Wilms tumor.pptx

genito urinary disorder

1. WILMS TUMOR Ms. K. Lavanya MSc(N)-CHN Associate Professor

2. Definition: Wilms tumor or nephroblastoma, is the common malignant renal and intra-abdominal tumor of childhood described by German surgeon Max Wilms. It is associated with chromosomal deletions, especially from chromosomes 11 and 16. It is unilateral or bilateral .   It may be associated with other congenital anomalies, like hemi hypertrophy of the vertebrae, genitourinary anomalies, aniridia , ambiguous genitalia etc.   This tumor develops within the kidney parenchyma, distorting it and invading the surrounding tissues.

4. Etiology: Chromosomal Hereditary   Risk Factors: African-American race Family history with wilm’s tumor  

5. Pathophysiology :

6. Stages : Stage 1.  The tumor is limited to kidney and is completely resected; the renal capsule is intact; the tumor was not ruptured or biopsied prior to removal; the vessels of the renal sinus are not involved, and no evidence of tumor is present at or beyond the margins of resection. Stage 2.  The tumor is completely resected; no evidence of tumor at or beyond the margins of resection is noted; and the tumor extends beyond the kidney (penetration of renal capsule, involvement of renal sinus). Stage 3.  A residual, nonhematogenous tumor is present following surgery and is confined to the abdomen; positive lymph nodes in the abdomen or pelvis are noted; penetration through the peritoneal surface is observed; peritoneal implants are present; gross or microscopic tumor remains postoperatively, including positive margins of resection; tumor spillage is noted, including biopsy; the tumor is treated with preoperative chemotherapy; and the tumor is removed in more than 1 piece.

7. Stages : Stage 4.   Hematogenous metastases ( eg , lung, liver, bone, brain) or lymph node metastases beyond the abdomen or pelvis are noted. Stage 5.  Bilateral renal involvement by the tumor is present at diagnosis.  

8. Clinical Manifestations: Abdominal mass.  The most common manifestation of Wilms tumor is an asymptomatic abdominal mass; an abdominal mass occurs in 80% of children at presentation. Abdominal pain.  Abdominal pain or hematuria occurs in 25%. Tumor hemorrhage.  A few patients with hemorrhage into their tumor may present with hypotension, anemia, and fever. Fatigue Metastasis, symptoms of lung involvement: Dyspnea Cough Shortness of breath Chest pain

9. Diagnostic Evaluation: History of illness Physical Examination X-ray- chest and abdomen Radiographic tests include- Abdominal ultrasound Abdominal and chest CT scan Hematological studies Biochemical studies Urinalysis Bone marrow aspiration to rule out metastasis Inferior venacavagram - to rule out tumor involvement adjacent to the venacava.

10. Management : Treatment consists of surgical removal as soon as possible, after the growth is discovered, combined with radiation and chemotherapy.   stage I-III Wilms tumors and diffuse anaplastic stage I Wilms tumors.  Nephrectomy followed by vincristine, actinomycin-D, and doxorubicin in addition to local radiotherapy.   Stage IV Wilms tumors and diffuse anaplastic stage II-III tumors.  Patients undergo the same treatment, with the addition of cyclophosphamide, etoposide, and carboplatin.

11. Contd …. Stage IV diffuse anaplastic Wilms tumors.  More aggressive treatment is delivered; nephrectomy is followed by initial irinotecan and vincristine administration, which in turn is followed by actinomycin-D, doxorubicin, cyclophosphamide, carboplatin, etoposide, and radiotherapy.   Activity. No precautions regarding activity are advised, although the patient and his or her parents should be aware that the patient will have only 1 kidney after therapy; activities that carry an inherent risk of kidney injuries, such as boxing and hockey, should be avoided.  

12. Contd …. Antineoplastics .  These chemotherapeutic agents used to treat patients with Wilms tumor depend on the stage and histology of disease; commonly used agents include  dactinomycin , vincristine, doxorubicin, cyclophosphamide, etoposide, and carboplatin; the dosage depends on the particular stage of the disease and on the child.

13. Nursing Management: Nursing Assessment Assessment of the child with Wilms tumor include: Assess for bleeding.  Assess for bleeding from any site and febrile episodes; Monitor WBC, platelet count, hematocrit, absolute neutrophil count. Assess the oral cavity.  Assess oral cavity for pain ulcers, lesions, gingivitis, mucositis or stomatitis and effect on the ability to ingest food and fluids. Assess for anxiety.  Assess source and level of anxiety and need for information and support that will relieve it.

14. Nursing Management: Ineffective protection  related to antineoplastic agents, radiation therapy, or leukopenia. Impaired oral mucous membrane  related to chemotherapy. Anxiety  related to change in health status and threat of death. Risk for injury  related to side effects of medications and complications.