Bollinger Stephen Scott, DeSautel Mark Gregory, Spanos William Chad, Tjarks Brian Joel
University of South Dakota Sanford School of Medicine, Sioux Falls, South Dakota.
Department of Surgery, University of South Dakota Sanford School of Medicine, Sioux Falls, South Dakota.
S D Med. 2018 Jul;71(7):294-298.
Adenoid cystic carcinoma (ACC) is a rare malignant secretory gland tumor. It is characterized by slow growth, long clinical course, local recurrences, and distant metastases. In the sinonasal tract, it most commonly arises in the maxillary sinus. It often presents at an advanced stage with perineural spread (PNS). Our patient presented with left-sided facial numbness without other symptoms. The numbness was localized to the left cheek, left side of nose, and left upper lip. Magnetic resonance imaging (MRI) of the brain revealed an enhancing lesion involving the left maxillary sinus with orbital invasion and posterior extension into the cavernous sinus. Transnasal endoscopic exploration with tissue removal revealed ACC. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) scan revealed no evidence of distant metastases. Presentation of sinonasal ACC (SNACC) is variable depending on the involved structures. Characteristic PNS with ACC may cause neuropathic symptoms. This case displays a unique presentation of an advanced ACC of the maxillary sinus manifesting as isolated unilateral trigeminal anesthesia without sinonasal symptoms. The patient also failed to demonstrate any ocular or oculomotor symptoms despite extensive involvement of the orbit and surrounding structures. This case highlights the importance of recognizing ACC due to its association with late symptomatic manifestations. It also reinforces the need for clinical diligence with the workup of new onset neuropathic symptoms in the maxillary distribution of the trigeminal nerve.
腺样囊性癌（ACC）是一种罕见的恶性分泌性腺瘤。其特点是生长缓慢、临床病程长、局部复发和远处转移。在鼻窦区域，它最常发生于上颌窦。它常以晚期伴有神经周围浸润（PNS）的形式出现。我们的患者表现为左侧面部麻木，无其他症状。麻木局限于左侧脸颊、左侧鼻子和左上唇。脑部磁共振成像（MRI）显示一个强化病变，累及左上颌窦，侵犯眼眶并向后延伸至海绵窦。经鼻内镜探查并取组织后显示为ACC。18F-氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）显示无远处转移迹象。鼻窦腺样囊性癌（SNACC）的表现因受累结构而异。ACC典型的PNS可能导致神经病变症状。该病例展示了上颌窦晚期ACC的一种独特表现，表现为孤立的单侧三叉神经麻醉，无鼻窦症状。尽管眼眶及周围结构广泛受累，但患者也未表现出任何眼部或动眼神经症状。该病例强调了认识ACC的重要性，因为它与晚期症状表现相关。它还强化了在对三叉神经上颌支分布区域新发神经病变症状进行检查时临床保持勤勉的必要性。
