Department of Otolaryngology - Head and Neck Surgery, Beth Israel Deaconess Medical Center, 300 Brookline Ave, Boston, MA, 02115, USA; Department of Otolaryngology-Head and Neck Surgery, Harvard Medical School, 25 Shattuck Street, Boston, MA, 02115, USA.
Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, 333 Longwood Avenue, Boston, MA, 02115, USA.
Int J Pediatr Otorhinolaryngol. 2024 Nov;186:112137. doi: 10.1016/j.ijporl.2024.112137. Epub 2024 Oct 16.
Burkitt lymphoma (BL) is an aggressive form of non-Hodgkin lymphoma with the sporadic subtype being predominant in North America. The clinical presentations and outcomes of pediatric BL within the head and neck were assessed using both an institutional case series and the Surveillance, Epidemiology, and End Results (SEER) Cancer database.
The electronic medical record at our quaternary children's hospital was queried over a 22-year period (2000-2022) for BL patients with head and neck manifestations. Demographics, clinical presentation, staging, treatment, and outcomes data were collected and analyzed. A corresponding review of the SEER database from 1975 to 2022 was also performed.
Our institutional case series identified 48 sporadic BL patients with a mean age of 8.7 years, the majority of whom were male (79 %) and white (74 %). The most common primary sites were the cervical lymph nodes (38 %) and (or) palatine tonsils (23 %). Thirty-five patients (73 %) were treated initially for a presumed inflammatory or infectious process before undergoing malignancy work-up, which did not significantly delay time to diagnosis (31.5 vs. 38.8 days, p = 0.27). The SEER database analysis identified 78 cases, 43.5 % of whom were 5-9 years of age, with a similar male (66 %) and Caucasian (76.9 %) predominance. Cervical lymph nodes were also the most common subsite (67 %), followed by the palatine tonsils (13 %). Remission rates were similar, 93.7 % and 94.8 %, respectively, in both the institutional and SEER database cohorts.
Unilateral cervical lymphadenopathy and asymmetric tonsillar hypertrophy are the most common presentations in sporadic BL in the head and neck. Clinical presentation in patients with BL is often similar to common, insidious pediatric otolaryngology symptoms and a majority of patients initially undergo treatment for presumed infectious or inflammatory disease. Although overall BL disease-free survival is high even for disseminated BL, the prognosis is better for local/regional disease, and minimizing time to diagnosis and treatment should remain a priority.
伯基特淋巴瘤(BL)是一种侵袭性非霍奇金淋巴瘤,散发性亚型在北美的发病率较高。本研究通过机构病例系列和监测、流行病学和最终结果(SEER)癌症数据库,评估了头颈部儿童 BL 的临床表现和结局。
在 22 年的时间内(2000 年至 2022 年),我们对我院四级儿童医院的电子病历进行了查询,以寻找具有头颈部表现的 BL 患者。收集并分析了人口统计学、临床表现、分期、治疗和结局数据。还对 1975 年至 2022 年的 SEER 数据库进行了相应的回顾。
本机构病例系列共纳入 48 例散发性 BL 患者,平均年龄为 8.7 岁,大多数为男性(79%)和白人(74%)。最常见的原发部位是颈淋巴结(38%)和(或)腭扁桃体(23%)。35 例患者(73%)在接受恶性肿瘤检查前,最初因疑似炎症或感染性疾病接受治疗,这并未显著延迟诊断时间(31.5 天 vs. 38.8 天,p=0.27)。SEER 数据库分析共纳入 78 例患者,其中 43.5%为 5-9 岁,男性(66%)和白种人(76.9%)比例较高。颈淋巴结也是最常见的部位(67%),其次是腭扁桃体(13%)。机构和 SEER 数据库队列的缓解率分别为 93.7%和 94.8%,相似。
单侧颈淋巴结病和不对称性扁桃体肿大是头颈部散发性 BL 最常见的表现。BL 患者的临床表现常与常见的、隐匿性儿科耳鼻喉科症状相似,大多数患者最初接受治疗的原因是疑似感染或炎症性疾病。尽管即使是播散性 BL,总体无病生存也很高,但局部/区域性疾病的预后更好,应优先考虑缩短诊断和治疗时间。