Journal of Kidney Cancer and VHL最新文献_第4页

Case Reports of TFE3-Rearranged Renal Cell Carcinoma: FDG-PET Uptake Might Help Diagnosis. TFE3重排肾细胞癌的病例报告：摄取FDG-PET可能有助于诊断。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-09-27 eCollection Date: 2023-01-01 DOI: 10.15586/jkcvhl.v10i3.266

Sho Murakami, Keita Nagawa, Takanori Inui, Aya Yamamoto, Mizuka Suzuki, Fumitaka Koga, Toru Motoi, Yasunobu Takaki

{"title":"Case Reports of TFE3-Rearranged Renal Cell Carcinoma: FDG-PET Uptake Might Help Diagnosis.","authors":"Sho Murakami, Keita Nagawa, Takanori Inui, Aya Yamamoto, Mizuka Suzuki, Fumitaka Koga, Toru Motoi, Yasunobu Takaki","doi":"10.15586/jkcvhl.v10i3.266","DOIUrl":"https://doi.org/10.15586/jkcvhl.v10i3.266","url":null,"abstract":"Translocation and transcription factor E3 (TFE3)-rearranged renal cell carcinoma (RCC) is a rare subtype of RCCs characterised by the fusion of the TFE3 transcription factor genes on chromosome Xp11.2 with one of the multiple genes. TFE3-rearranged RCC occurs mainly in children and adolescents, although middle-aged cases are also observed. As computed tomography (CT)/magnetic resonance imaging (MRI) findings of TFE3-rearranged RCC overlap with those of other RCCs, differential diagnosis is often challenging. In the present case reports, we highlighted the features of the fluorine-18-labelled fluorodeoxyglucose positron emission tomography with CT (FDG PET-CT) in TFE3-rearranged RCCs. Due to the rarity of the disease, FDG PET-CT features of TFE3-rearranged RCC have not yet been reported. In our cases, FDG PET-CT showed high standardised uptake values (SUVmax) of 7.14 and 6.25 for primary tumours. This might imply that TFE3-rearranged RCC has high malignant potential. This is conceivable when the molecular background of the disease is considered in terms of glucose metabolism. Our cases suggest that a high SUVmax of the primary tumour is a clinical characteristic of TFE3-rearranged RCCs.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"10 3","pages":"61-68"},"PeriodicalIF":1.6,"publicationDate":"2023-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542702/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41137372","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Systemic Treatment for Advanced and Metastatic Non-Clear Cell Renal Cell Carcinoma: Examining Modern Therapeutic Strategies for a Notoriously Challenging Malignancy. 晚期和转移性非透明细胞肾细胞癌的系统治疗：研究一种臭名昭著的挑战性恶性肿瘤的现代治疗策略。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-09-26 eCollection Date: 2023-01-01 DOI: 10.15586/jkcvhl.v10i3.295

Jake Drobner, Daniella Portal, Karie Runcie, Yuanquan Yang, Eric A Singer

{"title":"Systemic Treatment for Advanced and Metastatic Non-Clear Cell Renal Cell Carcinoma: Examining Modern Therapeutic Strategies for a Notoriously Challenging Malignancy.","authors":"Jake Drobner, Daniella Portal, Karie Runcie, Yuanquan Yang, Eric A Singer","doi":"10.15586/jkcvhl.v10i3.295","DOIUrl":"10.15586/jkcvhl.v10i3.295","url":null,"abstract":"Non-clear cell renal cell carcinoma (nccRCC) is a heterogeneous group of malignancies that represents 25% of renal cell carcinoma (RCC) cases. Treatment for non-clear cell histologies is mostly based on evidence from small phase II clinical trials or extrapolated from successful therapies in clear cell RCC because of the low incidence of non-clear cell pathology. Advances in genomic profiling have improved clinicians' understanding of molecular targets for nccRCC, such as altered mesenchymal epithelial transition (MET) gene status and fumarate hydratase (FH) gene inactivation, but patient outcomes remain poor and optimal management of this disease remains unclear. This review assesses outcomes by histologic subtype from 27 prospective and 13 ongoing clinical trials to identify therapeutic strategies for advanced or metastatic nccRCC. Vascular endothelial growth factor tyrosine kinase inhibitors (TKI), such as sunitinib, and mammalian target of rapamycin (mTOR) inhibitors, such as everolimus, have demonstrated efficacy and remain viable treatment options, with a preference for sunitinib. However, everolimus is preferred in patients with chromophobe RCC because folliculin (FLCN) gene mutations upregulate the mTOR pathway. Novel TKIs, such as cabozantinib, show improved outcomes in patients with papillary RCC because of targeted MET inhibition. Platinum-based chemotherapy continues to be the recommended treatment strategy for collecting duct and medullary RCC. Clinically meaningful antitumor activity has been observed across all non-clear cell histologies for immune checkpoint inhibitors, such as nivolumab, pembrolizumab, and ipilimumab. Ongoing trials are evaluating novel tyrosine kinase inhibitor and immunotherapy combination regimens, with an emphasis on the promising MET-inhibitor cabozantinib and pembrolizumab plus lenvatinib.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"10 3","pages":"37-60"},"PeriodicalIF":1.6,"publicationDate":"2023-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542704/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41151679","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Primary Well-Differentiated Neuroendocrine Tumor of the Kidney. 原发性肾高分化神经内分泌肿瘤。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-01-01 DOI: 10.15586/jkcvhl.v10i2.277

Vishnu Prasad, Charakkulam Vijay Sreelakshmi, K Ravi Chandran, Shashank Agrawal, Ginil Kumar Pooleri, Amrita Sao

{"title":"Primary Well-Differentiated Neuroendocrine Tumor of the Kidney.","authors":"Vishnu Prasad, Charakkulam Vijay Sreelakshmi, K Ravi Chandran, Shashank Agrawal, Ginil Kumar Pooleri, Amrita Sao","doi":"10.15586/jkcvhl.v10i2.277","DOIUrl":"https://doi.org/10.15586/jkcvhl.v10i2.277","url":null,"abstract":"Primary neuroendocrine tumors (NET) of the kidney are rare. They present with varied symptoms, making their diagnosis difficult clinically as well as pathologically. We present to you the case of a renal NET, which presented in a young female patient. A 48-year-old female patient came with an incidentally detected right renal mass during the evaluation of a nonspecific gynecological problem. She underwent contrast-enhanced computed tomography (CT) of the abdomen, which showed a 57*45*34 mm mass with enlarged retrocaval and aortocaval nodes (25*12 mm). Renal cell carcinoma was suspected as per the CT findings, and metastatic workup in the form of FDG PET CT was done in view of the unusually enlarged nodes. She underwent robot-assisted radical nephrectomy along with lymph node dissection. Surgery was uneventful, and she recovered well in the postoperative period. In the final pathology, there was confusion regarding the diagnosis, and further immunohistochemistry (IHC) was recommended by the pathologist. IHC showed synaptophysin positive, chromogranin negative, CD56 focally positive with Ki-67 of 2-3%, which was suggestive of low-grade NET of the kidney. Lymph nodes were negative. She was kept on follow-up and a Ga 68-DOTANOC scan at 3 months showed no evidence of disease. Diagnosis and management of NET of the kidney still remains a debatable and controversial topic in view of its rarity. High index of suspicion needs to be observed in patients presenting with carcinoid syndrome and a renal mass. Nuclear scans like PET scan and DOTANOC scan can accurately stage the disease. Management includes partial or radical nephrectomy depending on the tumor characteristics. Further studies are required to optimize the treatment protocols for these patients.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"10 2","pages":"8-12"},"PeriodicalIF":1.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10184039/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9491777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review. Von Hippel-Lindau与胰腺神经内分泌肿瘤相关:综述

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-01-01 DOI: 10.15586/jkcvhl.v10i2.272

Danilo Coco, Silvana Leanza

{"title":"Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review.","authors":"Danilo Coco, Silvana Leanza","doi":"10.15586/jkcvhl.v10i2.272","DOIUrl":"https://doi.org/10.15586/jkcvhl.v10i2.272","url":null,"abstract":"Multiorgan tumors are a hallmark of the autosomal dominant genetic disorder known as Von Hippel-Lindau syndrome (VHL), which is typically the result of inherited aberrations of the VHL tumor suppressor gene. The most frequent cancer is retinoblastoma, which can also occur in the brain and spinal cord, renal clear cell carcinoma (RCCC), paraganglioma, and neuroendocrine tumors. There may also be lymphangiomas, epididymal cysts, and pancreatic cysts or pancreatic neuroendocrine tumors (pNETs). The most frequent causes of death are metastasis from RCCC and neurological complications from retinoblastoma or central nervous system (CNS). Pancreatic cysts are present in 35-70% of VHL patients. Simple cysts, serous cysts, or pNETs are possible presentations, and the likelihood of malignant degeneration or metastasis is no greater than 8%. Although VHL has been associated with pNETs, their pathological characteristics are unknown. Furthermore, it is unknown whether variations in the VHL gene cause the development of pNETs. Hence, this retrospective study was undertaken with the main aim to examine whether pNETs are connected to VHL from a surgical perspective.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"10 2","pages":"13-20"},"PeriodicalIF":1.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10219837/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9550509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Recent Improvements in Adult Wilms Tumor Diagnosis and Management: Review of Literature. 成人肾母细胞瘤诊断和治疗的最新进展:文献综述。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-01-01 DOI: 10.15586/jkcvhl.v10i3.281

Vishnupriya Sakthivel, Ismail Z Adeeb, Devashree Vijayabalan

引用次数: 1

Leiomyoma of Kidney. 肾平滑肌瘤。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-01-01 DOI: 10.15586/jkcvhl.v10i2.264

Vishnu Karayil R, Mini Bhaskarashenoy, Geetha Sukumaran

引用次数: 0

Role of Angular Interface Sign in Characterizing Small Exophytic Renal Masses in Computed Tomography; Prospective Study. 角界面征象在计算机断层诊断肾外生性小肿块中的作用前瞻性研究。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-01-01 DOI: 10.15586/jkcvhl.v10i2.262

Mohamed Sharafeldeen, Mohamed Shaaban, Ahmed Hafez Afif, Mohamed Elsaqa, Nagy Naguib, Sara Elnaggar, Ahmad Beltagy

{"title":"Role of Angular Interface Sign in Characterizing Small Exophytic Renal Masses in Computed Tomography; Prospective Study.","authors":"Mohamed Sharafeldeen, Mohamed Shaaban, Ahmed Hafez Afif, Mohamed Elsaqa, Nagy Naguib, Sara Elnaggar, Ahmad Beltagy","doi":"10.15586/jkcvhl.v10i2.262","DOIUrl":"https://doi.org/10.15586/jkcvhl.v10i2.262","url":null,"abstract":"The widespread use of computed tomography (CT) has increased the incidence of small renal cell masses. We aimed to evaluate the usefulness of the angular interface sign (ice cream cone sign) to differentiate a broad spectrum of small renal masses using CT. The prospective study included CT images of patients with exophytic renal masses ≤ 4 cm in maximal dimension. The presence or absence of an angular interface of the renal parenchyma with the deep part of the renal mass was assessed. Correlation with the final pathological diagnosis was performed. The study included 116 patients with renal parenchymal masses of a mean (± SD) diameter of 28 (± 8.8) mm and a mean age of 47.7 (±12.8) years. The final diagnosis showed 101 neoplastic masses [66 renal cell carcinomas (RCC), 29 angiomyolipomas (AML), 3 lymphomas, and 3 oncocytomas] and 15 non-neoplastic masses [11 small abscesses, 2 complicated renal cysts, and 2 granulomas]. Angular interface sign was statistically comparable in neoplastic versus non-neoplastic lesions (37.6% versus 13.3%, respectively, P = 0.065). There was a statistically higher incidence of the sign when comparing benign versus malignant neoplastic masses (56.25 vs. 29%, respectively, P = 0.009). Also, comparing the sign in AML versus RCC was statistically significant (52% of AML versus 29% of RCC, P = 0.032). The angular interface sign seems beneficial in predicting the nature of small renal masses. The sign suggests benign rather than malignant small renal masses.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"10 2","pages":"33-39"},"PeriodicalIF":1.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10315668/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9799162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Von Hippel-Lindau Disease (VHL): Characteristic Lesions with Classic Imaging Findings. Von Hippel-Lindau病(VHL):典型影像学表现的特征性病变。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-01-01 DOI: 10.15586/jkcvhl.v10i3.293

Suryansh Bajaj, Darshan Gandhi, Divya Nayar, Ali Serhal

引用次数: 2

Renal Cell Carcinoma Arising from Isthmus of Horseshoe K. 马蹄峡肾细胞癌。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-01-01 DOI: 10.15586/jkcvhl.v10i2.267

Sanjay M Khaladkar, Sai Sabari Vinay Kumar Parripati, Deepak Koganti, Satvik Dhirawani, Urvashi Agarwal

引用次数: 0

Screening for Latent Polycythemia Vera in Renal Cell Carcinoma-Associated Erythrocytosis. 肾细胞癌相关红细胞增多症的潜伏性真性红细胞增多症筛查。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2023-01-01 DOI: 10.15586/jkcvhl.v10i1.276

Stephen E Langabeer

引用次数: 0