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Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC), is a new entity that is restricted to the sinonasal tract and is associated with high-risk HPV. This tumor is suggested to have an indolent behavior with a better prognosis than other carcinomas. We report a unique case of HMSC with a locally aggressive behavior. It is about a 61-year-old men presented with 12 months of unilateral progressive olfactory dysfunction accompanied by exophthalmia of the left eye, declining vision and headaches for 6 months. Computed tomography imaging revealed a voluminous mass occupying the ethmoid, maxillary and frontal sinus with bony destruction of the left ethmoidal blade. Histology showed a tumor composed of variably sized nests, separated by thick mucoid stroma. Tumor cells are plasmacytoid with hyperchromatic nuclei and frequent mitoses. Immunohistochemistry revealed that these cells were positive for cytokeratin AE1/AE3, p16 and negative for CK7, CK20, CD117, p40, p63, S100, synaptophysin and chromogranin.

Neurilemmomas (Schwannomas) of sinonasal tract are very unusual. They are benign, slow-growing, usually solitary encapsulated perineural tumors. They arise from Schwann cells of the neural sheath of the peripheral nervous system including motor, sensory and autonomic nerves. They can occur throughout the body, but the head and neck region demonstrate a higher incidence of occurrence (25–45%). The sinonasal region, however, has the lowest incidence rate with only 3–4%. We report here a rare case of fronto-ethmoid sinus neurilemmoma that is locally destructing the anterior skull base and the lateral orbital wall. A left eye proptosis, diplopia and chemosis were the presenting complaints. Images and histopathology examinations confirmed the diagnosis. The patient underwent tumor resection through Endoscopic Endonasal approach, followed by a functional sinus drainage of the retained secretions. The patient made a good postoperative recovery and remained disease free at a 1-year follow up period.

Juvenile nasopharyngeal angiofibroma (JNA) are rare, histologically benign, but locally aggressive, vascular tumors of the nasopharynx that affects predominantly male adolescents, its origins from other sites is denominated extranasopharyngeal angiofibroma (ENPA), which is an even rarer entity afflicting more females and young adults, representing a diagnostic and therapeutic challenge to rhinologists. In this report we present 2 cases of similar clinical presentation, with unilateral nasal obstruction and previous episodes of nasal bleeding. One patient is a 14-year-old male with a soft-tissue mass occupying nasopharynx, middle meatus, and right nasal cavity. The second patient is a 12-year-old male with a soft-tissue lesion rising from right sphenoid sinus and obliterating right nasal cavity, obstructing ipsilateral maxillary sinus, ethmoidal cells, and frontal sinus. Both patients underwent complete endoscopic excision of the lesions, the first one recurred on sphenoid sinus and the second showed no recurrence after 2 years of post-operative follow-up. The unusual sites and presentations make these rare cases and thus worth of reporting.

Background High grade basal-like breast carcinomas are triple negative, express basal cytokeratins, and are known for the overall poor prognosis and aggressive behavior. HPV related multiphenotypic sino-nasal carcinoma has overlapping histology with basal-like breast carcinomas, but carry the defining feature of association with high risk HPV. Case presentation We present a case of a perimenopausal woman with a non-healing ulcerated lesion involving the nipple and breast following a trauma. Biopsy performed showed an HPV-positive basal-like carcinoma with squamous differentiation involving the breast, analogous to multiphenotypic carcinoma previously described in the sinonasal tract. Conclusion This is the first report of a case of a high- risk HPV related basal-like carcinoma with squamous differentiation, described in the literature. We highlight the morphology and immunophenotype of this lesion and its recognition when compared to other multiphenotypic lesions of the breast, and suggest that pathologists should consider HPV evaluation when encountering similar basal-like tumors involving the breast.

Background HPV- related carcinoma with adenoid cystic like features of the sinonasal tract is a newly describe entity with histological and immunophenotypic features of surface derived and salivary gland carcinoma. It affects females more than males with age range of 40–70 years and is linked to high risk HPV infection. Most cases present with nasal obstruction and epistaxis. They consist of basaoid cells growing in various sizes separated by fibrocollagenous stroma.1 It is believed to have a good prognosis. Tumor-associated macrophages (TAMs) are activated macrophages associated with tumor progression in various cancers. TAMs can polarize M1 or M2 type. M1 has a pro-inflammatory function and kills pathogens. Conversely, M2 shows immunosuppressive action and promotes tumor growth. CD68 is known as a pan-macrophage marker.2 We evaluate the CD68 expression in three cases of HPV- related carcinoma with adenoid cystic like features of the sinonasal tract. Methods Three cases of HPV-related carcinoma with adenoid cystic like features were retrieved from our archives and stained with p16 and CD68 antibodies. Data was analyzed using spss version 21. Results Patient ages were 46, 48 and 56 years old respectively, with a female to male ratio of 2:1. Histology showed epithelial surface dysplasia overlying basaloid cell growing in tubular and crbriform patterns. All were strongly positive for p16 stains (figure 1). CD 68 showed intratumoral and peritumoral expression in two cases while, one case showed only peritumoral expression. Infiltration of tumour associated macrophages (M2) CD68 cell in this study is associated with increase recurrence of HPV-related adenoid cystic carcinoma of the sinonasal tract (figure 2). Conclusions The targeting of TAMs in HPV-related adenoid cystic carcinoma of the sinonasal tract and other cancers should be explored in the future using macrophage targeted approach. Ethics Approval Health research ethics committee ABUTH/HREC/Y/2017 References Burugu S, Asleh-Aburaya K, Nielsen TO. Immune infiltrates in the breast cancer microenvironment: detection, characterization and clinical implication. Breast Cancer 2017; 24:3–15. Fan QM, Jing YY, Yu GF, Kou XR, Ye F, Gao L, Li R, Zhao QD, Yang Y, Lu ZH, Wei LX. Tumor-associated macrophages promote cancer stem cell-like properties via transforming growth factor-beta1-induced epithelial-mesenchymal transition in hepatocellular carcinoma. Cancer Lett 2017;352:160–168.

Purpose To document a case of a biphenotypic sinonasal sarcoma that impinged on the orbit via a tumor-induced mucocele and review the previous literature devoted to this condition. Methods A clinicopathologic case report with detailed histopathologic, immunohistochemical, and radiological studies with tabulations of previously reported cases and immunohistochemical stains for ruling out mimicking lesions. Results A biphenotypic sinonasal sarcoma arose from the ethmoid sinus in a middle-aged man. The tumor induced a mucocele that bowed the medial orbital lamina papyracea into the orbit and caused diplopia and mild proptosis. The biopsy's dual positivity for S100 and smooth muscle actin together with positive paired box 3 immunohistochemical staining established the diagnosis of biphenotypic sinonasal sarcoma. Conclusion Biphenotypic sinonasal sarcoma, which can involve the orbit in 25% of cases, is a rare head and neck malignancy that has only recently been described. Biphenotypic sinonasal sarcoma is a low-grade, locally aggressive, nonmetastasizing sarcoma displaying both neural and muscle differentiation. It is further characterized by rearrangements of the PAX3 gene with multiple fusion partners, most commonly MAML3 (Mastermind like transcriptional coactivator 3). It occurs predominantly in middle-aged women and exclusively in the sinonasal tract. However, it can spread throughout the central facial structures to invade the brain if not detected early. Ophthalmologists should be aware of this new entity to avoid delays in diagnosis and treatment. The previous literature devoted to this condition was reviewed and analyzed for clinical, radiological, histopathological, and immunohistochemical features. In circumstances where molecular testing is not available, paired box 3 immunohistochemical staining can be used as an alternative diagnostic marker. The current case is most unusual because the orbital findings were induced by a mucocele caused by the tumor that obstructed the sinus ostium. This produced diplopia without direct tumor invasion into the orbital fat.

Sinonasal renal cell-like adenocarcinoma (SRCLA), which is an extremely rare but established entity, belongs to the non-salivary gland type of low-grade adenocarcinomas arising from the sinonasal tract. We report a case of high-grade SRCLA. The patient was a 63-year-old Japanese male, who complained of nasal obstruction. Histologically, the tumor exhibited solid nests and sheet-like structures composed of atypical clear or eosinophilic cells with central necrosis. The tumor cells contained periodic acid Schiff-positive glycogen. Immunohistochemically, they were positive for cytokeratin (CK)7, CD10, carbonic anhydrase (CA)-IX, the epidermal growth factor receptor (EGFR), mucin 1, and cancer antigen 125, whereas they were negative for CK20, caudal type homeobox 2, CK5/6, p40, vimentin, S-100, a renal cell carcinoma marker, paired box gene 8, and the human epidermal growth factor receptor 2. Both local recurrence and late lymph node metastases arose in this case. Therefore, the tumor was considered to be clinically aggressive and was diagnosed as a high-grade SRCLA. Immunostaining of CD10 and CA-IX was very diagnostically useful. Although 14 cases of SRCLA have been reported previously, all of them involved low-grade malignancies, and no descriptions of their EGFR statuses were provided. This is the first report about a high-grade SRCLA exhibiting EGFR overexpression.

HPV-related multiphenotypic sinonasal carcinoma (HMSC) is a recently described distinct tumor entity of the sinonasal tract associated with high-risk subtypes of human papilloma virus (HPV), predominantly type 33. The biological behavior seems to be less aggressive than the often high-grade, highly proliferative morphology implies; however, recurrences are frequent. Most of the cases present as polypoid tumors within the nasal cavity. Microscopic morphology frequently encompasses adenoid cystic-like features or features reminiscent of other salivary gland tumors. Here, we describe four cases of this rare entity, all observed in women. The polypoid tumors were within the nasal cavity, leading to obstruction, facial pain and epistaxis. The morphology was predominantly basaloid, solid and adenoid cystic-like in two of four cases, one with additional glomeruloid features. Another case showed basaloid tumor cells with prominent mature squamous differentiation and extensive keratinization. A single case showed a predominantly solid and reticular growth pattern. All cases were diffusely positive for p16 (100%), expressed SOX10, LEF-1 and partially S-100, and harbored HPV high-risk types 33, 56 (2×) and 82. No recurrences or metastases were detectable after 3-50 months of follow-up. Of note, three of four patients were nurses/nursing assistant. We expand the morphological spectrum by describing a glomeruloid growth pattern and extensive mature keratinization, and add HPV type 82 to the molecular spectrum. The finding of HMSC among predominantly nurses in our cohort warrants further epidemiological studies in larger cohorts.

Solitary fibrous tumor (SFT) is a fibroblastic tumor of the pleura, and subcutaneous and deep soft tissues of the extremities, abdomen, pelvis, and retroperitoneum. In the head and neck region, the sinonasal tract and soft tissues of the orbit are involved most frequently. However, the lacrimal sac is an extremely unusual location for this tumor, with occasional case reports in literature. Being superficially located and palpable, lacrimal sac masses are easily amenable to FNAC. Despite cytological features of SFT being well-documented, they are often underdiagnosed on FNAC. Immunocytochemistry for STAT6 is a sensitive and specific marker that can aid in accurate diagnosis, as is required for planning appropriate management. We describe the case of a 45-year-old lady who presented with a mass at the medial canthus of the right eye, and was diagnosed as SFT on FNAC, based on cytomorphological features and immunopositivity for STAT6. The diagnosis was subsequently confirmed on histological examination.

Background Narrow band imaging (NBI) endoscopy is an optical method that helps to characterise tissue vasculature. Its appli- cation in sinonasal pathology remains scarce and a systematic study of its application to rhinology is lacking. The aim of this study is to analyse and describe the normal sinonasal mucosa under NBI light and to characterise the microvascular features of various sinonasal pathologies. We also want to suggest a classification of the patterns, peculiar to this district, and to evaluate whether they can be indicative of a specific physiological or pathological condition. Methods Digital videos and images under white light and NBI of 103 patients (82 evaluated) with 29 sinonasal pathologies and 55 controls (33 evaluated). were independently analysed by three otolaryngologists and the final pattern was then arranged for each image, reaching an agreement between the individual evaluations. Results Once the appearance of normal sinonasal (SN) mucosa was established (SN1), four patterns for the pathological mucosa were described and a working classification was proposed (SN2, SN3, SN4, SN5). We calculated specificity (80.6% vs 90.6%), sensi- tivity (20% vs 38.5%), PPV (46.1% vs 50%), NPV (54.7% vs 85.7%) and accuracy (53% vs 80.3%) of the ability of SN4 and SN5 pattern to discriminate between benign and malignant nasal neoformations. Conclusions This is the first study to propose a systematic NBI description and a classification of the vasculature of healthy and pathological mucosa in the sinonasal tract. Our preliminary results show that this technique can help in the workup of several rhinologic conditions and especially in distinguishing benign from malignant tumors.

Current clinical practice algorithms for HPV testing make no effort to discern the impact of genotypes for patients with head and neck squamous cell carcinoma (HNSC). Data was collected for all patients with HNSCs that had undergone HPV testing at an academic hospital as part of clinical care (2012-2019). Screening was performed using real-time PCR targeting L1 of low and high-risk HPV types, followed by genotyping of positive cases. Genotype status was correlated with age, site and histologic parameters. Of the 964 patients tested, 68% had HPV-positive cancers. Most arose from the oropharynx (OP) (89%) and sinonasal tract (5%). The most frequent genotype was 16 (84.4%) followed by 35 (5.6%), 33 (4.1%), 18 (2.7%), 45 (1.1%), 69 (0.8%) and others (1.3%). There was an association between genotype (16 vs non-16) and tumor origin (OP vs non-OP) (p 0.0001). HPV18 was associated with transformation to an aggressive small cell phenotype, but HPV16 was not (22% vs 0%, p 0.0001). Patients with HPV-non-16 OP carcinomas were older than patients with HPV16 OP carcinomas, but the difference was not significant. HPV genotypes are variable and unevenly distributed across anatomic sites of the head and neck. The association of HPV18 with small cell transformation suggests that variants can track with certain phenotypes in ways that may account for differences in clinical behavior. This study challenges the prevailing assumption of HPV equivalency across all high-risk genotypes in ways that may inform preventive, diagnostic, therapeutic and surveillance strategies.

Nasal chondromesenchymal hamartoma (NCMH) is a rare, benign lesion of the sinonasal tract. It usually presents as a polypoid mass in infants and older children. Imaging studies and endoscopy are required to delineate the extent of the lesion and aid in its excision. This unusual lesion is composed of proliferating mesenchymal and cartilaginous elements. Recently, a genetic association between NCMH and DICER1 mutation has been established. It is important for pathologists to be familiar with this entity to avoid misdiagnosis since the lesion is benign and surgical excision is curative.

We present a case of primary systemic amyloidosis diagnosed by endoscopic sinus surgery. A 75-year-old woman had blurred vision in her left eye; computed tomography and magnetic resonance imaging showed shadows of the bilateral paranasal sinuses. Endoscopic sinus surgery was performed, and amyloidosis was diagnosed by histopathology. She had previously been diagnosed with amyloidosis of the stomach, and therefore, she was diagnosed with primary systemic amyloidosis. A systemic workup for additional amyloid deposits revealed no evidence of other diseases. The patient remained under follow-up without further treatment, as no further amyloid deposition or progression of the lesions was seen. Amyloidosis is a rare condition characterized by the deposition of abnormal protein filaments in the extracellular tissue. Generally, systemic amyloidosis does not involve the head and neck region, and the presence of amyloid in the nasal and paranasal sinus mucosa is more likely to be indicative of a localized process. However, in our patient, the lesions were located in both the sinonasal tract and the stomach, indicating systemic amyloidosis. To our knowledge, there have been no previous reports of systemic amyloidosis involving the sinonasal tract, and therefore, we consider this case to be extremely rare.

Objective The ongoing pandemic of coronavirus disease (2019 coronavirus disease [COVID‐19]), caused by the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS‐CoV‐2) virus, is highly contagious with high morbidity and mortality. The role of the nasal and paranasal sinus cavities is increasingly recognized for COVID‐19 symptomatology and transmission. We therefore conducted a systematic review, synthesizing existing scientific evidence about sinonasal pathophysiology in COVID‐19. Study Design Systematic review. Methods Systematic searches were performed of all indexed studies in PubMed/Medline and Cochrane databases through 28 March 2020 and studies searchable on http://preprints.com (including ArXiv and Scilit repositories) through 30 March 2020. Data extraction focused on sinonasal pathophysiology in COVID‐19. Results A total of 19 studies were identified. The sinonasal cavity may be a major site of infection by SARS‐CoV‐2, where susceptibility genes required for infection are expressed at high levels and may be modulated by environmental and host factors. Viral shedding appears to be highest from the nose, therefore reflecting a major source for transmission. This has been highlighted by multiple reports of health care‐associated infection (HAI) during rhinologic procedures, which are now consequently considered to be high risk for SARS‐CoV‐2 transmission to health care workers. While sinonasal symptomatology, such as rhinorrhea or congestion, appears to be a rarer symptom of COVID‐19, anosmia without nasal obstruction is reported as highly specific predictor of COVID‐19+ patients. Conclusion Sinonasal pathophysiology is increasingly important in our understanding of COVID‐19. The sinonasal tract may be an important site of infection while sinonasal viral shedding may be an important transmission mechanism—including HAI. Anosmia without nasal obstruction may be a highly specific indicator of COVID‐19. Level of Evidence 2a.

Introduction SMARCB1 encodes for a component of the SWI/SNF complex and is widely implicated in carcinogenesis. In the head and neck, SMARCB1-deficient carcinomas typically arise in the sinonasal tract but can be found at other sites. EZH2 inhibitors have emerged as potential targeted therapy against SWI/SNF-deficient tumors. We sought to characterize the cytomorphology of head and neck carcinomas with SMARCB1 deficiencies to identify potential candidates for targeted therapy. Materials and methods Head and neck carcinomas with SMARCB1 mutations were retrospectively identified and confirmed to be SMARCB1-deficient by both molecular (fluorescent in-situ hybridization or next generation sequencing) and immunohistochemical means. Cases with positive cytology were reviewed and their cytologic features cataloged. Results A total of 19 specimens from 13 patients were reviewed, including 8 specimens from 7 sinonasal carcinomas, 4 specimens from 3 thyroid carcinomas, 3 specimens from 2 skin carcinomas, and 4 specimens from 1 carcinoma of unknown primary origin. High-grade features were common, including mitoses (11 of 19) necrosis (13 of 19) and multinucleation (16 of 19). Tumors showed either dense cytoplasm with distinct cell borders (10 of 19) or delicate cytoplasm with indistinct cell borders (9 of 19). Most tumors showed no distinct epithelial differentiation (12 of 19), while some (7 of 19) showed glandular or signet ring features. A minor cohort demonstrated rhabdoid cells (4 of 19). Conclusions Head and neck carcinomas with SMARCB1 deficiencies have a wide array of morphologies and tend to demonstrate high-grade features. Only a minor cohort demonstrate rhabdoid-type cells. Evaluation of SMARCB1 deficiency for potential targeted therapy should not be limited to tumors with rhabdoid morphology.

Background Respiratory epithelial adenomatoid hamartoma (REAH) is a benign lesion of the sinonasal tract that may mimic more concerning pathology. Clinical factors associated with REAH have not been well characterized. Objective To report our findings on patients with this pathologic diagnosis. Methods A retrospective chart review of patients with REAH between September 2006 and November 2019 was conducted. Data collected included clinical allergic rhinitis and asthma history, additional sinonasal diagnoses, prior sinus surgery, and the location of the REAH within the sinonasal cavity. Results Twenty-six patients were identified (53.8% male, mean age 62 years [range, 29–93]). Bilateral REAH occurred in 50%. REAH was located at the superior nasal septum in 84.6% cases, with the remainder identified in sinus contents submitted for pathology, making definitive site uncertain. Concurrent sinonasal inflammatory disorders were identified in 18 patients (69.2%), including chronic rhinosinusitis with nasal polyps—not otherwise specified (6), chronic rhinosinusitis without nasal polyps (4), aspirin-exacerbated respiratory disease (2), allergic fungal rhinosinusitis (1), central compartment atopic disease (5), and IgG4-related sclerosing disease (1). Eight patients had isolated REAH. Adequate allergy records were available for 19 patients, of which 18 of 19 (94.7%) had clinical allergic rhinitis. Conclusions REAH is a benign sinonasal lesion commonly located within the central compartment of the nasal cavity, a site of significant allergen exposure. Affected patients have a high incidence of allergy along with chronic inflammatory conditions. The coexistence of REAH within inflammatory nasal mucosa in a consistent anatomic location, suggests REAH may have a similar etiology to central compartment atopic disease, with resultant respiratory glandular ingrowth within long-standing reactive changes of mucosa derived from ethmoid embryologic origin.

Sinonasal malignant mucosal melanoma (SNM) is a rare, aggressive malignancy. The diagnosis of SNM is often quite challenging due to anatomical limitations, frequent lack of pigmentation, variable histologic appearances, and aberrant differentiation (e.g., positivity for cytokeratin, desmin, or neuroendocrine markers). S100 protein is routinely used as a standard screening marker for SNM, but it may lack optimal sensitivity. Our objective was to study the extent of immunohistochemical expression of S100 protein in SNM, and determine its diagnostic value by comparing it to a newer melanoma marker, SOX10. Twenty-three cases of sinonasal MMM were retrieved from the archival files of the Department of Pathology at UT Southwestern Medical Center. The patients included 14 men and 9 women, and ranged from 36 to 90 years (mean 64.9 years). Sections from blocks of formalin-fixed, paraffin-embedded tissue were used for immunohistochemical analysis with S100 protein and SOX10. The extent and intensity of immunostaining was recorded, and H-score was calculated. For a subset of negative or focally positive cases, S100 protein was repeated at a high-volume reference laboratory. S100 protein immunoexpression was quite variable in the SNM cases, with H-scores ranging from 0 to 300 (mean 123). While 11 of 23 cases exhibited strong and diffuse staining (H-score > 100) as expected for melanoma, 7 were weak and/or focal (H-score 1–100), and 5 were completely S100 protein-negative. For 10 cases, the negative or focal results were confirmed by reference laboratory staining. In contrast, all 23 SNM cases were diffusely and strongly positive for SOX10 (H-scores 210–300, mean 296). Our study demonstrated that S100 protein immunoexpression is extremely variable in SNM. Weak or even absent S100 protein staining is not uncommon in SNM, and should not dissuade pathologists from that diagnosis. Our data demonstrates that S100 protein is insufficiently sensitive to be used as a screening marker for SNM, but that SOX10 is consistently and robustly positive, and should therefore replace S100 protein for that purpose. Indeed, for any high-grade sinonasal tumor, pathologists must have a low threshold for utilizing additional markers to exclude the possibility of SNM.

The anterior skull base can be divided into three segments: a midline and two symmetrically placed segments located laterally. The midline segment is the roof of the nasal cavity and serves as a watershed between the sinonasal tract and the intracranial space, whereas the lateral segments separate the intracranial compartment from the orbital content. Several peculiar anatomical areas make up the midline segment (posterior frontal plate, cribriform plate, ethmoidal roof, planum sphenoidale, and tuberculum sellae), while the lateral segments are more regular, formed by flat laminae (orbital plates of the frontal bones and lesser wings of the sphenoid). Here we detail each segment of the anterior skull base, emphasizing major landmarks, providing classifications and measurements of key areas, and cautioning the endoscopist about areas to avoid or minimize the occurrence of cerebrospinal fluid leaks, as well as providing recommendations and tips. Several endoscopic and sectional macroscopic anatomical images provide the reader with an informative, illustrative, and broad perspective of anterior skull base anatomy.

The aim of this study is to describe the clinicopathological characteristics of intranasal pleomorphic adenomas (PAs), as well as the role and outcomes of endoscopic endonasal resection. A retrospective review of the clinical data from patients with PA of the nasal cavity who were treated by the authors at three tertiary medical centers between June 1998 and December 2019. A total of five patients with PA were found. Three patients were male, two were female and their mean age was 62.2 years. All cases were resected "en bloc" with endoscopic endonasal approach. No evidence of disease was observed during a mean follow-up of 10.6 years. No case presented with malignant transformation into carcinoma ex-PA. PA of the sinonasal tract and the nasopharynx is difficult to diagnose due to nonspecific clinical and radiological findings. Endoscopic endonasal approaches can be considered the gold standard in the treatment of these tumors and provide excellent visual control of the surgical field and clear margins.

Cavernous haemangioma of the maxillary sinus often presents with recurrent epistaxis and nasal obstruction. Clinical features usually mimics sinonasal mass or antrochoanal polyp. We report this case of a 13 years old female patient with unilateral maxillary sinus cavernous haemangioma which was managed successfully by both endoscopic and Caldwell Luc approach. The patient presented with features of antrochoanal polyp. Radiologically, it was suspected to be an Angiomatous Antrochoanal polyp and histologically proved to be a cavernous haemangioma. Postoperative period was uneventful.

The sinonasal tract is the site of origin for an enormous variety of neoplasms. Sinonasal neoplasms are broadly classified as either epithelial or mesenchymal. Among sinonasal malignancies, squamous cell carcinoma (SCC) accounts for approximately 80%.

Patient: Female, 51-years-old Final Diagnosis: Choanal polyp with osseous metaplasia Symptoms: Nasal obstruction Medication:— Clinical Procedure: Combined surgical approach, endoscopic and trans-oral Specialty: Otolaryngology Objective: Rare co-existance of disease or pathology Background: Osseous metaplasia is a heterotopic normal bone in soft tissues. It is occasionally found in mucosal polyps of the external auditory canal, tongue, gut, stomach, nasal cavity, and uterus. Choanal polyp with osseous metaplasia originating from the lateral wall of the nasopharynx has not been previously reported. In fact, osseous metaplasia in nasal polyps represents a very uncommon histological finding with only 12 cases described in the literature. Case Report: We reported here, the clinical, radiological, and therapeutic management of a nasopharynx choanal polyp in a patient with severe nasal obstruction and rhinolalia history. Endoscopic examination of nasal cavities revealed a polypoid-like mass in the left nasal fossa, extending to the choanal area and nasopharynx. Computed tomography scan and contrast-enhanced magnetic resonance imaging confirmed the presence of a solitary and lobulated mass in the choanal area and nasopharynx. We performed the removal of the mass through careful hemostasis of left sphenopalatine artery using both transnasal and transoral paths, with no complications for the patient. Histopathological examination of the biopsy revealed a benign inflammatory polyp with osseous metaplasia. No recurrence was noted 24 months after surgery. Conclusions: The incidence of ossifying areas in nasal polyps is very low, with only 12 cases reported in the literature. Ossifying polyps originating from the lateral wall of nasopharynx has never been reported before. The use of a combined endoscopic and transoral surgical approach was shown to be reliable in terms of adequate exposure and visualization of the lesion, control of bleeding, and complete removal of the mass.

Context.— Biphenotypic sinonasal sarcoma (BSNS) is a rare, slow-growing soft tissue sarcoma of the sinonasal tract, typically presenting with nonspecific obstructive nasal symptoms. Although recurrences are common, no metastases have been reported, and only 1 patient has died of disease thus far. It characteristically demonstrates rearrangements of PAX3 with multiple fusion partners, the most common of which is MAML3. Objectives.— To highlight the most important diagnostic features, including morphologic, immunohistochemical, and molecular findings, and to provide comparisons to other entities in the differential diagnosis. We also aim to provide a summary of the clinical features and outcomes in cases reported to date. Data Sources.— Recently published literature encompassing BSNS and its synonym, low-grade sinonasal sarcoma with neural and myogenic differentiation. Conclusions.— BSNS is a sinonasal tumor that is important to recognize because its biologic behavior differs from most of the entities in the differential diagnosis. The diagnosis can typically be rendered through a combination of morphology, immunohistochemical stains, and ancillary testing for characteristic PAX3 rearrangements.

Tumors of the sinonasal tract are usually of epithelial in origin such as papilloma, salivary gland tumors or carcinoma. Soft tissue tumors of the sinonasal tract are rare and are predominantly vascular and fibrohistiocytic in origin. Neural tumors are unusual in this location. Schwannomas of the sinonasal tract are rare, benign, slow growing peripheral nerve sheath tumors representing less than 4% of the schwannomas of the head and neck region without age, race or gender predilection. We present a case of a 50 year old female patient with history of unilateral nasal obstruction and with a polypoidal mass of the right nasal septum, in whom a diagnosis of Schwannoma was rendered.

Background: Epigenetic modifications have been recognized as an important mechanism underlying carcinoma progression. DNA methylation plays an important role in cancer biology and represents potentially heritable changes in gene expression that do not involve DNA sequence. The aim of this study was to investigate promoter methylation of selected genes in sinonasal carcinoma by comparison with noncancerous sinonasal tissue. Methods: To search for epigenetic events (methylation in 25 tumor suppressor genes) we used MS-MLPA (Methylation-specific multiplex ligation-dependent probe amplification) to compare methylation status of 59 formalin fixed, paraffin embedded tissue samples of sinonasal carcinomas with 18 control samples. The most important changes in methylation were confirmed using MSP (Methylation specific PCR). Detected alterations in methylation were compared with clinicopathological characteristics. Results: Using a 20% cut-off for methylation (MS-MLPA), we found significantly higher methylation in GATA5 (P=0.0005), THSB1 (P=0.0002) and PAX5 (P=0.03) genes in the sinonasal cancer group compared to the control group. Methylation in five or more genes was associated with impaired overall survival (P=0.017). Conclusion: These findings provide evidence that alterations in methylation profile may be one of the major mechanisms in sinonasal carcinogenesis. In addition, changes in methylation could potentially be used as prognostic factors of sinonasal carcinoma and may have implications for future individualized therapy based on epigenetic changes.

Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC) is a distinct, newly-described sinonasal tract neoplasm characterized by a salivary gland tumor-like appearance with myoepithelial and ductal cells, frequent surface squamous dysplasia, and relatively indolent behavior. When considering a diagnosis of HMSC, aggressive high-grade salivary gland carcinomas, particularly those with a basaloid morphology such as basal cell adenocarcinoma and adenoid cystic carcinoma, enter the differential diagnosis. The full morphologic and immunophenotypic profile of HMSC continues to be unraveled. In this series of ten cases, we demonstrate that this tumor has consistent, strong immunohistochemical expression of LEF-1 yet lacks nuclear expression of β-catenin, and also has consistent yet variable expression of MYB protein. While LEF-1 expression may be a useful diagnostic adjunct, it can also be a pitfall, as other salivary tumors such as basal cell adenocarcinoma have been previously shown to express LEF-1. Additionally, MYB protein expression is not a discriminatory marker when trying to separate HMSC from adenoid cystic carcinoma.

Background Sinonasal sarcomas are rare and heterogeneous in nature. Continuously collecting data influencing the prognosis is fundamental for optimizing therapeutic assessment of this highly destructive neoplasm. Methods We conducted a single-institution retrospective cohort study considering 27 patients with sinonasal sarcoma. Results The overall survival (OS) rates for 1 year and 5 years were calculated as 74% and 36%, respectively. Extent of the primary tumor (P = .010), nodal spread (P = .009), and age (P = .004) significantly reduced the OS. Disease-free survival (DFS) was significantly reduced by age (P = .003), extent of the primary (P = .006), nodal (P = .004), and hematogenous (P = .048) spread. Multimodal therapy including surgery improved the OS and DFS rates (P Conclusion Prognosis is poor due to late disease recognition. However, multimodal therapeutic regimens, including surgery, may improve the outcome.

The last two decades have seen significant advances in the pathology of sinonasal tract neoplasms. This was the consequence of the availability of several innovative diagnostic tools, which resulted in a dynamic evolution of entities and splitting of newly defined or conceptualized entities and subtypes that have been included in the spectrum of old heterogeneous diseases. Most of these new tumor subtypes have distinctive demographic, clinicopathologic, and biological characteristics with prognostic and therapeutic implications for individual patients. NUT carcinoma (NUT midline carcinoma) was separated from the spectrum of sinonasal undifferentiated carcinoma (SNUC) and is defined by specific recurrent translocation. On the other hand, the recently described SMARCB1-deficient carcinoma (while probably representing a distinctive clinicopathologic entity) remained as a variant in the SNUC spectrum. A new neoplasm in the spectrum of non-keratinizing carcinomas is the human papillomavirus(HPV)-related adenoid-cystic-like sinonasal carcinoma with its distinctive, albeit diverse, morphology. In the group of small round-cell malignancies, adamantinoma-like Ewing sarcoma has been delineated as an important diagnostic pitfall given its prominent epithelial differentiation. Inclusion of the biphenotypic (myoneural) sinonasal sarcoma (BSS) as a low-grade malignancy defined by recurrent PAX3/MAML3-translocation represents an important feature of the new WHO classification given the distinctive biological behavior of this low-grade non-metastasizing rare entity, which has been uniformly misclassified as a peripheral nerve sheath tumor or leiomyosarcoma in the past. Recognition of CTNNB1 mutations and STAT6/NAB2 gene fusions as defining genetic markers for sinonasal hemangio‑/glomangiopericytoma and solitary fibrous tumors, respectively, represents another important achievement in recent years. This review summarizes the new aspects in the WHO classification and also addresses recently described entities that have not been included in the WHO classification.

BACKGROUND Intestinal-type adenocarcinoma (ITAC) of the nasal cavity and paranasal sinuses is an uncommon tumor associated with exposure to wood and leather dust, nickel, and possibly smoking. ITAC shares phenotypical features with colorectal carcinoma. In contrast to most non-intestinal-type sinonasal adenocarcinomas, ITAC is an aggressive adenocarcinoma with poor clinical outcome; therefore, its reliable separation from non-ITAC is very important. AIM The use of a combination of immunohistochemical markers of intestinal differentiation was tested in a cohort of sinonasal carcinomas of different types. The aim of this study was to explore a new intestinal marker, SATB2, in conjunction with CDX2 and CK20 in differential diagnosis of sinonasal adenocarcinomas. MATERIALS AND METHODS Seven ITACs, 66 non-ITACs, and 1 case of extensive intestinal metaplasia (IM) of the nasal mucosa were included in the study and stained with SATB2, CK20, CDX2, and CK7 antibodies. Detection of mismatch repair proteins was performed in all cases of ITAC. All 7 sinonasal ITACs have been tested for KRAS, NRAS, and BRAF gene mutations. RESULTS All ITACs showed positive expression for SATB2, whereas all non-ITAC cases were negative. The only 1 case of IM was found to be positive for SATB2, whereas the same case showed negative expression of CK20 and only focal immunostaining for CDX2. The genetic analysis showed that only 1 sinonasal ITAC (1/7) showed KRAS c.35G>C, p.(Gly12Ala) mutation, whereas BRAF and NRAS genes were wild type. Four ITACs revealed wild-type KRAS, NRAS, and BRAF, and 2 remaining cases were not analyzable. All ITACs showed preserved nuclear expression of mismatch repair proteins. CONCLUSIONS SATB2 in combination with CDX2 and CK20 differentiates sinonasal ITAC from non-ITAC with increased diagnostic sensitivity and specificity and detects IM in the sinonasal tract more easily.

Although schwannomas are commonly found in the head and neck region, sinonasal tract involvement is extremely rare, estimated as4%. The presence of these lesions in the frontal sinus is even less common.Two patients presented with schwannomas originating in the frontal sinus with variable degrees of extension. These patients underwent resection of the tumors via an endoscopic or combined approach. The histologic diagnosis confirmed schwannoma in both cases.Although these lesions have traditionally been approached through larger craniotomies and open procedures, we describe less invasive approaches for these rare frontal sinus schwannomas and demonstrate them as a valid alternative with minimal morbidity for patients.