SMARCB1-Deficient Sinonasal Carcinoma: Case Report and Review of the Literature.

BACKGROUND SMARCB1-deficient sinonasal carcinoma is a rare neoplasm with inactivation of the SWI/SNF complex, with an aggressive clinical course as most of the lesions present as advanced in pT3/T4 stages with frequent recurrence, and many patients succumb to the disease. Reported initially in 2014, the lesion has male predominance, with an age range of 19 to 89 years and predilection for the ethmoid sinus and nasal cavity. Histopathological findings show a proliferation of small- to medium-sized monomorphic basaloid cells with indistinctive cytoplasmic borders and round variably prominent nuclei with scattered cells that show rhabdoid morphology. Cytoplasmic vacuoles are common. It has similar morphological findings to a wide array of neoplasms in the sinonasal area. CASE REPORT We report a case of SMARCB1-deficient sinonasal carcinoma in a 30-year-old man referred to our hospital with a preliminary diagnosis of sinonasal adenocarcinoma, intestinal type. Computed tomography showed a huge destructive soft tissue mass in the left maxillary sinus, extended to involve the left nasal cavity with extension to the skull base and perineural spread along the foramen rotundum. Histological examination revealed a malignant basaloid neoplasm embedded in a myxoid stroma that showed loss of SMARCB1 stain. The patient was treated with induction chemotherapy using etoposide and cisplatin for disease control. CONCLUSIONS SMARCB1-deficient sinonasal carcinoma is a rare neoplasm with an aggressive clinical course and high-grade behavior despite having uniform cytological features. This poses complex diagnoses, especially in small biopsies. Incorporating morphological findings with ancillary tests is required to identify this high-grade malignancy.

Osteoma of the ethmoid sinus in a pediatric patient - a case report.

BACKGROUND: Osteomas of the paranasal sinuses occur rarely in the pediatric population, we find only a few reference of symptomatic osteomas in the literature. Opinions on the indication for surgical treatment are controversial. CASE: The authors present a case of symptomatic osteoma of the right ethoimoidal sinus in a 12-year-old boy, who was treated surgically, with endoscopic endonasal approach. The symptomatology, diagnosis and therapy of these tumors in the pediatric patient are discussed. CONCLUSION: Osteomas of the paranasal sinuses are slow-growing benign lesions. Symptomatic osteomas can grow expansively and cause serious complications. The treatment of osteoma is surgical and the endoscopic approach offers the possibility of removal with cosmetic benefits.

Intraosseous Hemangioma of the Ethmoid Sinus.

Intraosseous hemangiomas, also referred to as bony hemangiomas, arising within the nasal cavity are exceedingly rare with only 2 cases arising in the ethmoid region. Despite their rarity, they are important to consider in a patient presenting with long-standing nasal congestion. While characteristic findings on computed tomography imaging may be observed, biopsy is diagnostic. En bloc resection using a transnasal endoscopic approach with or without preoperative embolization is the treatment of choice for intranasal intraosseous hemangiomas.

Surgical Approach to Frontal and Ethmoid Sinus Osteomas: The Experience of 2 Metropolitan Italian Hospitals.

OBJECTIVES: The aim of this study was to report the surgical management experience of patients with osteomas of the frontal and ethmoid sinuses performed in 2 metropolitan Italian hospitals between 2012 and 2019. METHODS: A retrospective chart review of cases of frontal and ethmoid osteomas from the Ca' Granda Niguarda Hospital of Milan and the Policlinico Umberto I University Hospital of Rome was performed. All patients underwent preoperative computed tomography and, when orbital or intracranial extension was suspected, magnetic resonance imaging. Surgical treatment was performed according to Chiu classification. RESULTS: A total of 38 cases of frontal and ethmoid sinus osteomas were included in the study; 22 patients were men and 16 were women. The mean age at diagnosis was 49 years. Seven (18.4%) patients were treated using an open approach; 3 (7.9%) patients underwent open and endoscopic approach; the remaining 28 (73.7%) patients were treated with endoscopic approach. Seven (18.4%) patients had a cerebrospinal fluid leak intraoperatively and were treated with the placement of tissue graft through the defect. The mean follow-up time was 18 months; no recurrence was observed at 12-month follow-up. CONCLUSION: Osteomas of the frontal and ethmoid sinuses can be treated using different techniques, mostly endoscopically. The choice of surgical approach (endoscopic vs open) depends on the location and size of the osteoma, anatomical size, characteristic of the sinus, surgeon's experience, and available existing technical facilities. Cerebrospinal fluid leak is a possible complication of surgery.

A Von Hippel-Lindau Disease-Associated Microcystic Adenoma of the Ethmoid Sinus Mimicking Metastatic Clear Cell Renal Cell Carcinoma.

ABSTRACT: A 38-year-old man with von Hippel-Lindau (VHL) disease and a history of renal cell carcinoma presented with a 2-month history of recurrent epistaxis. MRI revealed a microcystic tumor in the left ethmoid sinus with strong contrast enhancement. 18 F-FDG PET/CT showed FDG uptake (SUV max , 4.2) in the lesion. Under the suspicion of renal cell carcinoma metastasis, the patient underwent 2 surgical resections. However, based on the morphological and immunohistochemical findings, the patient was finally diagnosed with a VHL-associated microcystic adenoma of the ethmoid sinus, which is an extremely rare tumor that occurs in VHL disease.

Ethmoidal mucocele causing proptosis in a pediatric patient with cystic fibrosis: A case report.

In cystic fibrosis (CF) patients, Ear Nose Throat (ENT) pathology is often undiagnosed despite its high prevalence and its possible life-threatening complications. We present the case of an ethmoidal mucocele leading to ocular manifestations in a 2-year-old girl with cystic fibrosis with no previous serious complications. She progressively developed non-axial proptosis, limitation of the adduction and exotropia of her left eye. Paranasal sinus magnetic resonance image (MRI) showed a left ethmoidal mucocele causing displacement of the ocular globe, compression of the medial rectus and the optic nerve. Eye fundus revealed disc edema and diffuse vascular congestion. Endoscopic sinus surgery was performed to remove the mass. The mucocele was drained and the discharge was sent for microbiology assessment. Escherichia coli (E. coli) was found in the culture and treated with cefotaxime and dexamethasone with complete resolution of non-axial proptosis and disc edema.

Sino-nasal hemangiopericytoma: a case series and systematic literature review.

BACKGROUND: Hemangiopericytoma is a rare tumor of the sino-nasal tract. Its clinical behavior is controversial. Whereas some describe an indolent course, others consider it to be an aggressive lesion with a tendency toward rapid local recurrence. Here, we describe our experience in the management of sino-nasal hemangiopericytoma (SN-HPC), comparing our experience with the current literature, and evaluating signs and tools to improve diagnosis and treatment. METHODS: All cases of SN-HPC between 2010 and 2020 were extracted and reviewed from our institutional electronic medical records. SN-HPC cases from PubMed and EMBASE between 2010 and 2020 were analyzed in a systematic literature review using the preferred reporting items for systematic review and meta-analysis (PRISMA) guidelines. Data regarding demographics, presentation, diagnosis, treatment, and outcome were collected. RESULTS: We identified four cases of SN-HPC in the nasal cavity in our institution and an additional 53 cases in previous reports. The mean age at the time of diagnosis was 59 years, with a 1.2:1 male to female ratio. SN-HPC mostly appears unilaterally, arising in the ethmoid sinus (42.1%). The most common presenting symptoms were epistaxis (47.3) and nasal obstruction (47.3%). Both computed tomography (CT) and magnetic resonance imaging (MRI) were required for diagnosis and for tailoring the treatment plan. Endoscopic surgical excision was used in 85.9% of the patients, and in 15.7%, an additional preoperative embolization was performed, which was associated with septal necrosis in one patient (2.6%). The recurrence rate was 7%. CONCLUSION: Although previous reports attribute an aggressive tumoral behavior to SN-HPC, our experience and the literature review support a more indolent course with low recurrence rates following complete endoscopic resection. Preoperative embolization can be useful in certain cases, but due to potential complications, it should not be routinely indicated.

Risk Model Establishment of Endoscopic Sinus Surgery for Patients with Chronic Rhinosinusitis: a Multicenter Study in Korea.

BACKGROUND: Endoscopic sinus surgery (ESS) is the mainstay treatment for refractory chronic rhinosinusitis (CRS). Since various factors may contribute to the surgical outcome, it is challenging for physicians to predict surgical outcomes. The aim of study was to analyze the prognostic factors of postoperative outcomes and to establish the prediction model with the risk factors that impact the postoperative outcomes. METHODS: Medical records of CRS patients who underwent ESS at 9 institutions in 2005, 2010, and 2016 were retrospectively reviewed. We classified the patients into 2 groups based on postoperative objective endoscopic outcomes. Demographics, nose-specific symptoms, olfactory function, eosinophil counts in blood (EoB) and nasal tissue (EoT), and Lund-Mackay CT score (LMS) were collected. Univariate and multivariate analyses were performed and established a prediction equation for postoperative endoscopic objective outcomes. RESULTS: In total (n = 1,249), 27.0% were not satisfied under postoperative endoscopic examination. Of 10 variables, LMS (> 5), sinus dominancy (maxillary sinus and ethmoid sinus), EoB (> 210), and EoT (> 100) were statistically significant in univariate analysis (P < 0.05, all). In multivariate analysis, EoT (> 100) and LMS (> 5) were significantly associated with poor postoperative outcome. Furthermore, 5 significant variables were employed to establish the risk model of postoperative outcomes and P (the value of prediction probability) = 1 / (1 + exp [-0.392 + 1.088 × EoT (> 100) + 0.123 × mean LMS (> 5) - 0.366 × sinus dominancy (maxillary) + 0.064 × sinus dominancy (similar) + 0.200 × EoB (4%) + 0.344 × EoB (> 210)] was developed. CONCLUSION: Tissue eosinophil count and radiographic severity predispose to a poorer outcome of ESS and the risk model established may be helpful to predict postoperative outcomes of ESS.

[Fibrous dysplasia of the frontal and ethmoid sinuses: a case report]. / Dysplasie fibreuse ethmoïdo-frontale: à propos d'un cas.

Fibrous dysplasia is a genetic and rare bone disorder affecting the young subject. Its pathophysiology involves genetic mutations leading to defective skeletal development with fibrous and medullary proliferations. Cephalic extremity is involved in one third of cases. We here report a case of surgically treated fibrous dysplasia of the frontal and ethmoid sinuses. The study involved a 31-year-old man presenting with chronic holocranial headaches. Computed tomography (CT) scan showed voluminous bilateral hyperdense expansive process in the frontal and ethmoid sinuses. Surgery was performed on the basis of functional signs and imaging data. Anatomopathological examination of bone fragments confirmed the diagnosis of fibrous dysplasia. Fibrous dysplasia is rare and characterized by slow disease progression. In the absence of a consensus on therapy, surgery remains the treatment of choice for unifocal forms.

Clinical, anatomopathological, and immunohistochemical findings of a transitional cell carcinoma from nasal cavity, frontal and ethmoidal sinus with meningoencephalic invasion in a dog.

Background: Primary neoplasms of the nasal cavity and sinuses are uncommon in domestic animals, most of which are of epithelial origin, being adenocarcinoma the most common tumor diagnosed in this region. Some malignant nasal cavity neoplasms may invade the brain causing clinical neurological signs, as well as purulent nasal secretion and epistaxis. Case Description: A case of neoplasm is reported in a 14-year-old pincher presenting dyspnea, epistaxis, and neurological alterations. Necropsy revealed the presence of a mass in the oral cavity vestibule, and another in the whole nasal cavity with invasion of the cribiform plate, meninges and brain. Squamous cells carcinoma was diagnosed in the oral cavity and transitional carcinoma in the nasal cavity. The immunohistochemistry confirmed that the brain infiltration was of the same origin as the nasal cavity neoplasm. Conclusion: The present report describes a rare case of transitional carcinoma of the nasal cavity as well as the frontal and ethmoidal sinuses with brain invasion, confirmed by immunohistochemistry. It is extremely important for veterinarians to include neoplasms in their differential diagnoses, when these animals show chronic respiratory signs and neurological alterations that do not improve with appropriate treatment, always associating with complementary exams, for correct diagnosis establishment and prognosis formulation.

Adult-onset Woakes' syndrome: a rare entity.

Woakes' syndrome (WS) is a rare entity, defined as severe recalcitrant nasal polyposis with consecutive deformity of the nasal pyramid. WS occurs mainly in childhood and its aetiology remains unclear. We report a case of a 68-year old woman, with aspirin-exacerbated respiratory disease, who presented with recurrent nasal polyposis and progressive broadening of the nasal dorsum. CT scan revealed extensive bilateral nasal polyposis and diffuse osteitis, with anterior ethmoidal calcified lesions. The patient underwent revision endoscopic sinus surgery and nasal pyramid deformity was successfully managed without osteotomies.

Is Ethmoidal Air Cell Count Relevant in Chronic Rhinosinusitis?

OBJECTIVES/HYPOTHESIS: Studies analyzing the association between the total number of ethmoid cells (EC) and chronic rhinosinusitis (CRS) are missing. Our aim was to analyze the total number of EC in patients with and without CRS. STUDY DESIGN: Retrospective case-control study. METHODS: A total of 50 computed tomography (CT) scans of patients with CRS, 14 CT scans of patients with odontogenic CRS, and 50 CT scans of healthy controls were retrospectively analyzed. The number of EC has been determined for each side separately. RESULTS: In total 228 sides have been analyzed. The bilateral total EC count in both planes was more than 2 cells higher, when comparing CRS patients to healthy controls or odontogenic CRS patients (P < .01). No difference was observed, when comparing healthy and odontogenic CRS patients. CONCLUSION: The number of EC on CT of CRS patients is significantly higher than in both, the healthy and the odontogenic CRS control group. Cell count could contribute to the pathogenesis of CRS. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1212-1216, 2021.

Small Cell Neuroendocrine Carcinoma of Paranasal Sinuses: Radiologic Features in 14 Cases.

PURPOSE: The purpose of this study was to explore the characteristic computed tomography (CT) and magnetic resonance (MR) features of small cell neuroendocrine carcinoma (SNEC) of paranasal sinuses. MATERIALS AND METHODS: Computed tomography (n = 8) and MR (n = 14) images and clinical findings from 14 patients with SNEC of paranasal sinuses were retrospectively reviewed. RESULTS: Eight lesions were located in the ethmoidal sinus, 4 in the maxillary sinus, and 2 in the sphenoid sinus. Small cell neuroendocrine carcinoma of the sphenoid sinus showed bilateral asymmetry patterns. On CT images, bony changes were visible in all 8 cases. On MR, 4 cases contained hemorrhage, and 10 cases contained cystic or necrotic areas. All cases demonstrated marked heterogeneous enhancement, with half showing a "cribriform-like" or "geographic" appearance. The nasal cavity was the most common site invaded by SNEC of paranasal sinuses, followed by the orbits. A time-signal intensity curve examination showed a washout-type pattern in all but 1 case. The mean ± SD apparent diffusion coefficient value was 0.702 ± 0.112 (×10-3 mm2/s). According to the Dulguerov staging system, 9 tumors were staged as N0 (1 T1, 1 T2, 5 T3, and 2 T4). The recurrence rate was 64.3%. CONCLUSIONS: Some characteristics of radiological findings can provide important clues for preoperative diagnosis.

Orbital cellulitis secondary to giant sino-orbital osteoma: A case report.

BACKGROUND: Although osteoma is a common benign tumor of the paranasal sinuses, its orbital extension is not common. Secondary orbital cellulitis has rarely been reported in association with sino-orbital osteoma. CASE: A 30-year-old woman presented with left side proptosis, orbital pain and inflammation. Orbital CT scan showed a well-defined giant osteoma in the superonasal part of the left orbit originating from the left ethmoidal sinus associated with opacity of the ipsilateral ethmoidal sinus and infiltration of orbital soft tissue. After treatment by systemic antibiotics, osteoma was resected with combined external and endoscopic surgery and the patient recovered uneventfully. CONCLUSION: Sino-orbital osteoma may manifest primarily as orbital cellulitis and needs early surgical intervention.

Epithelial Squamous Metaplasia and Dysplasia in Inflammatory Nasal Polyps: An Observational Study.

Nasal polyposis (NP) is characterized by polypoid outgrowths of chronically inflamed respiratory mucosa. The presence of squamous metaplasia and dysplasia on the mucosal surface of nasal polyps (NPs) represents different manifestations of epithelial atypia. The aim of this investigation was to evaluate the presence of epithelial squamous metaplasia and dysplasia in ethmoidal NPs. This retrospective analysis of prospectively collected data involved 212 patients with NP undergoing endoscopic ethmoidectomy. To evaluate possible etiological factors for epithelial atypia, the patients in whom we histopathologically detected the presence of epithelial atypia were compared with patients with "normal" NPs in accordance with the following characteristics as found in the patients' medical records: gender, age, main symptoms, preoperative extent of sinus disease on computed tomography, atopic status, aspirin sensitivity, cigarette smoking, and occupational exposure to different noxious factors. Epithelial atypia were detected histopathologically in 44 (20.7%) NP patients, whereas features of "true" dysplasia were found in only 1 (0.5%) patient. The presence of atypia was more frequent in males than in females (P = .008). The association with aspirin-exacerbated respiratory disease and with long-term occupational exposure to different noxious chemicals, especially in workers exposed to salts of heavy metals, was more frequent in NP patients with epithelial atypia than in patients without atypia (P = .023; P = .006, respectively). Our results suggest epithelial atypia in NPs are associated with aspirin sensitivity and occupational exposure to different noxious chemicals. Although extremely rare, epithelial dysplasia may occasionally be noted in NPs, a fact potentially useful for both rhinologists and pathologists.

Glomangiopericytoma of the sphenoethmoid complex.

Sinonasal glomangiopericytoma is a rare sinonasal tumour accounting for less than. 5% of all sinonasal tumours. This tumour often presents as another, more common type of vascular lesion and is similarly prone to haemorrhage. The optimal treatment includes complete surgical resection. We, herein, present two such cases adding to the world literature of this rare tumour.

Extramedullary plasmacytoma of the nasal cavity and ethmoidal sinus in human immunodeficiency virus-positive patient.

Extramedullary plasmacytoma (EMP) occurring in the nose and paranasal sinus regions are rare with a male preponderance in the fifth and seventh decades of life. We report a case of EMP of the nasal cavity and ethmoid sinus in a 28-year-old female with human immunodeficiency virus infection.