Sinonasal mucosal melanoma: Molecular profile and therapeutic implications from a series of 32 cases

BACKGROUND: Primary sinonasal mucosal melanomas are aggressive tumors with a poor clinical control by current treatments, raising the urgent need of novel strategies. METHODS: By fluorescence in situ hybridization (FISH), direct sequencing, and immunohistochemistry, we investigate the spectrum of molecular abnormalities in a cohort of 32 cases of primary sinonasal mucosal melanomas. RESULTS: We found that all primary sinonasal mucosal melanomas lack BRAF V600E mutation; in addition, they are characterized by somatic mutations of NRAS (22%) and KIT (12.5%), together with amplification of RREB1 (100%) and loss of MYB (76%). The large majority of cases showed KIT protein expression (96.9%). Among tumor suppressor genes, primary sinonasal mucosal melanomas showed loss of PTEN (48.1%) and p16/INK4a (55.2%). All tested cases showed expression of pAkt and pErk, suggesting a combined activation of PI3K/Akt and RAS-mitogen-activated protein kinase (MAPK) pathways. CONCLUSIONS: This molecular fingerprint strongly argues against the clinical efficacy of BRAF-inhibitors, but could candidate primary sinonasal mucosal melanomas to therapeutic strategies targeting RAS and KIT mutations or inhibiting PI3K-Akt-mTOR pathway

Palatovaginal (pharyngeal) artery: clinical implication and surgical experience

The palatovaginal or pharyngeal artery is a small branch of the internal maxillary artery supplying the nasopharynx. Bleeding from this artery is exceptional and only one case of traumatic epistaxis from this artery has been reported previously. We report a case of a 66-year-old male presenting with right recurrent posterior epistaxis. Endoscopic dissection of the pterygopalatine fossa and direct visualization of the palatosphenoidal canal permitted to identify the origin of bleeding, and coagulation of the pharyngeal artery solved the epistaxis. Although rare, intractable posterior epistaxis may arise from the pharyngeal artery. The anatomical knowledge of this artery and of the palatosphenoidal canal is of outmost importance in endoscopic transpterygoid and nasopharyngeal procedures, to identify the vidian canal, evaluate nasopharyngeal cancer spread in the pterygopalatine fossa, reduce bleeding during surgery of the nasopharynx, and harvest adequately the pedicle of the nasoseptal flap

Social Perception of Reconstruction following Orbital Exenteration

Background: Orbital exenteration, the removal of the entire globe, eyelids, and orbital content, is indicated in extensive neoplastic disease involving the orbital region. Although a functional reconstruction of orbital exenteration defects is mandatory, aesthetic concerns need to be considered. Facial disfigurement following reconstructive surgery often leads to great discomfort and social retirement, which can limit social interaction. The aim of this study was to explore how the society perceives the aspect of patients who underwent orbital exenteration and subsequent reconstruction, comparing two different types of reconstruction: standard anterolateral thigh (ALT) or "sandwich" fascial ALT (SALT) free flap. Methods: An online survey was created based on four questions regarding the perception of reconstruction (discomfort at looking at that patient, perception of unhealthiness, hypothesis of social life impairment, etc); five possible answers were provided, ranging from "completely" to "not at all." The survey was administered to the general population and to medical students. Results: In total, 255 people participated to the survey (130 medical students and 125 people of the general population); a total of 245 surveys were considered eligible (10 were incomplete and then discharged). Statistical significance was found (P < 0.001) regarding the better overall appearance of the SALT group over the ALT one. Conclusions: After analysis, the surgical outcome after SALT reconstruction has been found to be less disruptive in both groups, due to a reduced scar burden and a more pleasant orbital pocket. Our results encourage more research in the field of postexenteration reconstruction to achieve more aesthetic and social acceptability