Bień Ewa, Godziński Jan, Balcerska Anna, Rapała Małgorzata, Izycka-Swieszewska Ewa, Stachowicz-Stencel Teresa, Sulka Wojciech, Kazanowska Bernarda, Reich Adam, Chybicka Alicja, Madziara Wojciech, Bohosiewicz Janusz, Perek-Polnik Marta, Perek Danuta, Mańkowski Przemysław, Jankowski Andrzej, Nurzyńska-Flak Joanna, Kowalczyk Jerzy, Kurylak Andrzej, Wysocki Mariusz, Rybczyńska Aleksandra, Wachowiak Jacek, Zalewska-Szewczyk Beata, Bodalski Jerzy, Jaśkiewicz Kazimierz
Klinika Pediatrii, Hematologii, Onkologii i Endokrynologii, Akademia Medyczna, Debinki 7, 80-211 Gdańsk, Poland.
Med Wieku Rozwoj. 2004 Apr-Jun;8(2 Pt 1):145-58.
Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas.
To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms.
The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002. On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients).
Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease. Primary complete resection (PRC) was performed in 7 patients, not resulting in local control in any of them. Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all. 7 patients relapsed, 3 never entered clinical remission. 9 of the 10 patients of group I, died of disease progression. Group II: PRC was performed in 5 patients and led to local control in 2. Adjuvant RTX was used in 2 and CHT in 4 patients. 2 children relapsed and died of the disease. Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free. Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4. Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression.
The effectiveness of primary complete resection in all groups was doubtful. The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory. The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease). Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression. Prognosis in patients with haemangioendothelioma is intermediate, however the role of immunotherapy should be further investigated.
恶性血管肿瘤是一组罕见的肿瘤，通常按照软组织肉瘤的治疗方案进行治疗。
评估波兰患恶性血管肿瘤儿童的临床特征、病程及预后。
波兰儿科罕见肿瘤研究对1992年至2002年间登记的32例血管肿瘤儿童的多中心数据进行了回顾性分析。根据肿瘤的组织学类型，这些患者被分为三组：第一组——10例血管肉瘤（ASA）患者，第二组——7例血管内皮瘤（HE）儿童，第三组——15例血管外皮细胞瘤（HP）患者，包括婴儿型（7例）和成人型（8例）。
第一组：5例表现为局部病变，3例为全身病变，2例为区域病变。7例患者进行了原发灶完全切除（PRC），但均未实现局部控制。5例患者接受了放疗（RTX），所有患者均接受了化疗（CHT）。7例患者复发，3例从未进入临床缓解期。第一组的10例患者中有9例死于疾病进展。第二组：5例患者进行了PRC，2例实现了局部控制。2例患者使用了辅助RTX，4例患者使用了辅助CHT。2例儿童复发并死于该疾病。婴儿型HP：3例儿童进行了PRC，其余4例患者接受了辅助CHT。所有儿童均进入完全缓解期且无疾病。成人型HP：5例患者进行了PRC，4例实现了局部控制。5例儿童接受了辅助RTX，6例接受了辅助CHT。3例患者复发并死于疾病进展。
所有组中原发灶完全切除的有效性存疑。转移复发率高表明目前给予的全身治疗并不令人满意。唯一预后良好的肿瘤是婴儿型HP（所有患者均存活且无疾病）。血管肉瘤患儿的适当治疗方法仍不明确——10例患者中有9例死于疾病进展。血管内皮瘤患者的预后中等，然而免疫治疗的作用应进一步研究。
