Small cell neuroendocrine carcinoma with primary orbital involvement - The unseen, and a review of literature.

Neuroendocrine neoplasms are derived from the epithelial lineages mainly of respiratory tract, with predominant neuroendocrine differentiation. There are only a handful of documented cases of paranasal small cell neuroendocrine carcinomas (SNEC) with primary orbital involvement. Here, the authors describe a 33-year-old male patient with rapidly progressive swelling of the right lower lid with proptosis since 4 weeks. On contrast-MRI orbit, an ill-defined multilobulated mass measuring 3.6 × 3.1 cm with intense homogenous enhancement was seen in the right retrobulbar space involving the right ethmoid sinus. On incisional biopsy, a poorly differentiated mass containing numerous small round blue cells and scanty intervening stroma with prominent necrosis and apoptosis was seen. Immunohistochemistry was strongly positive for synaptophysin. He was diagnosed as a case of SNEC and received chemotherapy, with good response till date of 9 months of follow up. The authors present a literature review and describe challenges in management of a primary orbital SNEC.

SMARCB1-Deficient Sinonasal Carcinoma: A Case Report and Discussion of the Clinical Implications.

OBJECTIVE: 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. METHODS: Case report with review of the literature. RESULTS: 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. CONCLUSION: 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.

Chronic sinonasal tract inflammation as a precursor to nasopharyngeal carcinoma and sinonasal malignancy in the United States.

BACKGROUND: Chronic inflammatory states have been linked to the development of malignancy. Chronic rhinosinusitis (CRS) and allergic rhinitis (AR) have been associated with nasopharyngeal carcinoma (NPC) in population-based studies in Asia. A similar association with NPC and paranasal sinus malignancy (PSM) has not been defined in a North American population. Our purpose was to investigate the impact of CRS and AR on the risk of NPC and PSM. METHODS: The Surveillance, Epidemiology, and End Results (SEER)-Medicare linked database was queried as a case-control study of adults ≥65 years of age. The study cohort included 2009 patients diagnosed with NPC and/or PSM diagnosed between 2003 and 2011, and 2009 propensity-score-matched controls selected from a 5% random sample of Medicare beneficiaries without cancer. CRS and AR were examined as exposures. Multivariable unconditional logistic regression was employed. RESULTS: Overall, NPC and PSM patients were more likely to have previous CRS diagnosis than the controls (9.2% vs 3.0% and 11.1% vs 2.7%, respectively). CRS was associated with greater odds of developing NPC (odds ratio [OR], 3.51; 95% confidence interval [CI], 2.12-5.79) and PSM (OR, 5.30; 95% CI, 3.55-7.92). AR was associated with greater odds of developing NPC (OR, 4.23; 95% CI, 2.96 to 6.06) and PSM (OR, 3.35; 95% CI, 2.49-4.49). The number needed to harm in the exposed population was 311. CONCLUSIONS: CRS and AR are associated with the presence of NPC and PSM in the elderly population of United States. This epidemiologic association will need to be examined for causative pathophysiologic mechanisms and utility in clinical diagnosis.

Staging of Sinonasal and Ventral Skull Base Malignancies.

Tremendous pathologic diversity among sinonasal and ventral skull base malignancies complicates development of a uniform and prognostically relevant staging system. Because of the comparatively low incidence of these tumors, comprehensive evaluation and comparison of specific staging systems is difficult. The current American Joint Committee on Cancer TNM staging system for sinonasal malignancies is the most common and widely used system in current clinical practice. Alternative systems have been proposed for use with individual histopathologic subtypes. Many of these staging systems are of great utility and accurately predict patient survival. Further research and adjustment of these current staging systems remains an important area of research.

Functional outcome in the neurosurgical patient and its impact on quality of life.

Outcomes research has become an integral part of most clinical studies today. Extent of resection, increased median survival, and "time to progression" are no longer the only important end points in need of assessment. Identifying the long-term adverse effects of treatment has become increasingly important as patients try to resume previous activities and an independent lifestyle. The measurement of functional states and health-related quality of life issues are at the forefront of medicine and have become necessary measures of functional outcome following the treatment of a variety of medical and surgical disorders. In this article, we review the most recent studies on the functional outcome of patients undergoing neurological surgeries and its impact on quality of life.