双侧肾透明细胞肉瘤

Bilateral clear cell sarcoma of the kidney.

作者信息

Zekri Wael, Yehia Dina, Elshafie Maged M, Zaghloul Mohamed Saad, El-Kinaai Naglaa, Taha Hala, Refaat Amal, Younes Alaa A, Alfaar Ahmad Samir

机构信息

Pediatric Oncology Department, Children's Cancer Hospital Egypt 57357, Cairo, Egypt; National Cancer Institute, Cairo University, Cairo, Egypt.

Research Department, Children's Cancer Hospital Egypt 57357, Cairo, Egypt; Faculty of Science, Cairo University, Cairo, Egypt.

出版信息

J Egypt Natl Canc Inst. 2015 Jun;27(2):97-100. doi: 10.1016/j.jnci.2015.03.002. Epub 2015 Apr 28.

DOI:10.1016/j.jnci.2015.03.002

PMID:25934445

Abstract

Clear cell sarcoma of the kidney (CCSK) accounts for 2-5% of all pediatric renal malignancies, and is known for its propensity to metastasize to bone and other sites. We are reporting two cases with bilateral CCSK that were diagnosed at our institution. One patient initially presented with bilateral renal masses, as well as pulmonary, hepatic and bone metastasis; while other present only with bilateral masses with no evident distant metastasis. Both patients received aggressive neo-adjuvant chemotherapy to decrease tumor size. One patient completed his designated treatment and initially showed complete remission (CR); eventually suffering from relapse. The other patient's tumor progressed during the course of chemotherapy. Both cases manifested brain dissemination at the time of relapse or progression. This emphasizes the importance of staging stratification in CCSK. This also illustrates CCSK's ability to metastasize to bone and other sites including the brain (a primary relapse site in our cases).

摘要

肾透明细胞肉瘤（CCSK）占所有儿童肾恶性肿瘤的2%-5%，以易于转移至骨骼和其他部位而闻名。我们报告了在我们机构诊断出的两例双侧CCSK病例。一名患者最初表现为双侧肾肿块，以及肺、肝和骨转移；而另一名患者仅表现为双侧肿块，无明显远处转移。两名患者均接受了积极的新辅助化疗以减小肿瘤大小。一名患者完成了指定治疗，最初显示完全缓解（CR）；最终复发。另一名患者的肿瘤在化疗过程中进展。两例病例在复发或进展时均出现脑转移。这强调了CCSK分期分层的重要性。这也说明了CCSK转移至骨骼和其他部位（包括脑，在我们的病例中是主要复发部位）的能力。