Background. The acinic cell carcinoma (AciCC) of the parotid gland is a rare tumor with an indolent behavior; however, a subgroup of this tumor presents an aggressive behavior with a tendency to recur. The aim of this multicenter study was to identify and stratify those patients with AciCC at high risk of tumor recurrence. Methods. A retrospective study was carried out involving 77 patients treated with surgery between January 2000 and September 2022, in different Italian referral centers. Data about tumor characteristics and its recurrence were collected. The histological specimens and slides were independently reviewed by a senior pathologist coordinator (L.C.) and the institution’s local head and neck pathologist. Results. The patients’ age average was 53.6 years, with a female prevalence in the group. The mean follow-up was 67.4 months (1-258, SD 59.39). The five-year overall survival (OS) was 83.2%. The 5-year disease-free survival (DFS) was 60% (95% CI 58.2–61.7). A high incidence of necrosis, extraglandular spread, lymphovascular invasion (LVI), atypical mitosis, and cellular pleomorphism was observed in the high-risk tumors compared to the low-risk ones. Conclusion. AciCC generally had an indolent behavior, optimal OS, DFS with few cervical node metastases, and rare distant relapses. This multicenter retrospective case series provides evidence of the need for clinical–epidemiological–histological stratification for patients at risk of poor outcomes. Our results suggest that the correct definition of high-risk AciCC should include tumor size, the presence of necrosis, extraglandular spread, LVI, atypical mitosis, and cellular pleomorphism.
背景：腮腺腺泡细胞癌（AciCC）是一种具有惰性生物学行为的罕见肿瘤，但其中部分亚型表现出侵袭性特征并易复发。本多中心研究旨在识别并分层评估具有高复发风险的AciCC患者。方法：本研究回顾性纳入2000年1月至2022年9月期间在意大利多家转诊中心接受手术治疗的77例患者，收集肿瘤特征及复发数据。所有组织学标本和切片均由资深病理学协调员（L.C.）及各机构头颈病理学负责人独立复核。结果：患者平均年龄53.6岁，女性居多。平均随访时间67.4个月（范围1-258个月，标准差59.39）。五年总生存率（OS）为83.2%，五年无病生存率（DFS）为60%（95% CI 58.2–61.7）。与低风险肿瘤相比，高风险肿瘤中坏死、腺外扩散、淋巴血管侵犯（LVI）、非典型核分裂及细胞多形性的发生率显著增高。结论：AciCC通常呈惰性病程，总生存率理想，无病生存率良好，颈部淋巴结转移较少，远处复发罕见。本多中心回顾性病例系列研究证实，对预后不良风险患者进行临床-流行病学-组织学分层评估具有必要性。研究结果表明，高危AciCC的准确定义应包含肿瘤大小、坏死存在、腺外扩散、LVI、非典型核分裂及细胞多形性等特征。
肿瘤(癌症)患者之家