Lymphoepithelial carcinoma (LEC) is a rare malignancy. Histologically, it is an undifferentiated carcinoma with an intermixed reactive lymphoplasmacytic infiltrate. Herein, we report two cases of LEC in the head and neck region that presented to Oulu University Hospital. Our first case is a 30-year-old man with LEC in the left maxillary sinus. The second case is a 49-year-old man with LEC in the soft palate and uvula with regional lymph node metastases at diagnosis. In addition, a systematic review of the literature from 1980 to 2010 was performed with MEDLINE and cross-references were searched manually. Case reports and clinical series of oral, oropharyngeal, nasal, and paranasal sinus LECs were reviewed revealing a total of 110 cases. Most of the oral cases were found in the tonsils (n = 29), oropharynx (n = 19), and in oral mucosa (n = 18), while sinonasal cases (n = 40) were mainly in the paranasal sinuses and nasal cavity. From 37 case reports, including ours, the median age was 58 and 62 years for sinonasal and oral/oropharyngeal LECs, respectively. Oral and oropharyngeal LECs have a 70.0% tendency to metastasize and 16.6% spread locally. In contrast, none of the nasal and paranasal LECs metastasized, but 60% spread locally. Epstein-Barr virus (EBV) had been detected in 87.5% of all tested LEC cases. Treatment of LECs, during the last decade, has largely consisted of surgery, combined with radiotherapy or chemoradiation. Although local spread or nodal metastases are fairly common at the time of diagnosis, the mortality rate of adequately treated LEC patients is low.

Regional lymph node (LN) metastasis in oral cancer patients is the most significant grave prognostic factor. We evaluated the relationship between clinical outcomes and different histopathological changes in tumor-negative LNs (LN0) selected from neck dissections without metastatic disease (pN0). A total of 435 LN0 selected from pN0 neck dissections (up to three nodes in each level) were scored for histopathological parameters of LN areas, capsule thickness, subcapsular and medullary sinus ectasia, lobular architecture and percent of cortical reactive follicles. These were compared to 328 LN0 selected from neck dissections with metastases (pN+) after exclusion of metastatic LNs. Data were presented by maximum scores of each parameter in I-III (close) and in IV-V (distant) levels. Limited data from level V and regression analyses inferred that the values in level IV represented the worst changes for most patients. Cox proportional hazard regression on each parameter in close and distant levels demonstrated that capsule thickness, number of lobules and percent of reactive follicles were significantly associated with time to death from disease. The higher the change in distant levels, the shorter the time to death, while the higher the change in close levels (given a stable change in distant levels), the longer the time to death. After adjustment for gender, age and location, only the effect of the percent of reactive follicles retained their significant effect. Logistic regression of metastases demonstrated that all parameters except for percent of reactive follicles were significantly associated with risk of metastases, with differences between close and distant levels similar to those found for time to death. After adjustment for gender, age and location, only the area and number of lobes retained their significance. The findings of this study suggested that selective histopathological changes in tumor-negative LNs in metastatic-free patients provide new valuable prognostic parameters.

Background:Aggressive familial giant-cell granulomas of the jaws can be severely deforming. Surgical and nonsurgical treatments may be associated with multiple recurrences. Denosumab, a new generation antiresorptive drug, is an osteoclast inhibitor, which may be particularly useful to manage such potentially disfiguring lesions.Materials and Methods:Two sisters, both with a history of multiple recurrent aggressive central giant-cell granuloma (CGCG)-like lesions in both jaws, were referred for management. All lesions were histologically consistent with the diagnosis of CGCG. The lesions were treated surgically with curettage and perilesional injection of triamcinolone. In particular, the older sister had four separate anatomic sites where some of her lesions had multiple recurrences necessitating three repeat procedures. A course of subcutaneous denosumab was administered following the last giant-cell granuloma removal in both sisters.Results:Bony healing was normal. No further recurrences were observed over 3.5 years of follow-up after denosumab therapy in either sister.Conclusions:In this small cohort comprising two sisters with multiple aggressive recurrent giant-cell granuloma lesions at multiple sites in the mouth, subcutaneous denosumab administration was associated with success over 3.5 years of follow-up. This report cautiously adds to the clinical experience in the use of denosumab for the treatment of recurrent aggressive familial CGCG lesions.