Alman B A, Goldberg M J, Naber S P, Galanopoulous T, Antoniades H N, Wolfe H J
Department of Orthopaedics, New England Medical Center, Boston, Massachusetts.
J Pediatr Orthop. 1992 Jan;12(1):1-10.
Ten patients with aggressive fibromatosis of the extremities were prospectively followed for 2-6 years. Results of treatment methods were compared. Five patients underwent three-dimensional imaging with and without intravenous contrast, and the images were compared with the anatomic extent of the resected lesion. Pathologic specimens and control tissue were tested for the presence of estrogen and progesterone receptors and for expression of the c-sis oncogene and platelet-derived growth factor (PDGF), potent mitogens for fibrocytes. Wider surgical resection resulted in a lower recurrence rate, but current chemotherapeutic agents were not effective in eradicating the tumors. Intravenous contrast enhanced the lesions. Two thirds of the tumors tested had estrogen or progesterone receptors. All tumors tested had inappropriate expression of c-sis and PDGF. This inappropriate expression may be responsible for the underlying pathobiology and deregulation of control of growth in aggressive fibromatosis.
对10例四肢侵袭性纤维瘤病患者进行了为期2至6年的前瞻性随访。比较了治疗方法的结果。5例患者接受了有无静脉造影剂的三维成像，并将图像与切除病变的解剖范围进行了比较。对病理标本和对照组织进行雌激素和孕激素受体检测，以及c-sis癌基因和血小板衍生生长因子（PDGF，成纤维细胞的强效有丝分裂原）表达检测。更广泛的手术切除导致较低的复发率，但目前的化疗药物对根除肿瘤无效。静脉造影剂增强了病变。三分之二的检测肿瘤有雌激素或孕激素受体。所有检测的肿瘤均有c-sis和PDGF的异常表达。这种异常表达可能是侵袭性纤维瘤病潜在病理生物学和生长控制失调的原因。
