Ferigo N, Cottalorda J, Allard D, Gentil-Perret A, Fessy M, Berger C, Stéphan J L
Pediatric Hematology and Oncology Unit, Hôpital Nord, University of Saint Etienne, Saint Etienne 42055, France.
J Pediatr Hematol Oncol. 2006 Apr;28(4):237-40. doi: 10.1097/01.mph.0000212903.61276.4b.
Hemangiopericytoma (HPC) is a soft-tissue neoplasm composed of proliferating capillary pericytes. It has variable and unpredictable malignancy and most commonly occurs in the fifth or sixth decade of life. Diagnosis is based on the histological aspect. HPC is exceedingly rare in childhood. In both adults and children, curative surgery is the most important predictor of survival. The place of chemotherapy in the treatment of HPC is not well established. We describe a case of adult-type metastatic HPC of the thigh in a 13-year-old boy. The response to neoadjuvant chemotherapy was excellent, and local control of this initially unresectable tumor was achieved without radiation therapy or mutilating surgery. The child is alive and well and has had 8 years of follow-up after treatment.
血管外皮细胞瘤（HPC）是一种由增殖的毛细血管周细胞组成的软组织肿瘤。其恶性程度多变且难以预测，最常见于50或60岁。诊断基于组织学表现。HPC在儿童期极为罕见。在成人和儿童中，根治性手术都是生存的最重要预测因素。化疗在HPC治疗中的地位尚未明确。我们报告一例13岁男孩发生的大腿成人型转移性HPC。新辅助化疗反应良好，在未进行放疗或致残性手术的情况下实现了对这个最初无法切除肿瘤的局部控制。该患儿目前存活且状况良好，治疗后已随访8年。
