Epithelial–myoepithelial carcinoma of the maxillofacial and sinonasal region: a systematic review of presenting characteristics, treatment modalities, and associated outcomes

Epithelial–myoepithelial carcinoma (EMC) is a rare salivary gland malignancy. Controversy exists in the literature regarding the effectiveness of treatment modalities employed in the management of EMC. This systematic review was undertaken to understand the presenting characteristics of EMC and identify the most common treatment modalities and their associated outcomes, in order to help guide an evidenced-based approach to the algorithm of care. The MEDLINE (PubMed) and Embase databases were searched (up to February 23, 2022), and the review was performed in accordance with the PRISMA statement. Fifty-seven studies (51 case reports and six case series) describing 91 cases of EMC were included in this review. In the included studies, a slow-growing painless mass was the most common presenting clinical feature. EMC was most frequently treated with surgery alone (65%). Local disease recurrence occurred in 24% of the cases and metastatic disease in 11%. A positive surgical margin was found to be associated with a higher risk of recurrence (P < 0.001), while adjuvant radiotherapy was associated with a decreased risk of local disease recurrence (P = 0.034). Metastatic disease and multimodal therapy were found to be associated with decreased disease-free and overall survival (all P < 0.05). The current literature supports surgery with clear margins as the mainstay of treatment for EMC of the salivary and seromucous glands of the head and neck. In certain situations, radiotherapy may improve disease-free survival

Surgical margins in head and neck cancer: A contemporary review

Adequate resection margins are critical to the treatment decisions and prognosis of patients with head and neck squamous cell carcinoma (HNSCC). However, there are numerous controversies regarding reporting and interpretation of the status of resection margins. Fundamental issues relating to the basic definition of margin adequacy, uniform reporting standards for margins, optimal method of specimen dissection, and the role of intraoperative frozen section evaluation, all require further clarification and standardization. Future horizons for margin surveillance offer the possible use of novel methods such as "molecular margins" and contact microscopic endoscopy, However, the limitations of these approaches need to be understood. The goal of this review was to evaluate these issues to define a more rational, standardized approach for achieving resection margin adequacy for patients with HNSCC undergoing curative resection. (c) 2012 Wiley Periodicals, Inc. Head Neck, 2013

The mutational landscape of adenoid cystic carcinoma

Adenoid cystic carcinomas (ACCs) are among the most enigmatic of human malignancies. These aggressive salivary gland cancers frequently recur and metastasize despite definitive treatment, with no known effective chemotherapy regimen. Here we determined the ACC mutational landscape and report the exome or whole-genome sequences of 60 ACC tumor-normal pairs. These analyses identified a low exonic somatic mutation rate (0.31 non-silent events per megabase) and wide mutational diversity. Notably, we found mutations in genes encoding chromatin-state regulators, such as SMARCA2, CREBBP and KDM6A, suggesting that there is aberrant epigenetic regulation in ACC oncogenesis. Mutations in genes central to the DNA damage response and protein kinase A signaling also implicate these processes. We observed MYB-NFIB translocations and somatic mutations in MYB-associated genes, solidifying the role of these aberrations as critical events in ACC. Lastly, we identified recurrent mutations in the FGF-IGF-PI3K pathway (30% of tumors) that might represent new avenues for therapy. Collectively, our observations establish a molecular foundation for understanding and exploring new treatments for ACC

Hotspot activating PRKD1 somatic mutations in polymorphous low-grade adenocarcinomas of the salivary glands

Polymorphous low-grade adenocarcinoma (PLGA) is the second most frequent type of malignant tumor of the minor salivary glands. We identified PRKD1 hotspot mutations encoding p.Glu710Asp in 72.9% of PLGAs but not in other salivary gland tumors. Functional studies demonstrated that this kinase-activating alteration likely constitutes a driver of PLGA

Correction to: Potentially modifiable factors contributing to outcome from acute respiratory distress syndrome: the LUNG SAFE study

Correction to: Intensive Care Med (2016) 42:1865\u20131876 DOI 10.1007/s00134-016-4571-