病例報告: 腎臟神經外胚層母細胞瘤併下腔靜脈栓塞及肺轉移
蔡佳穆、余家政、吳東霖
高雄榮民總醫院 外科部 泌尿外科
Primitive neuroectodermal tumor of kindey with IVC thrombosis and lung metastasis
Chia-Mu Tsai, Chia-Cheng Yu, Tong-Lin Wu
Divisions of Urology, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
Abstract: Primitive neuroectodermal tumor (PNET/Ewing sarcoma) is a rare disease, which belongs to Ewing Sarcoma Family of Tumors (ESFT). Here we describe a case of PNET of kidney in a 17-year-old male adolescent, who came to our emergency department due to left flan pain and progressive enlargement of left abdomen. Initially, computed tomography scan revealed a huge cystic mass in left kidney, also magnetic resonance imaging showed IVC thrombosis and adult Wilm’s tumor was suspected. Computed tomography-guided needle biopsy showed primitive neuroectodermal tumor. Patient received neoadjuvant chemotherapy under national cancer institute (NCI) protocol for Ewing sarcoma. Tumor and thrombosis had shrinkage thirty percent and suspicious lung metastatic lesions had vanished. He underwent radical nephrectomy with IVC thrombectomy and completed adjuvant chemotherapy (total 51 weeks) and radiotherapy.
Primitive neuroectodermal tumor is male preponderance and rare in children of Asian descent. PNET is an aggressive malignancy and poor prognosis, especially renal origin, multimodality treatment is usually required. We reported this case because of neoadjuvant chemotherapy was applied and patient is disease free for nearly 2 years.