Introduction  Angioleiomyoma is a rare neoplasm that represents ∼ 0.2 % of all head and neck benign tumors and ∼ 2% of total cases of tumors of the sinonasal tract. It was once considered a possible subtype of leiomyoma, but, in the 2020 World Health Organization (WHO) classification of soft tissue tumors, it is accepted as a singular entity. Objective  To systematically review the existing literature on angioleiomyoma in the light of the new classification of soft tissue tumors. Data Synthesis  The present study was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A comprehensive search in the PubMed, Cochrane, Scopus, and Google Scholar databases was performed in January 2022. The search items included the following keywords: nasal angioleiomyoma OR sinonasal angioleiomyoma OR nasal vascular leiomyoma OR sinonasal vascular leiomyoma . A total of 87 patients were evaluated. He age of the patients in the studies ranged from 15 to 88 years (mean age at diagnosis: 55.6 years). The most common site of involvement was the nasal septum (28.4 %), followed by the inferior turbinate (22.5%). The most common symptom was nasal obstruction (66.7%), followed by epistaxis (47.1%). Surgical excision represented the main treatment, and there was recurrence of pathology in only 1 case (0.9%). Conclusion  To our knowledge, only 87 cases of sinonasal-tract angioleiomyoma have been previously described. The results of the present review seem to confirm the rarity and the benign nature of this neoplasm, and they seem to confirm the necessity to improve the available data about sinonasal-tract angioleiomyoma.

OBJECTIVE: The role of elective neck dissection (END) in the management of clinical N0 (cN0) squamous cell carcinomas (SCC) of the sinonasal tract is unclear. In this systematic review, we evaluate the risk of occult nodal metastasis in sinonasal SCCs with cN0M0 tumors to support clinical decision making.
METHODS: A literature search was conducted in the following three electronic databases: Medline/PubMed, ScienceDirect, and Google Scholar. Articles were assessed for eligibility in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement. Two independent authors extracted the data. The Methodological Items for Non-Randomized Studies (MINORS) tool was used for the assessment of biases of each included study.
RESULTS: Our systematic review included six studies that met the inclusion criteria, all retrospective in design. The rate of histologically proven metastasis of sinonasal SCC to the clinically negative neck is 12.5%. Almost half of the positive cases are pathologically staged as N2 (6.5%).
CONCLUSIONS: Our systematic review provides the rate of sinonasal SCC occult metastasis to the neck so that the surgeons can discuss with patients the risks and possible merits of adding an elective neck management in the surgical plan.

BACKGROUND: Low-grade papillary Schneiderian carcinoma (LGPSC) is a relatively new entity of the sinonasal tract and is characterized by a bland morphology simulating sinonasal papilloma, invasive growth pattern with pushing borders, and aggressive clinical behavior with multiple recurrences and metastatic potential. Recently, DEK::AFF2 fusions were identified in LGPSC. However, some LPGSCs lack DEK::AFF2 fusion, and the molecular features of these tumors have not been clarified.
METHODS: A 69-year-old man presented with a discharge of pus from his left cheek. Computed tomography revealed a mass involving the left maxillary sinus, ethmoid sinus, and nasal cavity with the destruction of the orbital wall. The biopsy specimens showed that the tumor had a predominantly exophytic, papillary growth and did not have an apparent stromal invasion. The tumor was composed of multilayered epithelium that showed bland morphology with a round to polygonal shape, abundant eosinophilic cytoplasm, and uniform nuclei. Dense neutrophilic infiltrates were focally present. Immunohistochemically, CK5/6 was strongly and diffusely positive, and p16 was negative. p63 was mainly positive in the basal layer, and EMA was predominantly expressed in the outermost cell layer. DNA-based targeted sequencing showed TP53 R175H mutation, whereas neither EGFR nor KRAS mutation was identified. Reverse transcription polymerase chain reaction and fluorescence in situ hybridization revealed no DEK::AFF2 fusion.
CONCLUSIONS: We describe the first case of TP53-mutant LGPSC and review the literature. LGPSC is a genetically heterogeneous entity, and the recognition of this rare entity and comprehensive assessment of clinicopathological and molecular findings are crucial for the correct pathological diagnosis and clinical management.

UNASSIGNED: HPV-related carcinoma with adenoid cystic carcinoma-like features currently known as HPV-related multiphenotypic sinonasal carcinoma (HMSC) is a rare distinct head and neck high-risk HPV-related carcinoma. The high-risk HPV subtypes implicated are particularly type 33,35 and 56. So far this is the first reported rare case of a variant of sinonasal carcinoma in Tanzania.
METHODS: We present a 59-year old female who presented with a history of right-sided nasal obstruction and intermittent epistaxis for about one year and later on had ipsilateral proptosis. A provisional diagnosis of advanced right-sided malignant sinonasal tumor was made. Trans nasal biopsy revealed HMSC.
UNASSIGNED: The patient had a right sided fixed neck mass that measured about 7.5 × 8.2 cm. Magnetic resonance imaging (MRI) of the paranasal sinuses revealed a T1 weighted contrasted images that showed a huge extensive enhancing mass (estimated to measure 4.5 × 4.4) involving the nasal cavity, bilateral ethmoid sinuses, frontal and sphenoid sinuses and also the tumor exhibit intracranial extension (anterior cranial fossa) and tend to compress medial walls of both orbits though more marked on the right side. Histopathology and immunohistochemistry confirmed HPV-related multiphenotypic sinonasal carcinoma. The disease was staged to be T4bN3aM0 and the patient was referred for palliative chemoradiation.
CONCLUSIONS: Although HMSC presents at advanced stage in close to half of cases and has a high-grade histological appearance it paradoxically exhibits a relatively indolent manner with frequent local recurrences. Prompt histopathological diagnosis is important to prevent metastases and HMSC-related deaths.

OBJECTIVE: Low-grade papillary Schneiderian carcinoma (LGPSC) is a rare and newly described entity of the sinonasal tract. The aim of this study was to evaluate the clinicopathological and molecular characteristics in order to identify typical features for differential diagnosis.
RESULTS: Of the 3000 cases of sinonasal tumour studied during a period of 6 years, five cases were reviewed and diagnosed as LGPSC. All five patients were female (mean age, 47.8 years; range, 18-64 years) and had undergone multiple surgeries (3-10 surgeries). Both the sinonasal tract and the middle ear were involved in four patients. Nodal metastasis occurred in two patients, and one patient developed a distant metastasis to the left lung. Histologically, tumours had branched and crowded papillae with pushing boundaries. Tumour epithelia were multilayered and arranged in an orderly pattern without cilia. No malignant cytological features were observed in any of the cases. Immunohistochemical findings revealed a scattered distribution of Ki67-positive cells and positive staining for epithelial membrane antigen, mainly in the outermost-layer cells. Human papillomavirus (HPV) DNA was found in two patients and genotyped as HPV type 16. Sanger sequencing did not reveal any epidermal growth factor receptor or Kirsten rat sarcoma viral oncogene homologue gene mutation in the five cases.
CONCLUSIONS: We report on five new cases of LGPSC, and confirm LGPSC as a new sinonasal carcinoma that behaves aggressively with metastatic potential. The combination of clinical behaviour and typical histological features can distinguish LGPSC from sinonasal papilloma and other carcinomas.

BACKGROUND: Solitary fibrous tumour (SFT), as are benign neoplasms of fibroblastic cells. Nasosinusal localisation is exremely rare, difficult to diagnose and to manage.
METHODS: We report a rare case of Solitary fibrous tumour in the nasal cavity in a 47-year-old- woman, with complete surgical resection.
CONCLUSIONS: SFTs are of mesenchymal origin, mainly from serous membranes. The head and neck region is affected with a percentage ranging from 5 to 27%. On the other hand, LTS unusually affects the nasal tract (NTS). Because of this rarity and its variable morphological appearance, it is difficult to distinguish TNS from other mesenchymal lesions.
CONCLUSIONS: Although there are no standard clinical guidelines, the preferred treatment for FLS is radical surgical resection.

OBJECTIVE: This study aimed to reveal diagnosis, management, and treatment outcome characteristics of sinonasal pleomorphic adenoma and to identify predictors for disease recurrence.
METHODS: This study comprised a retrospective review of data from seven patients with sinonasal pleomorphic adenoma who were treated by the authors at a tertiary medical center between January 2005 and May 2017. In addition, the MEDLINE database was searched for literature published in English. A systematic review of the published articles was performed.
RESULTS: A total of 42 articles were eligible for review. Data from the seven treated patients and 101 patients described in the literature were included in the analysis. An endoscopic approach was employed in all cases treated at our institution, and recurrence was found in one patient with a large, 5.5-cm tumor. In reviewing prior publications, the mean age at surgery was 45.4 years, and the primary sign at diagnosis was nasal obstruction. The most common primary tumor site was the nasal septum (57.4%), and the mean tumor size was 3.6cm (range, 0.5-10cm) in 76 cases for which detailed information was available. Endoscopic endonasal resection achieved successful oncologic control in applied cases (25/25, 100%). Recurrence was found in eight of 101 patients. Multivariate analysis identified tumor origin of the paranasal sinus (adjusted OR 31.000, p=0.016) as being significantly associated with a high prevalence of recurrence.
CONCLUSIONS: Pleomorphic adenoma is a rare benign tumor of the sinonasal tract. Surgery that achieves clear margins is the treatment of choice. The tumor may be accessible via an endoscopic endonasal approach in most cases, although incomplete resection of the tumor stem may allow for tumor recurrence. In addition, our findings suggest that a tumor originating from the paranasal sinus is significantly associated with a high prevalence of recurrence.

BACKGROUND: Sinonasal renal cell-like carcinoma (SRCLC) is an extremely rare low malignant tumor arising in the sinonasal tract, with histological mimicry of renal cell carcinoma.
METHODS: We present a case of sinonasal renal cell-like carcinoma in a 63-year-old male patient. Computer tomography(CT) scanning revealed a soft tissue mass at the left nasal cavity and choana. Histologically, the predominant tumor architecture was follicular to glandular with intervening fibrous septa. The tumor cells were uniform cuboidal to polyhedral with abundant clear or eosinophilic cytoplasm. Immunohistochemically, the tumor cells were strongly positive for CK7, EMA, vimentin, SOX10, S-100, and focally positive for CA9. During 6 months of follow-up, there was no clinical or radiological evidence of recurrence or metastasis.
CONCLUSIONS: SRCLC has microscopic features which overlap with tumors that contain clear cells. Thus, several other tumors must be considered in the differential diagnosis of a tumor of the sinonasal region with clear cells, especially metastatic renal clear cell carcinoma. SRCLC is an indolent tumor and none of the reported SRCLC patients had metastatic disease.

Angioleiomyoma (ALM; synonyms: angiomyoma, vascular leiomyoma) is an uncommon benign tumor of skin and subcutaneous tissue. Most arise in the extremities (90 %). Head and neck ALMs are uncommon (~10 % of all ALMs) and those arising beneath the sinonasal tract mucosa are very rare (<1 %) with 38 cases reported so far. We herein analyzed 16 cases identified from our routine and consultation files. Patients included seven females and nine males aged 25-82 years (mean 58; median 62). Symptoms were intermittent nasal obstruction, sinusitis, recurrent epistaxis, and a slow-growing mass. Fifteen lesions originated within different regions of the nasal cavity and one lesion was detected incidentally in an ethmoid sinus sample. Size range was 6-25 mm (mean 11). Histologically, all lesions were well circumscribed but non-encapsulated and most (12/16) were of the compact solid type superficially mimicking conventional leiomyoma but contained numerous compressed muscular veins. The remainder were of venous (2) and cavernous (2) type. Variable amounts of mature fat were observed in four cases (25 %). Atypia, necrosis, and mitotic activity were absent. Immunohistochemistry showed consistent expression of smooth muscle actin (12/12), h-caldesmon (9/9), muscle-specific actin (4/4), variable expression of desmin (11/14) and CD56 (4/6), and absence of HMB45 expression (0/11). The covering mucosa was ulcerated in 6 cases and showed squamous metaplasia in one case. There were no recurrences after local excision. Submucosal sinonasal ALMs are rare benign tumors similar to their reported cutaneous counterparts with frequent adipocytic differentiation. They should be distinguished from renal-type angiomyolipoma. Simple excision is curative.

Eosinophilic angiocentric fibrosis (EAF) is a rare, benign condition of unknown etiology involving the sinonasal tract and the upper respiratory airways, and rarely, larynx, and orbit. We report four cases of EAF identified, in three women and one man, aged 31, 57, 27, and 51 years, respectively. The patients complained of sinonasal obstructive symptoms of long duration, nasal masses, epiphora, and/or proptosis. Histologically, all cases demonstrated a dense fibrotic stroma with a perivascular \"onion-skin\" whorling pattern, and a dense inflammatory infiltrate consisting of lymphocytes, plasma cells, eosinophils, and some neutrophils. In addition, one patient demonstrated modest acute neutrophilic inflammation with focal endothelial proliferation. No patient exhibited clinical or histological evidence of Wegener\'s granulomatosis, granuloma faciale, Kimura\'s disease, and malignant lymphomas. Surgical excision was performed in all cases, and to date, medical therapy has been of limited help. The clinical and histopathological features and differential diagnoses of this underreported EAF condition are discussed.