Malignant Sinonasal Tumors: Update on Histological and Clinical Management

Abstract

Tumors of nasal cavity and paranasal sinuses (TuNSs) are rare and heterogeneous malignancies, presenting different histological features and clinical behavior. We reviewed the literature about etiology, biology, and clinical features of TuNSs to define pathologic features and possible treatment strategies. From a diagnostic point of view, it is mandatory to have high expertise and perform an immunohistochemical assessment to distinguish between different histotypes. Due to the extreme rarity of these neoplasms, there are no standard and evidence-based therapeutic strategies, lacking prospective and large clinical trials. In fact, most studies are retrospective analyses. Surgery represents the mainstay of treatment of TuNSs for small and localized tumors allowing complete tumor removal. Locally advanced lesions require more demolitive surgery that should be always followed by adjuvant radio- or chemo-radiotherapy. Recurrent/metastatic disease requires palliative chemo- and/or radiotherapy. Many studies emphasize the role of specific genes mutations in the development of TuNSs like mutations in the exons 4–9 of the TP53 gene, in the exon 9 of the PIK3CA gene and in the promoter of the TERT gene. In the near future, this genetic assessment will have new therapeutic implications. Future improvements in the understanding of the etiology, biology, and clinical features of TuNSs are warranted to improve their management.

Quality of life in sinonasal tumors: an up-to-date review

“…Orbital infiltration results in ocular complications both at presentation and throughout the course of treatment. Direct tumor extension through the lamina papyracea may present as visual acuity disturbances, diplopia, ocular pain, proptosis, and eyelid fullness [4,30,31,[39][40][41]. Soft tissue infiltration requires orbital exenteration.…”

“…Visual complications due to surgery involve diplopia, globe malposition, enophthalmos, chronic epiphora, iterative dacryocystitis and decrease in visual acuity [31]. Postoperative radiation could lead to keratopathy, visual field defect and visual acuity disturbances [4,[39][40][41]. Sinonasal tumours involving the orbit are common [39,42], however it is vital for physicians to be aware and prepared for such cases due to the difficulty of access and the aggressiveness of these tumours to offer the appropriate therapy and multidisciplinary team to minimise complications and optimise patient's QOL [39,41,43,44].…”

“…Sinonasal tumors have a complex morbidity profile. Their predisposition to recur, their local destructive capability along with the potential of extension into vital adjacent structures, such as cerebellum, pituitary gland, cranial nerves, carotid and vertebral arteries, spinal cord and craniofacial skeleton may lead to symptomatology that affects severely one's physical and mental functioning in varying ways [4,6,30,31].…”

“…Symptoms are similar to those of inflammatory sinonasal diseases and thus diagnosis may delay significantly. Poor prognosis with approximately 50-60% 5-year survival rate is mostly attributed to the advanced stage at presentation [1,2,4].…”

Metabolic Tumour Volume as a Predictor of Survival for Sinonasal Tract Squamous Cell Carcinoma

“…Squamous cell carcinoma (SCC) in the sinonasal tract is definitively treated by surgery or radiotherapy with or without chemotherapy [ 1 ]. In the initial staging of multiple organ cancer, volumetric fluorine-18-fluorodeoxyglucose uptake (F18-FDG uptake) on positron emission tomography with computed tomography (PET/CT) is acknowledged as a survival parameter [ 2 , 3 , 4 , 5 ].…”