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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.    Case presentation  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.

Right pneumonectomy for primary large acinic cell carcinoma (AciCC) with severe mediastinal deviation: a case report and literature review