Stachowicz-Stencel Teresa, Bień Ewa, Stefanowicz Joanna, Połczytńska Katarzyna, Sierota Danuta, Szołkiewicz Anna, Drozyńska Elzbieta, Kosiak Wojciech, Stankiewicz Czesław, Pietniczka Mirosława, Kukwa Andrzej, Czauderna Piotr, Balcerska Anna
Klinika Pediatrii, Hematologii, Onkologii i Endokrynologii Akademii Medycznej w Gdańsku.
Med Wieku Rozwoj. 2005 Jul-Sep;9(3 Pt 2):487-94.
Soft tissue sarcomas (MTM) localized within the nonorbital and non-parameningeal head and neck region in children are associated with favourable prognosis. However in our material we have observed many therapeutic failures in this group of patients. The aim of the study was to analyze the reasons for disappointing results of oncological therapy in children with MTM treated between 1992 and 2004.
Nine children (M/F: 6/3; mean age 5,5 years). Five patients were diagnosed with rhabdomyosarcoma, four--with non-rhlabdomyosarcoma, including: angiosarcoma, malignant triton tumour, fibrosarcoma and leiomyosarcoma.
mean duration of initial symptoms was 7 months. The first sign of the neoplastic disease in all children was tumour. In as many as five of nine patients initially a false histopathological diagnosis was made based on material obtained from aspiration biopsy of the tumour performed in non-oncological centres. This resulted in a significant delay of the proper diagnosis of malignant disease ranging from 2 to 15 months (mean 7 months). That is why all our patients presented with highly advanced stages of MTM or even in the phase of disease recurrence. Therapy was conducted according to the schemes: MMT-89, CWS-91, CWS-96 and CWS-2002. Durable complete remission after the first line therapy was obtained in one child only. Six patients developed MTM relapse and two--progression during chemotherapy. Finally five children remain disease-free after treatment termination with follow-up of 1 to 1,5 years. Four of then had microscopically complete delayed resection of the tumour or the relapse. Four patients died of neoplasm recurrence and progression. In three of them the proper diagnosis was delayed significantly and they were diagnosed in the first or even second relapse of the tumour.
Unfavourable course and treatment results in MTM located in nonorbital and nonparameningeal head and neck region in our patients result from initial wrong histopathological diagnosis and delayed therapy institution.
儿童非眼眶及非脑膜旁头颈部区域的软组织肉瘤（MTM）预后良好。然而，在我们的病例中，我们观察到这组患者中有许多治疗失败的情况。本研究的目的是分析1992年至2004年间接受治疗的儿童MTM肿瘤治疗结果令人失望的原因。
9名儿童（男/女：6/3；平均年龄5.5岁）。5例诊断为横纹肌肉瘤，4例为非横纹肌肉瘤，包括：血管肉瘤、恶性蝾螈瘤、纤维肉瘤和平滑肌肉瘤。
初始症状的平均持续时间为7个月。所有儿童肿瘤性疾病的首个体征均为肿瘤。9例患者中有多达5例最初基于在非肿瘤中心对肿瘤进行穿刺活检所获得的材料做出了错误的组织病理学诊断。这导致恶性疾病的正确诊断显著延迟，延迟时间为2至15个月（平均7个月）。这就是为什么我们所有的患者均呈现MTM的高度进展期，甚至处于疾病复发阶段。治疗按照MMT - 89、CWS - 91、CWS - 96和CWS - 2002方案进行。仅1名儿童在一线治疗后获得了持久的完全缓解。6例患者出现MTM复发，2例在化疗期间病情进展。最终，5名儿童在治疗结束后无疾病生存，随访1至1.5年。其中4例在显微镜下对肿瘤或复发灶进行了完全延迟切除。4例患者死于肿瘤复发和进展。其中3例的正确诊断被显著延迟，他们在肿瘤首次甚至第二次复发时才被诊断出来。
我们患者中位于非眼眶及非脑膜旁头颈部区域的MTM病程及治疗结果不佳是由于最初错误的组织病理学诊断和延迟的治疗开始。
