不同細胞形態之雙側腎細胞瘤
吳冠儒1、陳建志1
1台灣基督長老教會馬偕醫療財團法人馬偕紀念醫院泌尿科
Different Cell Type of Bilateral Renal Cell Carcinoma
Kuan-Ju Wu1、Marcelo Chen1
1Department of Urology, MacKay Memorial Hospital, Taipei, Taiwan
Introduction:
Renal cell carcinoma (RCC) was responsible for over 80% of all the primary renal tumor. It was rise from renal cortex. According to previous research, there were about two times amount of RCC incidence occurred in men compared to women. The prevalent age of RCC happened between fifty-five to seventy-five years old. Thus, it was uncommon to find young adults even children to have RCC. Most of the RCCs were found sporadic and very few of them were believed to be familial related. Usually, RCC happened unilateral and less common bilateral lesions. Only 1-4% of patients to be found bilateral renal tumors. Among those patients who had bilateral renal tumor, we can divide into hereditary cause and non-hereditary cause. We share our experience of a 52-year-old female who had different cell type of bilateral renal cell carcinoma.
Case Presentation:
A 52-year-old female who had incidentally found bilateral renal tumor when she was having regular health examination. According to the patient, she had family history of liver nodule and thus was regular having health examination. She had renal echo and was found negative last year. She denied any systemic disease. Also, she denied tobacco used. She came to ER due to flank pain for months. As a result, CT was arranged and showed one 3.7cm right renal mass and a 2.5cm left renal mass which were both suspicious for arising from renal cortex. Renal cell carcinomas were suspected. Then she was referred to our outpatient clinic for further survey. MRI and bone scan were then arranged for staging. MRI showed bilateral renal tumor and bone scan showed no bone metastasis. Thus, she was arranged laparotomy bilateral partial nephrectomy on September 27th, 2022. Right side warm ischemia time was 15 minutes and left side was 9 minutes. Pathology showed right side renal cell carcinoma, clear cell type and the greatest dimension was 4 cm. In the other hand, the pathology of left side tumor was renal cell carcinoma, but the cell type was papillary renal neoplasms with reverse polarity (PRNRP). The greatest dimension of the tumor was 3 cm. Outpatient clinic follow-up showed well wound healing without any active infection sign. Since the tumors were resected completely, we arranged 6 months interval of image follow-up including CT and renal echo to observe of any changes of the patient.
Discussion:
In the different cell type of renal cell carcinoma, clear cell type was responsible for up to 80% of them. The histology of clear cell type was often yellow in color and full of vascular consistent. About the genome mutation were BAP1, PBRM1, VHL, or SETD2 genes which were located on chromosome 3. Among these mutations, von-Hippel Lindau syndrome was a well-known hereditary renal cell carcinoma which often consist of clear cell type of renal cell carcinoma. In our case, the other cell type appears to be papillary renal neoplasms with reverse polarity (PRNRP). This pattern of description was recently used. In previous study, PRNRP was described with about 80% of KRAS missense mutations. This particular cell type of papillary renal cell carcinoma used to call oncocytic or oncocytoid papillary renal cell carcinoma was found to have a better outcome compared to other subtypes of papillary renal cell carcinoma. In previous study, GATA3 and 34βE12 were both positive in PRNRP. In contrast to clear cell renal cell carcinoma (ccRCC), PRNRP was suspected originated in the collecting system or distal convoluted tubule and thus was different from ccRCC. Since the different origin of these two cell type, it was less likely to consider our patient to have hereditary renal cell carcinoma. Also, non-familial bilateral renal cell carcinoma was told to have good overall prognosis. The classification between hereditary and non-familial renal cell carcinoma still need a better outline that could assist physicians making proper treatment plan.