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Wilms tumor

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sheena bhatia

Detailed Powerpoint Presentation on Wilms Tumour . It includes definition with images, causes, sign and symptoms all treatment modalities with nursing responsibilities and recent research related to this...

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PREPARED BY SHEENA BHATIA WILMS TUMOR
Definition Wilms Tumor is also known as Nephroblastoma. It is a highly malignant embryonal neoplasm. It may involve one or both kidney.
INCIDENCE AND ETIOLOGY Usually the tumor is unilateral, but in 5% cases it may be bilateral. The tumor involves left kidney more than right kidney. It affects children between 3-5 year of age. CONT.
CONT.. The disease occur in about 1 out of 2-2.5 lakh children. The exact cause of tumor is unknown, but it has been identified that tumor suppressor gene acts to promote normal kidney development. This gene may be absent or missing in wilms tumor.
Wilms tumor
PATHOPHYSIOLOGY Mostly wilms tumor is unilateral but it can be bilateral in 5% of cases Nephroblastoma are generally large and rapidly growing. Tumor generally start growing in renal parenchyma or at the tip of kidney. cont.
Cont. It causes suppression of normal tissue remaining Majority of tumors present a as single encapsulated mass, that separates the normal kidney and tumor. Although the tumor is encapsulated but the membrane may be very thin and get easily torn Rupture of tumor put patient at the risk of hemmorhage and dissemination of tumor
CLINICAL FEATURES Presence of abdominal mass. Pain ,if tumor is enlarging Anorexia Hematuria Nausea and vomiting Urinary tract infection
STAGES OF WILMS TUMOR STAGE I (43% CASES ) - Tumor limited to kidney and completely resectable. STAGE II (23% CASES) - Tumor extend beyond kidney , into nearby fatty tissue, but it is resectable. STAGE III (23% CASES) Non hematogenous spread in abdomen, like spread to lymph nodes in abdomen or pelvis, but this stage tumor is not completely resectable. CONT.
CONT STAGE IV (10% CASES) - Hematogenous metastatis to lungs and liver. STAGE V (5% CASES)- Bilateral renal involvement.
DIAGRAMATIC PRESENTATION OF STAGES
DIAGNOSTIC EVALUATION History- The child may have positive family history. Physical examination reveals presence of abdominal mass. Urinanalysis reveals presence of blood in urine. Abdominal x-ray
Cont. Ultrasound Chest X- ray to detect metastasis to lungs. Increased Blood urea nitrogen, creatinine values.
MANAGEMENT The management of children with wilms tumor include :- Radiation therapy Chemothrapy Surgical management
Radiation Therapy Wilms tumor may be bilateral or large in size , may be inoperable, for such cases radiation therapy may be used to reduce the size of tumor, so that surgery can be performed.
CHEMOTHERAPY The objective of chemotherapy is to treat any metastatic lesions that may exist and destroy any cells in blood stream, before they get implanted. The drugs used for chemotherapy are Actinomycin D, Doxorubicin and Vincristine.
SURGICAL MANAGEMENT Partial or complete nephrectomy is done for unilateral and for bilateral partial nephrectomy is done. After surgical management, chemotherapy and radiation therapy is given if indicated.
TREATMENT Treatment for wilms tumor is based mainly on the stages of the cancer:- STAGE I- These tumor are still only in the kidney. Standard treatment starts with surgery to remove the part of kidney containing tumor. These children needs to be watched closely because the chances of recurrence are higher.The chemotherapy is given for 18 weeks.
cont.. STAGE III- Treatment is usually surgery followed by radiation therapy to the abdomen over several days . This is followed by chemotherapy for about 6 months. STAGE IV- These tumors are already spread to distant parts of the body at the time of diagnosis, so standard treatment id surgery followed by radiation and chemotherapy.
Cont STAGE V- In this stage usually tumor is bilaterally present, standard treatment involves surgery, radiation and chemotherapy repeatedly until normal kidney tissue left behind. In case if not enough kidney tissue is left after surgery that child may need to place on dialysis. If there is no evidence of any cancer after year or two, a donor kidney transplant may be done.
NURSING MANAGEMENT PREOPERATIVE CARE Prepare the parents and child for surgery. Expalin parents not to palpate the abdomen of the child. Explain child about post operative care , if he or she is old enough. Caution should be taken while turning and handling the child.
POST OPERATIVE CARE Monitor vitals signs. Monitor renal functioning by monitoring weight, intake output and KFT values. Obsesrve for the signs of functioning. Use aseptic techniques while doing dressing Explain parents about follow up and continuing treatment at the time of discharge.
Current Research Research on diagnostic procedures and treatment for Wilms tumor is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your childs doctor.
New tumor markers. A tumor marker, also called a serum marker or biomarker, is a substance found in a person's urine, or body tissue. Doctors in the United Kingdom recently discovered that children with Wilms tumor who have a recurrence all have active genes on one area of a specific chromosome (the 1q area). Children can be tested to see if their 1q genes are active, which can help doctors plan treatment. Once the specific genes are identified, it is that new tests may be developed to predict which children likely to have a recurrence.
Genetics. A new Wilms tumor gene (WTX) has been identified on the X chromosome and plays a role in normal kidney development. This gene is not active in approximately one-third of children with Wilms tumor. Studies on the importance of this gene are ongoing. New therapy combinations. Researchers are looking at whether patients with a favorable histology Wilms tumor who have metastases in the lungs need radiation to the lungs if the lung tumors go away after six weeks chemotherapy.
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Wilms tumor

  • 2. Definition Wilms Tumor is also known as Nephroblastoma. It is a highly malignant embryonal neoplasm. It may involve one or both kidney.
  • 3. INCIDENCE AND ETIOLOGY Usually the tumor is unilateral, but in 5% cases it may be bilateral. The tumor involves left kidney more than right kidney. It affects children between 3-5 year of age. CONT.
  • 4. CONT.. The disease occur in about 1 out of 2-2.5 lakh children. The exact cause of tumor is unknown, but it has been identified that tumor suppressor gene acts to promote normal kidney development. This gene may be absent or missing in wilms tumor.
  • 6. PATHOPHYSIOLOGY Mostly wilms tumor is unilateral but it can be bilateral in 5% of cases Nephroblastoma are generally large and rapidly growing. Tumor generally start growing in renal parenchyma or at the tip of kidney. cont.
  • 7. Cont. It causes suppression of normal tissue remaining Majority of tumors present a as single encapsulated mass, that separates the normal kidney and tumor. Although the tumor is encapsulated but the membrane may be very thin and get easily torn Rupture of tumor put patient at the risk of hemmorhage and dissemination of tumor
  • 8. CLINICAL FEATURES Presence of abdominal mass. Pain ,if tumor is enlarging Anorexia Hematuria Nausea and vomiting Urinary tract infection
  • 9. STAGES OF WILMS TUMOR STAGE I (43% CASES ) - Tumor limited to kidney and completely resectable. STAGE II (23% CASES) - Tumor extend beyond kidney , into nearby fatty tissue, but it is resectable. STAGE III (23% CASES) Non hematogenous spread in abdomen, like spread to lymph nodes in abdomen or pelvis, but this stage tumor is not completely resectable. CONT.
  • 10. CONT STAGE IV (10% CASES) - Hematogenous metastatis to lungs and liver. STAGE V (5% CASES)- Bilateral renal involvement.
  • 12. DIAGNOSTIC EVALUATION History- The child may have positive family history. Physical examination reveals presence of abdominal mass. Urinanalysis reveals presence of blood in urine. Abdominal x-ray
  • 13. Cont. Ultrasound Chest X- ray to detect metastasis to lungs. Increased Blood urea nitrogen, creatinine values.
  • 14. MANAGEMENT The management of children with wilms tumor include :- Radiation therapy Chemothrapy Surgical management
  • 15. Radiation Therapy Wilms tumor may be bilateral or large in size , may be inoperable, for such cases radiation therapy may be used to reduce the size of tumor, so that surgery can be performed.
  • 16. CHEMOTHERAPY The objective of chemotherapy is to treat any metastatic lesions that may exist and destroy any cells in blood stream, before they get implanted. The drugs used for chemotherapy are Actinomycin D, Doxorubicin and Vincristine.
  • 17. SURGICAL MANAGEMENT Partial or complete nephrectomy is done for unilateral and for bilateral partial nephrectomy is done. After surgical management, chemotherapy and radiation therapy is given if indicated.
  • 18. TREATMENT Treatment for wilms tumor is based mainly on the stages of the cancer:- STAGE I- These tumor are still only in the kidney. Standard treatment starts with surgery to remove the part of kidney containing tumor. These children needs to be watched closely because the chances of recurrence are higher.The chemotherapy is given for 18 weeks.
  • 19. cont.. STAGE III- Treatment is usually surgery followed by radiation therapy to the abdomen over several days . This is followed by chemotherapy for about 6 months. STAGE IV- These tumors are already spread to distant parts of the body at the time of diagnosis, so standard treatment id surgery followed by radiation and chemotherapy.
  • 20. Cont STAGE V- In this stage usually tumor is bilaterally present, standard treatment involves surgery, radiation and chemotherapy repeatedly until normal kidney tissue left behind. In case if not enough kidney tissue is left after surgery that child may need to place on dialysis. If there is no evidence of any cancer after year or two, a donor kidney transplant may be done.
  • 21. NURSING MANAGEMENT PREOPERATIVE CARE Prepare the parents and child for surgery. Expalin parents not to palpate the abdomen of the child. Explain child about post operative care , if he or she is old enough. Caution should be taken while turning and handling the child.
  • 22. POST OPERATIVE CARE Monitor vitals signs. Monitor renal functioning by monitoring weight, intake output and KFT values. Obsesrve for the signs of functioning. Use aseptic techniques while doing dressing Explain parents about follow up and continuing treatment at the time of discharge.
  • 23. Current Research Research on diagnostic procedures and treatment for Wilms tumor is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your childs doctor.
  • 24. New tumor markers. A tumor marker, also called a serum marker or biomarker, is a substance found in a person's urine, or body tissue. Doctors in the United Kingdom recently discovered that children with Wilms tumor who have a recurrence all have active genes on one area of a specific chromosome (the 1q area). Children can be tested to see if their 1q genes are active, which can help doctors plan treatment. Once the specific genes are identified, it is that new tests may be developed to predict which children likely to have a recurrence.
  • 25. Genetics. A new Wilms tumor gene (WTX) has been identified on the X chromosome and plays a role in normal kidney development. This gene is not active in approximately one-third of children with Wilms tumor. Studies on the importance of this gene are ongoing. New therapy combinations. Researchers are looking at whether patients with a favorable histology Wilms tumor who have metastases in the lungs need radiation to the lungs if the lung tumors go away after six weeks chemotherapy.