Gajda Mariusz, Zagolski Olaf, Jasztal Agnieszka, Lis Grzegorz J, Pyka-Fosciak Grazyna
Department of Histology, Jagiellonian University, Medical College, Kopernika 7, 31-034, Krakow, Poland.
Cases J. 2009 Sep 16;2:8151. doi: 10.4076/1757-1626-2-8151.
Hamartomas are non-neoplastic lesions constituted by a mixture of tissues indigenous to the region. Respiratory epithelial adenomatoid hamartomas are characterised by glandular proliferation lined by ciliated airway epithelium. Their localisation in the nasal cavity is rare and most frequent cases described so far were associated with the posterior nasal septum.
A 9-year-old Caucasian boy presented with long-standing nasal obstruction. A large right nasal mass was evident on physical and CT examinations. It was surgically removed from the anterior nasal septum under general anaesthesia. Histologically, the diagnosis of REAH was established. The tumour lined by stratified squamous and ciliated respiratory epithelium was characterised by prominent glandular proliferation. By immunohistochemistry, the tumour was positive for cytokeratins, smooth muscle actin, vimentin, laminin, collagen type IV, CD8, and CD68. No S-100 immunoreactivity was observed. The patient has been asymptomatic for 12 months with completely healed lining of the nose.
Respiratory epithelial adenomatoid hamartoma, although rare, must be taken into consideration in differential diagnosis of nasal exophytic lesions.
错构瘤是由该区域固有组织混合构成的非肿瘤性病变。呼吸上皮腺样错构瘤的特征是由纤毛气道上皮衬里的腺体增生。它们在鼻腔中的定位很少见，迄今为止描述的大多数病例与鼻中隔后部有关。
一名9岁的白种男孩长期存在鼻塞。体格检查和CT检查发现右侧鼻腔有一个大肿块。在全身麻醉下从鼻中隔前部将其手术切除。组织学上，确诊为呼吸上皮腺样错构瘤。肿瘤由复层鳞状上皮和纤毛呼吸上皮衬里，其特征是显著的腺体增生。免疫组织化学显示，肿瘤细胞角蛋白、平滑肌肌动蛋白、波形蛋白、层粘连蛋白、IV型胶原、CD8和CD68呈阳性。未观察到S-100免疫反应性。该患者12个月来无症状，鼻腔内衬已完全愈合。
呼吸上皮腺样错构瘤虽然罕见，但在鼻腔外生性病变的鉴别诊断中必须予以考虑。
