scholarly journals Bilateral Renal Tumors in Children: The First 5 Years’ Experience of National Centralization in The Netherlands and a Narrative Review of the Literature

2021 ◽  
Vol 10 (23) ◽  
pp. 5558
Author(s):  
Sophie E. van Peer ◽  
Janna A. Hol ◽  
Alida F. W. van der Steeg ◽  
Martine van Grotel ◽  
Godelieve A. M. Tytgat ◽  
...  
Keyword(s):  
Renal Tumor ◽  
Relevant Literature ◽  
Renal Tumors ◽  
Chemotherapy Response ◽  
Review Of The Literature ◽  
Kidney Tumors ◽  
Tumor Patients ◽  
Nephron Sparing ◽  
Expert Center ◽  
National Expert

Survival of unilateral Wilms tumors (WTs) is exceeding 90%, whereas bilateral WTs have an inferior outcome. We evaluated all Dutch patients with bilateral kidney tumors, treated in the first five years of national centralization and reviewed relevant literature. We identified 24 patients in our center (2015–2020), 23 patients had WT/nephroblastomatosis and one renal cell carcinoma. Patients were treated according to SIOP-RTSG protocols. Chemotherapy response was observed in 26/34 WTs. Nephroblastomatosis lesions were stable (n = 7) or showed response (n = 18). Nephron-sparing surgery was performed in 11/22 patients undergoing surgery (n = 2 kidneys positive margins). Local stage in 20 patients with ≥1 WT revealed stage I (n = 7), II (n = 4) and III (n = 9). Histology was intermediate risk in 15 patients and high risk in 5. Three patients developed a WT in a treated nephroblastomatosis lesion. Two of 24 patients died following toxicity and renal failure, i.e., respectively dialysis-related invasive fungal infection and septic shock. Genetic predisposition was confirmed in 18/24 patients. Our literature review revealed that knowledge is scarce on bilateral renal tumor patients with metastases and that radiotherapy seems important for local stage III patients. Bilateral renal tumors are a therapeutic challenge. We describe management and outcome in a national expert center and summarized available literature, serving as baseline for further improvement of care.

scholarly journals ESC, ALK, HOT and LOT: Three Letter Acronyms of Emerging Renal Entities Knocking on the Door of the WHO Classification

Cancers ◽  
2020 ◽  
Vol 12 (1) ◽  
pp. 168 ◽  
Author(s):  
Farshid Siadat ◽  
Kiril Trpkov
Keyword(s):  
Renal Cell ◽  
Who Classification ◽  
Renal Tumor ◽  
Current Knowledge ◽  
World Health ◽  
Renal Tumors ◽  
Low Grade ◽  
Kidney Tumors ◽  
Anaplastic Lymphoma

Kidney neoplasms are among the most heterogeneous and diverse tumors. Continuous advancement of this field is reflected in the emergence of new tumour entities and an increased recognition of the expanding morphologic, immunohistochemical, molecular, epidemiologic and clinical spectrum of renal tumors. Most recent advances after the 2016 World Health Organization (WHO) classification of renal cell tumors have provided new evidence on some emerging entities, such as anaplastic lymphoma kinase rearrangement-associated RCC (ALK-RCC), which has already been included in the WHO 2016 classification as a provisional entity. Additionally, several previously unrecognized entities, not currently included in the WHO classification, have also been introduced, such as eosinophilic solid and cystic renal cell carcinoma (ESC RCC), low-grade oncocytic renal tumor (LOT) and high-grade oncocytic renal tumor (HOT) of kidney. Although pathologists play a crucial role in the recognition and classification of these new tumor entities and are at the forefront of the efforts to characterize them, the awareness and the acceptance of these entities among clinicians will ultimately translate into more nuanced management and improved prognostication for individual patients. In this review, we summarise the current knowledge and the novel data on these emerging renal entities, with an aim to promote their increased diagnostic recognition and better characterization, and to facilitate further studies that will hopefully lead to their formal recognition and consideration in the future classifications of kidney tumors.

scholarly journals Staged, Open, No-Ischemia Nephron-Sparing Surgery for Bilateral-Multiple Kidney Tumors in a Patient with Birt-Hogg-Dubé Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Ahmet Tefekli ◽  
Ayşe Deniz Akkaya ◽  
Kamil Peker ◽  
Terman Gümüş ◽  
Metin Vural ◽  
...  
Keyword(s):  
Kidney Cancer ◽  
Papillary Renal Cell Carcinoma ◽  
Minimal Invasive ◽  
Nephron Sparing Surgery ◽  
Renal Tumors ◽  
Invasive Treatment ◽  
Kidney Tumors ◽  
Lung Cysts ◽  
Increased Risk ◽  
Nephron Sparing

Hereditary kidney cancer patients with bilateral multiple kidney tumors represent challenges in the era of rapidly growing minimal invasive treatment techniques. Birt-Hogg-Dubé Syndrome (BHDS) is an autosomal dominant genodermatosis characterized by a triad of benign skin tumors (fibrofolliculomas, trichodiscomas, acrochordons) together with an increased risk of developing malignant renal tumors and pulmonary disease such as pneumothoraces and multiple lung cysts. The morbidity and mortality of the affected patients is determined by the presence of the kidney tumors, which tend to be multifocal and bilateral, as observed in other hereditary kidney cancer syndromes like von Hippel-Lindau disease, familial leiomyomatosis, and hereditary papillary renal cell carcinoma. Herein, a patient with BHDS, presenting with synchronous bilateral multiple kidney tumors, is reported. The report describes the management of kidney tumors with two-stage open nephron-sparing surgery in which the nonvascular clamping technique was utilized.

Cellular mesoblastic nephroma in infants and children: Report of four cases and review of the literature

2019 ◽  
Vol 87 (2) ◽  
pp. 91-96
Author(s):  
Bita Geramizadeh ◽  
Ali Kashkooe ◽  
Pedram Keshavarz ◽  
Soheila Zareifar ◽  
Hamidreza Foroutan
Keyword(s):  
Clinical Presentation ◽  
Renal Tumor ◽  
Metastatic Potential ◽  
Renal Tumors ◽  
Mesoblastic Nephroma ◽  
Review Of The Literature ◽  
Histologic Diagnosis ◽  
Immunohistochemical Markers ◽  
Proliferative Rate ◽  
In Infants And Children

Background: Cellular mesoblastic nephroma is rare after infancy, and there are many controversial reports about its clinical presentation and treatment as well as outcome in infants, young children, and adolescents. Objectives: In this report, we will discuss our experience with four cases of cellular mesoblastic nephroma presented from infancy to childhood (from 18 months of age to 11 years of age). Cases: During 10 years, we had the experience of 4 cases of pediatric renal tumor with the diagnosis of cellular mesoblastic nephroma, which have been followed between 1 year and 6 years. There were three male and one female patients with the age of 1.5, 2, 2, and 11 years. These tumors showed variable characteristics according to the number of mitosis, proliferative rate, necrosis, immunohistochemical markers, and metastatic potential; however, despite of all of these variabilities, all of these patients have done well and all have been well at the end of study. Conclusion: Pediatric renal tumors with the histologic diagnosis of cellular mesoblastic nephroma have good outcome even with metastasis, mitosis, and high proliferative rate.

scholarly journals Practice Trends in the Surgical Management of Renal Tumors in an Academic Medical Center in the Past Decade

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Matheus Tannus ◽  
Fábio Sepúlveda ◽  
Thomé Pinheiro ◽  
Cássio Andreoni
Keyword(s):  
Hospital Stay ◽  
Operative Time ◽  
Renal Tumor ◽  
Medical Center ◽  
Academic Medical Center ◽  
Renal Tumors ◽  
Time Frequency ◽  
Academic Medical ◽  
Nephron Sparing ◽  
Stay Time

Objectives. To evaluate the trends of surgical treatment of the renal tumor in an academic medical center. Methods. Between 2001 and 2010, 505 were treated surgically at the Federal University of Sao Paulo for renal tumors. The following variables were observed and analyzed according to their evolution through time: frequency and types of surgeries performed, operative time, hospital stay, and warm ischemia time for partial nephrectomy. Results. An increase in the frequency of laparoscopic radical nephrectomies, open partial nephrectomies, and laparoscopic partial nephrectomies was observed when comparing the periods from 2001 to 2005 (4.3%, 2.6%, and 12.6%, resp.) and from 2006 to 2010 (13.2%, 18.6%, and 20.2%, resp.; ). The average of operative time, hospital stay, and tumor size diminished (from 211.7 to 177.17 minutes, from 5.52 to 4.22 days, and from 6.72 to 5.29 cm, resp.) when compared to the periods from 2001 to 2005 and from 2006 to 2010 (, , resp.). Conclusion. As time goes by, there has been a significant reduction in the hospital stay time, surgery time, and size of renal tumor in patients treated surgically. The frequencies of minimally invasive and nephron-sparing surgeries have increased over the last years.

scholarly journals Clinical and Molecular Characteristics and Outcome of Cystic Partially Differentiated Nephroblastoma and Cystic Nephroma: A Narrative Review of the Literature

Cancers ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 997
Author(s):  
Sophie E. van Peer ◽  
Corine J. H. Pleijte ◽  
Ronald R. de Krijger ◽  
Marjolijn C. J. Jongmans ◽  
Roland P. Kuiper ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Genetic Testing ◽  
Renal Tumor ◽  
Molecular Characteristics ◽  
Extensive Literature ◽  
Cystic Nephroma ◽  
Limited Information ◽  
Review Of The Literature ◽  
Genetic Characteristics ◽  
Excellent Outcome

In children presenting with a predominantly cystic renal tumor, the most likely diagnoses include cystic partially differentiated nephroblastoma (CPDN) and cystic nephroma (CN). Both entities are rare and limited information on the clinical and molecular characteristics, treatment, and outcome is available since large cohort studies are lacking. We performed an extensive literature review, in which we identified 113 CPDN and 167 CN. The median age at presentation for CPDN and CN was 12 months (range: 3 weeks–4 years) and 16 months (prenatal diagnosis–16 years), respectively. No patients presented with metastatic disease. Bilateral disease occurred in both entities. Surgery was the main treatment for both. Two/113 CPDN patients and 26/167 CN patients had previous, concomitant, or subsequent other tumors. Unlike CPDN, CN was strongly associated with somatic (n = 27/29) and germline (n = 12/12) DICER1-mutations. Four CPDN patients and one CN patient relapsed. Death was reported in six/103 patients with CPDN and six/118 CN patients, none directly due to disease. In conclusion, children with CPDN and CN are young, do not present with metastases, and have an excellent outcome. Awareness of concomitant or subsequent tumors and genetic testing is important. International registration of cystic renal tumor cohorts is required to enable a better understanding of clinical and genetic characteristics.

scholarly journals Deep learning for end-to-end kidney cancer diagnosis on multi-phase abdominal computed tomography

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Kwang-Hyun Uhm ◽  
Seung-Won Jung ◽  
Moon Hyung Choi ◽  
Hong-Kyu Shin ◽  
Jae-Ik Yoo ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Deep Learning ◽  
Kidney Cancer ◽  
Renal Tumor ◽  
Renal Tumors ◽  
Abdominal Computed Tomography ◽  
Wide Range ◽  
End To End ◽  
Multi Phase ◽  
Histologic Subtypes

AbstractIn 2020, it is estimated that 73,750 kidney cancer cases were diagnosed, and 14,830 people died from cancer in the United States. Preoperative multi-phase abdominal computed tomography (CT) is often used for detecting lesions and classifying histologic subtypes of renal tumor to avoid unnecessary biopsy or surgery. However, there exists inter-observer variability due to subtle differences in the imaging features of tumor subtypes, which makes decisions on treatment challenging. While deep learning has been recently applied to the automated diagnosis of renal tumor, classification of a wide range of subtype classes has not been sufficiently studied yet. In this paper, we propose an end-to-end deep learning model for the differential diagnosis of five major histologic subtypes of renal tumors including both benign and malignant tumors on multi-phase CT. Our model is a unified framework to simultaneously identify lesions and classify subtypes for the diagnosis without manual intervention. We trained and tested the model using CT data from 308 patients who underwent nephrectomy for renal tumors. The model achieved an area under the curve (AUC) of 0.889, and outperformed radiologists for most subtypes. We further validated the model on an independent dataset of 184 patients from The Cancer Imaging Archive (TCIA). The AUC for this dataset was 0.855, and the model performed comparably to the radiologists. These results indicate that our model can achieve similar or better diagnostic performance than radiologists in differentiating a wide range of renal tumors on multi-phase CT.

scholarly journals Small Cell Ovarian Cancer in Adolescents: Report of Two Cases and Review of the Literature

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
M. Rovithi ◽  
A. G. Pallis ◽  
A. Kalykaki ◽  
E. Lagoudaki ◽  
L. Giannikaki ◽  
...  
Keyword(s):  
Ovarian Cancer ◽  
Cell Carcinoma ◽  
Small Cell Carcinoma ◽  
Combination Chemotherapy ◽  
Poor Prognosis ◽  
Relevant Literature ◽  
Malignant Neoplasm ◽  
Standard Of Care ◽  
Small Cell ◽  
Review Of The Literature

Ovarian small cell carcinoma is a rare and highly malignant neoplasm carrying a poor prognosis. Although combination chemotherapy remains the cornerstone of treatment due to the rarity of these tumors, no regimen can be recommended as standard of care although in the majority of cases platinum-based regimens are used. Herein, we report two cases of small cell carcinoma of the ovaries along with a review of the relevant literature.

Renal Oncocytoma With Both Lymphovascular Invasion and Prominent Intracytoplasmic Vacuole-Like Spaces: A Case Report and Review of the Literature

2021 ◽  
pp. 106689692110415
Author(s):  
Xunda Luo ◽  
Christopher Preciado ◽  
Anupma Nayak ◽  
Lauren E. Schwartz ◽  
Thomas J. Guzzo ◽  
...  
Keyword(s):  
Needle Biopsy ◽  
Vascular Invasion ◽  
Lymphovascular Invasion ◽  
Renal Tumor ◽  
Renal Oncocytoma ◽  
Review Of The Literature ◽  
Renal Oncocytomas ◽  
Microscopic Features ◽  
Intracytoplasmic Vacuole ◽  
Tumor Entities

Here we report a case of renal oncocytoma in a 68 year-old male. The diagnosis was initially made on a needle biopsy 6 years prior to the partial nephrectomy. The case is unique that in addition to the gross and microscopic features commonly seen in renal oncocytomas, both lymphovascular invasion and prominent intracytoplasmic vacuole-like spaces are also present in this tumor. Although vascular invasion is increasingly recognized as compatible with renal oncocytoma, intracytoplasmic vacuoles are a rare and unusual finding that may lead to diagnostic difficulty. The diagnosis of renal oncocytoma was confirmed after immunohistochemistry was performed to argue against succinate dehydrogenase deficient renal cell carcinoma (RCC) and chromophobe RCC. This case highlights the importance for practicing pathologists to recognize the rare co-occurrence of lymphovascular invasion and large intracytoplasmic vacuole-like spaces in renal oncocytoma. Other differential diagnoses may include emerging renal tumor entities, such as the recently-proposed eosinophilic vacuolated tumor.

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