Dymock R B, Allen P W, Stirling J W, Gilbert E F, Thornbery J M
Am J Surg Pathol. 1987 Apr;11(4):263-71.
Giant cell fibroblastoma, which was first described by Shmookler and Enzinger in 1983, is a rare fibroblastic tumor occurring mainly in male patients younger than 10 years of age. Only 28 cases have been reported so far. This paper describes seven new cases which were referred in consultation between 1968 and 1985. Five of the seven patients were male; their ages at the time of first surgery ranged from 2 to 18 years, but six of the seven were younger than 4 years. Tumors were all superficial and were situated in the chest (2 tumors), neck, axilla, scrotum, thigh, and finger; they generally grew slowly, were poorly circumscribed, and measured from 1 to 5 cm in maximum dimensions. Grossly, they were described variously as gray, gelatinous, firm, white, fibrous, pink, and watery. Histologically, there were varying proportions of moderately cellular solid areas and angiectoid areas, both featuring distinctive fibroblastic cells with what appeared to be multiple nuclei arranged in florets or wreaths. By electron microscopy, each wreath proved to be an excessively convoluted, but single, multilobed nucleus. The nuclei of the tumor cells were slightly hyperchromatic and not bizarre or pleomorphic. In follow-up times of from 12 months to 11 years (median 31 months), only one tumor recurred locally and none metastasized. The comparatively low recurrence rate in this series may well increase if the patients are followed for a longer period. Giant cell fibroblastoma should probably be classified with other nonmetastasizing, locally recurring fibroblastic proliferations of youth and childhood such as juvenile aponeurotic fibroma and recurring digital fibrous tumor of infancy.
巨细胞成纤维细胞瘤于1983年由施穆克勒和恩津格首次描述，是一种罕见的成纤维细胞肿瘤，主要发生于10岁以下男性患者。迄今为止，仅报道了28例。本文描述了1968年至1985年间会诊的7例新病例。7例患者中有5例为男性；首次手术时他们的年龄在2至18岁之间，但7例中有6例年龄小于4岁。肿瘤均位于体表，分别位于胸部（2例）、颈部、腋窝、阴囊、大腿和手指；它们通常生长缓慢，边界不清，最大直径为1至5厘米。大体上，它们被描述为灰色、胶冻状、坚实、白色、纤维性、粉红色和水样。组织学上，中等细胞密度的实性区域和血管样区域比例各异，两者均具有独特的成纤维细胞，其细胞核似乎多个呈小花状或环状排列。通过电子显微镜观察，每个环证明是一个过度卷曲但单一的多叶核。肿瘤细胞的细胞核轻度深染，无怪异或多形性。随访时间为12个月至11年（中位时间31个月），仅1例肿瘤局部复发，无转移。如果对患者进行更长时间的随访，本系列中相对较低的复发率很可能会增加。巨细胞成纤维细胞瘤可能应与其他非转移性、局部复发的青少年和成纤维细胞增殖性疾病，如青少年腱膜纤维瘤和婴儿复发性指状纤维瘤归为一类。
