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NCBI Bookshelf. Wilms Tumor [Internet]. Authors Basak Erginel.

Nephroblastoma [Wilms tumor WT ] is a rare, but the most common, primary renal tumor in children. WT is usually diagnosed between the ages of 1 and 5, with the most common diagnosis at the age of 3.

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Surgery is one of the cornerstones of WT treatment. Other aspects of management include chemotherapy and radiation therapy.

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In this chapter, the surgical therapy of WT is reviewed. Wilms tumor WTalso called nephroblastoma, was first described by Thomas F. Rance in 12.

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InCarl Max Wilhelm Wilms, a German surgeon and pathologist, gave a detailed histological description and since then the tumor bears his name 13. The incidence of WT is in children under 15 4. It is also the second most common intra-abdominal tru society weight loss reviews, and it is an embryonal malignancy of the kidney 5.

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Beckwith-Wiedemann syndrome is a syndrome characterized by macrosomia, macroglossia, omphalocele, and growth retardation, and hemihypertrophy is caused by a mutation located on 11p15, also known as the WT2 gene locus WT is thought to arise from these nephrogenic rests The prognosis tru society weight loss reviews WT is closely related to its histology. An unfavorable prognosis occurs when the tumor consists of anaplastic cells; the prognosis is favorable otherwise.

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This anaplastic type represents WT is associated with mutations in various tumor suppressor genes In the anaplastic type of WTs, p53 mutation is frequent WT is usually unilateral, and the mean age of presentation is 3.

The most common clinical presentation of WT is an abdominal mass.

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Other common symptoms are abdominal pain, macroscopic hematuria, fever, and hypertension Abdominal pain can be a symptom of a rupture or intratumoral hemorrhage. Macroscopic hematuria may occur when the tumor has extended to the collecting system Nonspecific symptoms, such as tru society weight loss reviews hematuria, urinary disturbances, malaise, weight loss, and anemia, may be present at initial presentation.

If spermatic veins are occluded, varicocele can occur 3 and a thorough examination of the abdomen is necessary. Varicocele tru society weight loss reviews always an alarming symptom, but even more if it occurs on the right side [symptom of inferior vena cava IVC thrombosis] or occlusion of the right spermatic vein by the lower part of the tumor right spermatic vein goes directly to IVC.

During the physical examination, a firm, nontender mass in the abdomen is usually identified. Due to elevated renin levels, a follow-up of the blood pressure is very important in WT patients.

Richard T Scott, M.D.,H.C.L.D.

The most common intravascular tumor extension sites are the renal vein, IVC, and atrium Although the lung reviews the most common metastatic site, respiratory symptoms are not common. In summary, during examination, the associated anomalies should be considered, and the patient should be examined for aniridia, hemihypertrophy, and genitourinary anomalies. Ultrasonography US is the primary diagnostic tool for children suspected of having WT.

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US allows for measuring the tumor size, identifying its origin, establishing the relationship between vena cava and aorta, as well as possible IVC or renal vein. The second important diagnostic tool is computerized tomography CT. During interpretation of the CT, tru society weight loss reviews also the US, the contralateral kidney and the liver should always be carefully examined.

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Magnetic resonance imaging MRI is also helpful in detecting the vascular involvement. MRI, although requires longer general anesthesia in the preschool age, offers the most accurate imaging of the kidney.

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It serves best when nephron-sparing resection is considered or in case of the need to distinguish between WT and nephroblastomatosis. A plain chest X-ray is a routine procedure for the evaluation of pulmonary metastases.

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A routine pulmonary CT is still controversial 22 ; however, many physicians prefer the thoracic CT loss reviews of its high sensitivity. The definite diagnoses of WT and its subtypes are made by histological evaluation. The preoperative laboratory tests that should be performed are the total blood count, renal and liver function tests, calcium level examination, and urinary examination.

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In rhabdoid types, the serum calcium increases High-risk patients for the growth of WTs, such as patients with Beckwith-Wiedemann syndrome, should be routinely examined with an US In addition, a hour urine catecholamine test is essential to avoid misdiagnosing with neuroblastoma.

Surgery is the cornerstone for the treatment of WT.

The SIOP recommends preoperative tru society weight loss reviews to decrease the risk of intraoperative rupture, downstage the tumor, and to reduce the need for irradiation.

The advantage of preoperative chemotherapy is the identification of chemoresistant high-risk blastemal predominant subtype that benefits from treatment intensification. The management of WT requires a multidisciplinary approach with a pediatric radiologist, an oncologist, a surgeon, and a radiotherapist.

First, the patient must be carefully evaluated using appropriate imaging techniques in the preoperative period to identify the origin of the tumor, its position in relation to the adjacent tissue, and vascular involvement.

The bilaterality of the tumor, and presence of nephrogenic rests, must be evaluated.

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  6. NCBI Bookshelf. Wilms Tumor [Internet].
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If the imaging is not suggestive of any bilateral lesion, there is no need to explore the contralateral kidney at surgery 28 In tumors with a renal tru society weight loss reviews and caval extension, it is advised to delay surgery and to start with chemotherapy If the thrombus extends to the thoracic vena cava, an echocardiography should be performed.

Some of these patients are cardiosurgical emergencies and therefore an oncology-oriented pediatric surgeon and a cardiac surgeon are necessary, and the equipment must include the cardiopulmonary bypass device.

For unilateral WTs, a transperitoneal radical nephrectomy is the standard operation.

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Nephron-sparing surgery NSS is advocated only in selected cases of patients with solitary kidney or bilateral WTs Before the incision, rolled sterile pads should be placed under the patient in supine position. A nasogastric tube, a transient catheter, an arterial line, and a Foley catheter are placed in our center Figure 2. A large transverse supraumbilical incision should be made, permitting the exploration of the entire abdomen and the contralateral kidney, if necessary Figure 3.

tru society weight loss reviews

In patients with an intracardiac extension, where an upfront excision tru society weight loss reviews not encouraged, a chevron incision or a combined transverse abdominal and sternal incision can be performed. Because the rupture of the tumor capsule and the spillage of the tumor increase with the tumor stage, and decrease survival, it is important to perform a suitably long incision to remove the tumor from the abdomen safely.

Richard T Scott, M.D.,H.C.L.D.

At the beginning of the operation, the abdomen should be explored for any intra-abdominal implants. If deemed necessary based on preoperative imaging, the opening of Gerota fascia and careful exploration of the whole contra lateral kidney is recommended.

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Any suspicious areas should be biopsied. The peritoneum covering the tumor is opened as laterally as possible for easy closure of the peritoneum after the tumor resection Figures 4 and 5.

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For left-sided WTs, laterocolonal access is usually sufficient Figure 6. The renal vein and the IVC should be palpated first to exclude thrombus.

The nephrectomy should begin by ligating the renal artery to avoid thrombus embolism. Afterward, the renal vein should be ligated immediately Figure 7.

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The ureter should be ligated as low as possible. Titanium clips are mostly used for the marking of the tumor area for possible further radiotherapy.

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An en bloc resection of the tumor without any tumor spillage is the most important aspect. Lymph node sampling is another important goal in WT surgery.

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Lymph node samples should be collected from the renal hilum, iliac, paracaval, or para-aortic areas for accurate staging. Spillage of the tumor or inadequate lymph node dissection results in incomplete chemotherapy and thus decreased survival rates.

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NWTS suggests seven regional nodes to get reliable information on lymph node involvement A large transverse supraumbilical incision, permitting the exploration of the entire abdomen and the contralateral kidney. The peritoneal reflection covering a right Wilms tumor.

P: peritoneum.

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The lateral peritoneal reflection opened from an avascular area on the lateral side. WT: Wilms tumor, P: peritoneum, C: colon.

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Image showing access to the right-sided WT in which the colon is reflected medially. There is an emerging consensus on performing routine adrenalectomy during unilateral nephrectomy for WTs.

One recent study tru society weight loss reviews that an adrenalectomy does not affect the 5-year survival rate, but it does increase the intraoperative spillage rates Most surgeons leave the adrenal gland intact if it is not infiltrated by the tumor In the study of van Waas et al.

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Performing NSS by partial nephrectomy or enucleation in unilateral cases is also a matter of debate We recommend this approach only in synchronous or metachronous bilateral cases or in solitary kidneys. Minimally invasive nephrectomy can offer the same outcome as the classical laparotomic tru society weight loss reviews 40 For bilateral cases, the current approach is preoperative chemotherapy followed by bilateral NSS Timing of surgery is important.

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Where possible, both sides can be operated in the same session. In difficult cases, the easier side can be operated first.

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The more difficult side can be operated after one or two courses of chemotherapy and stabilization of the renal function. The major goal of bilateral WT surgery tru society weight loss reviews to achieve a cure by removing all tumoral renal tissue while preserving the maximum functioning kidney. Some authors suggest in situ topical cooling or perfusion with preservation solutions for meticulous dissection without the loss of renal function from ischemia.