Hong Xinyuan, Khalife Sarah, Bouhabel Sarah, Bernard Chantal, Daniel Sam J, Manoukian John J, Nguyen Lily H P
Faculty of Medicine, McGill University, Montreal, Canada.
Department of Otolaryngology - Head and Neck Surgery, McGill University, Montreal, Canada.
Int J Pediatr Otorhinolaryngol. 2019 Jun;121:127-136. doi: 10.1016/j.ijporl.2019.03.013. Epub 2019 Mar 13.
Burkitt lymphoma (BL), an aggressive form of B-cell non-Hodgkin's lymphoma, arising from the nose and paranasal sinuses is relatively rare. It can present with various symptoms leading to potential misdiagnosis and delayed treatment. BL is fatal if left untreated, while early identification and treatment can improve prognosis.
A series of six cases of pediatric (0-18 years) BL with rhinologic manifestations is presented. Age, sex, ethnicity, symptoms, imaging, staging, treatment and outcome were recorded. A systematic review of literature was also conducted using PRISMA guidelines. The search strategy used keywords related to rhinologic manifestations of BL (nasal cavity, nasopharynx, paranasal sinus etc.; Burkitt etc.) and included studies published in English and French describing patients 0-18 years of age.
42 patients were included (six from case series and 36 from current literature). Most common presenting symptoms were: nasal obstruction (29%), facial swelling (24%), headache (21%) and proptosis (19%). Most frequent sites of presentation were: nasopharynx (40%), maxilla (40%) and sphenoid (33%). More than half (60%) had systemic involvement, of which the most common locations were: kidney (19%), pancreas (17%) and liver (17%). Mortality from BL in children from this study population was correlated with a longer duration of symptoms prior to presentation, as well as a misdiagnosis preceding the final diagnosis of BL.
This study brings understanding to the numerous presentations of the same aggressive disease, promotes high clinical suspicion when evaluating common otolaryngologic symptoms and can guide healthcare providers in diagnosing pediatric BL with rhinologic manifestations.
伯基特淋巴瘤（BL）是一种侵袭性B细胞非霍奇金淋巴瘤，起源于鼻腔和鼻窦者相对罕见。它可表现出多种症状，导致可能的误诊和治疗延误。若不治疗，BL会致命，而早期识别和治疗可改善预后。
1）回顾6例有鼻科表现的儿童BL的临床表现和受累部位，并与当前文献进行比较。2）提高对BL在这一特定解剖部位多种表现的认识。
报告了一系列6例有鼻科表现的儿童（0至18岁）BL病例。记录年龄、性别、种族、症状、影像学检查、分期、治疗及结果。还使用PRISMA指南对文献进行了系统回顾。检索策略使用了与BL鼻科表现相关的关键词（鼻腔、鼻咽、鼻窦等；伯基特等），并纳入了以英文和法文发表的描述0至18岁患者的研究。
共纳入42例患者（6例来自病例系列，36例来自当前文献）。最常见的首发症状为：鼻塞（29%）、面部肿胀（24%）、头痛（21%）和眼球突出（19%）。最常见的受累部位为：鼻咽（40%）、上颌骨（40%）和蝶窦（33%）。超过一半（60%）有全身受累，其中最常见的部位为：肾脏（19%）、胰腺（17%）和肝脏（17%）。该研究人群中儿童BL的死亡率与出现症状至就诊的时间较长以及在BL最终诊断之前的误诊相关。
本研究有助于了解同一种侵袭性疾病的多种表现，在评估常见的耳鼻咽喉科症状时提高临床高度怀疑，并可指导医疗服务提供者诊断有鼻科表现的儿童BL。
