BACKGROUND: Acinic cell carcinoma (ACC) is a salivary gland malignancy that rarely can involve the sinonasal cavity. There are limited outcomes data available to guide management.
OBJECTIVE: We sought to use the National Cancer Database (NCDB) to characterize treatment outcomes in sinonasal ACC.
METHODS: A retrospective analysis of the NCDB from 2004 to 2016 for patients with ACC involving the sinonasal cavity was conducted. Demographic, treatment, and survival information were obtained. Unadjusted Kaplan-Meier estimates, log-rank tests, and a multivariable Cox proportional hazard model were used to assess overall survival (OS).
RESULTS: A total of 28 patients were included in the analysis with an average age of 58.6 ± 15.5 years. Half the patients (n = 14, 50%) were male, mostly white (n = 23, 82.1%), and with private insurance (n = 16, 57.1%). The nasal cavity was the most common subsite (n = 18, 64.3%), followed by the maxillary sinus (n = 5, 17.9%). Most patient received surgery alone (n = 17, 60.7%), with the remaining patients undergoing surgery followed by radiation (n = 8, 28.6%), radiation alone (n = 1, 3.6%), and no treatment (n = 2, 7.1%). The 1-, 5-, and 10-year survival in this cohort was 100% (95% CI: 100%-100%), 84.3% (95% CI: 71.2%-99.7%), and 72.2% (95% CI: 55%-94.8%), respectively. On multivariate analysis, older age was associated with worse OS (hazard ratio (HR): 1.27; 95% CI: 1.11-1.46, P < .001). Disease of the sphenoid sinus correlated with worse survival (HR: 198, 95% CI: 10.4-3,739, P < .001) and large tumor size was associated with worse OS on log-rank test, but not on multivariate analysis.
CONCLUSIONS: Sinonasal ACC is a rare entity with relatively good long-term outcomes. Older age and primary disease of the sphenoid sinus are associated with worse outcomes. Most patients are treated with surgical resection. Future research is needed to assess the optimal timing and indications for radiation therapy.

BACKGROUND: Primary lung acinic cell carcinoma is very rare. Here we report a young female patient who suffered the largest primary lung acinic cell carcinoma with severe mediastinal deviation which has never been reported before. We also reviewed data and features of 20 previously reported cases of primary lung acinic cell carcinoma who underwent lobectomy.
METHODS: A 27-year-old female patient presented with recurrent coughing and hemoptysis for more than 10 years came to our hospital. A chest computed tomography (CT) showed a giant space-occupying lesion in the hilum of right lung. After a thorough and detailed preoperative examination, the patient then was performed a radical right pneumonectomy with mediastinal lymph node dissection. The size of the tumor was about 8.6 × 4.5 × 4.4 cm. The pathological results demonstrated a primary acinic cell carcinoma of right lung. The immunohistochemistry of the tumor showed AE1/AE3 (+), Ki-67 (2% +), CK7 (+), Vimentin (+), CK19 (+), α1-ACT (+), AB-PAS (+), S-100 (-), TTF-1 (-). The patient was discharged less than 2 weeks after the operation. So far, the patient has been followed-up for 2 years, and no evidence of tumor recurrence or metastasis was observed.
CONCLUSIONS: The primary acinic cell carcinoma of lung in this case is the biggest one ever reported and also the first case treated with radical right pneumonectomy. In addition, the patient had a very rare condition of severe mediastinal deviation at the same time. After surgical treatment, the patient recovered uneventfully and had stable disease without recurrence and metastasis after 2 years of follow-up. This case together with the reported case indicate that primary acinic cell carcinoma of lung is of low malignancy, the prognosis and therapy effect of surgical treatment are relatively satisfactory.

Acinar cell carcinoma (ACC) of the pancreas is a rare neoplasm with less aggressive behavior than ductal carcinoma. As a result, surgical resection for metastatic ACC is a therapeutic option which can result in long-term survival. There is a paucity of data describing institutional approaches to these challenging patients, and therefore, we herein describe our institution\'s approach to a patient with a distal pancreatic ACC and isolated liver metastasis. The patient underwent neoadjuvant chemotherapy (FOLFIRINOX), followed by a robot-assisted distal pancreatectomy/splenectomy and non-anatomic segment 6 resection. He was discharged to home post-operative day 2. Final pathology revealed complete tumor response of the liver metastasis and a margin negative resection of the primary tumor. He remains disease free and without complications at 3 months. We highlight that combined modality therapy for metastatic ACC can yield long-term survival in selected patients. Similarly, the robotic platform enables performance of complex multivisceral resections with rapid recovery. Future research investigating precision medicine for metastatic ACC is warranted given widely variable tumor biology in this disease.