Acinic Cell Carcinoma: An Unusual Cause of Bronchial Obstruction in a Child

Abstract

Primary acinic cell carcinomas of the lung are rare tumors, usually presenting in adulthood as parenchymal or endobronchial masses. These lesions are generally recognized by their morphological pattern and the presence of periodic acid-Schiff (PAS)-positive, diastase-resistant cytoplasmic granules. We describe a case of pri-mary acinic cell carcinoma of the bronchus in a 4-year-old girl. The tumor has the typical acinar structures:weakly PAS-positive, diastase-resistant cytoplasmic granules and intra-acinar laminated calcific structures. A lobectomy was done with a clear bronchial resection margin. The child is well with no evidence of recurrence or metastasis 2 years postresection.

Primary Pulmonary Acinic Cell Carcinoma: A Clinicopathological Study of 6 Cases and Literature Review

“…28 Therefore, weak positivity or negativity of PAS-D stain was observed. 12,19 Immunohistochemical stains are of limited value in evaluating pulmonary ACC and demonstrate an immunoprofile consistent with serous epithelial cells. 19 These neoplasms are usually immunoreactive for cytokeratins and EMA.…”

“…24 The patient age varies widely, and 2 pediatric cases have been reported in the literature. 13,19 Additional 2 pediatric cases were presented in this series. Both patients had a history of bronchial foreign body.…”

“…A thorough literature search was performed on PubMed and Google Scholar, using the key words “pulmonary,” “lung,” “tracheal,” “bronchial,” and “acinic cell carcinoma.” On review of the literature, 19 cases were identified (Table 1). 8-23…”

“…They are commonly small round mass with ill-defined margin, while some cases were encapsulated (6/25). Intratumoral fibrous septa, 12,15,16,19,23 presence of mitotic figure, 9,12,16,19 necrosis, 9,23 and psammoma bodies 23 were infrequent features ( Table 2). Prominent nuclear atypia and perineural invasion were rare and suggested high-grade transformation, metastasis, or recurrence.…”

Surgery for Primary Giant Acinic Cell Carcinoma of Right Lung with Severe Mediastinal Deviation: A Case Report

“…Primary acinic cell carcinoma of the lung is extremely rare, and so far very few cases have been reported worldwide.The pathogenesis of primary acinic cell carcinoma still remains unclear.Florian Haller believes that the pathogenesis of the disease may be related to the upregulation of NR4A3 [5].Primary lung acinic cell carcinoma is a kind of low-grade malignant tumor that usually presents as an isolated tumor adjacent to or close to the bronchus with little lymph node metastasis [6][7].Generally,the surface of tumor is covered with normal tracheal mucosa, it is di cult to get cytological diagnosis by routine bronchoscopy brush examination and biopsy [8].A postoperative pathological examination is critically needed to con rm the diagnosis. Meanwhile,Kazuo Watanabe [9], a Japanese scholar, believes that transbronchoscopic ne-needle aspiration (FNA) may help to obtain samples of tumor tissue to make a de nite pathological diagnosis of the disease preoperatively.Primary lung acinic cell carcinoma can occur from juvenile to old age and it is most common in the 30-75 years-old population (median age 49.5 years), but there are also two cases reported in young girls [10].The largest tumor size of primary acinic cell carcinoma ever reported is 5.2 cm [11], while the size of the tumor in this case is 8.6 cm, which is larger than that.At the same time, it is also rare for the patient in this case have a herniation of compensatory left lung expansion and heart into the right thoracic cavity.The clinical symptoms and signs of this kind of patients are lack of speci city.It often manifested as cough, expectoration, hemoptysis, dyspnea, wheezing.Therefore, primary lung acinic cell carcinoma was often misdiagnosed before operation.In some cases patients have a history of inhaling foreign bodies and the incidence of primary lung acinic cell carcinoma of right lung is higher than left. Thus,some scholars speculate that the occurrence of primary lung acinic cell carcinoma may be related to long-term chronic stimulation of inhaling foreign bodies [12].…”