病例報告：巨型囊泡性非亮細胞腎細胞癌合併十二指腸、橫結腸以及降結腸侵犯

李懿文、羅浩倫

高雄長庚紀念醫院泌尿科

 Cases Report: Huge cystic non-clear cell renal cell carcinoma with duodenum, ascending and transverse colon invasion associated with peritonitis

 I-Wen Lee, Hao-Lun Luo

Department of Urology, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan.

Introduction:

Combination (target therapy + immune check point inhibitor) therapy is the recommended first line therapy for clear cell renal cell carcinoma. However, the effect of combination therapy is not clear for non-clear cell renal cell carcinoma.

Case history:

This 29-year-old male with a history of uncontrolled hypertension, huge cystic unclassified RCC with carcinomatosis and ascites formation transiently response to Pazopanib for about 3 months. This time, he suffered from progressive abdominal fullness for 5 days. He also accompanied with dyspnea, vomiting and diarrhea. We arranged abdominal CT disclosing huge right renal mixed solid and cystic tumor (16x12cm in size), extends beyond Gerota's fascia and compresses the ascending colon, transverse colon and 2nd portion of duodenum. Multiple peritoneal cystic nodules and mass with ascites, favor peritoneal carcinomatosis progression.

Emergent laparotomy was performed due to peritoneal sign and septic shock. However, nearly frozen abdomen was found. Therefore, we performed gastro-jeujunostomy and end ileostomy to bypass the obstructed site. During the operation, bowel edematous change with poor circulation and dirty ascites was noted and the laparostomy wound couldn’t be closed due to the space occupying tumor and swelling bowel. We placed bilateral Penrose and covered the wound with sterilized IV bag. This patient suffered from postoperative consciousness disturbance, acute liver failure with total bilirubin level up to 30 mg/dL and persisted intestinal obstruction without any output from ileostomy. After discussed with his family, we shifted Pazopanib to Axitinib plus Nivolumab. After 3 cycles of Axitinib plus Nivolumab. The swollen bowel regression and recovery of ileostomy function. The followed up abdominal CT also demonstrated much regressive change of the right kidney tumor.  After laparotomy wound closure, his liver failure also gradually recovered without any sequalae. Finally, the response duration of Axitinib plus Nivolumab is around one year and patient eventually died of refractory liver metastasis.

Discussion:

Even with the presence of target therapy or immunotherapy, the treatment of non-clear cell RCC still remains a challenge. According to 2020 NCCN guidelines, the standard treatment of stage IV unresectable non-clear cell RCC is clinical trial or systemic therapy. As for systemic therapy, the 2020 NCCN guidelines recommends Sunitinib, Cabozantinib, Everolimus or Lenvatinib + Everolimus. Other regimens such as Axitinib, Nivolumab, Bevacizumab may also be useful in certain circumstances.

 In this case, the huge non-clear cell RCC with carcinomatosis and caused intestinal obstruction with peritonitis. The treatment strategy was bypassing the intestinal obstruction site and shifting to effective therapy. Upfront use of combination therapy (target therapy + immune check point inhibitor) is established evidence for clear cell RCC. Then, we also treated this metastatic non-clear cell RCC with combination therapy (Axitinib-Nivolumab) successfully.  As the dramatic result of tumor regression, we may consider to treat metastatic non-clear cell RCC with combination therapy (target therapy + immune check point inhibitor) in the future.