BACKGROUND: Intestinal adenocarcinoma accounts for less than 0.1-4% of all malignancies in the region. It is common among woodworkers and leather workers. Sinonasal adenocarcinoma usually arises from the ethmoid sinus (40%) or nasal cavity (25%). Extension to nearby structures is common, but intracranial spread is very rare. These tumors are usually treated with surgery, with a reported 5-year survival rate of 59% to 80%.
METHODS: This is a 60-year-old Black African male patient who presented with globalized headache, nasal obstruction with snoring during sleep, anosmia, change in mentation, sometimes agitation and left-side visual loss of one-year duration with worsening his above symptoms over the last one month. He couldn\'t smell soap bilaterally; in his left eye he could see only hand movement at nearly 30 cm. On brain magnetic resonance imaging, there was a T1 hypo- and T2 hyper-intense anterior cranial fossa mass arising from the left ethmoid sinuses and sphenoid sinuses and compressing the left optic structures, and brain computed tomography demonstrated heterogeneous hypo- to isodense mass. Complete tumor excision achieved and discharged with significant improvement and linked to oncology unit for radiotherapy.
CONCLUSIONS: The management of these patients is multidisciplinary, involving neurosurgeons, otolaryngologists, oncologists, and maxillofacial surgeons. Surgical resection is the main treatment strategy, followed by radiotherapy, particularly intensity-modulated therapy. Chemotherapy is used in highly advanced, metastatic, and unresectable tumors.

UNASSIGNED: Epithelial-myoepithelial carcinoma (EMC) was recognised as a distinct pathologic entity in World Health Organisation classification. It is an extremely rare low grade carcinoma of salivary gland, with characteristic biphasic tubular structures. It predominantly occurs in Parotid gland but can also be seen in nasopharynx, lacrimal gland, paranasal sinuses, larynx, lung. Nasal EMC (excluding the paranasal sinuses as primary tumour site) are very rare with only 13 cases reported till date. In this case report, we described a case of nasal EMC extending into nasopharynx, its clinical features and management. We have also done a literature review of all the relevant cases of nasal EMC.
UNASSIGNED: We searched the PubMed database for articles between January 1950 and December 2022 for nasal EMC for this review.
UNASSIGNED: We found 13 relevant case reports of nasal EMC and median age was 58 years with female preponderance. We found that our patient was the youngest to be reported till date. Two cases, including the current study, showed epicentre of the tumour in posterior nasal cavity, extending to choana and nasopharynx. Most common presentation was epistaxis, followed by nasal obstruction. Only 4 out of 14 cases had information on surgical margin status, out of which only one has positive surgical margin. Five patients (including the patient in the current study) received adjuvant radiotherapy; however 6 patients (42.8%) did not receive any adjuvant radiotherapy.

BACKGROUND: Sinonasal NUT carcinoma is an extremely rare, lethal malignancy with limited literature.
METHODS: A case series was conduction of all patients with sinonasal NUT carcinoma at a single institution between 2010 and 2022. Survival and associated were evaluated. A systematic review of the literature was performed.
RESULTS: In 12 patients, followed for a median of 1.5 years, the median overall survival (OS) and disease-specific survival (DSS) were both 14.6 months. Patients with maxillary sinus tumors were 91% more likely to survive (hazard ratio [HR]: 0.094, 95% confidence interval [CI]: 0.011-0.78, p = 0.011). Patients with higher-stage disease stage had worse OS (stage IVb-c vs. III-IVa, p = 0.05). All three patients who were alive with no evidence of disease received induction chemotherapy.
CONCLUSIONS: For patients with sinonasal NUT carcinoma, the median survival was 15 months but better with lower-stage and maxillary tumors. Induction chemotherapy may be beneficial.

UNASSIGNED: Carcinosarcomas are rare, aggressive malignancies that can arise in the nasal cavity and paranasal sinuses. There are limited outcome data available. Accordingly, we sought to use the National Cancer Database (NCDB) to characterize patient demographics and outcomes.
UNASSIGNED: A retrospective analysis of the NCDB from 2004 to 2016 for patients with sinonasal carcinosarcoma was conducted.
UNASSIGNED: Thirty patients were included. The patients were predominantly male (n = 20), white (n = 23), and privately insured (n = 15), with an average age of 62.4 years. The nasal cavity was the most common subsite (n = 14), followed by the maxillary sinus (n = 8). Most patient were treated with surgery followed by radiation (n = 23), with the remaining undergoing surgery alone (n = 4), radiation alone (n = 2), or no treatment (n = 1). One-third (n = 10) received adjuvant chemotherapy. The 1- and 5-year overall survival (OS) in the cohort were 79.2% and 43.3%, respectively. Univariate log-rank testing showed OS varied based on intervention (P < 0.029), sex (P < 0.042), and age (P < 0.025), while on multivariate analysis none of these factors independently predicted OS.
UNASSIGNED: We describe the demographics and presenting features of a national cohort of sinonasal carcinosarcoma patients. Future research is needed to identify predictors of overall survival, and to assess the optimal roles for radiation and systemic chemotherapy.

Amyloidosis is the process of extracellular deposition of protein fibrils and manifests pathologically as a systemic or localized process. Localized amyloidosis of the head and neck is uncommon, and involvement of the sphenoid sinus is exceedingly rare. We describe a case of localized amyloidosis isolated from the sphenoid sinus. A descriptive literature search was conducted to highlight presentation, management, and outcomes related to this pathology. Our patient was a 65-year-old male who presented to our clinic with nasal congestion and an incidental finding of a large expansile mass within the sphenoid sinuses. The mass was seen to displace the pituitary gland, and thus a multidisciplinary care approach ensued. The mass was removed via a transnasal endoscopic approach. Pathology revealed fibrocollagenous tissue with calcifications that were positive on Congo red staining. The patient underwent further workup to rule out systemic involvement, which was unremarkable. Based on the findings of his workup, he was ultimately diagnosed with localized amyloidosis. A comprehensive review of the literature revealed 25 other reported cases of localized amyloidosis within the sinonasal region, with only one other case of isolated sphenoid sinus disease. Common presenting symptoms are nonspecific and may mimic other, more frequently seen regional pathologies, including nasal obstruction, rhinorrhea, and epistaxis. The treatment for localized disease is surgical resection. While localized amyloidosis within the sinonasal region is rare, it is important to recognize, work up, and treat it appropriately. A multidisciplinary team approach is necessary for appropriate diagnosis and management, and these patients should be followed closely after treatment.

Sinonasal adenoid cystic carcinomas (SNACC) have high propensity for skull base (SB) infiltration. Unresectability or incomplete surgical resection in such cases make radiotherapy treatment paramount. Curative dose escalation is challenging because of adjacent organs at risk, especially in locally advanced cases.
Eighteen patients that had locally advanced SB SNACC with unresectable or incomplete surgical resection treated by proton therapy and/or helical tomotherapy at Institut Curie between 3/2010 and 8/2020 were retrospectively included.
After median follow-up of 52 months, 5-year OS, LRRFS, DMFS, DFS rates were, respectively, 47% (95%CI: 26-83), 50% (95%CI: 36-88), 39% (95%CI: 26-81), 33% (95%CI: 22-73). One patient had grade 4 late optic nerve disorder. Eight patients had grade 3 late toxicity including mainly hearing impairments.
Proton therapy and helical tomotherapy are effective and safe methods for curative dose escalation of locally advanced SB SNACC, which are a poor prognosis subgroup. Available literature suggests carbon-ion therapy could be an efficient alternative.

BACKGROUND: Leiomyomas are benign smooth muscle tumors that are rarely diagnosed in the nasal cavity and paranasal sinuses.
OBJECTIVE: This systematic review summarizes the histopathologic and clinical tumor characteristics, surgical management, and follow-up of sinonasal leiomyomas.
METHODS: A systematic review of the literature on sinonasal leiomyoma was performed by applying the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Studies that met the inclusion criteria were assessed for level of evidence. Patient demographics, clinical and pathological tumor characteristics, primary intervention, and results of follow-up were evaluated.
RESULTS: Forty studies including 84 patients with sinonasal leiomyoma were identified. The tumor was most often located in the nasal cavity (47/84, 56%) originating from the inferior turbinate (32/84, 38%). Patients mostly presented with symptoms originating from an intranasal mass, including recurrent epistaxis (41/84, 49%), nasal obstruction (43/84, 51.2%), and localized facial or head pain (25/84, 29.8%). Surgery was performed in all cases. An endoscopic approach was most frequently chosen. Recurrence occurred only twice (2.4%). Morbidity was noted in 2 cases (2.4%) following postoperative bleeding and 1 (1.2%) case following a CSF leak.
CONCLUSIONS: Sinonasal leiomyomas are neoplasms of the smooth muscle manifesting clinically with recurrent epistaxis and nasal obstruction. Management goal is total resection with clear margins to avoid local recurrence.

In this paper, we present a rare case of tumor-induced osteomalacia (TIO) and a literature review of this rare disease.
A case of TIO of the isolated sphenoid sinus was reported. Furthermore, the clinical features of TIO in the sphenoid sinus and other sinonasal sinuses were also reviewed and summarized.
A 35-year-old man with muscle weakness and lower back pain came to the Department of Neurology. No obvious neurological disease was found; however, magnetic resonance imaging of the extremities accidentally showed a tumor in the axilla. Bone scintigraphy showed suspicious bone metastasis. Hypophosphatemia was neglected. Interestingly, 2-deoxy-2-[fluorine-18]fluoro-d-glucose positron emission tomography/computed tomography (18F-FDG PET/CT) detected a tumor in the axilla and another in the sphenoid sinus, but only the tumor in the sphenoid sinus had somatostatin receptor (SSTR) expression in 68-gallium 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid octreotate (Ga-68 DOTATATE) PET/CT. The sphenoid sinus tumor was proven to be a phosphaturic mesenchymal tumor (PMT), and the phosphate levels returned to normal after surgery. The literature review showed only 17 cases of TIOs that occurred in the sphenoid sinus, with an average age of 43.3 ± 13.7 years. Only three cases of TIOs in the sphenoid sinus did not invade the nasal cavity or other paranasal sinuses, which could be identified as isolated sphenoid sinus diseases. We compared the clinical features of sphenoid TIOs with those of non-sphenoid sinonasal TIOs, and it was found that the concentration of 1,25-dihydroxy vitamin D in the group with sphenoid TIOs was much higher than that in the group with non-sphenoid sinonasal TIOs. A total of 153 cases of TIOs in the sinonasal sinus were reviewed. The ethmoid sinus was found to be the major site (64.7%), followed by the nasal cavity (50.3%), maxillary sinus (19.0%), frontal sinus (16.4%), and sphenoid sinus (11.8%). There were 66 patients (43.1%) who showed tumors invading more than one sinus. Most of the tumors (69.3%) were diagnosed as PMTs by pathology, followed by hemangiopericytoma (14.3%). Immunostaining was beneficial in the differential diagnosis of these tumors; however, larger sample sizes are needed for better accuracy.
TIO in the sinonasal sinus, especially in the sphenoid sinus, is rare. Moreover, isolated sphenoid sinus disease can be easily misdiagnosed. When the clinical manifestation of osteomalacia is atypical, associating it with sphenoid sinus disease is even more difficult. Thus, TIO in the sphenoid sinus needs further exploration.

UNASSIGNED: Biphenotypic sinonasal sarcoma is a rare malignant tumour exclusively involving the sinonasal cavity. These tumours have variable and atypical presentations. Early approach and correct treatment modalities are key factors in the management of such cases.
UNASSIGNED: A 48-year-old male patient presented with left-sided nasal obstruction and intermittent nasal bleeding for one year.
UNASSIGNED: Biphenotypic sinonasal sarcoma confirmed on histopathological examination and immunohistochemistry.
UNASSIGNED: The patient underwent surgical excision with left lateral rhinotomy and bifrontal craniotomy with skull base repair. The patient also received postoperative radiotherapy.
UNASSIGNED: The patient is on regular follow-up with no similar complaints.
UNASSIGNED: Treating team should keep the diagnosis of biphenotypic sinonasal sarcoma in mind while investigating a patient with nasal mass. Surgical management is the treatment of choice, due to its local aggressive nature and proximity to the brain and eyes. Postoperative radiotherapy is vital to prevent tumour recurrence.

Wiskott-Aldrich syndrome (WAS) is a rare primary immunodeficiency disease with a predisposition towards autoimmunity and lymphoproliferative diseases. Non-Hodgkin lymphoma (NHL) is reported to be the predominant form of malignant tumor in WAS sufferers. Diffuse large B-cell lymphoma (DLBCL) is one of the most common types of NHL while it is uncommon to occur in paranasal sinuses and especially when associated with WAS. In this article, we report a unique case of WAS associated with DLBCL in paranasal sinuses and review the major publications of WAS-related lymphomas that occurred in the head and neck area. This study extends the available therapies for WAS-related lymphomas and emphasizes the significance of recognition for sinonasal lymphomas in WAS patients presenting with sinusitis.