Clinicopathologic characteristics of Burkitt lymphoma of the head and neck in a non-endemic region—a Danish nationwide study

Abstract

Background

Burkitt lymphoma rarely presents in head and neck (H&N) in Western countries.

Aims/objectives

We aimed to characterise clinicopathological features of H&N Burkitt lymphoma in Denmark representing a non-endemic region.

Material and methods

Clinical records were reviewed for a nationwide cohort of patients diagnosed with H&N Burkitt lymphoma in Denmark between 1980 and 2018. The diagnosis was histologically validated.

Results

Thirty-four patients with H&N Burkitt lymphoma (highest incidence in age group 0–9 years, male-to-female ratio 4.7:1) were included. Thirty-three lymphomas (97%) were extranodal. The tumour was visible at the clinical examination in 81% (n = 22) of the cases. The palatine tonsils were the most frequent location (n = 13, 38%) and 52% (n = 17) of the patients were diagnosed in advanced stage. Lymphoma was the tentative clinical diagnosis in 23% of the cases. The 5-year overall- and disease-specific survival was 78% and 81%, respectively.

Conclusions

Due to the rarity of Burkitt lymphoma of the H&N, there is a high risk of clinical misdiagnosis. Our findings suggest which symptoms and clinical presentations to be aware of in the diagnostics work up that could lead to the diagnosis of Burkitt lymphoma.

Chinese abstract

背景：在西方国家, 伯基特（Burkitt）淋巴瘤很少发生在头颈部 (H&N)。

目的：描述丹麦这一代表非流行地区的头颈部伯基特淋巴瘤的临床病理特征。

材料和方法：对1980 年至 2018 年期间在丹麦全国范围内诊断为头颈部伯基特淋巴瘤的患者的临床记录进行复检。这些诊断经过组织学验证。

结果：34 名 头颈部伯基特淋巴瘤患者（0-9 岁年龄组发病率最高, 男女比例 为4.7:1）。三十三个淋巴瘤（97%）是结外的。81% (n = 22) 的病例在临床检查中可见肿瘤。腭扁桃体是最常见位置 (n ¼ 13, 38%) , 52% (n ¼ 17) 的患者被诊断为晚期。淋巴瘤是 23% 病例的初步临床诊断。 5 年总生存率和疾病特定性生存率分别为 78% 和 81%。

结论：由于头颈部伯基特淋巴瘤的罕见性, 临床误诊风险较高。我们的研究结果表明, 在诊断工作中需要注意哪些症状和临床表现, 这样的工作可能会产生伯基特淋巴瘤诊断。

Keywords:

• Head and neck
• Burkitt lymphoma
• clinical presentation
• symptom
• prognosis

Acknowledgments

We acknowledge the Danish National Lymphoma Registry and the Danish Childhood Cancer Registry for providing data to our study.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

Grants from the Aase and Ejnar Danielsen Foundation and Candys Foundation supported this study. The foundations had no role in any part of the study, including study design, analysis, and manuscript drafting.