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Case Presentation On Wilms Tumor
Case Presentation On Wilms Tumor
ON
WILM’S TUMOR
PRESENTER:
MODERATOR:
DEEPIKA TOMAR
MRS. RIMPLE SHARMA
MSc (N) 1ST YEAR,
ASSOCIATE PROFESSOR
CON, AIIMS, NEWDELHI
CON, AIIMS, NEWDELHI
Sociodemographic Data:
Name of the child: Nityansh Jain
Age: 4 years 1 month and 21 days
Gender: Male
Religion: Hindu
UHID NO.: 105528457
Address: RZ-B/9A Madanpuri, west Sagarpur, Delhi
Ward: AB5 Ward
Unit: Pediatric surgery
Bed no.: 19
Date of admission: 9/10/21 at 3:22 pm
Diagnosis: Lt. Wilm’s tumor
Classification: Pre-schooler
Surgery: Lt. NFU+LN Sampling under GA on 11/10/21
Date of discharge:
FAMILY HISTORY
Indicates patient
A) ANTENATAL HISTORY
This is not a consanguineous marriage, antenatal period was supervised, mother received
all immunization, There is no history of exposure to drugs,radiation,viral infection and
other infections like HIV,Syphillis,PIH,STD,Diabetes mellitus.
B) INTRANATAL HISTORY
LSCS at term, birth weight was 3kg, Baby cried immediately after birth, there is no
history asphyxia, cyanosis.
DEVELOPMENT HISTORY Normal gross motor and fine motor milestones with history of
delayed speech.
PHYSICAL EXAMINATION
WEIGHT- 15 kg
1. GENERAL APPEARANCE - Thin
2. BEHAVIOR - Conscious, alert, oriented
3. VITAL SIGNS - Temperature -37 c
Pulse rate -112/min
Respiratory rate - 36/mint
Blood pressure - 118/74 mmhg
4. HEAD SIZE AND SHAPE - Round in shape.
Alopecia seen
5. FACE - Normal
6. EAR - Normal and responds to sound.
7. EYES - Sclera is normal
8. MOUTH - Normal
9. NECK - There is no signs of thyroid swelling
10. TEETH - Clean and one incisor is dead in upper jaw.
11. SKIN - Normal color, turgor also good
12. LYMHNODES - Normal
13. RESPIRATORY SYSTEM - Bilateral air entry present, no abnormality detected.
14. CARDIOVASCULAR SYSTEM – S1S2 heard,
no murmur
Peripheral pulses palpable
HR – Regular
15. ABDOMEN - Firm, non-tender, no guarding/ rigidity. Hard lump
felt in left flank, full mass cannot be
assessed.
Spleen not palpable, Bowel sounds heard.
16. CENTRAL NERVOUS SYSTEM- Alert, conscious
17. GENITALIA - Normal
18. LOWER LIMBS - No abnormality detected.
INVESTIGATIONS
Each kidney is surrounded by three layers of tissue. A fibrous renal capsule covers the surface
of the kidneys. The adipose capsule is a mass of perirenal fat that surrounds the renal capsule. A
double layer of fascia called the renal fascia completely encloses the kidney and the adipose
capsule, firmly anchoring them to the abdominal wall.
Three general regions can be distinguished in each kidney. The cortex is the outer layer of the
kidney just deep into the renal capsule. The medulla is consist is consist of several triangular
renal pyramids and located deep into the cortex. The tips or papilla of the renal pyramids projects
into a funnel shaped chamber called a minor calyx. The minor calyces join up to form the renal
pelvis. Urine passes from the papillae into the minor calyces and then travels to the renal pelvis
through the ureter and to the urinary bladder.
The functional unit of the kidney is called the nephron. There are approximately 1 million
nephrons in each kidney. Each nephron is consisting of two parts: a glomerulus (a network of
capillaries) and a tubule. The tubule is an elongated hollow duct with the proximal end forming
a double walled cap known as the Bowman’s capsule. Another part of the tubule twists and
folds and another straightens after folding back in itself and yet another one forms characteristic
loops. These are the proximal and distal convoluted tubules and the loop of Henle.
Blood flows to the kidney via the renal artery, ultimately delivering to the glomerulus by means
of the afferent arteriole. Blood then drains from the glomerulus through the efferent arteriole.
This vessel carries blood from the glomerulus to the peritubular capillaries. It is a second larger
vessel network that surrounds the nephron tubule. From these vessels, blood moves into the veins
that drains the nephron and into the renal vein.
Physiology
The ability of the kidney to alter blood composition and maintain homeostasis is base on three
processes:
Glomerular Filtration– the physical separation of the blood’s formed elements and
its plasma proteins from the fluid component. It allows water and small molecules to
move from the glomerular capillary into the lumen of Bowman’s capsule. The fluid
product is called glomerular filtrate. Normal GFR is 180 liters per day.
Selective Reabsorption– substances in the filtrate pass from the nephrons back into
the blood of the peritubular capillaries avert their loss. Not all the filtrate is
reabsorbed, some are excreted. Selection depends on the relative permeability of the
tubule to various components.
Tubular Secretion– Upon completion of the reabsorption and secretion processes, the
remaining fluid is transported to components of the urinary system to be secreted as
urine.
Urine is composed of water and materials that has been filtered but not reabsorbed. Substances
present in urine represents amount in excess of those required for homeostasis.
DISEASE CONDITION
LT. WILM’S TUMOR
INTRODUCTION:
Wilms' tumor or nephroblastoma is malignant tumor of the kidneys that typically occurs in
children.
Dr. Max Wilms , the German surgeon (1867–1918) first described this kind of tumor.
Wilms tumor is the fifth most common pediatric malignancy (7% of all childhood tumors).
DEFINITION:
Wilm’s Tumor is also known as Nephroblastoma. It is a highly malignant embryonal neoplasm.
It may involve one or both kidney.
INCIDENCE:
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. The disease occurs in
about 1 out of 2-2.5 lakh children.
ETIOLOGY:
Unknown
Genetic abnormalities WT1 gene; dominant oncogene (at chromosome 11p13), WT2
gene (at chromosome 11p15)
It is believed that tumor begins to grow as a fetus develops in the womb, with some cells
that are destined to form into the kidneys malfunctioning and forming a tumor. The exact
etiology of the tumors are still being investigated.
Tumor is exceedingly vascular, soft, mushy, or gelatinous in character. • Wilms tumor
has capacity for rapid growth, usually grows to a large size.
Tumor is usually uniform, well demarcated by a pseudocapsule of compressed renal
tissue. • Tumor develops from primitive renal tissue and can have epithelial ( tubules and
glomeruli), stromal (fat, skeletal muscles, cartilage) and blastemal elements (An
immature material from which cells and tissues develop).
Wilms tumor may be associated with
o Hemihypertrophy-one side of body is larger than other
o Aniridia (complete loss of iris) and
o Genitourinary anomalies.
In this child: Unknown
PATHOPHYSIOLOGY:
Mostly wilm’s tumor is unilateral but it may be bilateral in less than 5% cases. Nephroblastomas
are generally large and rapidly growing lesions. They reach a considerable size before being
detected. They develop in Renal parenchyma, in either Central or polar location (tip of kidney).
The entire renal parenchyma may appear replaced by tumor with only a rim of compressed
normal tissues remaining. Majority of tumors present as a single expanding mass, surrounded by
a pseudocapsule of connective tissue, that separates the kidney and tumor. Although the tumor is
encapsulated, the membrane may be very thin and gets easily torn. A rupture of wilm's tumor
puts the patient at risk of hemorrhage and peritoneal dissemination of tumor. In cases of
metastasis, it is usually to lungs.
The first identified Wilms tumor gene, WT1 , located at 11p13, is homozygously mutated in 10–
15% of tumors, resulting in loss of function of the encoded zinc finger transcription factor. The
majority of WT1 mutations are somatic; however, WT1 mutations can also be germline.
Germline truncating mutations are usually associated with Wilms tumor in the context of
genitourinary anomalies or the WAGR syndrome (Wilms tumor, aniridia, genitourinary
anomalies, mental retardation).
CLINICAL FEATURES:
MANAGEMENT:
Management of wilm’s tumor includes:
1. Radiation therapy
2. Chemotherapy
3. Surgery
Most children with Wilms tumors will get more than one type of treatment.
Radiation Therapy
Wilm’s 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 for wilm’s tumor is based mainly on the stages of the cancer: -
STAGE I- These tumors are still only in the kidney. Standard treatment starts with surgery to
remove the part of kidney containing tumor. These children need to be watched closely because
the chances of recurrence are higher. If tumor comes back, chemotherapy (Actinomycin D and
Vincristine) is effective.
STAGE 11- These tumors have grown outside the kidney into nearby tissues, but surgery can
remove all visible signs of cancer. Standard treatment is surgery (radical nephrectomy), followed
by chemotherapy with Actinomycin D and Vincristine. The chemotherapy is given for 18 weeks.
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.
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.
IN THIS CHILD:
Underwent 6 chemotherapy prior to surgery and then followed by surgery (NFU + LN
Sampling).
NURSING MANAGEMENT:
PREOPERATIVE CARE
Prepare the parents and child for surgery.
Explain 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.
NURSING DIAGNOSIS
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.
Altered clotting
Bone marrow suppression
Deficient immunity against infection
Hematuria
Hemorrhagic cystitis
Petechiae, bleeding from nose and gums
Desired Outcomes
Child will be protected from illness or injury.
Hyperemia
Oral pain or discomfort
Oral plaque
Oral ulcers
Stomatitis
Desired Outcomes
CONCLUSION:
Wilms tumor (nephroblastoma) is recognized as the most common pediatric malignant renal
tumor in children. The incidence of Wilms tumor is slightly less frequent in boys than in girls.
The average age at diagnosis with unilateral tumors is 41.5 months and with bilateral tumors is
29.5 months. It occurs in association with congenital anomalies and chromosomal abnormalities.
Over the years, advancements in the diagnosis and treatment of Wilms' tumor have greatly
improved the outlook (prognosis) for children with this disease. With appropriate treatment, the
outlook for most children with Wilms' tumor is very good.
REFERENCES:
Hockenberry MJ., Wilson D., Rodgers Cheryl C. Wong’s Essentials of Pediatric Nursing.
10th ed. St. Louis, Missouri: Elsevier;2017. Page no. 1637-1639.
Kliegman RM.,et.al. Nelson Textbook of Pediatrics. 21st ed. Canada: Elsevier;2020. Page
no. 2681-2685,2781.
Sharma R., Essentials of Pediatric Nursing. 2nd ed. New Delhi: Jaypee publishers;2017.
Page no.399-402.
Paul V. K., Bagga A. Ghai Essential Pediatrics. 9 th ed. New Delhi: CBS
publishers,2019.Page no. 609-610.
https://www.slideshare.net/sheenabhatia3/wilms-tumor-152388934
https://www.cancer.org/cancer/wilms-tumor/treating.html
https://www.mayoclinic.org/diseases-conditions/wilms-tumor/symptoms-
causes/syc-20352655