Köhle Olivia, Abt Dominik, Rothermundt Christian, Öhlschlegel Christian, Brugnolaro Christiane, Schmid Hans-Peter
Department of Urology, St. Gallen Cantonal Hospital , Switzerland.
Department of Oncology and Hematology, St. Gallen Cantonal Hospital , Switzerland.
Rare Tumors. 2015 Mar 31;7(1):5635. doi: 10.4081/rt.2015.5635. eCollection 2015 Feb 11.
Soft tissue sarcomas are rare mesenchymal tumors. Amongst others, primitive neuroectodermal tumors (PNET) of the kidney and synovial sarcoma of the kidney belong to the group of soft tissue sarcomas. Synovial sarcomas can occur almost anywhere in the body, most frequently, however, in the lower (62%) or upper extremities (21%). Metastases occur in 50-70% of cases, and thus the prognosis is poor. PNETs are rare, highly aggressive neoplastic lesions which mainly occur in the torso or axial skeleton in young adults. The prognosis is poor with a 5-year disease-free survival rate of 45-55%. The primary therapeutic approach is surgical resection. Most randomized studies assessing adjuvant chemotherapy for all types of localized soft tissue sarcomas did not show statistically significantly better overall survival times after chemotherapy, although they did show longer progression-free survival. We report on two cases of primary renal synovial sarcoma and one case of PNET of the kidney.
软组织肉瘤是罕见的间叶组织肿瘤。其中，肾原始神经外胚层肿瘤（PNET）和肾滑膜肉瘤属于软组织肉瘤范畴。滑膜肉瘤几乎可发生于身体的任何部位，但最常见于下肢（62%）或上肢（21%）。50 - 70%的病例会发生转移，因此预后较差。PNET罕见，是具有高度侵袭性的肿瘤性病变，主要发生于年轻人的躯干或中轴骨骼。预后较差，5年无病生存率为45 - 55%。主要治疗方法是手术切除。大多数评估各类局限性软组织肉瘤辅助化疗的随机研究并未显示化疗后总体生存时间有统计学上的显著改善，尽管化疗确实显示出更长的无进展生存期。我们报告了两例原发性肾滑膜肉瘤和一例肾PNET病例。
