UNASSIGNED: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade cancer that was included from the 4th edition of WHO classification of head and neck tumours. The purpose of this study is to analyse clinical behaviour, pattern of recurrences and survival outcomes of this neoplasm.
UNASSIGNED: Retrospective review of patients affected by BSNS who were treated via an endoscopic-assisted approach in 6 European tertiary-care referral hospitals. Cases of BSNS described in literature since 2012 to date were fully reviewed, according to PRISMA guidelines.
UNASSIGNED: A total of 15 patients were included. Seven patients were treated via an endoscopic endonasal approach, 4 with endoscopic transnasal craniectomy, and 4 via a cranio-endoscopic approach. Adjuvant treatment was delivered in 2 cases. After a mean follow-up of 27.3 months, systemic metastasis was observed in 1 case; the 5-year overall survival and disease-free survival rates were 100% and 80 ± 17.9%, respectively.
UNASSIGNED: BSNS is a locally aggressive tumour with a low recurrence rate and encouraging survival outcomes if properly treated with surgical resection and free margins followed by adjuvant radiotherapy for selected cases. Endoscopic-assisted surgery is safe and effective as an upfront treatment within a multidisciplinary care protocol.
Sarcoma bifenotipico nasosinusale: case-series europeo multicentrico e revisione sistematica della letteratura.
UNASSIGNED: Il sarcoma nasosinusale bifenotipico (SNSB) è un raro tumore a basso grado, incluso a partire dalla 4° edizione WHO dei tumori testa-collo. L’obiettivo di questo studio è analizzare i tassi di sopravvivenza e i pattern di recidiva di questa neoplasia.
UNASSIGNED: Revisione retrospettiva dei pazienti affetti da SNSB, trattati mediante approccio endoscopico in 6 centri di riferimento europei. È stata condotta inoltre una revisione sistematica della letteratura dal 2012 ad oggi, secondo le linee guida PRISMA.
UNASSIGNED: Sono stati inclusi 15 pazienti (approccio endoscopico endonasale in 7 casi, craniectomia endoscopica transnasale in 4 casi, approccio combinato transcranico in 4 casi). In 2 casi è stata somministrata radioterapia adiuvante. Dopo un periodo di follow-up medio di 27,3 mesi, è stato riscontrato un caso di metastasi a distanza; i tassi di 5-year Overall Survival e Disease-Free Survival erano 100% e 80 ± 17,9%, rispettivamente.
UNASSIGNED: Il SNSB è un tumore localmente aggressivo con un basso tasso di recidiva e tassi di sopravvivenza incoraggianti se trattato con asportazione chirurgica radicale con radioterapia adiuvante per casi selezionati. La chirurgia endoscopica ha dimostrato di essere sicura ed efficace come trattamento iniziale all’interno di un protocollo di cura multidisciplinare.

BACKGROUND: Inflammatory myofibroblastic tumors are neoplasms that occur infrequently, mainly affects children and young adults. It is an intermediate grade fibrotic multinodular neoplasm.
UNASSIGNED: We present the case of a 47-year-old female patient, who underwent emergency umbilical hernioplasty, later developed intestinal obstruction secondary to an inflammatory myofibroblastic tumor.
CONCLUSIONS: In 1939 Brunn described it for the first time, later in 1954 Umiker named it \"Inflammatory Myofibroblastic Tumor\". The symptoms are nonspecific. In 15 to 40% of patients they are asymptomatic. Cells positive for actin, smooth muscle, vimentin and desmin, in 3367% of cases the cells are positive for ALK, which is present in some malignant lesions. The recommended treatment is radical resection.
CONCLUSIONS: The diagnosis is established by histopathological study, surgery is the cornerstone of treatment.