Inserra A, Silvano A, Morabito A, Boldrini R, Del Nonno F, Boglino C
Divisione di Chirurgia Generale Pediatrica, Ospedale Bambino Gesù, Roma, Italia.
Pediatr Med Chir. 1993 Jan-Feb;15(1):107-10.
A case of retroperitoneal Hemangiopericytoma in a 9 year old boy is presented. Hemangiopericytoma is a rare soft tissue tumor with unpredictable biological behaviour and a high local recurrency rate. Its ubiquity, the different grades of malignancy, the lack of correlation between clinical and histological feature are discussed. It is emphasized the hard preoperative diagnosis of the painless "mass", that can be reached only by histology. Increased cellularity, prominent mitotic activity, and foci of necrosis or hemorrhage suggest a malignant form rather than a benign one. Classifying Hemangiopericytoma among mesenchymal tumors, radical surgery is the treatment of choice whenever possible, associated with pre or post operative chemotherapy according to National Protocol RMS 88.
本文报告一例9岁男孩的腹膜后血管外皮细胞瘤。血管外皮细胞瘤是一种罕见的软组织肿瘤,其生物学行为难以预测,局部复发率高。文中讨论了其普遍性、不同的恶性程度以及临床与组织学特征之间缺乏相关性。强调了对无痛性“肿块”术前诊断困难,只有通过组织学检查才能确诊。细胞增多、有明显的有丝分裂活性以及坏死或出血灶提示为恶性而非良性。将血管外皮细胞瘤归类于间叶组织肿瘤,只要有可能,根治性手术是首选治疗方法,并根据国家RMS 88方案进行术前或术后化疗。