Al-Bitar Ahmad, Al-Abdulrazzak Bishr A, Alahmar Ruba
Faculty of Medicine, Damascus University, Damascus, Syrian Arab Republic.
Case Rep Oncol. 2025 May 28;18(1):809-816. doi: 10.1159/000546446. eCollection 2025 Jan-Dec.
Adenoid cystic carcinoma (ACC), a rare, aggressive malignancy of secretory epithelia, is often present with nonspecific symptoms, delaying diagnosis.
A 67-year-old male reported 10 months of nasal obstruction, mouth breathing, and sleep disturbances. The initial evaluation for foreign body obstruction revealed septal deviation and low-density sinonasal tissue on CT. MRI identified a 7 × 4.5 × 7 cm heterogeneous lesion invading nasal structures, paranasal sinuses, and nasopharynx with diffusion restriction. Tru-cut biopsy confirmed ACC via cribriform, tubular, and solid basaloid cell patterns, pseudocystic spaces, biphasic ductal-myoepithelial cells, and perineural invasion. Immunohistochemistry (CK7, CD117, p63, S100) supported the diagnosis, with tumor-free margins and no metastases. Multimodal therapy (30 VMAT sessions, 4 cisplatin-vinorelbine cycles) improved symptoms.
This case illustrates ACC's diagnostic complexity, requiring advanced imaging and histopathology to exclude mimics. Despite indolent early progression, ACC's neurotropic spread and late-stage detection demand aggressive treatment. While surgery with adjuvant radiotherapy remains standard, the absence of metastases here underscores its variable behavior.
腺样囊性癌（ACC）是一种罕见的、侵袭性的分泌上皮恶性肿瘤，常表现为非特异性症状，导致诊断延迟。
一名67岁男性报告有10个月的鼻塞、张口呼吸和睡眠障碍。对异物阻塞的初步评估显示鼻中隔偏曲以及CT上鼻窦组织密度减低。磁共振成像（MRI）发现一个7×4.5×7厘米的异质性病变，侵犯鼻腔结构、鼻窦和鼻咽部，具有弥散受限。粗针穿刺活检通过筛状、管状和实性基底样细胞模式、假囊性间隙、双相导管 - 肌上皮细胞以及神经周围浸润确诊为ACC。免疫组织化学（CK7、CD117、p63、S100）支持诊断，切缘无肿瘤且无转移。多模式治疗（30次容积调强弧形放疗疗程，4个顺铂 - 长春瑞滨周期）改善了症状。
本病例说明了ACC诊断的复杂性，需要先进的影像学和组织病理学检查以排除相似疾病。尽管早期进展缓慢，但ACC的嗜神经侵袭和晚期发现需要积极治疗。虽然手术加辅助放疗仍然是标准治疗方法，但此处无转移强调了其行为的多变性。
