Wilm's+Tumor


 * **Epidemiolgy:** || Wilms tumor is the fourth most common pediatric tumor with 500 cases per year. The peak age is between 3 and 4 years old with 95% before the age of 10[1]. African American children are at the higher risk and Asian children are at the lowest risk ||
 * **Etiology:** || A higher risk of Wilms' tumor occurs in patients with multiple genitourinary abnormalities, hemihypertrophy and aniridia, and the facial abnormality syndrome Beckwith-Wiedemann or as part of a multiple malformation syndrome.[] either hereditary or not, Wilms can appear when there has been a change in one or more of ten specific genes although only 2% of family history has been proven positive for Wilms tumor occurrence.[1] ||
 * **Signs & Symptoms:** || Children with Wilms' tumor may experience many different symptoms[7]. The following, however, are the most common:
 * Mass or lump, which causes no tenderness, in the abdomen
 * Pain in the abdomen from pressure on other organs near the tumor
 * Swelling of the abdomen
 * Veins that appear distended or large across the abdomen
 * Blood in the urine
 * Decreased appetite
 * Fever
 * High blood pressure
 * Weakness or tiredness ||
 * **Diagnostic Procedures:** || Your child's doctor will conduct a complete medical history and physical examination[7]. Diagnostic procedures may include[7]:
 * Abdominal Computed Tomography (CT or CAT) Scan — This is a diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images, both horizontally and vertically, of the body. These images, often called slices, are more detailed than X-rays. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat and organs.


 * Abdominal Ultrasound — This is a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues and organs. It can provide an outline of the kidneys and tumor as well as identify problems in the renal or other major veins in the abdomen. It also can determine if there are any lesions or tumors in a kidney.


 * Blood and Urine Tests — These tests are used to evaluate kidney and liver function.


 * Chest X-ray — This diagnostic test uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs on film. A chest X-ray can determine if there are metastases or cancer that has spread in the lungs.


 * Magnetic Resonance Imaging (MRI) — This diagnostic procedure uses a combination of large magnets, radio frequencies and a computer to produce detailed images of organs and structures within the body. MRI can determine if there are metastases or cancer that has spread, any tumor cells in the lymph nodes and if any other organs are involved. Wilm's tumors can compress other organs in the area, affecting their function.

|| Small, round, dark lymphocyte-like nuclei with without visible nucleoli ||  ||   ||
 * Surgery — Surgical removal of the tumor and kidney may be necessary for a definitive diagnosis and to determine the extent of the disease. [[image:http://img.medscape.com/pi/emed/ckb/pediatrics_general/985844-989398-3115.jpg width="460" height="460"]] ||
 * **Histology:** || All Wilm tumors are classified by the Second National Wilms Tumor Study (NWTS-2) as favorable histology (FH) which are responsive to treatment, or unfavorable (UF)which are less responsive to treatment.[1] ||
 * **Lymph node drainage:** || Renal Lymph nodes originate at the Hilum go inferiorly along the renal artery to the para-aortic and paracaval chains. Regional nodes drain from the diafram to the bifurcation of the aorta. Other nodes at risk are retrocaval, precaval, interaortovaval, preaortic, and retroaortic. All at risk with Left and right sided tumors.[3]
 * **Metastatic spread:** || The most common site of metastatic spread at diagnosis is lung with liver second most common. Metastesis can occur if the capsule ruptures preoperatively or from seeding during surgery.[1] ||
 * = **Grading:**
 * __ Grade 1 __** ||= __ **Simplified Fuhrman Grading:** __ [4]
 * < __** Grade 2 **__ || Inconspicuous nucleoli, visible only at 200-400X (nuclei usually small and uniform with open, finely granular chromatin) ||
 * < __** Grade 3 **__ || Prominent nucleoli, easily visible at 100X (nuclei usually mildly to moderately pleomorphic) ||
 * < __** Grade 4 **__ || Markedly pleomorphic, bizarre nuclei, giant cells, multiple nucleoli ||

__** I: Tumor limited to kidney and completely excised: **__ __** II: Tumor extends beyond the kidney but is completely removed: **__ (1 or more of the following features must be present) (Any of the following conditions may also exist) (Other features) __** III: >1 of the following criteria must be met: **__ __** IV: Hematogenous metastases: **__ __** V: Bilateral renal involvement at diagnosis: **__ (An attempt should be made to stage each side according to the above criteria on the basis of extent of disease before biopsy) __** Musculoskeletal: Growth abnormalities and hypoplasia: **__ (These effects are more pronounced the younger the patient is at time of RT) __** Pulmonary: **__ __** Thyroid: increased risk of: **__ __** Cardiac: these are rare using current protocols: **__ __** Hepatic: There is a small long term risk of veno-occlusive disease which consists of the clinical triad: **__ __** Renal: Stenosis of the contralateral renal artery may produce hypertension: **__ __** Gonadal: **__ **Ovary:** **Testis:** __** Female patients who had RT have a higher risk of: **__ __** Secondary neoplasms: **__ Flank irradiation [1] || Heart: Whole 4000 cGy, 2/3 4500 cGy, 1/3 6000 cGy Kidney: Whole 2300 cGy, 2/3 3000 cGy, 1/3 5000 cGy Liver: Whole 3000 cGy, 2/3 3500 cGy, 1/3 5000 cGy Lung: Whole 1750 cGy, 2/3 3000 cGy, 1/3 4500 cGy Spinal cord: 20cm 4700 cGy, 10cm 5000 cGy, 5cm 5000 cGy Stomach: Whole 5000 cGy, 2/3 5500 cGy, 1/3 6000 cGy Small intestine (perforation, obstruction): Whole 4000 cGy, 1/3 5000 cGy [2] || 2. RadiationOncology/Toxicity/Emami. []. Accessed June 25, 2012. a. Papillary renal cell 3. Carcinoma. Stanford Medicine. Available at: []. Accessed on June 25, 2012. 4. Wilms tumor. Pediatric Oncology Materials. Available at: []. Accessed on June 25, 2012. 5. Wilms tumor. Pediatric Oncology Education Materials. Available at: []. Accessed on June 25, 2012. 6. Cancer of the kidney. Available at: **__ [] __**. Accessed June 29, 2012. 7. Wilms’ Tumor. UCSF Benioff Children’s Hospital. 2012. Available at: []. Accessed on: June 28, 2012. ||
 * **Staging:** || Staging system used in National Wilms Tumor Study 3 and 4: [5]
 * Surface of renal capsule is intact
 * Tumor was not ruptured before or during removal
 * No residual tumor apparent beyond the margins of resection
 * Tumor extends beyond the kidney but is completely excised
 * No residual tumor is apparent at or beyond the margins of excision
 * Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma
 * Regional extension of the tumor- penetration through the outer surface of the renal capsule into the perirenal soft tissue
 * Vessels outside the kidney substance are infiltrated or contain tumor thrombus
 * No residual tumor apparent at or beyond the margins of excision
 * Unresectable primary tumor
 * Lymph node metastasis
 * Positive surgical margins
 * Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus (This includes if the tumor has been biopsied prior to removal or there is spillage of tumor during surgery, confined to the flank)
 * Residual non hematogenous tumor confined to abdomen (any one of the following occur):
 * Lymph nodes on biopsy are found to be involved in the hilus, the periaortic chains, or beyond
 * The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank
 * There has been peritoneal contamination by tumor such as by spillage of tumor beyond the flank before or during surgery, or by tumor growth that has penetrated through the peritoneal surface
 * Implants are found on the peritoneal surfaces
 * The tumor extends beyond the surgical margins either microscopically or grossly
 * The tumor is not completely resectable because of local infiltration into vital structures
 * Deposits beyond stage III (eg. Lung, liver, bone, and brain)
 * Staging, which is the same for tumors with favorable and with unfavorable histologic features
 * Tumors of unfavorable type are those with focal or diffuse anaplasia, or those with sarcomatous histology ||
 * **Radiation side effects:** || Some of the late effects of Radiation Therapy (RT) on a Wilms tumor (depending on the area of RT) are: [6]
 * Mild scoliosis and mild asymmetry
 * Decrease in height
 * Increased risk of osteoporosis (especially involving the spine) and degenerative disease
 * Mild restrictive problem due to reduced growth of the chest wall and ribs
 * Breast hypoplasia (in females)
 * Decreased lung capacity
 * Decreased vital capacity
 * Hypothyroidism
 * Thyroid nodules
 * Papillary carcinoma of the thyroid
 * Congestive heart failure
 * Cardiomyopathy
 * Hepatomegaly
 * Ascites
 * Icterus
 * It is very important to check Wilms tumor survivor’s blood pressure on a regular basis
 * Chronic renal failure (bilateral Wilms tumor with partial nephrectomy)
 * Ovarian failure
 * 70% are amenorrheic
 * Risk of early menopause
 * Testicular damage with elevated FSH
 * Azoospermia
 * Severe Oligospermia
 * Premature births
 * Underdeveloped uterine wall and anomalies
 * Surgical adhesions
 * Lower birth weight babies
 * Increased perinatal deaths
 * Significant risk of second malignancy
 * Increased risk of breast cancer after pulmonary RT (in females) ||
 * **Prognosis:** || Poor prognosis is noted in patients with extensive tumors, diploid tumors, unfavorable (anaplastic) histology, and chromosomal loss in 1p and 16q. Lymph node involvement is a prognostic factor as well. [1] ||
 * **Treatments:** || Surgery is commonly used; however the surgeon must excise all tumor, without spillage, if possible. A transperitoneal incision is an important surgical technique to explore the entire abdomen. Tumors with favorable histology (FH) are responsive to chemotherapy and radiation therapy; however, radiation therapy can cause potentially long-term deleterious effects. Radiation should start within 10 days of surgery. [1]
 * For stage III FH, a randomized study elected to treat all abdominal disease with 10 Gy, plus a 10 Gy boost to gross (>3 cm) residual disease. Femoral heads and acetabulum must be blocked.
 * When disease is confined to the operative site, flank irradiation is used with parallel-opposed fields using 4 or 6 MV. The treatment portals should include 2 cm - 3 cm margin around the tumor bed and excised kidney.
 * Whole lung irradiation for stage IV (if lung mets) using 12-16 Gy. [1]
 * **TD 5/5:** || Femoral head: Whole 5200 cGy
 * **References:** || 1. Chao KS, Perez CA, Brady LW. //Radiation Oncology: Management Decisions.//Philadelphia, PA: Lippincott Williams & Wilkins; 2002.

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