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Low-grade non-intestinal-type sinonasal adenocarcinoma. A At low-power, there is a markedly increased number of seromucinous glands within the nasal submucosa. B The tumor glands have minimal cellular atypia, but demonstrate architectural atypia in the form of fusion and cribriforming with no intervening stroma. C A rare variant known as renal cell carcinoma-like adenocarcinoma has water-clear cytoplasm and prominent cell membranes. D S100 protein is usually positive, supporting seromucinous differentiation
Source publication
The World Health Organization Classification of Head and Neck Tumours recently published the 5th edition. There are new entities, emerging entities, and significant updates to the taxonomy and characterization of tumor and tumor-like lesions, specifically in this article as it relates to nasal cavity, paranasal sinuses and skull base. Importantly,...
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Introduction and importance:
Renal cell carcinoma metastasis in the sinonasal cavities is rare. They account for less than 1 % of all metastases of these renal cancers.
Case presentation:
We report the case of a patient with an unremarkable pathological history, who consulted for recurrent right epistaxis. Nasal endoscopy revealed a reddish mass...
Citations
... Опухоли данной локализации могут иметь различные морфологические формы, однако превалирует плоскоклеточный рак (57-80 % случаев). Реже встречаются злокачественные новообразования малых слюнных желез, синоназальная недифференцированная карцинома, а также меланома слизистой оболочки полости носа [4,5]. ...
Introduction. Malignant tumors of the nasal cavity and sinonasal tract are rare, less than 3 % of all of the head and neck malignant neoplasm. The leading treatment is combined methods with surgery as a first step. However, operation is limited in a locally advanced process due to requires the use of complex reconstructive techniques and removal of aesthetic and functionally important structures. In this regard, the interest of specialists is understandable in use of organ-preserving techniques with comparable oncological results. Intra-arterial chemotherapy has shown high effectiveness in various localizations of malignant neoplasms, so its use in locally advanced tumors of the nasal cavity and sinonasal tract seems promising.
Aim. To evaluate the results of complex treatment of patients with locally advanced cancer of the nasal cavity and sinonasal tract using induction intra-arterial chemotherapy.
Materials and methods. The study included 28 from 2017 to 2023 with locally advanced cancer of the nasal cavity and sinonasal tract underwent Tpf induction intra-arterial chemotherapy, followed by radiation or chemoradiotherapy. The primary endpoint of the study was survival, secondary – objective response rate, treatment toxicity and the possibilityof organ preservation.
Results. The median follow-up was 19.1 months. we obtained results for all patients of 1-year overall survival – 85.7 % (T3 – 100 %, T4a – 92.7 %, T4b – 55.6 %), and 1-year progression-free survival – 66.7 % (T3 – 75 %, T4a – 71.4 %, T4b – 50 %), respectively. The response rate after intra-arterial chemotherapy was 85.2 %. Overall survival and progression-free survival did not differ significantly between patients with complete response, partial response, and stable disease. However, in patients with a complete response after chemoradiotherapy, 1-year overall survival was 100 % (p = 0.009) and progression-free survival – 90 % (p <0.001). The main side effect in patients in the study was the development of neutropenia (35.7 %), and occurrence of neurological complications was noted in 10.7 %. Of the 35.7 % of patients with tumor orbital invasion, survival with preserved visual function in the first year of life was 80 %; all patients retained vision; as a result of treatment, not a single patient underwent surgery with exenteration.
Conclusion. Induction intra-arterial chemotherapy in treatment of patients with locally advanced cancer of the nasal cavity and sinonasal tract demonstrates high efficiency and a high level of complete responses with a fairly low level of toxicity, and also allows preserve the eye function for the majority of patients.
... The 5th edition of the world health organization classification of head and neck tumors recommends that high-risk HPV must be demonstrated by in situ hybridization or PCR-based techniques, specifically to include type 33. p16 alone is not sufficiently specific to make the diagnosis [13]. ...
Background
The detection of human papillomavirus (HPV) has several implications in the diagnostic work-up and management of oropharyngeal squamous cell carcinoma (OPSCC). The choice of HPV detection assay and testing algorithms differ across institutions and vary in cost, detection targets, technical feasibility, and turnaround time. In this study, we aimed to validate the VisionArray® HPV Chip for formalin-fixed and paraffin-embedded (FFPE) samples of OPSCC using the previously applied standard pan-HPV DNA PCR assay as a reference.
Methods
The validation cohort consisted of FFPE tissue samples from patients previously diagnosed with HPV DNA-positive OPSCC (n = 80), HPV DNA-negative OPSCC (n = 21), and a benign group of tumor samples consisting of Warthin’s tumors (n = 20) and branchial cleft cysts of the lateral neck (n = 14). All samples were tested with p16 immunohistochemistry, pan-HPV DNA PCR, and the VisionArray® HPV Chip.
Results
The overall sensitivity and specificity of the VisionArray® HPV Chip assay were 100% [95% CI 95.5%; 100.0%] and 96.3% [95% CI 87.3%; 99.6%] and the positive predictive value and negative predictive value were 97.6% [95% CI 91.5%; 99.7%] and 100% [95% CI 93.2%; 100%], respectively.
Conclusions
The VisionArray® HPV Chip assay can be recommended for high-risk HPV testing in FFPE tissue samples from OPSCC, providing both a fast and simultaneous genotyping for 41 clinically relevant HPV types.
... Sinonasal adenocarcinomas (SNACs) are rare and heterogeneous tumors, corresponding to around 3% of upper aerodigestive tract malignant tumors [1][2][3]. They are more prevalent in males and are associated with poor overall survival (OS), as they are often diagnosed at an advanced stage when there are fewer therapeutic options [4]. ...
... In the epithelial subgroup, squamous cell carcinoma accounts for around 80% of these tumors [1]. Adenocarcinomas (10-20%) are the second most common subtype of SN epithelial tumors, the most common being intestinal-type adenocarcinoma (ITAC) [1][2][3]5]. ITAC is often related to occupational exposure to wood and leather dust (both the intensity of exposure and its duration), and it can be diagnosed up to 40 years after exposure [1][2][3]5]. ...
... Adenocarcinomas (10-20%) are the second most common subtype of SN epithelial tumors, the most common being intestinal-type adenocarcinoma (ITAC) [1][2][3]5]. ITAC is often related to occupational exposure to wood and leather dust (both the intensity of exposure and its duration), and it can be diagnosed up to 40 years after exposure [1][2][3]5]. A Portuguese study, which included 39 patients with ITAC, also identified cork occupational exposure as a risk factor for ITAC [6]. ...
Sinonasal (SN) malignancies are rare. Within SN adenocarcinomas, the most frequent are intestinal-type adenocarcinomas (ITACs). ITAC has been associated with wood and leather dust occupational exposure and TP53 mutations. Not much information is available regarding its characterization and treatment. The aim of this study is to characterize the clinicopathologic and prognostic factors of patients with sinonasal adenocarcinomas (SNACs) treated in our tertiary-level hospital. A retrospective, consecutive study including SNAC patients diagnosed between 2004-2023 was conducted. Clinicopathological data was collected, and p53 status was assessed in the tumor specimens. The association between p53 status and clinicopathological variables, as well as their impact on survival, was evaluated.
In total, 35 were included, most of them having ITAC (91.4%) with papillary subtype (37.5%); the majority were subjected to occupational risk exposure (82.9%). Overexpression of p53 was identified in 48.6% of the tumors. Papillary and colonic subtypes were associated with higher median progression-free survival (mPFS) than mucinous and solid subtypes (mPFS 37 months, 95% CI, 20.0-54.0, vs. 9 months, 95% CI, 7.15-10.85, p=0.01); the former was also associated with higher median overall survival (mOS) (mOS 64 months, 95% CI, 37.18-90.81 vs. 14 months, 95% CI, 0-41.58, p=0.02). Histologic grade 1-2 and macroscopic complete resection were associated with higher PFS (PFS of five months of 90.9% vs. 33.3%, p=0.01; mPFS of 37 months, 95% CI, 4.93-69.07 vs. 10 months, 95% CI, 6.43-13.57, p=0.04, respectively). Disease recurrence with distant metastases was associated with lower OS (11 months, 95% CI, 6.1-15.9 vs. 53 months, 95% CI, 22.70-83.30, p=0.04). This study reinforces the importance of protective occupational measures. Future studies will be important to validate the best treatment strategy in the advanced stages of this disease and also to identify new prognostic and/or therapeutic target biomarkers in SNAC.
... This finding is in line with previous research [17]. The other morphologic mimics of MEC, including SCC, and DEK::AFF2 fusion-associated papillary SCC, were renamed "DEK::AFF2 carcinomas" [20]. SCC of the sinonasal tract and nasopharynx is an important differential diagnosis, particularly in cases with prominent clear cell features. ...
Aims
Primary mucoepidermoid carcinomas (MECs) of the sinonasal tract and nasopharynx are rare entities that represent a diagnostic challenge, especially in biopsy samples. Herein, we present a case series of MECs of the sinonasal and skull base and its mimics to evaluate the clinicopathological and molecular characteristics in order to avoid misdiagnosis.
Methods
We reviewed the pathology records of patients diagnosed from 2014 to 2022. Thirty MECs were consecutively diagnosed during that period.
Results
Based on morphological and fluorescence in situ hybridization (FISH) analyses, 30 tumors originally diagnosed as MECs were separated into MAML2 fusion-positive (7 cases) and MAML2 fusion-negative groups (23 cases), in which 14 tumors were positive for the EWSR1::ATF1 fusion; these tumors were reclassified to have hyalinizing clear cell carcinoma (HCCC). The remaining nine MAML2 FISH negative cases were reconfirmed as squamous cell carcinoma (SCC, 3 cases) which showed keratinization and high Ki-67 expression; DEK::AFF2 carcinomas (2 cases), in which DEK gene rearrangement was detected by FISH; and MECs as previously described (4 cases) with typical morphological features. Including 7 MAML2 rearrangements tumors, 11 MEC cases had a male-to-female ratio of 4.5:1, and 6 tumors arose from the nasopharyngeal region, while 5 tumors arose from the sinonasal region. The prognosis of this series of salivary gland-type MECs was favorable.
Conclusions
Our study confirmed that HCCC runs the risk of being misdiagnosed as MEC in the sinonasal tract and nasopharynx, particularly with biopsy specimens. Careful histological evaluation with supporting molecular testing can facilitate pathological diagnoses.
... A serrated hyperplastic, ciliated epithelium is surrounded by a thick, eosinophilic basement membrane that displaces normal elements to form what is known as respiratory epithelial nests. 18 The glands arise in direct continuity with the surface epithelium, which invaginates downwards into the submucosa. This is significantly different from the absence of respiratory epithelial nests in the stroma of NCMH. ...
Nasal chondromesenchymal hamartoma (NCMH) is a rare benign polypoid mesenchymal tumor arising in the nasal cavity and/or paranasal sinuses. Recognizing these sporadic, rare lesions is crucial, as surgical complete removal of the mass is the common treatment approach. This retrospective study analyzed the demographics, symptoms, and imaging data of 9 patients diagnosed with NCMH between
... Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC) is a newly recognized distinct diagnostic entity by the 5th edition of the World Health Organization (WHO) Classification of Head and Neck tumors [1][2][3], with 84 published cases to date. Although the "multiphenotypic" nomenclature, this was previously called HPV-related carcinoma with adenoid cystic (ACC)-like features [1,4]. ...
... Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC) is a newly recognized distinct diagnostic entity by the 5th edition of the World Health Organization (WHO) Classification of Head and Neck tumors [1][2][3], with 84 published cases to date. Although the "multiphenotypic" nomenclature, this was previously called HPV-related carcinoma with adenoid cystic (ACC)-like features [1,4]. Despite the usual indolence, certain cases can be morphologically and clinically aggressive, raising uncertainty regarding the appropriate management [2,[4][5][6][7]. ...
... Involvement of adjacent structures was also common, as seen in our case, however, invasion of the orbital tissues as presented by our patient was rarely found [9]. Based on the criteria recently described by the WHO, HMSC has morphological and immunophenotypical aspects that contemplate the combination of areas suggestive of a salivary gland carcinoma (with evident ductal and myoepithelial differentiation) and squamous cell carcinoma [1]. The main differential diagnosis is sinonasal ACC due to the presence of areas with cribriform architecture in these tumors [1,4]. ...
A 54-year-old male patient complained of nasal obstruction and epistaxis for 2 years, with worsening of the symptoms in the preceding year. Physical examination revealed a friable, irregular mass, with yellowish secretion, in the left nasal fossa. Magnetic resonance imaging revealed an expansive lesion in the left nasal cavity, extending into the nasopharynx, ethmoid, right nasal cavity, and cortical bone of the hard palate. An incisional biopsy was then performed. Morphologically, a cellular malignant proliferation with a solid basaloid appearance admixed with adenoid cystic-like areas was observed. Immunohistochemistry revealed positivity for AE1/AE3, CK7, p63, and calponin, with focal labeling for CD117 and α-SMA. p16 had diffuse cytoplasmic and nuclear positivity. Ki-67 index was >80%. Given the morphological and immunohistochemical aspects, the diagnosis was conclusive for HPV-related multiphenotypic sinonasal carcinoma. The tumor was considered irresectable, and the patient was submitted to induction chemotherapy with docetaxel, cisplatin, and infusional 5-fluorouracil, with significant regression after therapy, followed by chemoradiotherapy with carboplatin, without limiting toxicities. The patient is currently under regular follow-up, with complete clinical and radiological response. To date, there are no reports in the literature of induction chemotherapy use or its complete therapeutic responsiveness related to this lesion. A brief literature review was included with the main epidemiological, clinical, therapeutic, and prognostic aspects regarding the 85 cases reported in the literature, including ours.
... As in other malignancies, unique subsets of sinonasal tumors have recently been characterized by their specific molecular modifications because of the advances in molecular and immunohistochemical techniques. The fifth edition of the World Health Organization Classification of the tumors of the nasal cavity and paranasal sinuses includes the following carcinoma types [3]: keratinizing and non-keratinizing squamous cell carcinoma, NUT carcinoma (i.e., a rare, highly aggressive malignancy defined by translocations involving the NUT gene), SWItch/Sucrose Non-Fermentable (SWI/SNF) complex-deficient sinonasal carcinoma, sinonasal lymphoepithelial carcinoma, sinonasal undifferentiated carcinoma, teratocarcinosarcoma, human papilloma virus (HPV)-associated multiphenotypic sinonasal carcinoma, and adenocarcinomas, which are further divided into intestinal-type and non-intestinal type adenocarcinoma. ...
Purpose of Review
An emerging subset of dismal sinonasal cancers are those characterized by the loss of a SWItch/Sucrose Non-Fermentable (SWI/SNF) complex unit, such as the SWI/SNF-related Matrix-associated Actin-dependent Regulator of Chromatin (SMARC), which includes two main subtypes: SMARCB1- and SMARCA4-deficient sinonasal carcinomas, ultimately leading to four distinct SWI/SNF-deficient sinonasal tumors. These cancers are rare entities and low treatment responsive malignancies. In fact, they are poorly differentiated and usually detected at a late stage, when invasion of facial and cranial regions had already occurred.
Recent Findings
From a histological standpoint, SWI/SNF-deficient sinonasal carcinomas belong to the group of sinonasal undifferentiated carcinomas (SNUC); however, their distinctive features disclose a special category for these cancers. The identification of biomarkers and signaling pathways has led to the development of emerging therapies, such as immunotherapy and personalized treatments. Finally, we report preliminary findings on 3D in vitro models of sinonasal cancers, as a multidisciplinary tool that could empower the understanding of SWI/SNF-deficient cancer biology.
Summary
Here, we review the current knowledge about histological and molecular features of SWI/SNF-deficient sinonasal cancers, with a focus on treatment options and multidisciplinary research perspectives. The possibility of studying SWI/SNF-deficient sinonasal tumors in-depth would be fostered by the establishment of tumor cell lines.
... As the cases were collected over a long period of time and at different institutions, the initial diagnoses were reviewed by two experienced pathologists (BV and VBL). All available slides were reviewed in each case, and tumors were diagnosed according to the diagnostic criteria of the 5th edition of the WHO classification [49]. Additional immunohistochemical staining of cytokeratin 5/6, p40, p16, synaptophysin, chromogranin, NUT, SMARCB1, and SMARCA4 were applied when necessary. ...
Poorly differentiated sinonasal carcinomas (PDCs) are tumors that have a poor prognosis despite advances in classical treatment. Predictive and prognostic markers and new personalized treatments could improve the oncological outcomes of patients. In this study, we analyzed SOX2 and βIII-tubulin as biomarkers that could have prognostic and therapeutic impacts on these tumors. The cohort included 57 cases of PDCs: 36 sinonasal undifferentiated carcinoma (SNUC) cases, 13 olfactory neuroblastoma (ONB) cases, and 8 sinonasal neuroendocrine carcinoma (SNEC) cases. Clinical follow-up data were available for 26 of these cases. Sox2 expression was detected using immunohistochemistry in 6 (75%) SNEC cases, 19 (53%) SNUC cases, and 6 (46%) ONB cases. The absence of Sox2 staining correlated with a higher rate of recurrence (p = 0.015), especially distant recurrence. The majority of cases showed βIII-tubulin expression, with strong positivity in 85%, 75%, and 64% of SNEC, ONB, and SNUC cases, respectively. Tumors with stronger βIII-tubulin expression demonstrated longer disease-free survival than those with no expression or low expression (p = 0.049). Sox2 and βIII-tubulin expression is common in poorly differentiated sinonasal tumors and has prognostic and therapeutic utility.
... The tissue samples were xed with 10% formaldehyde and processed into para n-embedded sections, which were subsequently stained with routine hematoxylin-eosin for microscopic examination. To ensure the accuracy and reliability of the pathological diagnosis, all cases were reviewed and reclassi ed by two senior pathologists according to the criteria of the WHO classi cation (Thompson and Bishop 2022). In addition, two patients included in the study underwent surgery at another hospital, and their pathological sections were transferred to our institution for review. ...
Objective: This study aims to comprehensively analyze of orbito-naso-cranial communicating lesions to elucidate clinical manifestations, pathological classification, and specific lesion sites. The findings will enhance the diagnosis and preoperative evaluation of orbito-naso-cranial communicating lesions.
Methods: The 74 patients were classified into three groups based on the involved locations: Group A (orbito-nasal), group B (orbitocranial), and group C (orbito-naso-cranial). The clinical characteristics, pathological classification, and variations in the involved sites among these three groups were subjected to statistical analysis.
Results: Among the enrolled patients, 49 and 25 had benign and malignant lesions, respectively. Among benign lesions, neurogenic tumors (17 cases) constituted the largest proportion. Among malignant lesions, hematogenous (seven cases) and glandular (seven cases) tumors constituted the largest proportion. In the three groups, the malignant ratio in group C (55.6%, 10/18) was significantly higher than that in groups A (7.2%, 5/29) and B (37.0%, 10/27). The ethmoid sinus (35 cases) was the most prevalent site, including 24 benign and 11 malignant cases. Furthermore, there was greater invasion in the nasal cavity (28.0% vs. 0.0%, P=0.000) and anterior cranial fossa (40.0% vs. 8.2%, P=0.003) in malignant lesions than in benign lesions.
Conclusions: There is a higher incidence of orbito-nasal communication and infiltration of the nose and anterior cranial fossa in malignant tumors compared to benign lesions.
Level of Evidence: Level 4
... These were moved to their own dedicated chapters, such as soft-tissue tumors, melanocytic tumors, neuroendocrine neoplasms (such as paraganglioma), and so forth, with few exceptions. 3 Adamantinomatous craniopharyngioma is the only entity that has been moved to the SNT (from the nasopharynx in the 4th edition), because the SNT is the exclusive ectopic location for these tumors. A new chapter with genetic tumor syndromes was added with 15 entities with predominant head and neck manifestations. ...
... There is a new category for mesenchymal tumors of the SNT and a category for "other" sinonasal tumors that includes olfactory neuroblastoma. [1][2][3] Imaging characterization of these new entities is still at an early stage with significant overlap in the radiographic presentation. Nevertheless, it is important for the radiologist to be aware of the clinical course of these tumors, including the epidemiologic features and how they respond to treatment. ...
Sinonasal and skull base tumors are a heterogeneous group of neoplasms with considerable histologic variation and overlapping imaging features. In 2022, the World Health Organization updated the head and neck tumor classification, further emphasizing the importance of molecular data and genetic alterations in sinonasal neoplasms. The changes include the addition of new entities and discussion of emerging entities, as well as changes to the taxonomy and characterization of tumors. The new classification focuses on entities that develop in these sites either exclusively (eg, olfactory neuroblastoma) or most frequently. Another change includes reduction in the number of categories by creating separate category-specific chapters for soft-tissue, hematolymphoid, and neuroectodermal lesions. In this review, we briefly discuss the various categories in the new classification with a more detailed description of the 2 new entities (SWItch/Sucrose Non-Fermentable complex-deficient sinonasal carcinomas and human papillomavirus-related multiphenotypic sinonasal carcinoma). We also highlight the emerging entities including IDH-mutant sinonasal malignancies and DEK-AFF2 carcinoma, presently classified as sinonasal undifferentiated carcinoma and nonkeratinizing squamous cell carcinoma, respectively.
