Adenoid cystic carcinoma of the lower respiratory tract is an uncommon tumor that can arise in the mainstem bronchus and often presents as an endobronchial mass lesion causing bronchial obstruction with post obstructive atelectasis and pneumonia. Exfoliative cytology is seldom useful in the diagnosis of primary bronchial adenoid cystic carcinoma, because these neoplasms usually have a submucosal location with often intact mucosa. Since most endobronchial adenoid cystic carcinomas are endoscopically visible, bronchoscope-guided fine-needle aspiration constitutes an excellent approach to establish a pathologic diagnosis. The fine-needle aspiration cytology of primary pulmonary adenoid cystic carcinoma has been rarely described. We report a case of primary adenoid cystic carcinoma of the lung having characteristic cytologic features and correlate with computed tomography, bronchoscopic, and histological findings. Bronchoscope-guided aspiration cytology provided a conclusive diagnosis of adenoid cystic carcinoma, which was further corroborated by histology in the pneumonectomy specimen.

Adenoid cystic carcinoma of lower lobe of right lung: report of a case. Kyobu Geka. 2003 Oct;56(11):977-80.

A 69-year-old male was admitted to the hospital for further examination of an abnormal shadow in the right lower lung fields. He was previously under medical treatment for right thoracic empyema. Chest computed tomography (CT) showed a solitary mass, 4.5 cm in diameter and broncofiberscopy evidenced a tumor in the right lower bronchus. The biopsy was performed and the tumor was diagnosed as a pleomorphic adenoma. Intraoperativefinding showed the tumor was 6 cm in gross, extended to the left atrium, and a daughter tumor was palpable in the middle lobe. The middle and lower lobe were resected. The tumor was located in S9, S10, 6 x 4 x 3.5 cm in size, 2 daughter tumor was found in the middle lobe, the pulmonary vein was thickened by tumor invasion. Pathohistologically, main tumor and daughter tumor showed malignant feature, were compatible with adenoid cystic cancer. Four years after operation, he is still now alive with home oxygen therapy.

Primary peripheral adenoid cystic carcinoma of the lung. A case report. Acta Chir Belg. 2003 Aug;103(4):414-5.

We report a rare case of peripheral adenoid cystic carcinoma of the lung, showing unusual pathological and clinical features, namely rapid growth, local aggressive behaviour, huge tumour size, no endobronchial component or submucosal infiltration, and a rapidly progressive clinical course. Extensive surgery resulted in considerable palliation of symptoms, but not in prolonged survival. The reported case emphasizes the malignant potential of peripheral adenoid cystic carcinoma of the lung.

Treatment outcome of resected and nonresected primary adenoid cystic carcinoma of the lung.Ann Thorac Cardiovasc Surg. 2002 Apr;8(2):74-7.

BACKGROUND AND OBJECTIVE: The incidence of primary adenoid cystic carcinoma of the lung is relatively rare and the optimal treatment strategy is still unclear. METHODS: Sixteen adenoid cystic carcinoma patients were treated at our institute from 1972 to 1998 and their clinical features, treatments and survivals were reviewed. RESULTS: Half of all patients were female and the median age was 46 ranging from 30 to 64. All primary lesions were located in the central bronchial tree and 80% of the patients had some symptoms. Eleven patients underwent a resection of the tumor with/without plasty of the trachea or bronchus. Although 6 (55%) of 11 patients had a microscopic residual tumor after resection, 5 patients who received postoperative radiotherapy survived without recurrence from 3 to 17 years. Five patients received radiotherapy as their initial treatment and all tumors responded well to the treatment. The 5-year and 10-year survival rates were 91 and 76% without local recurrence in the resected group and 40 and 0% in the nonresected group, respectively. CONCLUSION: These observations suggest that surgical resection should be selected first whenever possible, and, in addition, adenoid cystic carcinoma is sensitive to radiotherapy.

A case of adenoid cystic carcinoma of the left main bronchus, which was performed carinal resection and reconstruction while the aortic arch is pulled down. Kyobu Geka. 2001 Jan;54(1):31-5.

A 55-year-old woman, who had treated as bronchial asthma for eight months, was admitted to our hospital. Chest CT scan showed a mass in the left main bronchus. Bronchoscopic examination revealed a tumor obstructing the left main bronchus and invading the left lower portion of the trachea. The pathologic diagnosis was adenoid cystic carcinoma. Primary resection of the left main bronchus and the carina with the objective and one-stoma-type carinal reconstruction were performed by the left thoracotomy. We pulled down the aortic arch to obtain an operative field, and carried out the operative procedure at the upper side of the aortic arch. As the resection margin contained residual tumor, the postoperative irradiation (50 Gy) was added.

Primary adenoid cystic carcinoma in the lung: report of two cases and therapeutic considerations. Lung Cancer. 1998 Mar;19(3):211-7.

Primary adenoid cystic carcinoma in the lung is an uncommon disease, which is regarded as a slow growing low-grade malignancy. However, this disease has a high risk of incomplete resection because of its unique histological invasion patterns. The cases of two patients who recently underwent surgery for adenoid cystic carcinoma in the lung are reported. Both patients received postoperative radiotherapy treatment as a follow-up to a histologically incomplete resection. Both patients have survived for 51 months and 7 months, respectively, with no recurrence. Postoperative radiotherapy is an acceptable treatment of choice to control residual lesions and provides long-term survival even in cases of incomplete resection.

Adenoid cystic carcinoma arising in the intrapulmonary bronchus. Intern Med. 1996 May;35(5):407-9.

A 63-year-old male was admitted to our hospital because of a coin lesion in the intrapulmonary bronchus. The patient had no symptoms such as cough or sputum. Imaging studies showed that the tumor was present in the S10b,c of the right lung. There was no metastasis or salivary gland tumor. Transbronchial tumor biopsy revealed a primary adenoid cystic carcinoma. The patient underwent a lobectomy, and has been well since the operation. This is an uncommon case of primary adenoid cystic carcinoma arising from the intrapulmonary bronchus.

Adenoid cystic carcinoma of the airway: thirty-two-year experience.
J Thorac Cardiovasc Surg. 1996 Dec;112(6):1522-31; discussion 1531-2.

METHODS: We have reviewed our experience in 38 patients with adenoid cystic carcinoma of the upper airway seen between 1963 and 1995. The mean age was 44.8 years (15 to 80 years) with a male/female ratio of 1:1.1. Thirty-two of the 38 patients were treated by resection and reconstruction (primary anastomosis 28; Marlex mesh prosthesis 4). Twenty-six of the 32 patients undergoing resection received adjuvant radiotherapy. Six patients with unresectable tumors were treated primarily with radiotherapy only. RESULTS: Pathologic examination revealed local invasion beyond the wall of the trachea in all patients. In a majority, microscopic extension was found in submucosal and perineural lymphatics, well beyond the grossly visible or palpable limits of the tumor. Lymphatic metastases were relatively uncommon, occurring in only five of 32 (19%) patients undergoing resection. Metachronous hematogenous metastases occurred in 17 of 38 patients (44%). Thirteen of these 38 patients (33%) had pulmonary metastases. Sixteen of 32 resections were complete and potentially curative. There were two deaths within 30 days of operation. The mean survival in the 14 patients undergoing complete resection was 9.8 years (12 months to 29 years). Sixteen of 32 resections were incomplete (residual tumor at the airway margin on final pathologic examination), with one operative death occurring in this group. The mean survival in the 15 surviving patients was 7.5 years (4 months to 21 years). Six patients were treated with primary radiation only and had a mean survival of 6.2 years (2 months to 14.3 years). In the patients with pulmonary metastases, mean survival was 37 months (4 months to 7 years) from the time of diagnosis of the pulmonary metastasis until their death. CONCLUSION: Adenoid cystic carcinoma of the upper airway is a rare tumor, which is locally invasive and frequently amenable to resection. Although late local recurrence after resection is a feature of this tumor (up to 29 years), excellent long-term palliation is commonly achieved after both complete and incomplete resection. There was a small difference in survival between patients having complete and incomplete resection. Long periods of control can be obtained with radiotherapy alone. The best results, in this series of patients, were obtained by resection. Adjuvant radiotherapy is assumed to favorably influence survival.

Primary adenoid cystic carcinoma of the lung. A clinicopathologic and immunohistochemical study of 16 cases. Cancer. 1994 Mar 1;73(5):1390-7.

BACKGROUND. Adenoid cystic carcinoma (ACC) is a rare but distinctive salivary gland-type malignant neoplasm that arises infrequently as a primary tumor in the lung. METHODS. The clinical and pathologic features in 16 cases of primary ACC of the lung were reviewed, and immunohistochemical stains on paraffin sections were performed in 7 cases. RESULTS. The patients' ages ranged from 29 to 79 years (mean age, 54 years); 11 were men and 5 were women. Clinically, most patients were seen initially with obstructive symptoms, including cough, wheezing, shortness of breath, and hemoptysis. Eight tumors were in the left lung and eight in the right lung. The lesions were treated by pneumonectomy in seven patients, lobectomy in six, and lobectomy plus chemotherapy in two. One patient was treated with chemotherapy alone after undergoing a diagnostic biopsy that revealed advanced disease. Grossly, most lesions were described as endobronchial and measured from 0.9 to 4.0 cm in greatest dimension; two cases, however, showed poorly circumscribed infiltrative tumors. Histologically, three main growth patterns were identified admixed in various proportions: cribriform (cylindromatous), tubular, and solid. Immunohistochemical study in six of seven cases showed a prominent myoepithelial cell component, as evidenced by immunoreactivity for keratin, actin, and S-100 protein in numerous tumor cells. Clinical follow-up ranging from 2 to 15 years in six patients showed that three were alive and well without evidence of recurrence or metastases at 5, 10, and 12 years, respectively, and three were alive with recurrence at 2, 5, and 15 years, respectively. Three other patients died of unrelated conditions at 2, 7, and 9 years, respectively, after diagnosis. Two patients in the study were seen initially with metastatic spread at the time of initial diagnosis and died 2 months and 1 year later with widespread metastases to lymph nodes, liver, spleen, kidney, and bone despite intensive chemotherapy. CONCLUSIONS. Disease stage at the time of diagnosis may play an important role in predicting the clinical outcome of patients with these tumors. Despite their generally slow and indolent growth in other locations, ACC arising in the lung may in certain cases be more aggressive.

Bilateral multiple pulmonary coin lesions--adenoid cystic carcinoma of the lung with 14-year follow-up.Pneumologie. 1994 Oct;48(10):769-71.

The authors report on an adenoid cystic carcinoma (ACC) of the lung that had been discovered by chance 14 years before the death of the patient and that had at first been interpreted morphologically as a metastatic lung or as a bronchioalveolar carcinoma. The patient remained without any complaints for 13 years. Diagnosis of ACC became possible only by means of a renewed peripheral lung biopsy. An extrapulmonary primary tumour was excluded by postmortem examination. The disease pattern and the special course of the ACC are described.

Peripheral pulmonary adenoid cystic carcinoma with substantial submucosal extension to the proximal bronchus.Thorax. 1991 Feb;46(2): 147-8.

A rare case of a peripheral pulmonary adenoid cystic carcinoma is presented. Histological examination after lobectomy showed pronounced submucosal invasion of the carcinoma to proximal bronchi.

A case of peripheral adenoid cystic carcinoma. Nihon Kyobu Shikkan Gakkai Zasshi. 1990 May;28(5):773-6.

A case of peripheral adenoid cystic carcinoma in a 70-year-old woman is presented. She had a coin lesion in the left upper lung field on chest X-ray film. Before one month on admission to our hospital, she noticed a tumor in the left thigh and underwent simple resection. Histological diagnosis was liposarcoma. Lung metastasis of the liposarcoma or primary lung cancer were suspected. Lung wedge resection was performed and adenoid cystic carcinoma was diagnosed. Generally adenoid cystic carcinoma originates from bronchial gland, arising in the trachea or main bronchus. In this case, the tumor originated from the peripheral lung field.

Peripheral adenoid cystic carcinoma of the lung.South Med J. 1990 May;83(5):577-9.

The large peripheral lung mass found in a 63-year-old female veteran proved to be adenoid cystic carcinoma of the lung. These rare primary lung tumors should be distinguished from adenocarcinoma, as the biologic activity and prognosis are much more favorable.

Atypical manifestations of pulmonary adenoid cystic carcinoma.Br J Dis Chest. 1986 Oct;80(4):396-9.

Pulmonary adenoid cystic carcinoma (PACC) typically arises in large airways. A patient who presented with a peripheral lung mass due to PACC is reported. She was found to have multiple pulmonary nodules due to PACC 11 years after resection of the original tumour. We emphasize that 10-15% of patients with PACC present with peripheral tumours.

Adenoid cystic carcinoma: a comparative pathologic study of tumors in salivary gland, breast, lung, and cervix.Hum Pathol.1982;13(10):916-24.

Histologic, histochemical, and ultrastructural features of eight adenoid cystic carcinomas arising at diverse sites were compared in order to determine diagnostic values and to investigate histogenetic mechanisms. These tumors originated in the salivary glands, breast, uterine cervix, and tracheobronchial tree. By light microscopy each tumor was seen to have morphologic features of adenoid cystic carcinoma, yet only five of the eight cases showed differential staining for the two mucin types, stromal and epithelial, which are reportedly present in these tumors. In contrast, every case showed a set of fine structural features which, in aggregate, are specific for adenoid cystic carcinoma. These features include pseudocysts, intercellular spaces, basal lamina, and true glandular lumens. The most prominent feature is the pseudocyst, which mimics a glandular lumen by light microscopy but is actually a rounded extracellular space containing basal lamina. Ultrastructurally, the variation in composition of the extracellular compartments, including pseudocysts and true lumens, appears to explain the lack of uniformity in the histochemical staining. The tumors also contained cytoplasmic microfilaments in parallel bundles, consistent with myofilaments. The presence of these filaments combined with basal lamina suggests myoepithelial differentiation, yet it is not known whether these tumors truly originate from myoepithelium or show differentiation toward myoepithelium as a part of the neoplastic process. Regardless of their histogenesis, this study shows that true adenoid cystic carcinomas do arise in different organs. Knowledge of the specific ultrastructural features of adenoid cystic carcinomas can be useful in classifying these tumors in some cases.

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