Abstract：
The purpose of the present study was to perform a systematic review focusing on oral squamous cell carcinoma (OSCC) and oral potentially malignant disorders (OPMD) in Fanconi anemia (FA) individuals. Electronic searches were undertaken in five databases supplemented by manual scrutiny and gray literature. Case reports and/or cases series were included. The searches yielded 55 studies describing 112 cases of OSCC (n = 107) and/or OPMD (n = 5) in FA individuals. The mean age at diagnosis of OSCC/OPMD was 27.1 (±9.6) years, and females (51.8 %) were slightly more affected. Ulcer (n = 37) or mass (n = 25) were described as clinical presentations for OSCC and OPMD. White lesions (n = 4) were the most common manifestation in OPMD. Tongue (47.2 %) was the most frequent location. Sixty-one (54.5 %) individuals underwent HSCT. Surgical resection (n = 75) was the main treatment adopted. The estimated rate of OPMD malignant transformation was 1.8 % and recurrences following OSCC excision occurred in 26.8 % of individuals. Overall, at 60 months of follow-up, the probability of survival fell to 25.5 % and at 64 months the probability of recurrence increased to 63.2 %. The present data support the need for strict surveillance of patients with FA, even in the absence of OPMD, for early OSCC detection and reduction of mortality.
摘要：
本研究的目的是对范可尼贫血（FA）个体的口腔鳞状细胞癌（OSCC）和口腔潜在恶性疾病（OPMD）进行系统评价。在五个数据库中进行了电子搜索，并辅以人工审查和灰色文献。包括病例报告和/或病例系列。搜索产生了55项研究，描述了FA个体中112例OSCC（n=107）和/或OPMD（n=5）。诊断OSCC/OPMD的平均年龄为27.1(±9.6)岁,而女性(51.8%)受影响稍大。溃疡（n=37）或肿块（n=25）被描述为OSCC和OPMD的临床表现。白色病变（n=4）是OPMD中最常见的表现。舌头（47.2％）是最常见的位置。61例（54.5%）患者接受HSCT。手术切除（n=75）是主要的治疗方法。OPMD恶性转化的估计率为1.8％，OSCC切除后复发的人数为26.8％。总的来说,在60个月的随访中，生存概率降至25.5%,64个月时复发概率增至63.2%.目前的数据支持对FA患者进行严格监测的必要性，即使没有OPMD,早期OSCC检测和降低死亡率。
