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Primary acinic cell carcinomas of the lung are rare tumors, usually presenting in adulthood as parenchymal or endobronchial masses. The tumour has also been reported in children.

Visit: Lung Tumour-Online ; Salivary gland-type tumours of Lung.

Histologically, the tumours are composed of clear cells with abundant granular cytoplasm growing as solid sheets with focal acinar, microcystic, and papillocystic areas.  

These lesions are generally recognized by their morphological pattern and the presence of large cells with abundant bright cytoplasm and periodic acid-Schiff (PAS)-positive, diastase-resistant cytoplasmic granules.

Signet cell formation sometimes occurs. 

Electron microscopic examination reveals abundant secretory granules.

Immunohistochemical stains are of limited value in this tumour.

Immunohistochemical stains may show strong positivity of the tumor cells for low-molecular-weight cytokeratins and epithelial membrane antigen (EMA). In some cases focal weak cytoplasmic positivity has been observed with alpha-1-antichymotrypsin and with amylase. Stains for vimentin, S-100 protein, chromogranin, and lysozyme are usually negative.

Because of their relatively indolent behavior and favorable prognosis, primary acinic cell carcinoma of the lung must be distinguished from other primary and metastatic clear cell tumours of the lung.

                 

Acinic cell carcinoma of the lung (“Fechner tumor”): a clinicopathologic, immunohistochemical and ultrastructural study of five cases. Am J Surg Pathol 1992;16:1039–1050.

The clinical, pathological, immunohistochemical, and ultrastructural features in five cases of primary acinic cell carcinoma of the lung are presented. The patients' ages ranged from 44 to 75 years (mean, 56); four were women and one a man. The lesions were discovered incidentally on routine chest x-ray and ranged from 1.2 to 4 cm in greatest diameter. Three tumors were located in the right middle lobe, one in the right upper lobe, and one in the left upper lobe. In three cases, the lesions presented as asymptomatic subpleural nodules in proximity to secondary bronchi, one case presented as an endobronchial tumor that led to obstructive symptoms, and one case as a well-circumscribed deep parenchymal nodule. Histologically, the tumors were composed of clear cells with abundant granular cytoplasm growing as solid sheets with focal acinar, microcystic, and papillocystic areas. Immunohistochemical stains showed strong positivity of the tumor cells for low-molecular-weight cytokeratins and epithelial membrane antigen (EMA). Focal weak cytoplasmic positivity was observed in three cases with alpha-1-antichymotrypsin and in one case with amylase. Stains for vimentin, S-100 protein, chromogranin, and lysozyme were negative in all cases examined. Electron microscopy performed in four cases showed abundant zymogen-type cytoplasmic granules of variable electron density characteristic of acinar-type secretory cells. All patients were treated by lobectomy alone. Follow-up of 3 to 10 years in four cases revealed that all patients were alive and well, with no evidence of recurrence or metastases. Because of their relatively indolent behavior and favorable prognosis, primary acinic cell carcinoma of the lung must be distinguished from other primary and metastatic clear cell tumors of the lung.

Acinic cell carcinoma: an unusual cause of bronchial obstruction in a child. Pediatr Dev Pathol. 2004 Sep-Oct;7(5):521-6.

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 primary 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.

Fine-needle aspiration cytology of bronchial acinic cell carcinoma: a case report. Diagn Cytopathol 2004;30:359–361.

Salivary gland-type carcinomas of the lung are rare but well-known tumors. Among them, acinic cell carcinoma (ACC) is extremely rare and its cytological features have not been reported. We present a case of bronchial ACC and describe its cytological characteristics. The tumor occurred in a 58-yr-old man as a 15-mm polypoid lesion at the right middle lobar bronchus and filled its lumen. Transbronchial brush cytology and a biopsy failed to collect tumor cells but transbronchial fine-needle aspiration (FNA) cytology was successful. The smear obtained was richly cellular and a large number of thick-layered or monolayered sheet-like tumor cell clusters and dissociated tumor cells were observed. Cribriform globular spaces were common and a lobulated acinar structure was found focally. The tumor cells had a fine granular large polygonal cytoplasm and rather uniform round or ovoid nuclei. The nuclei were situated eccentrically or centrally and the nuclear/cytoplasmic ratio was consistently low. These cytological features were essentially similar to those of ACC of the head and neck region. The patient underwent a lobectomy and the tumor was resected completely. Transbronchial FNA cytology was useful for diagnosing bronchial ACC and differentiating it from other conventional and salivary gland type carcinomas.

Primary acinic cell carcinoma of the lung with lymph node metastasis. Arch Pathol Lab Med. 2003 Apr;127(4):e216-9.

We report the case of a 30-year-old woman who presented with a subpleural mass in the lower lobe of the right lung, which was found incidentally on a routine chest radiograph. A wedge biopsy followed by lobectomy and lymph node staging revealed an acinic cell carcinoma with involvement of a hilar lymph node. The subcarinal and mediastinal lymph nodes were free of tumor. Perineural invasion was identified at the periphery of the tumor. There was no evidence of origin from salivary gland or involvement of any other organ. The patient was well 12 months postoperatively. To our knowledge, this is the second case report featuring lymph node metastasis in an acinic cell carcinoma of the lung and the first to demonstrate perineural invasion.

Acinic cell carcinoma of the lung with metastasis to lymph nodes.Chest. 1999 Feb;115(2):591-5.

A 64-year-old man presented with an asymptomatic left lower lobe mass. At bronchoscopy there was a tumor in the superior segment. Biopsy revealed an acinic cell carcinoma. There was no evidence of salivary gland or other site of origin. Lobectomy and lymph node staging showed involvement of interlobar (N1) nodes, while higher stations were benign. The patient remains well 20 months postoperatively. This is the only instance of primary pulmonary acinic cell carcinoma with lymph node metastasis among 15 cases in the literature. We review the clinical features, histology, and treatment of the reported cases.

Acinic cell carcinoma of the trachea. Auris Nasus Larynx 1994;21:193–195.

Acinic cell carcinoma (ACC) appears primarily in the parotid gland. It is extremely rare in the respiratory tract and only a few cases of ACC in the lungs, larynx, nose, and paranasal sinuses have been described. We present here a case of ACC located in the trachea and discuss possible therapeutic strategies in the light of this tumor behavior relating to other sites.

Predominantly mucous-differentiated acinic cell adenocarcinoma of the trachea. Zentralbl Allg Pathol 1989;135:751–756.

Reported in this paper is a predominantly mucous differentiated acinic cell adenocarcinoma of seromucous tracheal glands in a male patient aged 64 years. Local surgical treatment was performed several times within one year and was always followed by recurrence. The naked eye inspection showed a partial polypous appearance of the tumor. Histologically the carcinoma is composed of lobular arranged solid and cribrous cell formations only separated by a sparse fibrous stroma. The tumor is not capsulated, and invasive growth is evident. Large cells with abundant bright cytoplasm and a strongly positive reaction to PAS form the main constituent of the tumor. Signet cell formation sometimes occurs. Electron microscopic examination reveals abundant secretory granules. These cells are in full accordance with mucous cellular elements of adjacent seromucous tracheal glands from which this rare tumor has obviously originated.

Acinic cell carcinoma of the trachea: a case report. Gan No Rinsho 1984;30:1412–1416.

A 27-year-old woman was admitted to our hospital complaining of dyspnea. Bronchoscopic examination revealed an endotracheal mass in the lower trachea. Endoscopic removal of the lesion was done by the electrocoagulation method followed by wide circumferential excision and reconstruction of the trachea three days later. The patient made an uneventful recovery and has had no recurrence in two years of follow-up. The diagnosis of acinic cell carcinoma was made by light microscopy. This is the first description, to our knowledge, of a primary neoplasma of the intrathoracic trachea having the histological appearance of a salivary gland acinic cell tumor.

                       

 

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