儿童和青少年侵袭性纤维瘤病：意大利经验。

Aggressive fibromatosis in children and adolescents: the Italian experience.

机构信息

Pediatric Oncology Unit, National Cancer Institute, Milano, Italy.

出版信息

Cancer. 2010 Jan 1;116(1):233-40. doi: 10.1002/cncr.24679.

DOI:10.1002/cncr.24679

PMID:19950127

Abstract

BACKGROUND

Aggressive fibromatosis (AF) is a rare tumor of intermediate malignancy that has a strong potential for local invasiveness and recurrence. To date, there are no general recommendations for the clinical management of pediatric AF.

METHODS

The authors retrospectively analyzed 94 patients aged < or =21 years, including 23 patients who underwent complete surgery (Group I), 42 patients who underwent incomplete surgery with microscopic residual tumor (Group II), and 29 patients who underwent either biopsy or macroscopically incomplete surgery (Group III).

RESULTS

The 5-year event-free survival (EFS) and overall survival rates were 44% and 99%, respectively. Local recurrences developed in 22% of patients in Group I, in 76% of patients in Group II, and in 76% of patients in Group III. Two of 7 patients with abdominal disease died of tumor progression, whereas none of the patients with extra-abdominal AF died of their disease. Systemic treatment was given to 15 patients as first-line treatment and to 34 patients at time the time they developed recurrent disease: The response rate was 47% in the former patients and 50% in the latter patients. Objective responses were observed in 11 of 19 patients who received combined methotrexate plus vinblastine/vinorelbine, in 7 of 15 patients who received alkylating-agent chemotherapy, and in 4 of 11 patients who received other therapies (tamoxifen, sulindac, interferon alfa).

CONCLUSIONS

The current analysis suggested that the clinical course of AF in children may resemble that of AF in adults. Local recurrences did not affect the chance of responding to systemic therapy or the survival rate. The completeness of initial resection was the main factor that influenced EFS, whereas disease control after marginal resection was much the same as that achieved after intralesional surgery/biopsy. Good responses to systemic treatments, and particularly to low-dose chemotherapy, were observed as reported previously in adults.

摘要

背景

侵袭性纤维瘤病（AF）是一种罕见的中度恶性肿瘤，具有很强的局部侵袭性和复发性。迄今为止，尚无针对儿科 AF 临床治疗的一般建议。

方法

作者回顾性分析了 94 名年龄≤21 岁的患者，其中 23 例患者接受了完全手术（I 组），42 例患者接受了显微镜下有肿瘤残留的不完全手术（II 组），29 例患者接受了活检或肉眼上不完全手术（III 组）。

结果

5 年无事件生存率（EFS）和总生存率分别为 44%和 99%。I 组患者中有 22%局部复发，II 组患者中有 76%，III 组患者中有 76%。7 例腹部疾病患者中有 2 例因肿瘤进展而死亡，而无 1 例非腹部侵袭性纤维瘤病患者因疾病而死亡。15 例患者作为一线治疗，34 例患者在复发时接受了系统治疗：前者的缓解率为 47%，后者为 50%。接受甲氨蝶呤联合长春碱/长春新碱、烷化剂化疗、他莫昔芬、舒林酸、干扰素 alfa 治疗的 19 例患者中，有 11 例观察到客观缓解，15 例接受烷化剂化疗的患者中有 7 例，11 例接受其他治疗的患者中有 4 例（他莫昔芬、舒林酸、干扰素 alfa）。