scholarly journals Current role of imaging in the management of children with Wilms tumor according to the new umbrella protocol

2020 ◽  
pp. 51-63
Author(s):  
Varchetta Giovanni ◽  
Tanzillo Paolina ◽  
Mei Sara

Wilms tumor is the most common primary renal tumor in childhood. Children with Wilms tumor typically present with an asymptomatic abdominal mass, usually detected on a routine medical checkup or discovered coincidentally by parents. The initial differential diagnosis is with extrarenal abdominal masses; once a tumor of renal origin is established, distinguishing between Wilms tumor and other primary renal neoplasms such as congenital mesoblastic nephroma, clear cell sarcoma, malignant rhabdoid tumor and renal cell carcinoma may not be easy. However, in many cases imaging findings in conjunction with the patient's clinical and epidemiological data, allow the diagnosis of Wilms tumor. Wilms tumor care offers one of the most striking examples of success of pediatric oncology. Over the last decades the European SIOP studies have been the key to developing standardized diagnostic procedures, improved risk stratification, and adjusted treatment recommendations for children with Wilms tumor and this has resulted rate of overall survival is currently greater than 90%. As in previous SIOP trials and studies, the new protocol for the diagnosis and treatment of childhood renal tumors, the UMBRELLA SIOP–RTSG 2016, mandates preoperative chemotherapy without preceding mandatory histological assessment. Therefore, imaging studies are essential to obtain a presumptive diagnosis of WT, to provide disease staging information and to measure the tumor volume after neoadjuvant chemotherapy for the purposes of postoperative treatment stratification. This review describes role of imaging in the management of children with Wilms tumor, according to the current recommendations of the UMBRELLA protocol.

2017 ◽  
Vol 5 (3) ◽  
pp. 110-115
Author(s):  
Bruno Rafael Kunz Bereza ◽  
Tariane Friedrich Foiato Maneti ◽  
Vitor Arce Ferreira Cathcart ◽  
Raimundo Romilton Leal do Rosário ◽  
Paula Nunes ◽  
...  

Wilms Tumor (WT) is an extremely rare neoplasia in adults. It represents 95% and 1% of all renal tumors, in adults and children respectively. Clinical symptoms are also distinct between adults and children. In adults, symptoms are usually abdominal pain and hematuria, on the other hand, pediatric patients frequently present with an asymptomatic abdominal mass. The recommended treatment for WT in adults follows protocols established for pediatric patients. Currently, the 5-year overall survival rate for adults with WT is of approximately 90%. The adoption of multimodal therapy systematized, and proposed by current protocols, allowed the results to become much more robust, with a significant improvement from less than 30% to 90%. However, recent reviews suggest a worse outcome for adults when compared to children, even when submitted to the same treatment regimen, and comparable according to histology and tumor stage.We present a case of a 26 years-old male patient, who underwent surgery to treat a solid renal mass. Pathological report demonstrated a Wilms tumor. The patiente also received adjuvant chemotherapy with dactinomycin and vincristine. After 12 months follow-up, patient has no evidence of disease.


Author(s):  
Graciela Argote-Romero

Wilms tumor, known as well as nephroblastoma, is the most common primary malignant renal tumor in children. Over 95% of all renal tumors in patients under the age of 15 are Wilms tumors. The mean age at the time of diagnosis is 3.5 years. Wilms tumors are usually an incidental finding, a large abdominal mass discovered by a family member or pediatrician. Hematuria and hypertension can be present at the time of initial diagnosis. Up to 8% of the patients will have von Willebrand disease; therefore, all patients should have baseline coagulation studies. All patients should have either computed tomography of the abdomen and pelvis with oral and intravenous contrast or magnetic resonance imaging of the abdomen and pelvis with gadolinium. Treatment includes radical nephrectomy, chemotherapy, and, in some cases, radiotherapy. Emergency surgery is rarely. The disease-free survival rate is 86% for favorable-histology tumors and 64% for anaplastic tumors.


2002 ◽  
Vol 40 (1) ◽  
pp. 18-22 ◽  
Author(s):  
Gordan M. Vujani? ◽  
Anna Kelsey ◽  
Chris Mitchell ◽  
Rosemary S. Shannon ◽  
Peter Gornall
Keyword(s):  

2019 ◽  
Vol 104 (7-8) ◽  
pp. 348-351
Author(s):  
Junichiro Kawamura ◽  
Hokuto Ushijima ◽  
Yasumasa Yoshioka ◽  
Koji Daito ◽  
Kazuki Ueda ◽  
...  

Introduction Mesenteric cysts are rare intra-abdominal masses. They do not show classic clinical findings and are detected incidentally during imaging because of their absent or nonspecific clinical presentation. We herein report 2 cases of mesenteric cysts that were successfully diagnosed and resected through a laparoscopic approach. Case presentation In a 35-year-old man underwent a comprehensive medical checkup at another hospital, during which ultrasound incidentally revealed a cystic mass in his right retroperitoneum. The patient was referred to our hospital for surgical treatment. He was asymptomatic and his medical history was unremarkable. Physical examination revealed no palpable abdominal mass, and all laboratory test results were within normal ranges. Contrast-enhanced computed tomography showed a 7.2-cm-diameter smooth-surfaced, well-demarcated mass behind the right colon without a contrast effect (Fig. 1). We considered the cyst to be benign and localized in the mesentery of the ascending colon. In one patient, the cyst was located in the mesentery of the ascending colon and removed via a right hemicolectomy; in the other patient, the cyst was located in the sigmoid mesentery, which was completely resected through the operative wound under an open procedure after laparoscopic mobilization of the sigmoid mesentery. We chose the laparoscopic approach in both cases because the cysts were considered benign, noninfectious, and nonhemorrhagic. Histologic examination revealed cystic lymphangioma in both cases. Conclusion For mesenteric cysts, laparoscopy could be a preferred approach in properly selected patients.


2020 ◽  
Vol 7 (4) ◽  
pp. 945
Author(s):  
Hisham I. El Zanati ◽  
Waleed A. Aboelwafaa ◽  
Yasser M. Hamza

Background: Diagnostic laparoscopy is an added tool that has become widely available for the assessment of abdominal masses in addition to conventional imaging. It is the best real time imaging technique due to the magnification and intense illumination provided. The aim of this work is the assessment of the impact of performing diagnostic laparoscopy at the start of operations intended for resection of an abdominal mass.Methods: This prospective study included 40 patients admitted for surgical resection of an abdominal mass. All Patients were subjected to thorough conventional investigations followed by diagnostic laparoscopy performed prior to the start of the definitive operation. We assessed the extra time needed, complications encountered, effect on decision making and the overall benefit of laparoscopy in this context.Results: Overall 21 patients (52.5%) did benefit from diagnostic laparoscopy somehow between upgrading the staging, affecting the laparotomy incision site and confirming feasibility of laparoscopic resection. Of the patients who proceeded to a laparotomy (n=30), diagnostic laparoscopy missed local invasion in 7 patients, which precluded the resection of the tumor in 5 of them. Only 1 complication related to diagnostic laparoscopy was encountered in the form of a port-site hematoma (2.5%).Conclusions: While diagnostic laparoscopy doesn't carry significant added morbidity, it might save the patient an unnecessary laparotomy by altering the preoperative staging and improving the accuracy of anatomical and pathological diagnoses. Laparoscopy has its limitations mainly in the assessment of the retroperitoneal space as well as direct tumor invasion to adjacent organs and vessels.


2015 ◽  
Vol 2 (3) ◽  
pp. 84-89 ◽  
Author(s):  
Justin Scott Lee ◽  
Thomas Ray Sanchez ◽  
Sandra Wootton-Gorges

Renal malignancies are common in children. While the majority of malignant renal masses are secondary to Wilms tumor, it can be challenging to distinguish from more aggressive renal masses. For suspicious renal lesions, it is crucial to ensure prompt diagnosis in order to select the appropriate surgical procedure and treatment. This review article will discuss the common differential diagnosis that can be encountered when evaluating a suspicious renal mass in the pediatric population. This includes clear cell sarcoma of the kidney, malignant rhabdoid tumor, renal medullary carcinoma and lymphoma. 


1998 ◽  
Vol 1 (3) ◽  
pp. 243-248 ◽  
Author(s):  
Liliane A. Boccon-Gibod

In the majority of European countries, children with renal tumors now enter the SIOP-93-01 Trial and Study. The objective of this Study is to refine methods of treatment especialy in stage I patients. The role of institutional pathologists is important in this trial. There are new criteria for stages I and II, a new SIOP Working Classification of Renal Tumors of Childhood, and morphologic and prognostic similarities of pretreated and non-pretreated anaplastic cases. Specific problems encountered in assessing tumors treated with preoperative chemotherapy, administered to the majority of children over 6 months of age entering the SIOP Study, are discussed. The identification of a new low-risk group, the completely necrotic Wilms tumor, is outlined.


JAMA ◽  
1966 ◽  
Vol 195 (12) ◽  
pp. 1005-1009 ◽  
Author(s):  
D. J. Fernbach
Keyword(s):  

2007 ◽  
Vol 177 (4S) ◽  
pp. 305-305
Author(s):  
Shane Daley ◽  
Michael Ritchey ◽  
Robert Shamberger ◽  
Robert Sawin ◽  
Thomas Hamilton ◽  
...  

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Adel Attia ◽  
Moosa Suleman ◽  
Hesham Mosleh

Malignant rhabdoid tumor (MRT) is one of the most aggressive and lethal malignancies in pediatric oncology. Malignant rhabdoid tumor was initially described in 1978 as a rhabdomyosarcomatoid variant of a Wilms tumor because of its occurrence in the kidney and because of the resemblance of its cells to rhabdomyoblasts. The absence of muscular differentiation led Haas and colleagues to coin the term rhabdoid tumor of the kidney in 1981, Haas et al..


Sign in / Sign up

Export Citation Format

Share Document