Kossai Myriam, El Zein Sophie, Wassef Michel, Guichard Jean-Pierre, Pouliquen Christelle, Herman Philippe, Vérillaud Benjamin, Classe Marion
Departments of Pathology.
Faculty of Medicine, Paris Diderot University.
Am J Surg Pathol. 2018 Jan;42(1):9-17. doi: 10.1097/PAS.0000000000000967.
Sinonasal epithelial hamartomas occurring in adults are classified as seromucinous hamartoma (SMH) or respiratory epithelial adenomatoid hamartoma (REAH). We describe herein a novel subtype of adult sinonasal hamartoma that contains olfactory epithelium, a histologic feature not previously reported in the literature. Our pathology department database was retrospectively searched for sinonasal hamartomas containing areas of olfactory epithelium. Six relevant cases (3 male and 3 female patients; age, 30 to 77 y) were retrieved, and available pathology slides and clinical and imaging data from patient charts were reviewed. Five of the lesions were unilateral solitary, polypoid, pedunculated masses, 38 to 80 mm in length, lodged in the nasal olfactory cleft. The sixth lesion was associated with bilateral nasal polyposis, and its precise localization was not known. All patients were treated by transnasal endoscopic surgery. None of the 3 patients who had received adequate follow-up evaluation exhibited recurrence. Histologically, all lesions resembled SMH or REAH, with areas of olfactory epithelium comprising olfactory receptors and sustentacular and basal cells. Olfactory epithelium was observed at the lesion surface or in invaginated gland-like structures, and it contained focal aggregates of filamentous cell processes. Some olfactory receptor cells or cell processes were also present in the seromucinous gland component of lesions. Olfactory receptor cells expressed CD56 (neural cell adhesion molecule), and the filamentous aggregates contained CD56, neurofilaments, and synaptophysin. Aside from SMH and REAH, we have described a third subtype of adult sinonasal hamartoma-olfactory epithelial hamartoma-which shares the benign character of the other 2.
发生于成人的鼻窦上皮性错构瘤分为浆液黏液性错构瘤（SMH）或呼吸上皮性腺瘤样错构瘤（REAH）。我们在此描述一种成人鼻窦错构瘤的新亚型，其含有嗅上皮，这是一种此前文献中未报道过的组织学特征。我们回顾性检索了病理科数据库中含有嗅上皮区域的鼻窦错构瘤。检索到6例相关病例（3例男性和3例女性患者；年龄30至77岁），并复查了可用的病理切片以及患者病历中的临床和影像资料。其中5个病变为单侧孤立性、息肉样、有蒂肿物，长38至80毫米，位于鼻腔嗅裂。第6个病变与双侧鼻息肉病相关，其确切位置不详。所有患者均接受了经鼻内镜手术治疗。3例接受了充分随访评估的患者均未出现复发。组织学上，所有病变均类似SMH或REAH，具有包含嗅觉受体、支持细胞和基底细胞的嗅上皮区域。在病变表面或内陷的腺样结构中观察到嗅上皮，其含有丝状细胞突起的局灶性聚集物。病变的浆液黏液性腺成分中也存在一些嗅觉受体细胞或细胞突起。嗅觉受体细胞表达CD56（神经细胞黏附分子），丝状聚集物含有CD56、神经丝和突触素。除了SMH和REAH，我们还描述了成人鼻窦错构瘤的第三种亚型——嗅上皮性错构瘤，它与其他两种亚型一样具有良性特征。
