Popadiuk Stefan, Korzon Maria, Chybicka Alicja, Szmyd Krzysztof, Balwierz Walentyna, Trelinska Joanna, Kowalczyk Jerzy, Wisniewska-Slusarz Hanna, Woźniak Wojciech, Bilska Katarzyna, Wachowiak Jacek, Wysocki Mariusz, Krawczuk-Rybak Maryna, Szumera Małgorzata, Sznurkowska Katarzyna, Renke Joanna
Klinika Pediatrii, Gastroenterologii i Onkologii Dzieciecej, Akademia Medyczna, ul. Nowe Ogrody 1-6, 80-803 Gdańsk, Poland.
Med Wieku Rozwoj. 2007 Jul-Sep;11(3 Pt 2):301-6.
The aim of the study was the analysis of risk factors of therapeutic failures in children with malignant germ cell tumours treated within the multicentre programme of PPGGL from 1999--2006.
The investigated group included 18 (14.3%) patients, of 123 who have finished the treatment of malignant germ cell tumour, in whom no remission was obtained or relapse occurred. All the patients were treated according to the TGM 95 programme. Both clinical and morphological data of the group have been analysed.
Among 18 patients with therapeutic failures 12 died. Two patients from the high risk group died of complications of the treatment--sepsis during neutropenia after chemotherapy and one after haemorrhage to the central nervous system. The other 9 died from progression of malignancy, 6 of them belonged to the high risk group. 10 (82%) of 12 patients who died had extragonadal location and in 11 (92%) the tumour was in stage III or IV of the disease. The most frequent histology in this group was mixed germ cell tumour with component of yolk sac tumour or carcinoma embrionale. 92% patients had elevated AFP, in 4 it was above 15000 ng/ml. In 11 (92%) patients primary chemoresistance was observed, and radical surgery was not possible for the reason of advanced stage of the disease. In 6 patients relapse occurred. In 3 patients testis was the primary location (I and II stage), in 3 patients the tumour was localized in the sacrococcygeal region (III and IV stage). All the patients are alive in remission after second line therapy, with 78 months (median) of follow-up.
本研究旨在分析1999年至2006年在PPGGL多中心项目中接受治疗的恶性生殖细胞肿瘤患儿治疗失败的危险因素。
研究组包括123例完成恶性生殖细胞肿瘤治疗的患者中的18例(14.3%),这些患者未获得缓解或出现复发。所有患者均按照TGM 95方案进行治疗。对该组患者的临床和形态学数据进行了分析。
18例治疗失败的患者中有12例死亡。高危组的2例患者死于治疗并发症——化疗后中性粒细胞减少期间的败血症以及1例中枢神经系统出血后死亡。另外9例死于恶性肿瘤进展,其中6例属于高危组。12例死亡患者中有10例(82%)肿瘤位于性腺外,11例(92%)肿瘤处于疾病的III期或IV期。该组中最常见的组织学类型是伴有卵黄囊瘤或胚胎癌成分的混合性生殖细胞肿瘤。92%的患者甲胎蛋白升高,4例高于15000 ng/ml。11例(92%)患者观察到原发性化疗耐药,由于疾病分期较晚无法进行根治性手术。6例患者出现复发。3例患者肿瘤原发于睾丸(I期和II期),3例患者肿瘤位于骶尾区域(III期和IV期)。所有患者在二线治疗后均处于缓解期存活,中位随访时间为78个月。