Sinonasal Tumors Research Articles

Editorial introductions.

Editorial introductions.

Temporal evolution of quality of life in patients endoscopically treated for sinonasal malignant tumors.

The aim of our study is to assess which factors may affect the quality of life (QoL) and its fluctuation over time in adult patients who received endonasal endoscopic oncologic sinus surgery (EOSS) for sinonasal malignancies (SNM) in our center. We analyzed EOSS cases for primary SNM from January 2015 to June 2020. For each patient, we have recorded the age at treatment, gender, smoking habits, use of psychotropic drugs for mood disorders, stage, histotype, type of surgical resection, need for skull-base reconstruction, development of postoperative major complications, and the use of adjuvant intensity-modulated radiotherapy (IMRT). We evaluated the patient's performance status pre-treatment using the ECOG scale. Quality of life was measured using three questionnaires (SNOT-22; ASK-9; EORTC QLQ-C30 version 3). Fifty-five patients were enrolled in our study, of whom thirty-two (58.18%) received adjuvant IMRT. Overall, a significant improvement in all QoL outcomes was observed at eighteen months, while, female sex, higher ECOG scores, advanced stage of disease, and adjuvant IMRT were associated with worse QoL. After 18 months the delta in QoL between women and men worsened (in SNOT-22 and EORTC QLQ-GLOBAL) while if only the most fragile patients according to ECOG are considered, this difference was reduced for both tools. Our analysis revealed that IMRT is the element that has the greatest impact on patient's quality of life, in association with the female sex, ECOG >2, and advanced stage of the disease.

Imaging Recommendations for Diagnosis, Staging, and Management of Sinonasal Tumors

AbstractSinonasal tumors are a relatively rare and heterogeneous group of tumors. Owing to their nonspecific presentation and rarity, they can be potentially overlooked resulting in delayed diagnosis and management, and increased patient morbidity. Imaging is crucial for the detection, staging, surgical planning, follow-up as well as surveillance of sinonasal masses, wherein computed tomography (CT) and magnetic resonance imaging (MRI) play complementary roles. CT is better at depicting bony changes, while MRI is useful for delineating the extent of soft tissue lesion, detect perineural, intracranial, or intraorbital spread as well as differentiate trapped sinus secretions from tumor tissue. Other modalities like fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) and arteriography can be selectively employed. FDG-PET is useful for metastatic workup and detection of residual/ recurrent disease. Arteriography and endovascular image-guided interventions are useful to delineate supply of vascular tumors and perform preoperative embolization. A systematic evidence-based approach to a possible case of sinonasal tumor can go a long way in streamlining the detection and management of these tumors, while optimizing the use of available healthcare resources.

Tumors of the Nose and Paranasal Sinuses: Promoting Factors and Molecular Mechanisms-A Systematic Review.

Sinonasal neoplasms are uncommon diseases, characterized by heterogeneous biological behavior, which frequently results in challenges in differential diagnosis and treatment choice. The aim of this review was to examine the pathogenesis and molecular mechanisms underlying the regulation of tumor initiation and growth, in order to better define diagnostic and therapeutic strategies as well as the prognostic impact of these rare neoplasms. A systematic review according to Preferred Reporting Items for Systematic Review and Meta-Analysis criteria was conducted between September and November 2022. The authors considered the three main histological patterns of sinonasal tumors, namely Squamous Cell Carcinoma, Intestinal-Type Adenocarcinoma, and Olfactory Neuroblastoma. In total, 246 articles were eventually included in the analysis. The genetic and epigenetic changes underlying the oncogenic process were discussed, through a qualitative synthesis of the included studies. The identification of a comprehensive model of carcinogenesis for each sinonasal cancer subtype is needed, in order to pave the way toward tailored treatment approaches and improve survival for this rare and challenging group of cancers.

Orbital and periocular complications in patients with sinonasal tumours with orbital invasion

AimsThe purpose of this study was to determine the frequency and associated risk factors of orbital/periocular complications in patients with sinonasal tumour with orbital invasion managed with eye-sparing treatments.MethodsA retrospective...

Emerging concepts in sinonasal and salivary gland tumour pathology: nothing to sneeze or spit at

The past decade has seen a dizzying array of new tumours and concepts in both sinonasal and salivary gland tumour pathology. As is true in most organ systems, the changes have largely been brought on by insights discovered through molecular analysis. In the sinonasal tract, what was once a simple classification scheme with only a handful of entities has evolved into a complex web of novel neoplasms driven by a molecular alteration, many of which are closely related to each other. In the salivary glands, awareness of tumor-defining genetic alterations has led to the expansion of the histologic spectra of known entities (e.g., mucoepidermoid carcinoma or adenoid cystic carcinoma) as well as the discovery of entirely new tumour types (e.g., microsecretory adenocarcinoma and microcribriform adenocarcinoma).

Chondroblastoma- a rare sinonasal tumor: case report and review of literature

Chondroblastoma is a benign primary bone tumor that typically develops in the epiphyses of long bones. Chondroblastoma of the craniofacial skeleton is extremely rare, with most cases occurring in the squamosal portion of the temporal bone. In this report, we describe a case of chondroblastoma of the nasal cavity presenting with a left sided nasal mass which was excised with open approach. We review the literature on craniofacial chondroblastomas with particular emphasis on sinonasal lesions.

Sinonasal Lymphoma: A Rare Pathology Presenting with Common Post-Influenza Symptoms

Sinonasal malignancies are uncommon and only account for 3% of head and neck cancers. Despite diffuse large B-cell lymphomas (DLBCL) being the most common type of non-Hodgkins lymphoma occurring at various extranodal sites, its occurrence as a sinonasal tumor is exceedingly rare. Early detection and treatment of sinonasal lymphoma, have favourable outcomes with improvements in the 2-year and 5-year survival rates. We are reporting a case of primary sinonasal DLBCL in a 63-year-old lady who initially presented with nasal obstruction and epistaxis, both common symptoms in patients who have had a recent Influenza A infection. However, due to the unilaterality and progressive nature of her nasal obstruction, she warranted a specialist otorhinolaryngology assessment. Naso endoscopy revealed a unilateral nasal mass extending from the right middle meatus to the nasal floor and was friable with contact bleeding. These clinical findings are similarly seen in most of the various types of sinonasal malignancies, and diagnosis can be challenging. Pathologists rely upon immunohistochemical staining profiles to differentiate this highly heterogeneous group of malignancies. Histopathological examination confirmed the diagnosis of sinonasal lymphoma. The treatment of this rare pathological entity is discussed. International Journal of Human and Health Sciences Supplementary Issue: 2023 Page: S28

A Case of Sinonasal Low-Grade Nonintestinal Adenocarcinoma That Was Difficult to Diagnose Preoperatively

AbstractWe report a case of sinonasal low-grade, nonintestinal adenocarcinoma (LG non-ITAC). The patient, a 56-year-old woman, was referred to our university hospital for a left nasal tumor with nasal obstruction, epistaxis, and nasal discharge. Nasal endoscopy revealed a nasal polypoid tumor with a smooth margin occupying the left nasal cavity. The sinonasal tumor was diagnosed as an exophytic papilloma by histopathology. Sinonasal contrast computed tomography (CT) confirmed homogeneous opacification of the left frontal, maxillary, and ethmoidal sinuses. There was no evidence of a potential bone defect. T2-weighted magnetic resonance imaging (MRI) showed a relatively uniform high signal intensity area in the left maxillary and frontal sinuses. An area of high signal intensity was also found in a part of the left ethmoid sinus. Left transnasal endoscopic ethmoidectomy was performed under general anesthesia. The tumor in the nasal cavity was prone to bleeding. We defined the site of tumor attachment as the suprabullar cell. The anterior ethmoidal artery was exposed, and the tumor was carefully dissected and removed. Based on the findings of these radiological and histological examinations, the sinonasal tumor was diagnosed as a LG non-ITAC. Sinonasal non-ITAC is an adenocarcinoma without the histopathological characteristics of salivary or intestinal-type adenocarcinoma. Non-ITAC is additionally classified into the high-grade and LG types. LG non-ITAC is very uncommon. Thus far, there is only one published report in the literature on the clinical and therapeutic characteristics of LG non-ITAC in Japan. Non-ITAC has a good prognosis, with a low risk of metastasis. Therefore, it is imperative to suspect and distinguish non-ITAC from other sinonasal tumors with similar symptoms preoperatively.

Characteristics of Extranodal NK/T-Cell Lymphoma, Nasal Type, Compared with Nasal Diffuse Large B-cell Lymphoma.

Extranodal NK/T-cell lymphoma, nasal type (ENKL), is an aggressive tumour with poor prognosis. Its early diagnosis may improve the prognosis of patients; however, it is often overlooked in many cases and misdiagnosed as an inflammatory sinus disease during its initial stage. Identifying the clinical characteristics of ENKL may aid otorhinolaryngologists in indicating cases early for a pathologic examination. In this study, we aimed to investigate the clinical characteristics of ENKL compared with that of diffuse large B-cell lymphoma (DLBCL), which is the most common nasal malignant lymphoma. The backgrounds, clinical symptoms, blood test results, and computed tomography images of patients with nasal/paranasal malignant lymphoma in our hospital between 2012 and 2017 were investigated. The characteristics of ENKL and nasal DLBCL were compared to differentiate them. A total of 27 patients with nasal cavity and/or paranasal sinus lymphoma were included. Extranodal NK/T-cell lymphoma, nasal type, was diagnosed in 10 patients, while DLBCL was diagnosed in 17 patients. The median age of patients with ENKL was significantly lower than that of patients with DLBCL. All patients with ENKL had a unilateral lesion in the nasal cavity, with most located at the inferior turbinate. They also experienced nasal symptoms with significantly higher incidence of nasal obstruction and tendency of bleeding. ENKL was often unilateral and caused nasal obstruction, unlike DLBCL. Those who are younger in age and have sinonasal tumour with unilateral nasal obstruction and bleeding should be considered for early and repeated biopsies at multiple sites, with ENKL taken into consideration.

Применение интраоперационной навигации в эндоскопической эндоназальной хирургии мукоцеле околоносовых пазух

Objective. Evaluation of the effectiveness, safety, and benefit of the use of electromagnetic computer navigation in endoscopic endonasal surgery of paranasal mucocele. Materials and methods. At the Burdenko National Medical Research Center, from 2019 to 2022, 30 endoscopic endonasal removal of paranasal mucocele were performed: 10 operations were performed under CT-guided navigation, 12 with CT-MRI navigation, and 8 without navigation. The clinical findings of the disease, the history of life of patients, the data of endoscopic examination of the nasal cavity, olfactometry, intraoperative parameters, recurrence rate of the mucocele were analyzed. Results. Localization of the mucocele in the frontal sinus was significantly more common in the groups using navigation (p = 0.044). According to olfactometry before surgery, anosmia on the side of the pathology was more often detected in patients in groups using navigation (p = 0.005). According to endoscopy before surgery, scarring in the nasal cavity was found only in groups using navigation. There were no intraoperative complications in the three groups. The average intraoperative blood loss in the three groups did not differ significantly. The duration of the operation was slightly longer in the groups using navigation. The median navigation setup time in the CT navigation group was 8.5 [7.3; 9.8] min, in the CT-MRI navigation group it was 7.5 [7.0; 8.3] min. The median error in each group was 0 mm. According to the operating surgeon, computer navigation was necessary and the most beneficial in 100% of cases. The median long-term follow-up was 746 [478.3; 1133.0] days in the non-navigation group, 673 [176.0; 791.0] days in the CT-navigation group, and 274 [195.5; 371.0] days in the CT-MRI navigation group. Conclusion. The use of intraoperative navigation is justified in endoscopic debridement of mucocele with destruction of the walls of the orbit and the base of the skull, with scarring in the nasal cavity. The use of CT-MRI navigation is useful in a case of scarring in the nasal cavity and deformation of the orbit walls in the case of a fronto-orbital mucocele as well as while removing a sinonasal neoplasm bordering on the mucocele.

Correlation between Computed Tomography and Histopathology Findings of Sinonasal Tumors

Correlation between Computed Tomography and Histopathology Findings of Sinonasal Tumors

Clinical and histopathological findings of a rare sinonasal glomangiopericytoma

Glomangiopericytoma is a rare vascular neoplasm of the nasal cavity and paranasal sinuses that occurs during the sixth or seventh decade of life. It is categorized as a borderline tumor with low malignant potential and classified as a distinct entity of sinonasal tumors with perivascular myoid phenotype by the World Health Organization (WHO). We report the case of a 50-year-old woman with nasal obstruction and severe epistaxis. The nasal sinuses computed tomography (CT), and magnetic resonance imaging (MRI) demonstrated a 3.1 cm soft tissue mass occupying the upper part of the left nasal cavity invading the left paranasal sinuses and nasal septum, and the left eye medial rectus muscle. A total mass resection was performed by nasal endoscopy. The histological and immunohistochemical examination yielded the diagnosis of glomangiopericytoma. This case report aims to contribute to the knowledge of nasal neoplasms. The need for more data on this entity is the main obstacle to developing standardized treatment guidelines.

Synchronous sinonasal tumour: double trouble?

Schneiderian papilloma is a benign sinonasal tumour, which has three morphological variants: fungiform, inverted and oncocytic. Inverted papilloma, being the most prevalent variant, is three times more common among males in their fifth to seventh decade of life. On the other hand, nasopharyngeal carcinoma is a malignant tumour arising from the nasopharyngeal epithelium. Albeit rare, synchronous sinonasal tumour has been reported and remains a quandary till date, especially when it involves malignant and benign synchronous nasal mass. In this paper, we reported a case of a successfully treated synchronous sinonasal inverted papilloma with nasopharyngeal carcinoma in an elderly male. We would like to highlight the need to investigate suspicious bilateral nasal masses due to the possible coexistence of two different pathologies, as in our case.

Synchronous nasal mass: a wolf in sheep’s disguise

Aim: To highlight the possibility of synchronous benign and malignant nasal masses. Schneiderian papilloma, also known as inverted papilloma, despite being a common benign epithelial sinonasal tumour, remains a conundrum to otorhinolaryngologists due to its locally aggressive nature, tendency to recur as well as potential of malignant transformation. A synchronous nasal lesion adds on to the complexity of patient management. Case presentation: Herein, we are reporting an unusual case of synchronous lesions: sinonasal inverted papilloma and low-grade mucoepidermoid carcinoma. Initial biopsy of the sinonasal mass revealed inverted papilloma. However, histopathological examination post endoscopic medial maxillectomy revealed evidence of both inverted papilloma and low-grade mucoepidermoid carcinoma. Conclusion: We want to highlight that seemingly benign sinonasal inverted papillomas ought to be taken seriously considering the possibility of a synchronous malignant nasal mass, as in our case.

Analysis of KRAS, BRAF, and EGFR mutational status in respiratory epithelial adenomatoid hamartoma (REAH).

Respiratory epithelial adenomatoid hamartoma (REAH) is a sinonasal glandular overgrowth arising from the surface respiratory epithelium and invaginating into the stroma. Clinically, it appears as a polypoid mass that may cause nasal obstruction, anosmia, and epistaxis. The presence of cartilaginous and/or osseous areas move the lesion to a chondro-osseous respiratory epithelial (CORE) hamartoma subtype. Scattered small seromucinous glands may be observed between typical REAH glands and when it is the only feature, it represents seromucinous hamartoma (SH). The molecular pathogenesis of REAH has been poorly explored and remains unclear. Given that KRAS, BRAF, and EGFR mutations have been detected in a variety of sinonasal tumors, we aimed to assess these mutations in REAH and SH. Ten REAH (including one CORE subtype), in addition to two SH cases, were Sanger sequenced by standard techniques. The targeted regions included KRAS exons 2-4 (encompassing hotspots codons 12, 13, 61, and 146), BRAF exons 11 and 15 (spanning the V600 codon), and EGFR exons 19 and 20. All REAH and SH samples showed wild-type sequences for KRAS, BRAF, and EGFR genes. Our results demonstrate a lack of KRAS, BRAF, or EGFR pathogenic variants with further evaluation of REAH and SH needed to elucidate driver genetic events.

Primary mucosal melanomas of the head and neck are characterised by overexpression of the DNA mutating enzyme APOBEC3B.

Primary head/neck mucosal melanomas (MMs) are rare and exhibit aggressive biologic behaviour and elevated mutational loads. The molecular mechanisms responsible for high genomic instability observed in head/neck MMs remain elusive. The DNA cytosine deaminase APOBEC3B (A3B) constitutes a major endogenous source of mutation in human cancer. A3B-related mutations are identified through C-to-T/-G base substitutions in 5'-TCA/T motifs. Herein, we present immunohistochemical and genomic data supportive of a role for A3B in head/neck MMs. A3B protein levels were assessed in oral (n =13) and sinonasal (n =13) melanomas, and oral melanocytic nevi (n =13) by immunohistochemistry using a custom rabbit α-A3B mAb (5210-87-13). Heterogeneous, selective-to-diffuse, nuclear only, A3B immunopositivity was observed in 12 of 13 (92.3%) oral melanomas (H-score range=9-72, median=40) and 8 of 13 (62%) sinonasal melanomas (H-score range=1-110, median=24). Two cases negative for A3B showed prominent cytoplasmic staining consistent with A3G. A3B protein levels were significantly higher in oral and sinonasal MMs than intraoral melanocytic nevi (P < 0.0001 and P=0.0022, respectively), which were A3B-negative (H-score range=1-8, median=4). A3B levels, however, did not differ significantly between oral and sinonasal tumours (P > 0.99). NGS performed in 10 sinonasal MMs revealed missense NRAS mutations in 50% of the studied cases and one each KIT and HRAS mutations. Publicly available whole-genome sequencing (WGS) data disclosed that the number of C-to-T mutations and APOBEC3 enrichment score were markedly elevated in head/neck MMs (n=2). The above data strongly indicate a possible role for the mutagenic enzyme A3B in head/neck melanomagenesis, but not benign melanocytic neoplasms.

DNA methylation-based classification of sinonasal tumors

The diagnosis of sinonasal tumors is challenging due to a heterogeneous spectrum of various differential diagnoses as well as poorly defined, disputed entities such as sinonasal undifferentiated carcinomas (SNUCs). In this study, we apply a machine learning algorithm based on DNA methylation patterns to classify sinonasal tumors with clinical-grade reliability. We further show that sinonasal tumors with SNUC morphology are not as undifferentiated as their current terminology suggests but rather reassigned to four distinct molecular classes defined by epigenetic, mutational and proteomic profiles. This includes two classes with neuroendocrine differentiation, characterized by IDH2 or SMARCA4/ARID1A mutations with an overall favorable clinical course, one class composed of highly aggressive SMARCB1-deficient carcinomas and another class with tumors that represent potentially previously misclassified adenoid cystic carcinomas. Our findings can aid in improving the diagnostic classification of sinonasal tumors and could help to change the current perception of SNUCs.

Molecular Basis and Rationale for the Use of Targeted Agents and Immunotherapy in Sinonasal Cancers.

Despite the progress of surgery, radiotherapy, and neoadjuvant chemotherapy, the prognosis for advanced sinonasal cancers (SNCs) remains poor. In the era of precision medicine, more research has been conducted on the molecular pathways and recurrent mutations of SNCs, with the aim of understanding carcinogenesis, helping with diagnosis, identifying prognostic factors, and finding potentially targetable mutations. In the treatment of SNC, immunotherapy is rarely used, and no targeted therapies have been approved, partly because these tumors are usually excluded from major clinical trials. Data on the efficacy of targeted agents and immune checkpoint inhibitors are scarce. Despite those issues, a tumor-agnostic treatment approach based on targeted drugs against a detected genetic mutation is growing in several settings and cancer subtypes, and could also be proposed for SNCs. Our work aims to provide an overview of the main molecular pathways altered in the different epithelial subtypes of sinonasal and skull base tumors, focusing on the possible actionable mutations for which potential target therapies are already approved in other cancer types.

Endoscopic Management of Ethmoidal Schwannoma: A Case Report.

Schwannomas of sinonasal origin are rare tumours with an incidence as low as 4% and may present with a wide range of clinical features. Due to non-specific endoscopic and radiological findings, diagnosis becomes difficult. We present a case of an ethmoidal schwannoma with nasal and nasopharyngeal extension in an elderly female patient who had a slow course of the long-standing disease. Her main complaints were nasal obstruction, nasal discharge, mouth breathing, snoring and recurrent nasal bleed. Nasal endoscopy showed a pale, firm, polypoidal mass with dilated vessels on the surface which bled on probing. It was a non-enhancing sinonasal mass with scalloping of adjacent paranasal sinuses and erosion of posterior nasal septum on contrast-enhanced computed tomography. Endoscopic excision of the mass was done in toto and histopathology report confirmed it as schwannoma. Long standing sinonasal masses especially in the elderly age group with an indolent medical history should arise suspicion of benign neoplasm especially schwannomas due to their high rate of incidence amongst benign sinonasal neoplasms.