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极性反转的乳头状肾肿瘤:一例罕见肾肿瘤病例报告

Papillary renal neoplasm with reversed polarity: A case report of a rare renal tumor.

作者信息

Kannan Swetha, Akram Wania Mohammad, Elmussareh Muhammad, Saleh Rana

机构信息

Final Year Medical Students, Gulf Medical University, United Arab Emirates.

Final Year Medical Students, Gulf Medical University, United Arab Emirates.

出版信息

Int J Surg Case Rep. 2025 Aug;133:111517. doi: 10.1016/j.ijscr.2025.111517. Epub 2025 Jun 14.

Abstract

INTRODUCTION AND IMPORTANCE

Papillary Renal Neoplasm with Reversed Polarity (PRNRP) is a rare renal tumor first described in 2019. It is characterized by low-grade nuclei, eosinophilic cytoplasm, and an inverted nuclear arrangement. Unlike papillary renal cell carcinoma (PRCC), PRNRP frequently harbors KRAS mutations and lacks the typical trisomies of chromosomes 7 and 17. Histopathology and immunohistochemistry remain the gold standard for diagnosis.

CASE PRESENTATION

A 52-year-old woman with Type 2 Diabetes Mellitus, hypertension, and chronic kidney disease presented with worsening renal function. Imaging revealed a Bosniak type IV cyst in the right kidney, raising suspicion for malignancy. She underwent a robotic partial nephrectomy, and histopathology confirmed PRNRP (pT1a, WHO/ISUP grade 1). The tumor was completely resected, and no recurrence or metastasis was noted. PRNRP poses diagnostic challenges due to its rarity and overlap with other renal tumors. It is often discovered incidentally, with imaging offering limited preoperative specificity. Histopathologic criteria include thin papillary structures, interstitial vitrification, eosinophilic cytoplasm, and nuclear reversal. PRNRP follows an indolent course, with no reported cases of recurrence or metastasis post-resection.

CLINICAL DISCUSSION

Papillary Renal Neoplasm of Reversed Polarity (PRNRP) is a rare variant of renal cell carcinoma characterized by its distinct histological feature of reversed polarity, where the epithelial cells exhibit basally located nuclei and apical cytoplasm. This uncommon tumor type poses diagnostic challenges due to its overlapping features with other renal neoplasms, underscoring the importance of histopathological examination for accurate identification and management.

CONCLUSION

Current treatment parallels renal cell carcinoma management, primarily surgical excision, with surveillance recommended for long-term follow-up. PRNRP is a distinct renal neoplasm with an excellent prognosis. Further research is needed to refine diagnostic criteria, understand molecular mechanisms, and optimize treatment strategies. Increased awareness among clinicians and pathologists can aid in accurate diagnosis and management.

摘要

引言与重要性

极性反转乳头状肾肿瘤(PRNRP)是一种罕见的肾肿瘤,于2019年首次被描述。其特征为细胞核级别低、嗜酸性细胞质以及核排列倒置。与乳头状肾细胞癌(PRCC)不同,PRNRP经常存在KRAS突变,且缺乏典型的7号和17号染色体三体性。组织病理学和免疫组织化学仍是诊断的金标准。

病例介绍

一名患有2型糖尿病、高血压和慢性肾病的52岁女性,肾功能恶化。影像学检查显示右肾有一个博斯尼亚克IV型囊肿,怀疑为恶性肿瘤。她接受了机器人辅助部分肾切除术,组织病理学确诊为PRNRP(pT1a,WHO/ISUP 1级)。肿瘤被完全切除,未发现复发或转移。由于PRNRP罕见且与其他肾肿瘤有重叠,因此带来了诊断挑战。它通常是偶然发现的,术前影像学检查的特异性有限。组织病理学标准包括细乳头状结构、间质玻璃样变性、嗜酸性细胞质和核反转。PRNRP病程惰性,切除术后尚无复发或转移的报道病例。

临床讨论

极性反转乳头状肾肿瘤(PRNRP)是肾细胞癌的一种罕见变体,其特征是具有独特的极性反转组织学特征,即上皮细胞核位于基底,细胞质位于顶端。这种不常见的肿瘤类型因其与其他肾肿瘤的重叠特征而带来诊断挑战,凸显了组织病理学检查对于准确识别和管理的重要性。

结论

目前的治疗与肾细胞癌的管理相似,主要是手术切除,并建议进行监测以进行长期随访。PRNRP是一种独特的肾肿瘤,预后良好。需要进一步研究以完善诊断标准、了解分子机制并优化治疗策略。提高临床医生和病理学家的认识有助于准确诊断和管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/528a/12221828/def83972ea49/gr1.jpg

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