileptic seizures).
CONCLUSION
The present results support the importance of earlier treatment for advanced ONB and the fact that it is safe and efficacious to treat ONBs EEA. The preservation of the dura can be considered for select patients-specifically those without skull base involvement and who underwent postoperative comprehensive therapy.
PubMed: 35178425
DOI: 10.3389/fsurg.2021.799405 -
Advances in Anatomic Pathology Mar 2023Genetic profiling has caused an explosion in the subclassification of sinonasal malignancies. Distinguishing several of these tumor types by histomorphology alone has... (Review)
Review
INTRODUCTION
Genetic profiling has caused an explosion in the subclassification of sinonasal malignancies. Distinguishing several of these tumor types by histomorphology alone has been quite challenging, and although pathologic classification aims to be as specific as possible, it remains to be seen if this recent move toward tumor speciation bears clinical relevance, most particularly focused on subtyping for the sake of prognostication and treatment. One such recently described cohort, predominantly lumped under the moniker of sinonasal undifferentiated carcinoma (SNUC) is IDH2 -mutated sinonasal carcinoma, a high-grade carcinoma associated with mutations in the isocitrate dehydrogenase-2 ( IDH2 ) gene. A hotspot mutation in the R172 codon has been described in 50% to 80% of the tumors classified as SNUC, large cell neuroendocrine carcinomas, and rarely in cases classified as olfactory neuroblastoma. The use of immunohistochemical and molecular approaches is required to correctly identify this subset of sinonasal tumors, with further study necessary to elucidate their unique pathophysiology, ultimately determining whether a revision is required toward the current therapeutic approach.
AIMS
Here, we provide an overview of the IDH2- mutated sinonasal tumors, discuss histopathologic and clinical features, and focus on molecular diagnostics and novel immunohistochemical markers.
RESULTS
A review of the literature reveals 82 reported cases with IDH2 -mutated sinonasal tumors (IST), confirmed either by molecular studies or diagnostic immunohistochemical markers. The mean patient age is 60 years (female/male: 1/1.4), the median tumor size is 5 cm (range: 2.5 to 7.0 cm), and the most common location is the nasal cavity (81%). IST displays tumor necrosis and increased mitotes. Histopathologically, IST shows SNUC-like, large cell neuroendocrine carcinomas-like, or poorly differentiated carcinoma-like features (77%, 12%, and 9%, respectively). The molecular hotspot alterations in mitochondrial IDH2 are: R172S (61%), R172T (19%), R172G (7%), and R172M (3%). Sixty-five percent of tumors are surgically resectable, and all patients received chemotherapy, radiation therapy, or both. Rates of locoregional recurrence and distant metastasis are 60% and 40%, respectively. One-, 3- and 5-year survival rates are 83%, 50%, and 43%, respectively. In all but 1 study, IST is associated with better outcomes than IDH2 wild-type tumors and SMARCB1 -deficient sinonasal tumors.
Topics: Humans; Male; Female; Middle Aged; Maxillary Sinus Neoplasms; Carcinoma, Neuroendocrine; Neoplasms, Glandular and Epithelial; Mutation; Biomarkers, Tumor
PubMed: 36537260
DOI: 10.1097/PAP.0000000000000391 -
Modern Pathology : An Official Journal... May 2023Olfactory neuroblastoma (ONB, esthesioneuroblastoma) is a sinonasal cancer with an underdeveloped diagnostic toolkit, and is the subject of many incidents of tumor...
Olfactory neuroblastoma (ONB, esthesioneuroblastoma) is a sinonasal cancer with an underdeveloped diagnostic toolkit, and is the subject of many incidents of tumor misclassification throughout the literature. Despite its name, connections between the cancer and normal cells of the olfactory epithelium have not been systematically explored and markers of olfactory epithelial cell types are not deployed in clinical practice. Here, we utilize an integrated human-mouse single-cell atlas of the nasal mucosa, including the olfactory epithelium, to identify transcriptomic programs that link ONB to a specific population of stem/progenitor cells known as olfactory epithelial globose basal cells (GBCs). Expression of a GBC transcription factor NEUROD1 distinguishes both low- and high-grade ONB from sinonasal undifferentiated carcinoma, a potential histologic mimic with a distinctly unfavorable prognosis. Furthermore, we identify a reproducible subpopulation of highly proliferative ONB cells expressing the GBC stemness marker EZH2, suggesting that EZH2 inhibition may play a role in the targeted treatment of ONB. Finally, we study the cellular states comprising ONB parenchyma using single-cell transcriptomics and identify evidence of a conserved GBC transcriptional regulatory circuit that governs divergent neuronal-versus-sustentacular differentiation. These results link ONB to a specific cell type for the first time and identify conserved developmental pathways within ONB that inform diagnostic, prognostic, and mechanistic investigation.
Topics: Humans; Mice; Animals; Esthesioneuroblastoma, Olfactory; Paranasal Sinus Neoplasms; Neurons; Nose Neoplasms; Nasal Cavity
PubMed: 36841178
DOI: 10.1016/j.modpat.2023.100122 -
Seminars in Diagnostic Pathology Mar 2016Various hematolymphoid lesions involve the sinonasal tract, including aggressive B, T, and NK-cell neoplasms; myeloid sarcoma; low-grade lymphomas; indolent... (Review)
Review
Various hematolymphoid lesions involve the sinonasal tract, including aggressive B, T, and NK-cell neoplasms; myeloid sarcoma; low-grade lymphomas; indolent T-lymphoblastic proliferations; and Rosai-Dorfman disease. Differentiating aggressive lymphomas from non-hematopoietic neoplasms such as poorly differentiated squamous cell carcinoma, olfactory neuroblastoma, or sinonasal undifferentiated carcinoma may pose diagnostic challenges. In addition, the necrosis, vascular damage, and inflammatory infiltrates that are associated with some hematolymphoid disorders can result in misdiagnosis as infectious, autoimmune, or inflammatory conditions. Here, we review hematolymphoid disorders involving the sinonasal tract including their key clinical and histopathologic features.
Topics: Biomarkers, Tumor; Biopsy; Histiocytosis, Sinus; Humans; Immunohistochemistry; Leukemia-Lymphoma, Adult T-Cell; Lymphoma; Nasal Cavity; Nose Neoplasms; Paranasal Sinus Neoplasms; Prognosis
PubMed: 26472692
DOI: 10.1053/j.semdp.2015.09.008 -
Indian Journal of Otolaryngology and... Oct 2022Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Olfactory neuroblastoma centered in the posterior right orbit with prominent...
Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Olfactory neuroblastoma centered in the posterior right orbit with prominent orbital protrusion is even rare. Grading ONB is extremely important as individualized treatment plans must be formulated according to tumor grade. We report the case of a 67-year-old female who presented with the chief complaints of persistent nasal congestion with intermittent epistaxis and unilateral proptosis over the past five years. Radiological imaging was suggestive of a large heterogeneous mass in the right superior nasal cavity with extensions into the right medial orbit, nasopharynx, the right maxillary sinus, the anterior cranial fossa, right ethmoidal, frontal and bilateral sphenoidal sinuses, as well as into the right frontal lobe. Assessment of the radiologic features revealed the diagnostic possibility of olfactory neuroblastoma. A nasopharyngeal biopsy confirmed an olfactory neuroblastoma. Frontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities, paranasal sinuses and right medial orbit from below was done. Evaluation of histopathological characteristics and immunohistochemical findings revealed a diagnosis of WHO grade IV olfactory neuroblastoma. Because of poor economic condition, the patient did not take adjuvant radiotherapy and chemoradiation and post-operative examination. We report a huge ONB centered in the posterior right orbit with prominent orbital protrusion. Magnetic resonance image and computed tomography are helpful for evaluating the appearance and the extent of ONB, as well as grading this tumor, which may aid therapeutic decisions and improve survival.
PubMed: 36452830
DOI: 10.1007/s12070-020-02359-x -
Journal of Neurological Surgery. Part... Feb 2016This presentation outlines the clinical and imaging characteristics of esthesioneuroblastoma. (Review)
Review
This presentation outlines the clinical and imaging characteristics of esthesioneuroblastoma.
PubMed: 26949582
DOI: 10.1055/s-0035-1564053 -
Journal of Neurological Surgery Reports Jul 2022Esthesioneuroblastoma is a rare malignancy originating from the olfactory epithelium. Treatment consists of surgical resection with strong consideration for adjuvant...
Esthesioneuroblastoma is a rare malignancy originating from the olfactory epithelium. Treatment consists of surgical resection with strong consideration for adjuvant treatment in advanced Kadish stage and high Hyams grade. In the modern era, overall outcomes for esthesioneuroblastoma are favorable compared with many other sinonasal malignancies with 5-year overall survival estimated to be 80%. When selecting the optimal surgical approach, the surgeon must consider the approach that will allow for a negative margin resection and adequate reconstruction. In appropriately selected patients, endoscopic outcomes appear at least equivalent to open approaches and unilateral endoscopic approach may be used in select olfactory preservation cases.
PubMed: 35832684
DOI: 10.1055/s-0042-1753519 -
Head and Neck Pathology Mar 2016Neuroendocrine neoplasms (NENs) can occur in organs or tissues that do not contain neuroendocrine cells normally and do not necessarily imply embryologic derivation from... (Review)
Review
Neuroendocrine neoplasms (NENs) can occur in organs or tissues that do not contain neuroendocrine cells normally and do not necessarily imply embryologic derivation from the neuroectoderm; but rather reflect a shared phenotype characterized by the expression of multiple genes encoding both endocrine and neuronal features. NENs are rare in the sinonasal tract and are subdivided into epithelial and neural subtypes based on the presence of keratins or neurofilaments, respectively. Although relatively rare, neuroendocrine carcinomas (NECs) and olfactory neuroblastoma (ONB) are the most common neuroendocrine neoplasms of the sinonasal tract. The focus of this review is to highlight recent developments in the pathology of sinonasal NECs and ONB in light of the upcoming update of the World Health Organization (WHO) 2005 classification of tumors of the head and neck.
Topics: Carcinoma, Neuroendocrine; Esthesioneuroblastoma, Olfactory; Humans; Nasal Cavity; Nose Neoplasms; Paranasal Sinus Neoplasms
PubMed: 26830400
DOI: 10.1007/s12105-016-0696-7 -
Neuro-ophthalmology (Aeolus Press) 2022We report a case of 51-year-old medically healthy male who presented with gradual painless diminution of vision in both eyes for 3 months. His visual acuity was hand...
We report a case of 51-year-old medically healthy male who presented with gradual painless diminution of vision in both eyes for 3 months. His visual acuity was hand movement perception in the right eye, and no light perception in the left eye. The intraocular pressure, external eye examination, ocular motility and anterior segment examinations were normal. Fundus examination revealed severe bilateral papilloedema and engorged tortuous veins in both eyes. Imaging exhibited a large intracranial tumour causing raised intracranial pressure. This was debulked by neurosurgery and histological examination revealed that the tumour was an olfactory neuroblastoma (ONB). This case is noteworthy since ONBs rarely present with isolated papilloedema without any accompanying ophthalmoplegia, proptosis, epistaxis, nasal, or neurological symptoms.
PubMed: 35574165
DOI: 10.1080/01658107.2021.1958872 -
Bulletin Du Cancer Dec 2012Esthesioneuroblastoma is an uncommon malignancy originating from olfactive epithelium. Men are more frequently affected than women. Nasal symptoms are the most common... (Review)
Review
Esthesioneuroblastoma is an uncommon malignancy originating from olfactive epithelium. Men are more frequently affected than women. Nasal symptoms are the most common revealing signs. Immunohistochemistry helps diagnosis. There is no randomized trial evaluating treatment due to the low incidence of this tumor. Radiotherapy and surgery are the standard of care. Radiotherapy is benefic even in early stage disease. Chemotherapy is indicated in case of locally advanced or metastatic disease.
Topics: Antineoplastic Agents; Esthesioneuroblastoma, Olfactory; Female; Humans; Male; Nasal Cavity; Nose Neoplasms; Prognosis; Radiotherapy; Rare Diseases
PubMed: 23022763
DOI: 10.1684/bdc.2012.1642