Sinonasal undifferentiated carcinoma (SNUC) is a rare tumor which is aggressive in nature, has a different clinical course in contrast to sinonasal carcinoma and poor prognosis. Here, we are reporting first case of isolated frontal SNUC which was managed by upfront surgery followed by adjuvant treatment. We want to emphasize the importance of early presentation and timely intervention in form of conservative surgery in this highly aggressive tumor.

Sinonasal undifferentiated carcinoma (SNUC) is an exceedingly rare head and neck malignancy. No consensus exists on treatment for metastatic disease.
A 56-year-old female was diagnosed with SNUC after endorsing sinus congestion, diplopia, and right orbital pain. Initially treated with surgery and radiation, she later developed significant metastatic disease. She demonstrated progression of her hepatic metastases under pembrolizumab therapy. However, the addition of ipilimumab and a COX-2 inhibitor resulted in significant improvement in her lesions as well as an ongoing durable response. Her regimen was complicated by immune-related adverse events successfully treated with steroids.
Dual checkpoint inhibition deserves consideration when treating metastatic SNUC, especially after single agent therapy has failed. The positive effect of this treatment may be augmented by IDO1 inhibition.

Sinonasal undifferentiated carcinoma (SNUC) is a rare tumor highly aggressive most frequently arise in the maxillary sinus and nasal cavity. Oral involvement is extremely rare.
A 62-years-old male presents a large infiltrative mass involving the hard palate and left alveolar ridge. Computed tomography showed bone destruction and invasion of paranasal sinuses and orbits. Histology revealed a malignant neoplasm consisting of small round cells with minimal cytoplasm and hyperchromatic nuclei without any connection with the oral mucosal epithelium. Immunohistochemical analysis showed epithelial origin (CK-7+, CK-20+, AE1/AE3+, EMA+) and lacked strong evidence of squamous and neuroendocrine differentiation (p63-, 34βE12-, NSE-/+, chromogranin-, synaptophysin-). TTF-1 negative ruled out the metastatic origin. A diagnosis of SNUC subtype positive for SMARCB1 (INI1) was reached. The patient was submitted to concurrent radiotherapy and chemotherapy without signs of recurrence after 2 years.
SNUC involving the oral cavity is a rare malignancy that may mimic symptoms of dental infection or sinusitis. A careful correlation of clinical, microscopic, and immunohistochemical characteristics is mandatory for early diagnosis.

Sinonasal undifferentiated carcinoma (SNUC) is a rare malignancy arising from the nasal cavity with neuroendocrine differentiation and aggressive behavior. When first diagnosed, SNUC is often locally advanced with nodal and distant metastases. The present study indicates a rare case of sole metastases to the liver from maxillary sinus undifferentiated carcinoma. Diagnosis was confirmed by total body computed tomography and immunohistochemical analysis on tissues from both maxillary and liver neoplastic tissues. SNUC is an aggressive malignancy characterized by a poor prognosis. Distant metastases commonly involve bones and lungs and should always be suspected in patients with locally advanced stages. Distant metastases can be found in unexpected organs due to dissemination via collateral venous plexuses; isolated liver metastatic disease may also occur. Although SNUC is considered a chemo-sensitive malignancy, chemotherapy for metastatic SNUC needs to be further explored.

UNASSIGNED: SMARCB1-deficient sinonasal tract carcinomas are an emerging subset of rare tumors recently described in the literature, with less than 100 reported cases. Given the aggressive nature of this tumor, timely diagnosis is especially important. We present a case report of a SMARCB1-deficient carcinoma of the sinonasal tract.
UNASSIGNED: Case report with review of the literature.
UNASSIGNED: The patient was a 53-year-old male with computed tomography (CT)-proven mass of the right ethmoid and sphenoid sinuses. Rigid nasal endoscopy revealed a purple mass completely obstructing the right nasal cavity that extended inferiorly from the posterior ethmoids and sphenoid sinuses. Initial biopsy in the emergency room was nondiagnostic due to extensive tumor necrosis. Magnetic resonance imaging (MRI) revealed T2 hypointense enhancing mass centered in the right posterior ethmoids with invasion into the right orbital apex, classifying it as a T4b tumor. The patient underwent repeat biopsy with frozen section and tumor debulking. Immunohistochemical analysis of subsequent biopsy revealed complete loss of INI-1 and negative staining for other pertinent markers, alluding to the diagnosis of SMARCB1-deficient sinonasal tract carcinoma.
UNASSIGNED: Tumor necrosis may be problematic in obtaining a diagnosis for SMARCB1-deficient sinonasal carcinomas. Thus, sampling various regions of the tumor during initial biopsy can prevent delays in diagnosis and treatment.

Sinonasal undifferentiated carcinoma (SNUC) is a rare aggressive neoplasm arising in the nasal cavity and paranasal sinuses. We report a unique case of an 80-year-old man who presented with a locally advanced SNUC involving the ethmoid, sphenoid, and maxillary sinuses and bilateral lymph nodes, clinical T4N2M0. Given his age and the initial extent of his primary tumour, he was treated with neoadjuvant chemotherapy followed by chemoradiation with a split course of 50 Gray (Gy) in 40 fractions delivered twice a day. Four months after his treatments, he developed a recurrence at the left lower eyelid and left frontal sinus, intrabdominal metastases, and a left cerebellar metastasis. A single fraction of 22 Gy was delivered to the cerebellar lesion using stereotactic radiosurgery. He survived 17 months from the initial presentation. We review the available literature regarding treatment of brain metastases and use of hyperfractionated radiotherapy in this rare head and neck cancer.

Sinonasal undifferentiated carcinoma (SNUC) is an aggressive malignancy first described by Frierson et al. in 1986. As the tumor is very rare, current treatment recommendations are based on institutional case reports. We thus felt the need to perform a comprehensive systematic review and meta-analysis to investigate how treatment modalities are associated with survival.
Case-series, systematic review and meta-analysis METHODS: We searched the OvidMedline, OvidEmbase, Web of Science, Biosis, Scopus and the Cochrane Library database libraries. We extracted aggregate and individual patient data for statistical analysis. To study the association between treatment modalities and survival, we used random-effects meta-regression for the aggregate- and cox mixed-effects models.
379 citations were found; 29 case series could be included in the final analysis, including a total number of 390 single patients (34.6% female). Median age at diagnosis was 52 years. 80.9% of patients presented with a T4 tumor and 16.0% with nodal metastasis at diagnosis. In individual patient data (IPD) meta-analysis, single modality (surgery alone or radiation alone) treatment was associated with reduced survival compared to double modality (surgery & radiation or chemoradiation) treatment (adjusted Hazard Ratio [aHR] 2.97, 95% ConfidenceInterval [1.41-6.27]) and compared to triple modality (surgery & radiation & chemotherapy) treatment (aHR 2.80 95%-CI 1.29-6.05 for triple vs. single modality). Triple modality treatment was not superior to double modality treatment. (aHR 1.06, 95%-CI 0.59-1.92).
Double and triple modality treatment are associated with improved survival over single modality but there is no evidence that triple modality is superior to double modality treatment.

Sinonasal undifferentiated carcinoma is an extremely rare malignancy of the paranasal sinuses and nasal cavity. It is of unknown etiology, and occurs more commonly in the elderly men, with a routinely shown aggressive behavior and poor prognosis for survival. Radiographically, it looks like severe osteomyelitis. Histopathologic study is essential to confirm diagnosis, and the undifferentiated histologic appearance often necessitates immunohistochemical studies for differentiation from other high-grade neoplasms. We present an 83-year-old man complaining of pain and unilateral swelling on the left side of the face due to a rare malignant tumor of maxillary sinus origin, a sinonasal undifferentiated carcinoma. He underwent hemimaxillectomy and radiotherapy, but refused chemotherapy. Maxillary sinus malignancy may be presented with unspecific symptoms mimicking sinusitis or dental pain. Coming across such symptoms, the physician or dentist must consider malignancies as well, and carry out medical and dental workups.