Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, 55905, MN, USA.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, 55905, MN, USA.
Hum Pathol. 2021 Jan;107:46-57. doi: 10.1016/j.humpath.2020.10.013. Epub 2020 Nov 6.
Pelvicalyceal system (PS) involvement by renal cell carcinoma (RCC) is staged as pT3a disease (American Joint Committee on Cancer [AJCC], 8th edition). As papillary RCC (PRCC) has been infrequently represented in studies looking at the prognostic impact of PS involvement, we reviewed our institutional cohort of 8225 cases for PS involvement by PRCC. Nine such cases were subjected to histopathologic review and immunohistochemistry. Fluorescence in situ hybridization for TFE3/TFEB alterations was performed if indicated. One case each (1 of 9, 11%) was classified as TFE3-rearranged and FH-deficient RCC. The majority were high grade (World Health Organization/International Society of Urologic Pathology grade 3: 8 of 9, 89%) or had features of aggressive disease, including hilar fat (6 of 9, 67%) and regional lymph node involvement (5 of 7, 71%). One low-grade 3.3-cm tumor with isolated PS involvement with a germline heterozygous FH p.Lys477dup alteration with retained FH, lack of increased S-(2-succino)-cysteine expression, BRAF V600E immunohistochemistry positivity, and lack of trisomy 7/17 on chromosomal microarray was identified, arguing against an FH-deficient and conventional PRCC. Our study shows that PS involvement by renal neoplasia with papillary architecture is a rare event. Aside from PRCC, it is important to note that these may include other aggressive and nonaggressive subtypes of renal neoplasia with papillary architecture. One case of isolated PS involvement by a low-grade, noninvasive tumor that we refer to as nephrogenic papillary neoplasm was identified. At present, there are insufficient data to stage such tumors as pT3a (AJCC, 8th edition), and additional studies are needed to address this question.
肾盂肾盏系统（PS）受累的肾细胞癌（RCC）分期为 pT3a 期（美国癌症联合委员会 [AJCC]，第 8 版）。由于乳头状 RCC（PRCC）在研究 PS 受累对预后影响的研究中很少被报道，我们回顾了我们机构的 8225 例病例中 PS 受累的 PRCC。9 例进行了组织病理学检查和免疫组织化学检查。如果需要，进行 TFE3/TFEB 改变的荧光原位杂交。1 例（9 例中的 1 例，11%）被归类为 TFE3 重排和 FH 缺失的 RCC。大多数为高级别（世界卫生组织/国际泌尿病理学会分级 3：9 例中的 8 例，89%）或具有侵袭性疾病的特征，包括肾门脂肪（9 例中的 6 例，67%）和区域淋巴结受累（7 例中的 5 例，71%）。1 例低级别 3.3cm 肿瘤，孤立性 PS 受累，伴有种系杂合性 FH p.Lys477dup 改变，保留 FH，缺乏增加的 S-（2-琥珀酰）半胱氨酸表达，BRAF V600E 免疫组化阳性，染色体微阵列无三体 7/17，排除 FH 缺失和传统的 PRCC。我们的研究表明，具有乳头状结构的肾肿瘤 PS 受累是一种罕见事件。除了 PRCC，还需要注意的是，这些可能包括其他具有乳头状结构的侵袭性和非侵袭性肾肿瘤亚型。我们鉴定了 1 例孤立性 PS 受累的低级别、非浸润性肿瘤，我们称之为肾源性乳头状肿瘤。目前，尚无足够的数据将此类肿瘤分期为 pT3a（AJCC，第 8 版），需要进一步研究来解决这个问题。
