Clinicopathological characteristics and outcome of 31 patients with ETV6-NTRK3 fusion gene confirmed (mammary analogue) secretory carcinoma of salivary glands.

OBJECTIVES: In 2010, a new subtype of salivary gland cancer (SGC), (mammary analogue) secretory carcinoma (SC), was defined, characterized by the ETV6-NTRK3 fusion gene. As clinical behavior and outcome data of this histological subtype tumor are still sparse, we aimed to describe the clinicopathological course and outcome of a series of translocation positive SC patients. PATIENT AND METHODS: We re-evaluated the pathological diagnosis of a subset of SGCs, diagnosed in 4 of 8 Dutch head and neck centers. Subsequently, tumors with a morphological resemblance to SC were tested for the ETV6-NTRK3 fusion gene using RT-PCR. Furthermore, patients prospectively diagnosed with SC were included. The clinical characteristics and outcomes were retrieved from the patient files. RESULTS: Thirty-one patients with ETV6-NTRK3 fusion gene positive SC were included. The median age was 49 years, 17 patients (55%) were male. Eighteen tumors (58%) arose in the parotid gland. One patient presented with lymph node metastasis. All patients underwent tumor resection and 4 patients had a neck dissection. Four patients had re-resection and 15 patients (48%) received postoperative radiotherapy. One patient developed a local recurrence, no regional recurrences or distant metastases were observed. After a median follow-up of 49 months the 5- and 10-year overall survival were 95%, the 5- and 10-year disease free survival were 89%. CONCLUSION: The clinical course of SC is favorable with a low rate of locoregional recurrence and excellent survival. Given the low incidence of nodal metastases, elective neck treatment, i.e. surgery and/or radiotherapy, does not seem to be indicated.

Salivary gland pleomorphic adenoma in the Netherlands: A nationwide observational study of primary tumor incidence, malignant transformation, recurrence, and risk factors for recurrence.

INTRODUCTION: Whereas salivary gland pleomorphic adenoma (SGPA) is the most common type of salivary gland tumor, little is known about its epidemiology because national cancer registries do not register this disease. OBJECTIVES: To establish SGPA incidence trends, rates of secondary malignant transformation and recurrence and associated factors in the Netherlands. MATERIALS AND METHODS: Data on incidence, epidemiology, secondary malignant transformation and recurrence were retrieved from the Dutch pathology registry (PALGA) for the years 1992, 1997, 2002, 2007, and 2012. Multivariate analysis was performed to discover the risk factors for recurrence. RESULTS: 3506 cases of SGPA were recorded implying an overall European standardized rate of 4.2-4.9 per 100,000 person-years. Our figures showed a female preponderance (1:1.43) with an annual 1% rise in female incidence (95% confidence interval [CI]: 0.2-1.8) and a bimodal age distribution in women (p<0.0001). The overall 20-year recurrence rate was 6.7%, and median time to first recurrence was 7years. Positive and uncertain resection margins and younger age at diagnosis were risk factors for recurrence, with odds ratios (ORs) of 4.62 (95%CI 2.84-7.51), 4.08 (95%CI 2.24-7.43), and 0.42 (95%CI 0.29-0.63) respectively. Tumor locations in the minor salivary glands had lower odds of recurrence than tumors in the parotid (OR 0.24; 95% CI: 0.07-0.77; p<0.016). Malignant transformation occurred in 0.15% of SGPAs (3.2% of recurrences). CONCLUSION: This first nationwide study clearly showed sex differences in SGPA epidemiology, possibly suggesting some underlying hormonal mechanism. Long-term recurrence risks were low, and secondary malignant transformation risks were very low.

Maxillary sinus recovery and nasal ventilation after Le Fort I osteotomy: a prospective clinical, endoscopic, functional and radiographic evaluation.

The condition of the maxillary sinus is not routinely assessed before a Le Fort I osteotomy. Performing this procedure in an infected sinus might account for a considerable proportion of the complications, such as excessive bleeding and sinusitis. The aim of this study was to evaluate the maxillary sinus and nasal ventilation after Le Fort I osteotomy. Twenty patients were evaluated before and 2 months after surgery using validated questionnaires for sinonasal complaints (RSOM-31 and VAS score), nasal endoscopy, peak nasal inspiratory flow (PNIF), and a computed tomography (CT) scan. There were no differences in complaints before and 2 months after surgery (P>0.24). Also, the PNIF did not change significantly (P=0.10). On CT evaluation before surgery, a previously unnoted sinusitis was diagnosed in two patients. Postoperatively, a thickened sinus mucosa was present in all patients near the osteotomy line, the osteosyntheses, and around sequesters. This report describes maxillary sinus evaluation after Le Fort I osteotomy in a more comprehensive way by using CT. The Le Fort I procedure did not influence already existing physical or mental complaints, and nasal ventilation was not negatively affected. However, evaluation of sinonasal pathology should be emphasized in the preoperative work-up.

[Insufficient diagnostics in a patient with a fracture of the mandibular collum]. / Ontoereikende diagnostiek bij een fractuur van het collum mandibulae.

A 13-year-old boy was presented to oral and maxillofacial surgeon with chin and preauricular pain after afallfrom his skateboard. A panoramic radiograph did not show any indication of a fracture of the mandibular collum. Two weeks later, the boy was referred by his orthodontist to the department of Oral and Maxillofacial Surgery of a medical centre in connection with a relapse ofa class III malocclusion despite a recently completed treatment of that problem. A computertomogram displayed a fracture of the mandibular collum. In case afracture of the mandibular collum is suspected, imaging from 2 directions is always required.

Patent nasopalatine duct: a diagnostic pitfall.

We describe a patient in whom the combination of excessive air in the maxillary sinus and the presence of a patent nasopalatine duct led to misdiagnosis and subsequent treatment of a non-existent oroantral perforation. When a radiograph indicates that antral involvement during removal of a maxillary molar is unlikely, and yet a routine postoperative nose-blowing test contradicts this, the possible presence of a pre-existent oronasal communication such as a patent nasopalatine duct should be considered.