Peckova Kvetoslava, Martinek Petr, Pivovarcikova Kristyna, Vanecek Tomas, Alaghehbandan Reza, Prochazkova Kristyna, Montiel Delia Perez, Hora Milan, Skenderi Faruk, Ulamec Monika, Rotterova Pavla, Daum Ondrej, Ferda Jiri, Davidson Whitney, Ondic Ondrej, Dubova Magdalena, Michal Michal, Hes Ondrej
Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
Department of Pathology, University of British Columbia, Royal Columbian Hospital, Vancouver, Canada.
Ann Diagn Pathol. 2017 Feb;26:23-30. doi: 10.1016/j.anndiagpath.2016.10.007. Epub 2016 Oct 20.
Conflicting data have been published on the prognostic significance of tumor necrosis in papillary renal cell carcinoma (PRCC). Although the presence of necrosis is generally considered an adverse prognostic feature in PRCC, we report a cohort of 10 morphologically distinct cystic and extensively necrotic PRCC with favorable biological behavior. Ten cases of type 1 PRCC with a uniform morphologic pattern were selected from the 19 500 renal tumors, of which 1311 were PRCCs in our registry. We focused on precise morphologic diagnosis supported by immunohistochemical and molecular-genetic analysis. Patients included 8 men and 2 women with an age range of 32-85 years (mean, 62.6 years). Tumor size ranged from 6 to 14 cm (mean, 9.4 cm). Follow-up data were available in 7 patients, ranging from 0.5 to 14 years (mean, 4 years). All tumors were spherical, cystic, and circumscribed by a thick fibrous capsule, filled with hemorrhagic/necrotic contents. Limited viable neoplastic tissue was present only as a thin rim in the inner surface of the cyst wall, consistent with type 1 PRCC. All cases were positive for AMACR, OSCAR, CAM 5.2, HIF-2, and vimentin. Chromosome 7 and 17 polysomy was found in 5 of 9 analyzable cases, 2 cases demonstrated chromosome 7 and 17 disomy, and 1 case showed only chromosome 17 polysomy. Loss of chromosome Y was found in 5 cases, including 1 case with disomic chromosomes 7 and 17. No VHL gene abnormalities were found. Papillary renal cell carcinoma type 1 can present as a large hemorrhagic/necrotic unicystic lesion with a thick fibroleiomyomatous capsule. Most cases showed a chromosomal numerical aberration pattern characteristic of PRCC. All tumors followed a nonaggressive clinical course. Large liquefactive necrosis should not necessarily be considered an adverse prognostic feature, particularly in a subset of type 1 PRCC with unilocular cysts filled with necrotic/hemorrhagic material.
关于肿瘤坏死在乳头状肾细胞癌（PRCC）中的预后意义，已发表的数据存在冲突。尽管坏死的存在通常被认为是PRCC的不良预后特征，但我们报告了一组10例形态学上不同的囊性且广泛坏死的PRCC，其生物学行为良好。从19500例肾肿瘤中选出10例具有统一形态模式的1型PRCC，在我们的登记处中，其中1311例为PRCC。我们专注于在免疫组织化学和分子遗传学分析支持下的精确形态学诊断。患者包括8名男性和2名女性，年龄范围为32 - 85岁（平均62.6岁）。肿瘤大小为6至14厘米（平均9.4厘米）。7例患者有随访数据，范围为0.5至14年（平均4年）。所有肿瘤均为球形、囊性，由厚纤维包膜包绕，充满出血性/坏死性内容物。仅在囊壁内表面有一层薄的存活肿瘤组织，符合1型PRCC。所有病例AMACR、OSCAR、CAM 5.2、HIF - 2和波形蛋白均呈阳性。9例可分析病例中有5例发现7号和17号染色体多体性，2例显示7号和17号染色体二体性，1例仅显示17号染色体多体性。5例发现Y染色体缺失, 包括1例7号和17号染色体二体性病例。未发现VHL基因异常。1型乳头状肾细胞癌可表现为带有厚纤维平滑肌瘤样包膜的大的出血性/坏死性单囊肿性病变。大多数病例显示出PRCC特征性的染色体数目畸变模式。所有肿瘤临床过程均不具侵袭性。大的液化性坏死不一定应被视为不良预后特征，特别是在一部分具有充满坏死/出血性物质的单房囊肿的1型PRCC中。
