Masih I, Porter G, Porter S, Clarke R, Sidhu P, Harney J, McCarthy A, Convery R
Department of Respiratory Medicine, Craigavon Area Hospital, Port down, Craigavon, UK.
BMJ Case Rep. 2010 Nov 29;2010:bcr0820103252. doi: 10.1136/bcr.08.2010.3252.
Primary adenoid cystic carcinoma (ACC) of the lung is an extremely rare malignant lung neoplasm. ACC of salivary glands of the head and neck, lachrymal glands, breast, skin, vulva and trachea have been frequently reported disease patterns in the literature, but it is unique to see this rare lung tumour in a patient as young as 14 years old. No double blind placebo, multicentre treatment data are available. Surgery is considered as the cornerstone of the treatment. Prognosis is variable and adjuvant radiotherapy has been found beneficial for prolonged survival. Our report of primary lung ACC in a young girl is a complex case due to young age, a different way of presentation and staging on diagnosis. It has been a quite challenging clinical scenario for the multidisciplinary lung cancer treating team involved in the clinical care. Prognosis remains unpredictable and uncertain despite the best present day evidence-based treatment.
原发性肺腺样囊性癌（ACC）是一种极其罕见的肺部恶性肿瘤。头颈部唾液腺、泪腺、乳腺、皮肤、外阴和气管的ACC在文献中是常见的疾病类型，但在一名年仅14岁的患者中发现这种罕见的肺部肿瘤却很独特。目前尚无双盲安慰剂多中心治疗数据。手术被认为是治疗的基石。预后因人而异，辅助放疗已被证明对延长生存期有益。我们报告的一名年轻女孩的原发性肺ACC病例，因其年龄小、诊断时呈现方式和分期不同而较为复杂。对于参与临床护理的多学科肺癌治疗团队来说，这是一个极具挑战性的临床情况。尽管采用了当今最佳的循证治疗方法，预后仍然不可预测且不确定。
