Klekawka Tomasz, Balwierz Walentyna, Moryl-Bujakowska Angelina, Stanuch Helena, Matysiak Michał, Rokicka-Milewska Roma, Sopyło Barbara, Kołakowska-Mrozowska Beatrycze, Krenke Katarzyna, Chybicka Alicja, Chaber Radosław, Sońita-Jakimczyki Danuta, Janik-Moszants Anna, Wachowiak Jacek, Kaczmarek-Kanold Małgorzata, Kowalczyk Jerzy, Odój Teresa, Balcerska Anna, Adamkiewicz-Drozyińska Elibieta, Wysocki Mariusz, Koltan Andrzej, Krawczuk-Rybako Maryna, Muszyńska-Rosłan Katarzyna, Stolarska Małgorzata
Klinik Onkologii i Hematologii Dzieciecej Polsko-Amerykańskiego Instytutu Pediatrii Collegium Medicum Uniwersytetu Jagiellońskiego w Krakowie.
Przegl Lek. 2006;63(1):21-4.
Prognostic significance of residual mediastinal tumor mass in children treated for HD as well as the choice of the optimal management of these cases still remains unknown. In years 1994-2001 in 10 PPLLSG participating centers 480 children (age 2-19.7 years) were treated for HD (stages I-IV). In 338 cases initial mediastinal/lung hilus involvement was present. All patients with initial mediastinal/lung hilus involvement were treated with multidrug chemotherapy combined with involved field radiotherapy. In five cases remission was not achieved. Complete remission (CR) was achieved in 226 patients and uncertain complete remission (UCR) in 107 patients, in whom after completion of planned treatment residual changes in mediastinum/lung hilus were identified in radiological examinations. Twenty four children with persistent mediastinal tumor underwent thoracoscopy or thoracotomy. In only one case histopathological examination revealed the presence of neoplastic cells in mediastinal mass tissue, in 2 another cases cystic changes in mediastinum were present, in one case thymic tissue was identified, necrotic tissue was present in 20 cases. Among 107 children with residual mediastinal tumor and 226 patients with CR achieved, relapses occurred in 6 and 18 patients respectively. Over 5-year relapse-free survival was 92.4% and 91.3% respectively. Patients with the presence of mediastinal/lung hilus tumor after the completion of the treatment do not have an increased risk of relapse, but before the completion of therapy they require careful, clear-sighted and repeated examinations including computed tomography (CT), magnetic resonance imaging (MRI) and especially positron emission tomography (PET) to evaluate the nature of persistent lesions. Only in clinically and radiologically doubtful cases tumor biopsy with subsequent histopatological examination should be performed.
霍奇金淋巴瘤(HD)患儿纵隔残留肿瘤块的预后意义以及这些病例的最佳治疗选择仍不清楚。1994年至2001年期间,10个参加北欧儿科淋巴瘤研究组(PPLLSG)的中心对480例(年龄2至19.7岁)HD(I - IV期)患儿进行了治疗。其中338例最初有纵隔/肺门受累。所有最初纵隔/肺门受累的患者均接受了多药化疗联合受累野放疗。5例未达到缓解。226例患者达到完全缓解(CR),107例患者达到不确定完全缓解(UCR),这些患者在完成计划治疗后,影像学检查发现纵隔/肺门有残留改变。24例纵隔肿瘤持续存在的患儿接受了胸腔镜检查或开胸手术。只有1例组织病理学检查显示纵隔肿块组织中有肿瘤细胞,另外2例纵隔有囊性改变,1例发现胸腺组织,20例有坏死组织。在107例纵隔残留肿瘤患儿和226例达到CR的患者中,分别有6例和18例复发。5年无复发生存率分别为92.4%和91.3%。治疗结束后存在纵隔/肺门肿瘤的患者复发风险没有增加,但在治疗结束前,他们需要仔细、清晰且反复地进行检查,包括计算机断层扫描(CT)、磁共振成像(MRI),尤其是正电子发射断层扫描(PET),以评估持续病变的性质。仅在临床和影像学诊断存疑的病例中,才应进行肿瘤活检及后续组织病理学检查。