Wilm's+Tumor

Excretory Urogram, Ultrasonography, CT. CT shows early lesions. 1 ||
 * Epidemiology: || The peak age is between 3 and 4 years old. More common in black children than white children. More common in girls than in boys. 6 ||
 * Etiology: || A higher risk of Wilms' tumor occurs in patients with multiple genitourinary abnormalities, hemihypertrophy and aniridia, and the facial abnormality syndrome Beckwith-Wiedemann. A Wilms' tumor gene has recently been discovered and represents a deletion on chromosome 11p13. 8 ||
 * Signs & Symptoms: || Swelling in abdominal area. Gross hematuria in 25% of cases. Malaise or fever. Symptomatic metastases is rare. 1 ||
 * Diagnostic Procedures: || Films may show calcification in 15% of patients.
 * Histology: || Wilms' tumor is, in general, solid and confined by the renal capsule, although it may contain necrotic or cystic areas. However, the tumor frequently infiltrates through the kidney capsule into adjacent structures, and there may be direct extension into the pelvis and ureter or the renal vein and vena cava. Microscopically, the tumor may contain varying amounts of three tissue elements: blastema, epithelia, and stroma. On occasion, skeletal muscle, cartilage, and squamous epithelium have been found.

There are two pathologic entities associated with Wilms' tumor: congenital mesoblastic nephroma and nephroblastomatosis. Clear cell sarcomas and rhabdoid tumors of the kidney are considered separate entities from Wilms' tumor. 5 || Hematogenous metastases can occur in the lung, liver, bone, or brain. 6 || Favorable Histology (FH) Unfavorable Histology (UH) 3 || (A) +abdominal nodes; (B) penetration of peritoneal surface or tumor spillage not confined to flank; (C) peritoneal implants; (D) +margins (gross or microscopic); (E) not completely resectable due to extension to other organs.
 * Lymph Node Drainage: || Lymph node drainage is involved in the hilum or periaortic chains . 6 ||
 * Metastatic Spread: || “The lungs are the most common metastatic site, followed by the liver. In NWTS-2, 57 patients (11.4%) had metastases at diagnosis; 47 of these had pulmonary metastases only”. 1 
 * Grading: || The NWTS classifies all tumors into 2 groups:
 * Staging: || __NWTS - 5 staging system (National Wilms' Tumor Study)__
 * I:** Tumor limited to kidney, completely resected. Renal capsule intact. Tumor not ruptured or biopsied prior to resection. Vessels of renal sinus not involved or <2mm. Margins negative.
 * II:** Tumor extends beyond kidney but is completely excised. Regional extension (e.g., penetration of renal capsule, or blood vessels involved __>__ 2mm); blood vessel outside of renal parenchyma contain tumor. Tumor was biopsied or tumor spillage confined to the flank. Negative margins.
 * III:** Residual non-hematogenous tumor confined to abdomen:
 * IV:** Hematogenous mets or lymph node mets outside of abdomen or pelvis.
 * V:** Bilateral renal tumors at diagnosis. 2 ||
 * Radiation Side Effects: || <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">Due to radiation doses of 20 Gy or less, there are minimal side effects from radiation. <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">8 <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">

-Scoliosis can occur at doses over 24 Gy. <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">1 <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;"> -Musculoskeletal abnormalities, cardiac abnormalities, secondary malignancies, intestinal obstruction, arterial hypertension, and renal insufficiency have also been observed. <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">1 <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;"> -If the thorax is irradiated (due to lung metastases), lung fibrosis and impaired breast development (in female patients) may occur with doses over 15 Gy. <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">8 ||
 * Prognosis: || <span style="font-family: Arial,Helvetica,sans-serif;">**<span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">4-year survival rates of NWTS-3 patients ** <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">8

<span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">__Relapse Free Rate__ I (FH): 90% II (FH): 88% III (FH): 79% IV (FH): 75% I-III (UH): 65% IV (UH): 55%

<span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">__Overall Rate__ I (FH): 96% II (FH): 92% III (FH): 86% IV (FH): 82% I-III (UH): 68% IV (UH): 55% || * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">The diagnosis of Wilms' tumor is usually made preoperatively and confirmed at surgery. * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Anesthesia or sedation is often required for daily treatment of these children.
 * Treatments: || **<span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">General Management **<span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Preoperative therapy is not commonly practiced, although it has been examined in clinical trials.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Meticulous surgical techniques for exploring the abdomen through a transperitoneal incision are essential. The surgeon must excise all tumor, without spillage, if possible.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Thorough assessment and sampling of lymph nodes and inspection of the liver and opposite kidney should be performed.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Most favorable histology (FH) tumors are responsive to irradiaiton and chemotherapy.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Because of the potential long-term deleterious effects of radiaton therapy, it plays a relatively minor role compared with that of chemotherapy.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Unfavorable histology (UH) tumors are less responsive to either modality and generally are treated with aggressive multimodality regimens.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Radiation Therapy Techniques **<span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Although radiation doesn not need to be given immediately after operation, treatment timing is important. Patients in whom irradiation was delayed for 10 days or more from surgery ahad a significantly higher chance of abdominal relapse, particularly those with UH tumors.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Because the pathologist cannot always rule out UH quickly, all patients with Wilms' tumors should be scheduled to start radiation within 10 days after surgery. Most patients with ultimately not be treated, but it is easier to cancel than to make arrangements to intiate irradiation for a small child on short notice.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">No radiation therapy is needed for stage I and stage II FH tumors.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">All other tumors should be treated to a dose of 10 Gy to the abdomen plus a 10 Gy boost to gross residual disease after surgery.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Patients with disease confined to the operative site need only flank irradiaiton, even if there has been local spillage of tumor.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Parallel-opposed fields using 4 to 6 MV photon are preferred.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Treatment portals should encompass the tumor bed and site of the excised kidney with a 2 to 3 cm margin. The medial border must cross the midline to include the entire width of the vertebrae to minimize growth disturbances.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">A tangential abdominal wall shield can be used.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">When whole-abdomen irradiation is administered, shaped portals must be used, and the femoral heads and acetabulum must be shielded.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Whole lung irradiation is used if there are lung metastases.
 * <span style="color: #800000; font-family: Arial,Helvetica,sans-serif;">Dosages for FH bilateral Wilms' tumor should be limited to 10 Gy to the second kidney . 1 ||
 * TD5/5: || <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">The following organs have the potential to fall within the treatment field for the primary tumor or lung metastases. <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">7 <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">

Bladder: 65Gy (whole); 80Gy (2/3) Cauda equina: 60Gy (whole) Colon: 45Gy (whole); 55Gy (1/3) Esophagus: 55Gy (whole); 58Gy (2/3); 60Gy(1/3) Heart: 40Gy (whole); 45Gy (2/3); 60Gy (1/3) Kidney: 23Gy (whole); 30Gy (2/3); 50Gy (1/3) Liver: 30Gy (whole); 35Gy (2/3); 50Gy (1/3) Lung: 17.5Gy (whole); 30Gy (2/3); 45Gy (1/3) Rib Cage: 50Gy (1/3) Skin: 50Gy (100cm <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">2 <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">); 60Gy (30cm <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">2 <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">); 70Gy (10cm <span style="color: #008000; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">2 <span style="color: #008000; font-family: Arial,Helvetica,sans-serif;">) Spinal Cord: 47Gy (20cm); 50Gy (10cm); 50Gy (5cm) Small Intestine: 40Gy (whole); 50Gy (1/3) Stomach: 50Gy (whole); 55Gy (2/3); 60Gy (1/3) ||
 * Planning Photos || <span style="color: #0000ff; font-family: Arial,Helvetica,sans-serif;">Digitally Reconstructed Radiograph (DRR) example of anterior-posterior right flank portal showing inclusion of entire width of vertebral body in irradiated volume to minimize growth disturbances. <span style="color: #0000ff; font-family: Arial,Helvetica,sans-serif; font-size: 80%; vertical-align: super;">4

|| <span style="font-family: Arial,Helvetica,sans-serif;">**References** 1. Chao KS, Perez CA, Brady LW. //<span style="font-family: Arial,Helvetica,sans-serif;">Radiation Oncology - Management Decisions. // <span style="font-family: Arial,Helvetica,sans-serif;"> 2nd ed. Philadelphia: Lippincott, Williams & Wilkins, 2002: 641-647. 2. Hansen E, Haas-Kogan D. Pediatric (Non-CNS) Tumors. In: Hansen EK, Roach M. //<span style="font-family: Arial,Helvetica,sans-serif;">Handbook of Evidence-based Radiation Oncology, //<span style="font-family: Arial,Helvetica,sans-serif;"> 1st ed. New York: Springer, 2007: 432-471. 3. eMedicine. Wilms Tumor. []. Accessed February 4, 2010. 4. Digitally Reconstructed Radiographs courtesy of Ginnie Dea, RT(T), Alta Bates Summit Comprehensive Cancer Center. 5. Rubin P. //<span style="font-family: Arial,Helvetica,sans-serif;">Clinical Oncology - A Multidisciplinary Approach for Physicians and Students //. 8th ed. Philadelphia: W.B. Saunders Company, 2001:353. 6. Gunderson and Tepper. Clinical Radiation Oncology. 2nd Edition. Philadelphia, PA: Churchill Livingstone. 2007. 7. <span style="font-family: Arial,Helvetica,sans-serif;">Radiation Oncology/Toxicity/Emami. []. Accessed February 10, 2010. 8. <span style="font-family: Arial,Helvetica,sans-serif;">Washington CM, Leaver D. //<span style="font-family: Arial,Helvetica,sans-serif;">Principles and Practice of Radiation Therapy. // <span style="font-family: Arial,Helvetica,sans-serif;">2nd ed. Philadelphia, PA: Mosby Inc; 2004: 884-6.

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