Background/Objectives: Head and neck cancer is one of the most common malignancies in patients with Fanconi anemia (FA), with a greater than 500-fold incidence of head and neck squamous cell carcinoma. Treatment of HNSCC in these patients is particularly challenging because of poor tolerance to radiation therapy and to some chemotherapeutic agents such as cisplatin. For these reasons, new research is needed to determine the best treatment course for these patients. With this goal in mind, we assessed the characteristics of head and neck squamous cell carcinoma (SCC) in Fanconi anemia (FA). Subjects and Methods: Data for 11 patients (mean age 31 years) with head and neck SCC and FA attended to between 2005 and 2021 were analyzed. Results: The primary tumor site was the oral cavity in eight patients, and five had advanced stages. All patients underwent primary tumor resection. Four patients received adjuvant radiotherapy (mean 57.2 Gy), but three developed toxicity. The mean follow-up was 48.4 months. Six patients experienced 19 episodes of primary tumor recurrence and five developed secondary head and neck tumors. The 3-year disease-free survival was 47%, and the 5-year cause-specific survival was 48%. These findings are similar to the data for 72 patients collected from the literature. Conclusions: The prognosis of head and neck cancer in patients with Fanconi anemia is poor, with an overall survival lower than 50% at 5 years.
背景/目的：范可尼贫血患者头颈部恶性肿瘤发病率极高，其中头颈部鳞状细胞癌的发病率超过普通人群500倍以上。由于此类患者对放射治疗及顺铂等化疗药物耐受性较差，头颈部鳞状细胞癌的治疗尤为棘手。因此亟需开展新研究以确定最佳治疗方案。基于此目标，本研究旨在评估范可尼贫血患者头颈部鳞状细胞癌的临床特征。研究对象与方法：分析2005年至2021年间收治的11例范可尼贫血合并头颈部鳞状细胞癌患者（平均年龄31岁）的临床资料。结果：原发肿瘤位于口腔者8例，其中5例为晚期。所有患者均接受原发肿瘤切除术，4例患者接受辅助放疗（平均剂量57.2 Gy），其中3例出现放射性毒性反应。平均随访48.4个月期间，6例患者出现19次原发肿瘤复发，5例发生继发性头颈部肿瘤。3年无病生存率为47%，5年病因特异性生存率为48%，该结果与文献报道的72例患者数据相符。结论：范可尼贫血患者头颈部肿瘤预后不良，5年总生存率低于50%。
Head and Neck Cancer in Fanconi Anemia: Report of 11 Cases and a Systematic Review
肿瘤(癌症)患者之家