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

Abstract

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.

DOG-1 positive primary acinic cell carcinoma of the lung and investigation of molecular status

“…Similarly, DOG-1 staining in salivary ACC shows intense apical membranous positivity around lumina as well as complete membranous and variable cytoplasmatic positivity 4 . In seven cases of primary ACC of the lung, in which DOG-1 was evaluated, no positivity was detected 2 , 3 . Surprisingly, our case was the first one characterized by DOG-1 positive stain; this finding suggests considering DOG-1 positivity with caution to distinguish between primary from metastatic ACC.…”

“…This entity was described for the first time by Fechner et al in 1972 1 ; since then, only 29 cases have been reported in the English literature. DOG-1 positive stain was described in ACC of the salivary glands and its negativity was proposed to distinguish between primary and metastatic ACC of the lung 2 , 3 . Here, we report a new case of primary ACC of the lung, evaluating the utility of DOG-1 in diagnosis and describing the molecular status of the neoplasm.…”

Primary acinic cell carcinoma of the trachea: A case report and literature review

“…[5] To the best of our knowledge, including the present case, only 32 patients with primary ACC of the lung or trachea have been reported so far. [5–26] Clinicopathological features of the patients are summarized in Table 1. The most common symptoms were hemoptysis and coughing, but 1-third (11/32) of the patients were asymptomatic and the tumor was identified incidentally.…”

“…The most common symptoms were hemoptysis and coughing, but 1-third (11/32) of the patients were asymptomatic and the tumor was identified incidentally. [5–26] Of the patients with primary ACC of the lungs, 14 were female and 17 were male. The patient age varied widely between 4 to 75 years (mean age: 43.4 years).…”

“…Symptoms Yr Sex Age Size (cm) Site Therapy Outcome 1 [5] Asymptomatic 1972 M 63 4.2 RLL S A, 22 mo 2 [6] Recurrent pulmonary infections 1976 F 12 0.8 RML, bronchus intermedius S A, 1 yr 3 [7] Three-year history of hemoptysis 1982 M 54 2.2 Trachea S A, 2 yr 4 [8] Persistent cough 1985 M 36 4 RML Unknown Unknown 5 [9] Incidental 1992 F 44 3.5 RML, subpleural S A, 10 yr 6 [9] Incidental 1992 F 48 4 LUL, parenchymal S LF 7 [9] Incidental 1992 F 50 1.7 RML, subpleural S A, 3 yr 8 [9] Persistent cough 1992 M 63 1.2 RML, endobronchial S A, 4 yr 9 [9] Incidental 1992 F 75 1.5 RUL, subpleural S A, 4 yr 10 [10] Dyspnea, cough, stridor 1994 M 31 2 Trachea S A, 8 yr 11 [11] Hemoptysis 1996 M 59 3 Trachea S ND 12 [12] Incidental 1999 M 64 3 LLL, endobronchial S, radiation A, 1.5 yr 13 [13] Incidental 2003 F 30 2 RLL, subpleural S R, 20 mo 14 [14] Incidental 2003 M 70 4 RLL, intraparenchymal S ND 15 [15] Hemoptysis and night sweats 2004 F 4 3 LUL, endobronchial S ND 16 [16] Hoarseness 2004 M 54 5 Trachea S A, 23 mo 17 [17] Persistent cough 2004 M 58 1.5 RML, endobronchial S A, 4 mo 18 [18] Incidental 2005 F 37 3.3 RUL S ND 19 [19] Incidental 2006 M 47 1.8 LLL, endobronchial S A, 1 yr 20 [20] Asymptomatic 2010 M 68 1.8 LLL S, chemotherapy ND 21 [21] Incidental 2011 M 63 5.2 RUL S A 22 [22] Persistent cough 2011 F 55 1.3 RUL S, chemotherapy A, 3 yr 23 [23] Persistent cough 2017 M 31 4.5 RLL, subpleural S ND 24 [24] Persistent cough, hemoptysis 2019 F 10 1.5 LUL S A, 30 mo 25 [24] Persistent cough, chest pain 2019 M 25 2.2 RUL S A, 48 mo 26 [24] Hemoptysis 2019 M 37 1 RLL S A, 8 mo 27 [24] Persistent cough, hemoptysis 2019 F 8 0.4 LML S A, 33 mo 28 [24] Persistent cough, hemoptysis 2019 M 28 1 RML S A, 31 mo 29 [24] Hemoptysis 2019 F 53 1.5 RML S A, 28 mo 30 [25] Persistent cough, hemoptysis 2021 F 27 8.6 RUL, endobronchial S A, 24 mo 31 [26] Persistent…”