無原位腎臟腫瘤之轉移性乳突狀腎臟細胞癌之治療經驗及追蹤

董劭偉¹、何承勳²

¹台北醫學大學附設雙和醫院 泌尿科，²新光吳火獅紀念醫院外科部泌尿科

Metastatic papillary renal cell carcinoma without primary renal tumor: treatment experience and follow up

Shao-Wei Dong¹, Chen-Hsun Ho ²

¹Department of Urology, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan

²Division of Urology, Department of Surgery, Shin Kong WHS Memorial Hospital, Taipei, Taiwan

A 56-year-old man with underlying of hepatitis B accompanied by acute abdominal pain this year.

He visited emergency department and abdominal and pelvic Computed Tomography (CT) scan accidentally found multiple paraaortic and retrocural lymph node enlargement suspected lymphoma and metastatic carcinoma with multiple metastatic lymphadenopathy.

We performed lymph node CT guide biopsy and laparoscopy lymph node dissection. Pathology showed the morphology and immunoprofile could be compatible with papillary renal cell carcinoma. And whole-body CT and Positron Emission Tomography scan showed large sellar mass with suprasellar extension and no other metastatic lesion. Due to the diplopia progressive was also noted, the patient underwent trans-sphenoidal surgery and pathology revealed pituitary adenoma. Renal CT guide biopsy was performed but pathology showed no renal cell carcinoma.

Papillary renal cell carcinoma accounts for about 10-15% of all renal cell carcinomas. There was much previously case report about papillary renal cell carcinoma. To our knowledge, this is the first reported case of metastatic papillary renal cell carcinoma without primary renal tumor.

This patient was received CABOMETYX ^® 60mg QD (cabozantinib: receptor tyrosine kinase inhibitor with activity against MET, VEGFR2, FLT3, c-KIT, and RET) and close follow up at our out-patient department.

We follow up laboratory examination of blood every one month and abdominal and pelvic CT every three month. Due to the side effect of hand-foot symdrome, reduce the dose to 40mg and 20mg QD alternately. GOT and GPT was mild elevated in first six months. Lymph node was shrinkage in first CT follow up and stationary in second CT follow up. There was no significant changes or uncomfortable until seven months after CABOMETYX ^® used. Body weight increased and abdominal fullness was noted. Therefore, we performed abdominal and pelvic CT and showed liver lesion and massive ascites. Pigtail was inserted to drainage ascites and liver biopsy was done. Pathology revealed liver metastasis. GOT/GPT, bilirubin and creatine was elevated dramatically. Emergency hemodialysis and plasma exchange was performed. KEYTRUDA® (pembrolizumab) 200mg Q3W was prescribed but patient was expired after first dose.