Journal of Kidney Cancer and VHL最新文献_第8页

Hereditary Leiomyomatosis and Renal Cell Cancer-Recognizing Patterns May Save Lives. 遗传性平滑肌瘤病和肾细胞癌识别模式可能挽救生命。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-05-26 eCollection Date: 2022-01-01 DOI: 10.15586/jkcvhl.v9i2.222

Tavares Catarina, Maria Sofia Quental, José Ricardo Brandão, Miguel Silva-Ramos

{"title":"Hereditary Leiomyomatosis and Renal Cell Cancer-Recognizing Patterns May Save Lives.","authors":"Tavares Catarina, Maria Sofia Quental, José Ricardo Brandão, Miguel Silva-Ramos","doi":"10.15586/jkcvhl.v9i2.222","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i2.222","url":null,"abstract":"In 2001, Finish investigators described a rare familiar syndrome characterized by an inherited susceptibility to cutaneous leiomyomas, uterine leiomyomas, and renal cell carcinoma (RCC). Hereditary leiomyomatosis and renal cell cancer (HLRCC) is now linked to a germline mutation in the fumarate hydratase (FH) gene that encodes a Krebs cycle enzyme, transforming fumarate to malate. The accumulation of fumarate, an oncometabolite, promotes tumorigenesis. We present a case of a 41-year-old female diagnosed with HLRCC after a radical nephrectomy due to renal cell cancer. Genetic analyses confirmed a novel FH mutation. Close follow-up allowed for a precocious diagnosis of a metachronous renal tumor and later a hepatic metastasis. Her family was also counseled and offered genetic testing. As observed in this case, the diagnosis of HLRCC is of paramount importance for patients and their families: there is a 15% cumulative lifetime risk of developing RCC, which frequently occurs in young patients and metastasizes at an early stage. Implementing a regular follow-up with adequate imaging examinations may help save lives.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"27-31"},"PeriodicalIF":1.6,"publicationDate":"2022-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9430984/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40368314","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: 2

Renal Cell Carcinoma with Cardiac Metastases: A Case Report and Review of the Literature. 肾细胞癌合并心脏转移:1例报告及文献复习。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-05-25 eCollection Date: 2022-01-01 DOI: 10.15586/jkcvhl.v9i2.229

Ellen M Cahill, Alexandra Tabakin, Brian Shinder, Mark Bramwit, Biren Saraiya, Xinyang Xu, Cristo G Salazar, Zhongren Zhou, Eric A Singer

引用次数: 2

Renal Cell Carcinoma with Testicular Metastases: A Case Report and Review of the Literature. 肾细胞癌伴睾丸转移:1例报告及文献复习。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-05-06 eCollection Date: 2022-01-01 DOI: 10.15586/jkcvhl.v9i2.212

Sho Yoshitake, Brian M Shinder, Kevin Dazen, Colton Smith, Tina M Mayer, Evita Sadimin, Eric A Singer

引用次数: 1

Simultaneous Bilateral Testicular Metastases from Renal Clear Cell Carcinoma: A Rare Presentation in Von Hippel-Lindau disease. 肾透明细胞癌同时双侧睾丸转移:Von Hippel-Lindau病的罕见表现。

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-05-05 eCollection Date: 2022-01-01 DOI: 10.15586/jkcvhl.v9i2.211

Asaad Moradi, Delaram Farhoumand, Behnaz Bouzari, Behnam Shakiba

{"title":"Simultaneous Bilateral Testicular Metastases from Renal Clear Cell Carcinoma: A Rare Presentation in Von Hippel-Lindau disease.","authors":"Asaad Moradi, Delaram Farhoumand, Behnaz Bouzari, Behnam Shakiba","doi":"10.15586/jkcvhl.v9i2.211","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i2.211","url":null,"abstract":"In this article, we present a Von Hippel-Lindau (VHL) patient with synchoronus bilateral testicular metastasis from renal cell carcinoma (RCC). A 50 year-old man, a known case of VHL syndrome was referred with palpable masses in both the testes. His medical history demonstrated that he had undergone the brain surgery for cerebellar hemangioblastoma. He had undergone simultaneous Whipple's pancreatectomy and left radical nephrectomy becuase of well-differentiated neuroendocrine tumors in head and body of the pancreas and a 6-cm clear cell-type grade-3 RCC in the left kidney. Scrotal sonography demonstrated vascular and heteroechogen masses measuring 19×14 mm in lower pole of the right testicle, 19×16 mm in upper pole of the right testicle, and 23×16.5 mm in upper pole of the left testicle. After having patient's consent, bilateral orchiectomy was performed by inguinal incision. Histopathologic examination and immunohistochemistry staining revealed metastasis from RCC. The most common neoplasm of reproductive system in VHL patients is epididymal papillary cystadenoma. Owing to it's benign nature, the management is conservative with routine physical examination and ultrasonography. Our patient indicated that every scrotal mass in patients with VHL is not to be considered as epididymal papillary cystadenoma.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"19-21"},"PeriodicalIF":1.6,"publicationDate":"2022-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9430985/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40368313","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: 2

Massive Malignant Epithelioid Angiomyolipoma of the Kidney 肾脏大量恶性上皮样血管平滑肌脂肪瘤

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-04-22 DOI: 10.15586/jkcvhl.v9i2.210

Isaac M. Tessone, Benjamin Lichtbroun, A. Srivastava, Alexandra L. Tabakin, Charles F Polotti, R. Groisberg, Evita T. Sadimin, E. Singer, M. Grandhi

引用次数: 2

Incidence and Distribution of New Renal Cell Carcinoma Cases: 27-Year Trends from a Statewide Cancer Registry 新发肾细胞癌病例的发病率和分布:来自全国癌症登记的27年趋势

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-04-18 DOI: 10.15586/jkcvhl.v9i2.219

Ahmad Alzubaidi, Stephen P Sekoulopoulos, J. Pham, Vonn Walter, Jay G. Fuletra, J. Raman

{"title":"Incidence and Distribution of New Renal Cell Carcinoma Cases: 27-Year Trends from a Statewide Cancer Registry","authors":"Ahmad Alzubaidi, Stephen P Sekoulopoulos, J. Pham, Vonn Walter, Jay G. Fuletra, J. Raman","doi":"10.15586/jkcvhl.v9i2.219","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i2.219","url":null,"abstract":"Nationwide databases have implicated an increased incidence of renal cell carcinoma (RCC). The Pennsylvania (PA) Cancer Registry was queried to better define incidence, geographic distribution, and statewide trends of new RCC cases over a 27-year period. JoinPoint Trend Analysis Software modeled average annual percent changes (APCs) in age-adjusted rates (AAR). Maps plotting county-level incidence rates and stage distribution of disease across the state in 5-year time intervals were created using R 4.0.2 software. Overall, 59,628 cases of RCC were recorded in PA from 1990 to 2017. Eighty six percent of patients were >50 years of age, 61% were males, and 89% were Caucasian. Stage distribution using the SEER staging system included 64% local, 17% regional, and 16% distant. Over the study interval, AAR of all RCC cases increased from 9.9 to 18.0 patients per 100,000 population with an APC of 2.3% (p < 0.01). AAR of local disease increased from 5.4 to 12.7 patients per 100,000 population with an APC of 3.2% (p < 0.01). AAR of regional disease also increased from 1.9 to 2.9 patients per 100,000 population with an APC of 1.0% (p = 0.01). Younger patients (<50 years) had a greater rate of increase than older counterparts (APC 3.8% vs. 2.0%, p < 0.05). Geospatial investigation of new RCC cases noted certain geographic concentrations of greater disease incidence. The incidence of RCC in PA has increased over the past 27 years in PA. One-third of the cases are regional or metastatic at presentation and rates of increase were most notable in younger patients.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"9 1","pages":"7 - 12"},"PeriodicalIF":1.6,"publicationDate":"2022-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43355062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 6

High Levels of PD-L1+ and Hyal2+ Myeloid-derived Suppressor Cells in Renal Cell Carcinoma 肾细胞癌中高水平的PD-L1+和Hyal2+髓源性抑制细胞

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-04-16 DOI: 10.15586/jkcvhl.v9i2.208

S. Kusmartsev, Elizabeth P. Kwenda, Paul R. Dominguez-Gutierrez, P. Crispen, P. O'Malley

{"title":"High Levels of PD-L1+ and Hyal2+ Myeloid-derived Suppressor Cells in Renal Cell Carcinoma","authors":"S. Kusmartsev, Elizabeth P. Kwenda, Paul R. Dominguez-Gutierrez, P. Crispen, P. O'Malley","doi":"10.15586/jkcvhl.v9i2.208","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i2.208","url":null,"abstract":"Renal cell carcinoma (RCC) patients frequently have increased number of immunosuppressive myeloid cells in circulation. High number of myeloid-derived suppressor cells (MDSCs) in the blood are associated with immune suppression as well as with cancer-related inflammation which drives the mobilization of myeloid cells to tumor tissue. Here, we show that peripheral blood from a previously untreated RCC patient has increased the number of monocytic CD33+CD11b+ MDSCs, which also co-expressed PD-L1 and membrane-bound enzyme hyaluronidase 2 (Hyal2). PD-L1 expression is associated with immune suppression, whereas expression of Hyal2 is associated with inflammation, because Hyal2+ myeloid cells can degrade the extracellular hyaluronan (HA), leading to the accumulation of pro-inflammatory HA fragments with low molecular weight. These findings implicate the potential involvement of monocytic MDSCs in both tumor-associated immune suppression and cancer-related inflammation. Analysis of organotypic tumor-tissue slice cultures prepared from cancer tissue of the same patient revealed the significant presence of PD-L1+ HLA-DR+ macrophage-like or dendritic cell-like antigen-presenting cells in tumor stroma. Interestingly, stroma-associated PD-L1+ cells frequently have intracellular hyaluronan. Collectively, data presented in this study suggest that the interplay between tumor-recruited myeloid cells and stromal HA may contribute to the inflammation and immune tolerance in kidney cancer.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"9 1","pages":"1 - 6"},"PeriodicalIF":1.6,"publicationDate":"2022-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46108715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4

Primary Rhabdomyosarcoma of Kidney with Local Recurrence and Liver Metastasis in Adults: A Case Report 成人原发性肾横纹肌肉瘤伴局部复发及肝转移1例报告

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-04-14 DOI: 10.15586/jkcvhl.v9i1.218

H. Nasrollahi, Ali Eslahi, Ali Ariafar, F. Ahmed, A. Monabati

{"title":"Primary Rhabdomyosarcoma of Kidney with Local Recurrence and Liver Metastasis in Adults: A Case Report","authors":"H. Nasrollahi, Ali Eslahi, Ali Ariafar, F. Ahmed, A. Monabati","doi":"10.15586/jkcvhl.v9i1.218","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i1.218","url":null,"abstract":"Primary rhabdomyosarcoma (RMS) of the kidney in an adult is rare, with only a few cases published in the literature. It is a mesenchymal tumor associated with an aggressive and rapid clinical progression course. We present a case of primary renal RMS in a 58-year-old female who presented with intermittent abdominal pain in the past year. The computed tomography (CT) scan revealed a 20×25×8 cm heterogeneous solid mass in the middle pole extended to the lower pole of the right kidney. Therefore, the patient underwent a right radical nephroureterectomy. Histopathology examination and immunohistochemistry studies confirmed the diagnosis of RMS with pleomorphic components. Postoperatively, the patient was discharged without any complications and was referred to an oncologist for chemotherapy. However, a follow-up CT scan in 2 months showed widespread liver metastasis and local recurrence. The patient received Gemcitabine and Docetaxel, but her condition worsened, and she passed away 5 months later. Primary renal RMS is rare in adults. In addition, liver metastasis is uncommon and poorly understood. Hence, we describe the clinicopathologic characteristics, including clinical follow-up of our case, focusing on the disease progression, treatment, and outcome.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"9 1","pages":"55 - 58"},"PeriodicalIF":1.6,"publicationDate":"2022-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67104234","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Renal Leiomyosarcoma, a Rare Presentation 肾脏平滑肌肉瘤，一种罕见的表现

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-03-21 DOI: 10.15586/jkcvhl.v9i1.216

T. Djiwa, K. Sabi, P. Simgban, Mayi Bombonne, B. Sama, Mazamaesso Tchaou, T. Darré

引用次数: 1

Inflammatory Hibernoma of the Renal Hilum Mimicking a Renal Pelvis Tumor 类似肾盂肿瘤的肾门炎性冬眠瘤

IF 1.6

Journal of Kidney Cancer and VHL Pub Date : 2022-03-16 DOI: 10.15586/jkcvhl.v9i1.225

Kelsey Larkin, Kevin J. Chua, S. Doppalapudi, Colton Smith, Evita T. Sadimin, V. Fitzhugh, E. Singer

引用次数: 0