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.

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.

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.

Frontotemporal dementia: an unusual cause.

Intracerebral pneumocephalus is commonly associated with head and facial trauma, ear infection, tumors and surgical interventions. Osteomas are relatively common, benign tumors that occur mainly in the paranasal sinuses, the frontal sinus in particular. Pneumocephalus has been commonly reported with frontal osteoma but isolated presentation as frontotemporal dementia is uncommon. Patient was admitted with complaints of change of behavior and forgetfulness for the last one year. He had progressively become more apathetic and presented with behavioral abnormalities. General physical examinations were within normal limits including the motor and sensory system although neuropsychiatry assessments were below the average level, with features of dementia. Further, MRI brain revealed pneumocephalus in bilateral frontal lobe. CT cisternography revealed a well defined lobulated densely sclerotic lesion of approximate size 20 × 17 × 27mm transverse and cranio-caudal axis respectively arising from right ethmoid sinus. Clinically, the association of pneumocephalus and isolated presentation as frontotemporal dementia has not been described to the best of our knowledge. A single case has been described with ethmoid osteoma. Radiological features were suggestive of osteoid osteoma. The uniqueness of the case is the development of dementia with frontotemporal involvement and resemblance with Frontotemporal Dementia. This is the only case with dementia and pneumocephalus (secondary to osteoid osteoma) to best of our knowledge.

Wounding potential of 4.4-mm (.173) caliber steel ball projectiles.

From time to time, severe or fatal injuries caused by small caliber air rifle projectiles are seen. In forensic sciences, the theoretical wounding potential of these weapons and projectiles is widely known. Usually, shots against the skull were reported and, in these cases, penetrating the eyes or thin bone layers of the temporal region. Amongst a huge number of different projectiles available for air guns, sub-caliber 4.4-mm (.173) caliber steel ball projectiles were used in an unusual suicide case. This case led to fundamental questions concerning wound ballistics. An 82-year-old man shot once against his right temporal region and twice into his mouth with a 4.5-mm (.177) caliber air rifle. Because of the exceptionally deep penetration of the base of the skull and the use of spherical-shaped sub-caliber air rifle projectiles, terminal ballistic features were analyzed and compared to results published in forensic literature. Test shots using the same weapon and similar projectiles were fired into ballistic gelatin to measure and calculate basic wound ballistic variables of cal. 4.4-mm (.173) steel balls. In comparison, further test shots with cal. 4.5-mm (.177) steel balls BB (ball bearing), flat-headed and pointed air rifle pellets ("diabolos") were carried out. The theoretical penetration depth in solid bone was calculated with 36.4 mm, and test shots in gelatin from hard contact produced an on-average wound track of 120 mm underlining the potential wounding effect. Furthermore, spherical projectiles could roll back and forth within the barrel, and an air cushion between projectile and breechblock can reduce muzzle velocity by more than half, explaining the retained missile in the temporal region.

Surgical Management of a Recurrent Orbit-Eroding Mucocele Associated With Frontal-Ethmoidal Sinus Inverted Papilloma.

The development of orbit-eroding mucocele associated with inverted papilloma has been rarely reported., Here, the authors present a case and surgical management of a patient with orbit-eroding mucocele associated with inverted papilloma who declined craniotomy. A combined approach utilizing frontal endoscopic sinus surgery and external sub-brow anterior orbitotomy was used to explore, drain, and excise the mucocele and inverted papilloma. Gelatin sponges soaked in gentamicin were used to cover the exposed dura and to protect the orbital content from the frontal sinus cavity.A fronto-ethmoidal sinus inverted papilloma associated with recurrent orbit-eroding mucocele was excised by combined transnasal endoscopic and external sub-brow anterior orbitotomy approach using stereotactic navigation.

Three-dimensional computed tomography analysis of frontoethmoidal cells: A critical evaluation of the International Frontal Sinus Anatomy Classification (IFAC).

OBJECTIVES: Two major classifications of frontoethmoidal cells, Lee and Kuhn and the IFAC (International Frontal Sinus Anatomy Classification), distinguish anterior, posterior and medial cells. The European anatomical position paper includes also lateral cells. According to the IFAC, anterior cells push the frontal sinus drainage pathway (FSDP) medially, posteriorly or posteromedially. Posterior cells push the FSDP anteriorly. The only medial cell, pushing the FSDP laterally is the frontal septal cell, which is attached to or located in the interfrontal sinus septum. The aim of this study was to verify the IFAC and characterise cells, which are inconsistent with this classification. DESIGN: A radioanatomic analysis. SETTING: Tertiary university hospital. PARTICIPANTS: One hundred and three Caucasian adult patients with no inflammatory changes in paranasal sinuses CT. MAIN OUTCOMES MEASURE: Results of assessment of multiplanar reconstruction of thin slice CT. RESULTS: Two types of cells that cannot be classified using the IFAC were found: (a) Lateral cells extending between the skull base and the anterior buttress, pushing the FSDP anteromedially or medially, present in 34 (16.5%) of the sides, (b) Paramedian cells: medially based, not adjacent to the interfrontal septum, pushing the FSDP laterally and posteriorly, present in 33 (16%) of sides. Suprabulla cells and suprabulla frontal cells were found to push the FSDP in directions other than anterior 28% and 31% of the time respectively. CONCLUSIONS: Neglecting lateral and paramedian cells may lead to inconsistent results between radioanatomical studies and impede communication between surgeons. They should be included in existing classifications of frontoethmoidal cells.

Anatomic variations associated with antrochoanal polyps.

OBJECTIVE: Although more than a century has passed since antrochoanal polyps (ACPs) were first defined, etiopathogenesis still remains unclear. The aim of this study was to investigate the relationship between ACPs and sinonasal cavity variations. SUBJECTS AND METHODS: One hundred and forty-four patients with ACP on paranasal sinus computed tomography scans (ACP group) and 160 paranasal sinuses without ACP (control group) were included into the study. The study group was evaluated in respect of the presence of retention cyst in the contralateral maxillary sinus and sinus bone wall sclerosis thickening. Both groups were also compared with respect to the frequency of sinonasal anatomic variations, nasal septal deviation, variations of the uncinate process insertion, concha bullosa, paradoxical middle turbinate, and accessory maxillary sinus ostium. In the ACP group, the cases with septal deviation (SD) were also evaluated whether the deviation convexity was towards the polyp side or the opposite side. In addition, the posterior extension of ACPs were evaluated in three groups as middle meatus, nasopharynx, and oropharynx extension. RESULTS: The prevalence of retention cyst, sinus wall sclerosis thickening, SD, and accessory maxillary ostium was significantly higher in the ACP group. A negative directional correlation was determined between the SD side and ACP side. When the ACP extensions were examined, middle meatus extension was seen in 32.6%, nasopharynx in 56.3%, and oropharynx in 11.1%. CONCLUSION: Accessory ostium may be an accelerating factor in the transformation of retention cyst to ACP. Furthermore, the changes in the nasal passage airflow on the opposite side suggest that SD contributes to this process.

Impact of anatomic origin of primary squamous cell carcinomas of the nasal cavity and ethmoidal sinus on clinical outcome.

PURPOSE: Since squamous cell carcinomas (SCCs) of the nasoethmoidal complex are rare and aggressive malignancies, the purpose of this study was to evaluate whether anatomic subsites of SCCs of the nasal cavity and ethmoid sinuses affect clinical outcome. METHODS: We retrospectively analyzed data from 47 patients with primary SCCs of the nasal cavity and ethmoid sinuses who were treated at the Department of Otorhinolaryngology, Head and Neck Surgery, Medical University of Vienna, between 1993 and 2018. The impact of anatomic subsites of nasoethmoidal SCCs was evaluated with respect to tumor and nodal classification, disease-free survival (DFS) and disease-specific survival (DSS). RESULTS: Of the 47 cases, 17 SCCs (36.2%) originated from lateral nasal wall followed by 13 (27.7%) tumors of the edge of naris to mucocutaneous junction, 11 (23.4%) SCCs of the nasal septum, 3 tumors of the nasal floor (6.4%) and 3 SCCs of the ethmoid sinuses (6.4%), respectively. SCCs of the nasal septum were associated with significantly higher rates of neck node metastasis (p = 0.007), which represented a significantly worse prognostic factor for DSS (HR 7.87; p < 0.001). Moreover, advanced tumor stage (HR 5.38; p = 0.014) and tumor origin of nasal septum (HR 4.05; p = 0.025) were also significantly worse prognostic factors for DSS. Fourteen patients (29.8%) developed recurrent disease, including eight local (17.0%), five regional (10.6%) and one distant (2.1%) recurrence. Elective neck dissection (ND) was associated with lower (0 vs. 20.0%) but not significantly different regional and distant DFS (p = 0.075). CONCLUSION: Anatomic origin of nasal SCC has significant impact on clinical outcome. SCCs of the nasal septum were associated with higher rates of positive neck nodes and worse outcome.

Nasoethmoid schwannoma with intracranial extension. Case report and review of the literature.

Nasoethmoid schwannomas with significant intracranial extension are rare tumours that typically present with visual and olfactory deficits. We describe a nasoethmoid schwannoma in a patient who was completely asymptomatic. Despite impressive intracranial involvement, nasoethmoid schwannomas may present asymptomatically and should be considered in the differential of contrast-enhancing anterior skull base lesions.

A Giant Osteoma of the Ethmoid Sinus.

Osteomas are slow-growing, benign bony tumors. Most are asymptomatic. Paranasal sinus osteoma in the pediatric population is rare; hence, there are few reports of symptomatic osteomas. The authors report the case of a giant ethmoid sinus osteoma in an 11-year-old girl who presented with diplopia and proptosis. Total excision and reconstruction of the medial orbital wall were performed via a coronal approach. The postoperative period was uneventful, the child's vision improved, and a good cosmetic result was obtained.

Osteosarcoma of the Ethmoid Sinus.

Osteosarcoma (OS) is a malignant bone tumor and it is very rare in head and neck region. If it arises in this area, most common localizations of this tumor are mandible and maxilla and ethmoid sinus is very rare site for this malignancy. A 43-year-old female admitted to our clinic with the complaint of pain in the right half of the face and headache persisting for 2 months. According to the clinical symptoms and imaging studies, sinonasal ossifying fibroma was suspected. For this reason, patient was operated endoscopically and histopathological examination revealed that osteoid matrix producing atypical osteoblastic cells so these findings identified osteoblastic variant OS. This article is about a very rare case of OS of ethmoid sinus. The differential diagnosis, general characteristics, and classification of this tumor, radiological features, and surgical techniques are also presented.

Hypertelorism Secondary to Mucocele in the Paranasal Sinuses.

Ocular hypertelorism was introduced by Greig as an increased interpupillary distance. The paranasal sinus mucoceles are acquired lesions for various reasons; however, their behavior is progressive, capable of eroding the bone and extending to the orbital and intracranial regions. The objective is to present a clinical case of orbital hypertelorism secondary to mucocele in the paranasal sinuses. This is a 72-year-old male patient who came presenting an increase in volume in the right nasoorbitoethmoidal region. The isodense lesion occupying the maxillary and right ethmoidal sinuses was confirmed by an intimate relationship with the ipsilateral frontal and sphenoidal sinus, with osteolytic involvement of the orbit and nasal region. After incisional biopsy with mucocele results, a wide resection plus facial reconstruction was performed with autologous grafts and osteosynthesis material. Currently, the patient has 1 year of evolution, without significant functional commitment. It is important to consider giant mucoceles as part of the differential diagnoses in patients with deformities in the middle and upper third of the face.

A rare case of large sphenoethmoidal osteoma.

Osteoma is the most common benign tumour of the nose and paranasal sinuses. However, involvement of the sphenoid sinus by osteoma is rare. Most of the time, patients with paranasal sinus osteoma are asymptomatic. However, symptoms may present, depending on the location and extension of the tumour. We report a case of a patient with sphenoethmoidal osteoma found incidentally on imaging, with evidence of impingement onto the optic nerve at the optic canal. However, the patient was asymptomatic. He was surgically treated using the endoscopic transnasal approach.

Venous malformation of the ethmoid and sphenoid sinuses.

OBJECTIVES: The aim of this study was to present a rare case of a venous malformation that occupied the ethmoid and sphenoid sinuses. Prior to resection, it was believed to be a hemangioma. METHODS: This study includes a case report and review of the literature. CONCLUSION: There is often confusion between "hemangiomas" and "vascular malformations," but they are important to differentiate because they have unique approaches to treatment. Venous malformations in the paranasal sinuses are very rare. To our knowledge, this is the first case report that explicitly describes a venous malformation in the ethmoid and sphenoid sinuses. It was treated using endoscopic sinus surgery with intraoperative computer-assisted stereotactic navigation.

IgG4-related disease in the head and neck region: report of two cases and review of the literature.

IgG4-related disease (IgG4-RD) is a rare immune-mediated condition characterized by extensive tissue fibrosis and infiltration by immunoglobulin G4 positive plasma cells in a single organ or systemic appearance. Two cases are presented including an unusual case of a 30-year-old man with IgG4-RD appearing simultaneously in the cervical lymph nodes, ethmoid, maxillary sinuses, and upper gingiva, with spontaneous loss of teeth. According to the literature, this is the first case with loss of teeth occurring in the course of the disease. The second case is a 46-year-old man suffering from IgG4-related chronic sclerosing sialadenitis of the right submandibular gland.

Osteoblastoma originating from frontoethmoidal sinus causing personality disorders and superior gaze palsy.

Osteoblastoma is a rare, solitary benign tumor that is usually situated in axial skeleton mainly in vertebra. It is rarely seen in ethmoid and frontal sinuses. A 40-year-old man who had osteoblastoma originated from frontal and ethmoidal sinuses that extends up to frontal lobe and gave rise to personality disorders by compressing the frontal lobe, and caused superior gaze palsy by compressing the superior rectus muscle. We present this rare case with clinical, radiological and histopathological findings.

Orbital Compartment Syndrome in Eosinophilic Angiocentric Fibrosis.

A 43-year-old man reported right visual loss after biopsy of an intranasal mass, which improved after treatment at another hospital. On first examination at our hospital, his visual acuity was 1.2 OD, and MRI showed a right intraorbital mass involving the ethmoid sinus. His visual acuity decreased to 0.08 OD 12 days after orbital biopsy, with right globe tenting shown by MRI. An emergent lateral canthotomy and cantholysis were performed. The histopathological diagnosis was eosinophilic angiocentric fibrosis, and treatment with intravenous methylprednisolone (125 mg/day) and oral diaminophenyl sulfone (75 mg/day) was started immediately after surgery. One month later, the diaminophenyl sulfone was discontinued and cyclophosphamide (50 mg/day) was started. The methylprednisolone and cyclophosphamide doses were tapered over 9 months and 7 months, respectively. At the 1.5-year follow-up examination, his visual acuity was 1.0 OD. The lesions did not grow in size.

Orbital presentation of a nasal midline destructive lesion in a young boy.

Midline Destructive Lesions (MDL) are well known to cause nasal problems. There is a long differential diagnosis of such lesions. However, in the pediatric population, the 2 main diseases to be aware of are Non-Hodgkin's T-cell lymphoma and granulomatosis with polyangiitis (previously known as Wegener's granulomatosis). The authors present the report of a 15-year-old boy who presented with epiphora, chemosis, and limitation of left abduction. CT scan of his orbits suggested a destructive lesion of the ethmoid sinuses. His laboratory investigations revealed a positive ANCA. The patient underwent endoscopic sinus surgery, and this was characteristic for granulomatosis with polyangiitis. He was treated with systemic steroids and then maintained on cyclophosphamide, which controlled his disease activity. This case highlights the need for ophthalmologists to have a high index of suspicion for MDL and concomitant orbital disease.