Shimmoto K, Ushigome S, Nikaido T, Kikuchi Y, Kobayashi N, Yamazaki Y
Department of Pathology, Jikei University School of Medicine, Tokyo, Japan.
Pediatr Hematol Oncol. 1991 Apr-Jun;8(2):147-57. doi: 10.3109/08880019109033442.
A case is reported of Wilms' tumor associated with multiple pulmonary metastases histologically showing maturation of the tumor cells at 9 years after the resection of the primary tumor and intensive therapy. A huge tumor of a 22-month-old patient's right kidney was resected. The tumor was diagnosed as Wilms' tumor of mesenchymal type (stage 1), which consisted of predominantly immature mesenchymal tissue including rhabdomyoblasts, smooth muscle and fibrous tissue, and few blastemal and epithelial components. Intensive preoperative and postoperative chemotherapy with actinomycin D and vincristine and postoperative irradiation therapy totaling 16 Gy were carried out. The patient was regularly followed up uneventfully until 9 years after the surgery. On routine chest x ray at the age of 10 years 11 months, multiple pulmonary nodules were found. The excised nodules from the bilateral lungs disclosed similar histology, exclusively composed of dense collagen bundles and fibrocytes intermingled with mature striated muscle bundles. No immature tumor components were detected. The possible effect of intensive therapy in this maturation was stressed, although spontaneous benign differentiation of tumor cells cannot be excluded.
报告了一例与多发肺转移相关的肾母细胞瘤病例,组织学显示在原发肿瘤切除及强化治疗9年后肿瘤细胞成熟。一名22个月大患者的右肾巨大肿瘤被切除。该肿瘤被诊断为间充质型肾母细胞瘤(1期),主要由包括横纹肌母细胞、平滑肌和纤维组织的未成熟间充质组织组成,仅有少量胚芽和上皮成分。术前及术后进行了强化化疗,使用放线菌素D和长春新碱,术后放疗总量为16Gy。患者术后定期随访,直至术后9年情况平稳。在10岁11个月时进行常规胸部X光检查,发现多个肺结节。从双侧肺切除的结节显示出相似的组织学特征,仅由密集的胶原束和纤维细胞组成,并夹杂着成熟的横纹肌束。未检测到未成熟肿瘤成分。强调了强化治疗在这种成熟过程中的可能作用,尽管不能排除肿瘤细胞的自发良性分化。