Clinical Presentation and Radiological Features of Intrathoracic Non-Hodgkin’s Lymphoma in Paediatric Patients: an Institutional Review

Full Article

JHY Leung, KY Lai, MK Shing, CK Li, WCW Chu

Hong Kong J Radiol 2012;15:15-21

Objective: Non-Hodgkin’s lymphoma is the most common tumour causing the superior mediastinal syndrome and superior vena caval obstruction in paediatric patients. Our objective was to review the clinical parameters, radiological features and outcomes of children presenting with intrathoracic non-Hodgkin’s lymphoma and superior mediastinal syndrome in a tertiary cancer centre over a period of 11 years.

Methods: Retrospective review of clinical and radiological features of 11 patients with intrathoracic non- Hodgkin’s lymphoma admitted to a tertiary children’s cancer centre between 1999 and 2010 was undertaken.

Results:Most patients presented with superior mediastinal symptoms, including dyspnoea (n = 9, 82%) and neck swelling (n = 3, 27%). All the tumours were located within the anterior mediastinum. Their most common radiological features included heterogeneous enhancement (n = 7, 64%) and necrosis (n = 7, 64%). Neither abnormal fat/calcium nor chest wall invasion was evident in these patients. Associated complications included superior vena caval obstruction (n = 6, 55%) and tracheobronchial compression (n = 7, 64%). Correlation between clinical oxygenation status and radiological evidence of tracheobronchial compression was poor (Fisher’s exact test, p = 0.49).

Conclusion: Data from this large patient cohort of paediatric intrathoracic non-Hodgkin’s lymphoma in Hong Kong are presented. Non-Hodgkin’s lymphoma is the most common tumour causing superior mediastinal syndrome in children, who usually present late in the course of the disease. This study illustrates the spectrum of clinical manifestations and associated findings in such children. Clinicians should have high awareness of these mediastinal symptoms and assess the risk of airway compromise by computed tomographic quantification.

 

中文摘要

胸部非何杰金氏淋巴瘤小兒患者的臨床症狀及影像特徵:一所機構的報告

梁凱瑩、黎家攸、成明光、李志光、朱昭穎

目的:非何杰金氏淋巴瘤是導致小兒縱隔綜合症及上腔靜脈阻塞一種常見病因。本研究回顧一所 機構在11年內,患有上縱隔綜合症及胸部非何杰金氏淋巴瘤的小童的臨床症狀、影像特徵及治療結 果。

方法:回顧1999年至2010年間,因胸部非何杰金氏淋巴瘤而到一所提供專科醫療服務的小兒腫瘤中 心應診的11名病人的臨床症狀及影像特徵。

結果:大部分患者出現上縱隔綜合症的症狀,包括呼吸困難(9例,82%)及頸部腫脹(3例, 27%)。所有腫瘤的位置均在前縱隔內。最常見的影像特徵包括不均勻強化(7例,64%)及壞死(7 例,64%)。患者均無異常脂肪/鈣質或胸壁受侵的徵狀。有關的併發病包括上腔靜脈阻塞綜合症 (6例,55%)及氣管支氣管狹窄(7例,64%)。臨床氧合狀態和氣管支氣管狹窄影像結果並無相關 (Fisher確切檢驗,p = 0.49)。

結論:這是一項對於香港小兒胸部非何杰金氏淋巴瘤的大型研究。非何杰金氏淋巴瘤是導致小兒縱 隔綜合症及上腔靜脈阻塞一種常見病因。患者通常在上縱隔綜合症晚期才到診。本研究顯示小兒上 縱隔綜合症廣泛的病變臨床表現。醫生應對這些縱隔綜合症的症狀有高度警惕,應根據CT來量化評 估呼吸道風險。