Tang Yan-jing, Tang Jing-yan, Pan Ci, Xue Hui-liang, Chen Jing, Shen Shu-hong, Dong Lu, Zhou Min, Wang Yao-ping, Gu Long-jun, Jiang Hua, Ye Qi-dong
Department of Hematology/Oncology, Shanghai Children's Medical Center Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.
Zhonghua Er Ke Za Zhi. 2009 Sep;47(9):687-90.
Non-Hodgkin's lymphoma (NHL) presenting as mediastinal mass is usually progressive and may cause severe respiratory distress and death. This study aimed to summarize the clinical features and prognosis of NHL arising from mediastinum.
Totally 36 patients with NHL arising from mediastinum reported herein were diagnosed between 1999 and 2007. Their clinical characteristics, pathologic classification, diagnosis, outcome of different treatment protocol were retrospectively analyzed. Of these 36 patients, 25 were male, 11 were female (2.2:1). The mean age was 7.9 (range 1 - 12) years. Diagnosis was established on pathology that was achieved by mediastinal mass or peripheral lymph nodes biopsy, while some were diagnosed based on bone marrow or pleural effusion cytology study and immunophenotyping. For staging, the St. Jude system was applied. Patients received T-NHL-CCCG97, T-NHL-2002 or B-NHL-2001 protocol according to morphology and immunophenotyping. Patients who experienced superior vena cava syndrome (SVCS) and/or superior mediastinum syndrome (SMS) received induction chemotherapy with cyclophosphamide (C), vincristine (O) and prednisone (P) for one week.
Twenty-seven cases experienced mediastinal mass or peripheral lympho nodes biopsy and were diagnosed by histopathology and immunohistochemistry. Of them, 24 were lymphoblastic lymphoma and 3 were anaplastic large cell lymphoma. Nine patients were diagnosed by cytological study of bone marrow aspiration or pleural fluid. All the 36 cases were T-cell type. Twenty-four cases were in stage III, 12 in stage IV. Twenty-four patients had urgent situation of SVCS and airway obstruction, 22 patients reached good response after emergency management including COP induction chemotherapy and pleural effusion suction. Twenty-nine cases achieved complete remission (CR) while in 6 patients the disease relapsed. Thirteen patients died from disease progression, relapse or severe infection during chemotherapy. The Kaplan-Meier estimate of 5-year progression-free survival (PFS) was 61% +/- 8% (median follow up 35 months) for these 36 patients.
Establishment of a diagnosis as soon as possible was important to reduce the mortality and improve long term survival of patients. Induction chemotherapy for emergency situation was efficacious. The regimen of T-NHL-CCCG97, T-NHL-2002, and B-NHL-2001 for NHL arising from mediastinum based on pathological classification is feasible.
以纵隔肿块为表现的非霍奇金淋巴瘤（NHL）通常呈进行性发展，可导致严重的呼吸窘迫甚至死亡。本研究旨在总结纵隔起源的NHL的临床特征及预后情况。
本文报道的36例纵隔起源的NHL患者于1999年至2007年间确诊。对其临床特征、病理分类、诊断方法以及不同治疗方案的结果进行回顾性分析。这36例患者中，男性25例，女性11例（男女比例为2.2:1）。平均年龄为7.9岁（范围1 - 12岁）。通过纵隔肿块或外周淋巴结活检进行病理诊断，部分患者根据骨髓或胸腔积液细胞学检查及免疫表型分析确诊。分期采用圣裘德系统。患者根据形态学和免疫表型接受T-NHL-CCCG97、T-NHL-2002或B-NHL-2001方案治疗。出现上腔静脉综合征（SVCS）和/或上纵隔综合征（SMS）的患者接受环磷酰胺（C）、长春新碱（O）和泼尼松（P）诱导化疗1周。
27例患者接受了纵隔肿块或外周淋巴结活检，并通过组织病理学和免疫组化确诊。其中，24例为淋巴母细胞淋巴瘤，3例为间变性大细胞淋巴瘤。9例患者通过骨髓穿刺或胸腔积液细胞学检查确诊。36例均为T细胞型。24例处于Ⅲ期，12例处于Ⅳ期。24例患者存在SVCS和气道梗阻的紧急情况，22例患者在包括COP诱导化疗和胸腔积液抽吸的紧急处理后取得良好反应。29例达到完全缓解（CR），6例疾病复发。13例患者在化疗期间因疾病进展、复发或严重感染死亡。这36例患者的Kaplan-Meier法估计5年无进展生存率（PFS）为61%±8%（中位随访35个月）。
尽早确诊对于降低患者死亡率和提高长期生存率至关重要。紧急情况时的诱导化疗有效。基于病理分类的T-NHL-CCCG97、T-NHL-2002和B-NHL-2001方案用于纵隔起源的NHL是可行的。
