Primary adenoid cystic carcinoma of the lung: Clinicopathological features, treatment and results

Abstract

Adenoid cystic carcinoma of the lung (ACCL) is a rare salivary gland-type malignant neoplasm that occurs infrequently as a primary tumor of the airway. Owing to its low incidence, the clinicopathological features, immunohistochemical expression spectrum, treatment and long-term survival have not been fully elucidated. The present study retrospectively assessed the clinical features, immunohistochemical characters, treatment strategy and long-term survival of 34 patients diagnosed with ACCL at the Beijing Chest Hospital, Capital Medical University (Beijing, China) between January 1993 and June 2014. ACCL tended to occur in younger patients, with an approximate male/female ratio of 1:1. The majority of ACCL arose from the central airway. Positive immunochemical staining was found in wide-spectrum keratin (n=17), cytokeratin (CK)7 (n=11), p63 (11/12), S-100 (7/8), vimentin (10/12) and smooth muscle actin (6/9). No staining of thyroid transcription factor-1 (0/14), synaptophysin (0/7), cluster of differentiation 56 (0/7), CK20 (0/4) or chromogranin A (0/4) was observed. In the operable group (n=26), the addition of adjuvant radiotherapy to a positive margin resection (R1 resection) obtained long-term survival times equivalent to that found in patients with a negative margin resection (R0 resection). No significant survival benefit from post-operative radiotherapy was observed in the R0 resection group. For advanced cases, palliative radiotherapy and chemotherapy did not work efficiently. In addition, epidermal growth factor receptor mutation was a rare event in the ACCL patients. The results indicated that surgical resection is the optimal management for ACCL whenever feasible. Adjuvant radiotherapy with R1 resection is able to obtain long-term survival times comparable with those found using an R0 resection. The recommendation of post-operative radiotherapy for all patients with ACCL undergoing resection appears controversial. Owing to a poor response to radiotherapy and chemotherapy, more focus should be placed on the study of advanced ACCL in order to improve overall survival.

Primary Pulmonary Salivary Gland-type Tumors

“…Negative markers include synaptophysin and TTF-1. 32 More recently, these tumors have demonstrated immunohistochemical positivity for CD117 (c-kit protein) 2,16,33,34 and myeloblastosis (MYB) antigen 35 both of which can help to differentiate ACC from other basaloid neoplasms. ACC are cytogenetically characterized by a tumor-specific t(6;9) (q22-23;p23-24) translocation generating a MYB (v-myb avian MYB viral oncogene homolog) proto-oncogene fusion with the transcription factor gene NFIB (nuclear factor IB).…”

Case report of apatinib mesylate treatment in rare advanced tracheal adenoid cystic carcinoma

“…Tracheal ACC is a rare low‐grade malignant neoplasm and primarily occurs in the trachea and bronchi, originating from ducts in the mucous glands. Tracheal ACC accounts for 0.04–0.2% of lung cancer cases and is distributed equally between genders . The age of onset ranges from 40 to 59 years; our patient was 59.…”

“…Tracheal ACC accounts for 0.04-0.2% of lung cancer cases 5 and is distributed equally between genders. 6 The age of onset ranges from 40 to 59 years; our patient was 59. Lung ACC is characterized by slow disease progression and a long duration.…”

“…The course of the disease is usually two to three years, and in some cases it can last more than 10 years;our patient had the disease for 10 years. Treatments for lung ACC include surgical resection, radiotherapy, and chemotherapy . In some cases, fiberoptic bronchoscopic laser, flucticuli, electrotomy, and stent implantation have also been reported to be effective .…”

“…Treatments for lung ACC include surgical resection, radiotherapy, and chemotherapy. 6 In some cases, fiberoptic bronchoscopic laser, flucticuli, electrotomy, and stent implantation have also been reported to be effective. 8 Lung ACC is clinically rare and as such there is no uniform standard therapy.…”