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Askep

TUMOR WILMS

Ni Nyoman
Udiani,
S.Kep.Ns.,M.Kep
BATASAN
Tumor ganas ginjal yang berasal dari sisa
jaringan embrional primitive di ginjal

Sinonim :

Nephroblastoma
Embrioma Ginjal
Adenosarkoma Ginjal
Karsinosarkoma Ginjal
Tumor ganas campuran ginjal
EPIDEMIOLOGI
Amerika :
- Insiden TW : 7,8/1 juta populasi usia 1-14 tahun.
- Setiap tahun terdapat 500 penderita baru
- 77% usia penderita ≤ 5 th
- 90% usia penderita ≤ 7 th
- Penderita ♂∽♀
- Faktor Ras ⊝
- Faktor lingkungan ⊝

RS. Dr. Sutomo:


- 4 tahun 20 penderita
- usia < 5 th : 17 penderita
- ♂ : ♀ = 13 : 7
Description
 Wilms tumor, or nephroblastoma, is the
most common childhood abdominal
malignancy.
 Wilms tumor is an adenosarcoma in the 
kidney region.
 The tumor arises from bits of embryonic
tissue that remain after birth.
 This tissue can spark rapid cancerous
growth in the area of the kidney.
Causes
 Wilms tumor is thought to be caused by
alterations of genes responsible for normal
genitourinary development.
 WT1 gene. WT1, the first Wilms tumor
suppressor gene at chromosomal band
11p13, was identified as a direct result of
the study of children with Wilms tumor
who also had aniridia, genitourinary
anomalies, and mental retardation (WAGR
syndrome).
 Additional genetic loci. A second gene
that predisposes individuals to develop the
Wilms tumor has been identified (but has
not yet been cloned) telomeric of WT1, at
11p15; this locus was proposed on the
basis of studies in patients with both
Wilms tumor and Beckwith-Wiedemann
syndrome (BWS), another congenital
Wilms-tumor predisposition syndrome
linked to chromosomal band 11p15.
Pathophysiology
 Wilms tumor is a malignant mixed tumor
containing metanephric blastoma, stromal,
and epithelial derivatives.

 There are mutations of WT1 gene on


chromosome 11 and nephroblastematosis
(persistence of renal blastema in kidney
tissue.
 The tumor consists of tumor epithelial
component (abortive tubules and
glomeruli) surrounded by metanephric
blastema and tumor immature spindle cell
stroma.
 The stroma may include differentiated (
muscle, cartilage, bone, fat tissue, fibrous
tissue) or anaplastic elements.
 The tumor compresses the normal kidney
parenchyma.
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.
KELUHAN UTAMA

Terdapat/ teraba masa padat di perut (80%)


Nyeri perut (30%) → biasanya karena perdarahan
intra tumor
Mual dan muntah (20%)
Dibawa ke dokter karena menderita akut abdomen
karena tumor ruptur.
Pada 50% penderita disertai hipertensi
Assessment and Diagnostic
Findings
 Laboratory studies. 
Complete blood count, chemistry profile,
urinalysis, coagulation studies, and cytogenic
studies are made to determine Wilms tumor;
results may reveal an 11p13 deletion, as in
WAGR syndrome, or a duplication of the
paternal allele 11p15, as in Beckwith-
Wiedemann syndrome (BWS).
 Renal ultrasonography. 

Renal ultrasonography is often the initial study


because it does not expose children to the
detrimental effects of radiation
 CT scanning. Abdominal computed
tomography (CT) scanning helps in
determining the origin of the tumor,
involvement of the lymph nodes, bilateral
kidney involvement, invasion into major
vessels (eg, inferior vena cava), and liver
metastases.
 MRI scanning. Abdominal magnetic
resonance imaging (MRI) is reportedly the
most sensitive imaging modality for
determination of caval patency and may
be important in determining whether the
inferior vena cava is directly invaded by
the tumor.
PEMERIKSAAN RADIOALOGI
USG : - Membedakan kistik atau solid
- Melihat ginjal kontralateral
IVP : Foto polos : ukur bayangan massa/suram → lewat garis
tengah ?
Foto kontras : - Detorsi/pendesakan pcs.
- Delatasi kaliks.
- Dapat non visualized (7-30%)
- Perhatikan ginjal kontralateral.
CT Scan abdomen (dengan kontras) :
- Lebih preferable daripada IVP
- Dapat untuk melihat hubungan dengan adjacent structure →
operabilitas
GAMBARAN HISTOLOGI
Beckwith dan Palmer, membagi histologik TW menjadi :

I. Favourable → prognosa lebih baik


II. Unfavourable → prognosa lebih jelek
A. Anaplastik : - Inti besar, sp 3 x N.
- Hiperkromatik
- Mitosis ↗
B. Rhabduid : - Sel besar, uniform
- Inti besar
- Nukleoli prominen
C. Sarkomatoid: - Sitoplasma sedikit dan jernih
Kelainan lain yang dapat menyertai
Tumor WILMS :
Aniridia
Hemihipertrofi
Anomali Genital :
- Kriptorkhismus
- Hipospadia
- Varicocele
Anomali Tr. Urinarius :
- Duplikasi
- Ginjal Tapal Kuda
- Ginjal Ektopik

Frekwensi kelainan tersebut : 15%


STADIUM TUMOR WILMS
( menurut NWTS )
Stadium Batasan
I Tumor terbatas dalam ginjal, dapat dieksisi in toto
tanpa spillage/robekan kapsul.

II Tumor telah menembus kapsul ginjal tetapi dapat


dieksisi semua atau ada spillage tetapi hanya di dalam
fossa renalis.
III Ada sisa tumor (non hematogenik) terbatas dalam
rongga perut.
IV Terdapat metastase hematogen, misalnya :
di otak, di paru atau ekstra abdominal.

V Tumor bilateral.
TERAPI TUMOR WILMS
Penanganan multimodal : operasi, kemoterapi dan
radiasi
Pada stadium dini : kesembuhan dapat 100%

1. Pembedahan :
 Nephrektomi transperitoneal
 Bila N ⊕ → Remove
 Bila tumor besar atau operabilitasnya
meragukan → kemoterapi preoperatif →
me ↓spillage intraoperatif
TERAPI TUMOR WILMS

2. Kemoterapi : Obligatory untuk semua kasus


 Untuk stadium I &II diberi :
- Vincristine & Actinomycin D
 Untuk stadium III, IV, V, ditambah
- Doxorubicin
- Siklophosfamide
- Etoposide atau carboplatin
3. Radioterapi
Indikasi :
1. Favourable histology; stadium III, IV,V,
2. Unfavourable histology, stadium II,III,IV,V.

PROGNOSA : Stadium dini (I&II) dengan terapi multimodal →


100% sembuh.
Side Effects of Treatment
Acute:
 Loose stools

 Nausea –provide prophylactic anti-emetics

 Fatigue
Long term:
Bowel adhesions
Infertility (females > males) or future
pregnancy complications
Greater in females treated with whole
abdomen with both ovaries and uterus in
the field.
Scoliosis/vertebral body foreshortening
Hypertension
 Secondary to fibrosis of contralateral renal
artery
 Renal failure (low incidence if treating
flank)
 CHF –risk is ~4% in patients receiving
adriamycin1
 Liver failure (chemotherapy associated)
 2ndmalignancy (1.6% cumulative risk)
Asuhan Keperawatan
Tumor Wilms???
Nursing Assesment
 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.
Nursing Diagnoses
 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.
Nursing Care Planning and
Goals
 Child will be protected from illness or
injury.
 Child will be free of oral mucous
membrane irritation.
 Clients will experience decreased anxiety.
 Child will not experience injury.
Nursing Interventions
 Prevent transfer of microorganism. 

Perform handwashing prior
giving care, utilize mask and gown when
needed, provide a private room, monitor
for any signs and symptoms of infection.
 Prevent oral trauma. 
Instruct the use of a soft-sponge
toothbrush or sponge toothette or gauze
when rinsing the mouth; instruct to avoid
foods which are hot, spicy, or high
in ascorbic acid (vitamin C); provide oral
hygiene 30 minutes prior or after meals;
instruct to refrain from eating or drinking
for 30 minutes after completion of oral
hygiene; and offer moist, soft, bland foods.
 Prevent anxiety. 
Have the parents to stay with the child or
encourage open visitation, provide a telephone
number to call for information; explain all
procedures and care in simple, direct, honest
terms and repeat as often as necessary;
reinforce physician information if needed and
provide specific information as needed; and
provide consistent nurse assignment with the
same personnel; encourage parents to
participate in care.
 Prevent anxiety. 
Have the parents to stay with the child or
encourage open visitation, provide a telephone
number to call for information; explain all
procedures and care in simple, direct, honest
terms and repeat as often as necessary;
reinforce physician information if needed and
provide specific information as needed; and
provide consistent nurse assignment with the
same personnel; encourage parents to
participate in care.
 Prevent injury. 
Avoid any palpation of abdominal mass; post
sign on bed stating not to palpate
preoperatively; assess incision site for
redness, swelling, drainage, intactness,
and healing and change dressing when soiled
or wet; assess oral and perineal area; and
encourage parents to appropriately dress
child based on weather conditions and to
refrain from participating on rough activities
or sports.
Evaluation
Goals are met as evidenced by:

 Child is protected from illness or injury.


 Child is free of oral mucous membrane
irritation.
 Clients experienced decreased anxiety.
 Child did not experience injury.
Thank you for your attention

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