BACKGROUND: Malignancy of the nose and paranasal sinuses is a highly heterogeneous tumor group that arises from various cell types commonly seen in the fifth to sixth decades of life, with twice as much commonness in males. Patients present with varied clinical presentations like nasal obstruction, facial swelling, orbital complications, etc. Squamous cell carcinoma and adenocarcinoma are the most common variant. Surgery followed by adjuvant chemo or radiotherapy is the treatment of choice.
METHODS: The study was undertaken in the Department of Otorhinolaryngology All India Institute of Medical Sciences, Bhopal, India, from 2021 to 2023. It was a retrospective study in which patients diagnosed and underwent treatment in the last 2 years were enrolled. Data were retrieved from the medical record department and surgical registry. Twenty-eight patients were recruited for the study. Detailed history, clinical examination, imaging findings, surgical plans, postoperative adjuvant therapy details, and histopathological findings were recorded.
RESULTS: There were 18 (64.2%) males and 10 (35.8%) females, with a male-to-female ratio of 1.8: 1. The mean age of patients was 50.5 years. Facial swelling was the most frequent symptom (n=15, 54%). Twenty-one (75%) patients use chewable tobacco, while sixteen (57%) are smokers. All our patients belong to the lower socioeconomic group. Endoscopic resection was done in 15 (62.5%) patients, and combined open and endoscopic approaches were used in 9 (37.5%) patients. The most common histological variant was squamous cell carcinoma (n=8, 28%).
CONCLUSIONS: Malignancy of the nose and paranasal sinus is very rare. They presented with varied masked clinical presentations of benign diseases. Early identification and high clinical suspicion, along with imaging studies, are pivotal in managing malignancy of the nose and paranasal sinuses.

Traditional management of olfactory neuroblastoma (ONB) includes margin-negative resection with removal of cribriform plate, dura, and olfactory bulb, regardless of intracranial disease. This approach may be overtreating certain patients. Our investigation examines risk factors associated with occult intracranial disease to optimize therapeutic outcomes.
This retrospective, multi-institutional cohort study examined clinical covariates associated with occult intracranial involvement. Patient demographics, staging, Hyam\'s grade, and pathologic involvement of dura, olfactory bulb/tract, and brain were collected. Diagnostic imaging was reviewed. Positive and negative predictive value (NPV) were estimated along with effect size estimates. Cox hazard regression examined associations with overall survival (OS) and disease-free survival (DFS).
A total of 224 subjects with new diagnoses of ONB (2005-2021) were identified. Skull base bone involvement on computed tomography (CT) had the highest NPV for pathologic dura (88.0%), olfactory bulb (88%), and brain involvement (97.3%). Hyam\'s grade category was significantly associated with dural involvement (φC  = 0.26; 95% confidence interval [CI]: 0.16, 0.42). Subjects without radiologic skull base involvement (n = 66) had pathologic positivity of 12.1%. Within this subgroup, Hyam\'s grade was clinically significant for dural positivity (φ = 0.34; 95% CI: -0.12, 0.71) with 28.6% involvement in high grade tumors. Neither clinical nor pathologic positivity of intracranial structures were associated with significantly different OS or DFS.
Both CT and magnetic resonance imaging (MRI) had reasonably good NPV for involvement of dura and olfactory bulb. Higher Hyam\'s grade was associated with dural involvement. Patients with low-grade tumors not involving the skull base may be suitable for avoiding skull base resection; however, further investigation is warranted.

Lymphoblastic lymphoma is a rare and aggressive form of non-Hodgkin lymphoma (NHL). The tumor can derive from T-cell or B-cell and is clinically similar to acute lymphoblastic leukemia with minimal to no bone marrow involvement distinguishing the two. We present a rare case of lymphoblastic lymphoma originating from the paranasal sinuses. A 40-year-old male presented to the emergency department and was diagnosed with right-sided acute sinusitis complicated by pre-septal cellulitis. After failing medical management, he underwent endoscopic sinus surgery. Pathologic analysis of nasal contents stained for CD79a, CD34, and PAX5, suggesting a B-cell lymphoblastic lymphoma (B-LBL). He was referred to hematology-oncology and was treated with hyperfractionated cyclophosphamide, vincristine, Adriamycin, dexamethasone (Hyper-CVAD). Short-term follow-up has thus far demonstrated near-complete resolution of the tumor. Non-Hodgkin lymphomas of the paranasal sinuses are rare, and B-cell lymphoblastic lymphomas comprise just 0.3% of adults with NHL. Immunohistochemical phenotyping for B-LBL is typically positive for B-cell markers CD19, CD20, CD22 and CD79a. Classic treatment involves a chemotherapy regimen of Hyper-CVAD with an overall survival rate of 66%. B-cell lymphoblastic lymphoma is rarely reported in the paranasal sinuses. A thorough history and physical exam along with a complete workup, including biopsies with histopathological and immunohistochemical analysis, needs to be obtained. Little is known about its prognosis when the primary site is within the paranasal sinuses, and therefore, patients need prompt and aggressive treatment when the diagnosis is made.

Primary ectopic meningiomas of the nasal cavity are rare tumours and thus, often not diagnosed and treated properly. In this case report we are going to discuss about our experience with a primary nasal meningioma involving bilateral nasal cavity with its diagnosis, management and histopathological features. A 28 year old female presented with nasal obstruction and nasal discharge for the past 1 year and swelling over right side of face for the past 8 months. Patient underwent surgical resection by a combined endoscopic and external approach under general anaesthesia. Histopathological evaluation confirmed the diagnosis of Transitional meningioma WHO grade 1. A repeat nasal endoscopy done one week after surgery, showed no evidence of any residual tumour. Due to ectopic meningiomas being relatively less common one must always exclude the presence of a central meningioma, which makes radiological investigations a must in such cases. As primary extra-cranial meningiomas have an unpredictable behaviour, this study should further aid in diagnosis and management of these tumours.

Sinonasal teratocarcinosarcoma is a rare aggressive malignant tumor with a primary setting involving the nasal cavity followed by the ethmoid sinus and maxillary sinus. It accounts for approximately 3% of all head and neck cancers and less than 1% of all tumors. Nasal obstruction, recurrent epistaxis and headache represent the typical clinical presentation. Imaging shows the presence of a mass in the nasal cavity. The treatment usually consists of surgery and adjuvant intensity modulated radiotherapy. The rarity and the variability of the histological features make its diagnosis particularly difficult.
In this paper, we report a case of sinonasal teratocarcinosarcoma in a 62-year-old male treated with a multidisciplinary approach. As an alternative to intensity modulated radiotherapy, we proposed proton beam therapy for the first time. The patient benefited from the new and personalized protocol that provided excellent results and few adverse effects. At 45 months follow-up there is no evidence of relapse and the patient is in good health.

UNASSIGNED: A solitary fibrous tumour (SFT) is a rare neoplasm that commonly arises in the pleura and can occur in other extrathoracic sites. Extrapleural SFT, particularly in the sinonasal cavity, is extremely rare. There are no definite diagnostic criteria for sinonasal SFT as it is rare. Histologic analysis with immunohistochemistry plays an important role in diagnosing SFT.
UNASSIGNED: We report herein a case of SFT of the sinonasal cavity, which later spread to the oral cavity in a 67-year-old male with underlying papillary thyroid carcinoma (PTC) stage IV. He complained of recurrent epistaxis from a mass in his left nasal cavity for two weeks. The mass grew bigger, and spread to the oral cavity, causing dysphagia and upper airway obstruction. Tracheostomy was done under local anaesthesia and a biopsy of the mass was taken to rule out metastasis from the PTC. However, histopathological examination revealed a mesenchymal tumour of fibroblastic type, consistent with an SFT. He was planned for surgical resection of the tumour. However, he refused the operation and was lost to follow-up.
UNASSIGNED: We describe the clinical presentation of this rare tumour of the sinonasal and oral cavity, including upper airway obstruction, and the importance of immunohistochemical markers such as CD34 and BCL-2 in diagnosing SFT. Complete resection of the tumour is the definitive treatment for SFT.
UNASSIGNED: SFT of the sinonasal and oral cavity is extremely rare. Upper airway obstruction may occur due to the location of the tumour in the airway region. Immunohistochemistry is crucial to distinguish this tumour from other mesenchymal tumours.

BACKGROUND: Pretreatment assessment of local extension in sinonasal cancer is essential for prognostic evaluation and surgical planning. The aim of this study was to assess the diagnostic performance of two common imaging techniques (CT and MRI) for the diagnosis of skull base and orbital invasion by comparing imaging findings to histopathological data.
METHODS: This was a retrospective two-center study including patients with sinonasal cancer involving the skull base and/or the orbit operated on between 2000 and 2019. Patients were included only if pre-operative CT and/or MRI, operative and histopathologic reports were available. A double prospective blinded imaging review was conducted according to predefined radiological parameters. Radiologic tumor extension was compared to histopathological reports, which were considered the gold standard. The predictive positive value (PPV) for the diagnosis of skull base/orbital invasion was calculated for each parameter.
RESULTS: A total of 176 patients were included. Ethmoidal intestinal-type adenocarcinoma was the most common type of cancer (41%). The PPV for major modification of the bony skull base was 78% on the CT scan, and 89% on MRI. MRI signs of dural invasion with the highest PPVs were: contact angle over 45° between tumor and dura (86%), irregular deformation of dura adjacent to tumor (87%) and nodular dural enhancement over 2 mm in thickness (87%). Signs of orbital invasion had low PPVs (<50%).
CONCLUSIONS: This retrospective study provides objective data about the diagnostic value of pretreatment imaging in patients with sinonasal cancer.

UNASSIGNED: Sinonasal malignancies (SNMs) are a rare and heterogeneous group of cancers with an incidence of 3%-5% of all head-and-neck tumours. The aim of the present study was to identify the characteristic features of SNMs and to analyze the treatment outcomes and the prognostic factors affecting it.
UNASSIGNED: A retrospective review of patients with SNM treated at a dedicated cancer center of Pakistan between 2004 and 2018 was carried out. All patients with histologically proven diagnosis of malignancy arising from nasal cavity or paranasal sinuses and being treated with curative intent were included. The medical records of 184 cases were utilized for final analysis. Chi-square test was applied to identify significant differences. Five-year disease-specific survival (DSS) was calculated using Kaplan-Meier curve via log-rank test and comparison was made between squamous and nonsquamous histologies. P ≤0.05 was considered statistically significant.
UNASSIGNED: Squamous cell carcinoma was the most common histopathology (n = 62, 33.7%). 130 (70.7%) patients presented with stage IV disease. Nodal metastasis was seen in 24 (13%) patients. Treatment failure was seen in 96 (52.2%) patients and was significantly associated with tumour size and the overall stage. DSS of patients with squamous cell carcinoma was 29% as compared to 52% for patients with nonsquamous histologies (P = 0.001).
UNASSIGNED: SNM is a diverse group of cancers presenting in late stages. A higher rate of treatment failure associated significantly with stage of the disease was identified in our study.

Neuroendocrine tumors (NETs) have a heterogeneous pathology and indolent behavior, with the most common location being the gastrointestinal tract and then the lungs. The head and neck are rare sites of NET presentation with varied clinical signs and symptoms, which occasionally delay the diagnosis, thereby leading to an advanced stage at presentation. We present a rare case of paranasal sinus small cell neuroendocrine tumor and perform a review of the literature. A 71-year-old man presented with continuous bleeding from the left nostril and nasal congestion without any prior medical history. Clinical examination revealed nasal congestion, rhinorrhea, postnasal drip, and active bleeding. The laboratory data were within normal limits. Imaging studies showed a left sphenoid sinus mass extending to the left ethmoid sinus with a break in the cribriform plate encephalocele. An enlarged lymph node measuring 2.2 cm was noted in the left neck and supraclavicular region. The evaluation through stereotactic endoscopic sinus surgery and biopsy revealed left nasopharyngeal, sphenoid sinus, and ethmoid sinus masses. Pathologic biopsy findings were consistent with high-grade, malignant, small, round blue cell tumors. Immunohistochemical analysis confirmed high-grade small cell neuroendocrine carcinoma. The patient was diagnosed with stage IV (TXN2bM0, T: tumor size, N: lymph node, M: metastasis) high-grade neuroendocrine tumor of the left paranasal sinus. He was treated with concurrent chemoradiation therapy with two cycles of etoposide and cisplatin and radiation therapy with proton beam radiation therapy followed by two cycles of adjuvant etoposide cisplatin chemotherapy. The patient showed a good response to the treatment, as confirmed using imaging. He is currently being regularly followed up with serial imaging.

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.