Common Pediatric

Malignancies
Two Content Layout with Table

Wilm’s Tumor
introduction
• Wilms tumor, or nephroblastoma, is the most
common childhood abdominal malignancy.
• The median age at diagnosis of Wilms tumor is
approximately 3.5 years and becomes much less
common after age 5.
• With current multimodality therapy, approximately
80-90% of children with a diagnosis of Wilms tumor
survive.
Etiology
 the tumor may arise in 3 clinical settings, the study of which resulted in
the discovery of genetic abnormalities that lead to the disease.
 Setting for WT are:
 Sporadic: unknown causes (most of cases)
 Familial: familial WT arises with high frequency in certain families
 Genetic syndrome
Alterations of genes responsible for normal genitourinary development.
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.
• WAGR syndrome
• Denys-Drash syndrome
• Beckwith-Wiedemann syndrome
Pathology
 Intra renal solid or cystic mass, which
displacing collecting system
 Has pseudo-capsule and may contain
area of hemorrhage and necrosis
 May extend to renal vein in 40% of
cases
 Rarely extend into ureter and bladder
Favorable histology
Favorable histology means that
there is no anaplasia.
Over 90 percent of Wilms’ tumors
have favorable histology.
This means most tumors are
easier to cure.
Unfavorable histology
Unfavorable histology means that
the tumors have a nucleus in the
cells that looks very large, nuclear
hyperchromasia & abnormal
mitoses.
This is referred to as anaplasia.
The more anaplasia, the harder
the tumor is to cure.
Clinical Picture
§ Abdominal Mass
§ Abdominal pain or hematuria
(25%)
§ Urinary tract infection
§ Hypertension, gross hematuria,
and fever (5-30%)
§ Hypotension, anemia, and fever
Diagnosis
The following lab studies are indicated:
• Liver function tests (alanine transaminase [ALT], aspartate transaminase [AST], total and
direct bilirubin)—to detect liver involvement
• Complete blood count (CBC)—to assess anemia and possible bone marrow involvement
• Urinalysis—to assess for hematuria
• Urinary catecholamine levels—tumor markers used to rule out neuroblastoma
• Blood urea nitrogen, creatinine, and electrolyte levels—to assess renal function
• Additional testing of liver, bone, and brain only if additional symptoms indicate involvement
Ultrasound
•Initial Dx of renal or abdominal
mass
•Possible renal vein or IVC
thrombus
•Information regarding other
kidney
CT scan
Differential diagnosis of renal
versus adrenal tumor
(neuroblastoma)
Liver metastases
Status of opposite kidney, LN,
chest metastases
MRI
Bone scan+plain x-ray & CT :
determine stage of cancer
Staging
 A nephrectomy, or removal of the affected kidney, is performed to remove the tumor and to provide tissue for
diagnosis, histologic examination, and staging.
It also provides an opportunity to explore and biopsy lymph nodes and abdominal organs for involvement.

Stage I adjuvant Vincristine and actinomycin D without radiotherapy except

for patients with unfavourable histology. No need for maintenance and radiotherapy.

Stage II adjuvant vincristine and actinomycin D without radiotherapy except for

those with unfavourable histology. No need for maintenance and
radiotherapy

Stage III and Three drug adjuvant treatment with vincristine, actinomycin D and
earlier stage Adriamycin for a longer duration. Radiotherapy of renal bed and whole
disease with abdomen for those with positive nodes and tumour spillage. Radiotherapy
unfavourable should be avoided in children under the age of 2 years.
histology –
Stage IV – Three drug adjuvant treatment with vincristine, actinomycin D and
Adriamycin as in stage III. Radiotherapy is limited to residue
metastatic lesions, especially in the lungs. Surgical excision should be
considered prior to radiotherapy whenever feasible
Neuroblastoma
Neuroblastoma
introduction
 Most common extra cranial tumour in infants & 3rd children after leukemia and brain cancer
 An Autosomal Dominant through deletion of short arm chromosome 1
 An embryonal malignancy of the sympathetic nervous system arising from neuroblasts (
neural crest )
 NB is solid, highly vascular tumor with friable pseudo-capsule
 The poorly differentiate neoplasm derived from neural crest cell
 Neuroblastomas originate in (retroperitoneum) the adrenal medulla and paraspinal or
periaortic regions. The presentation varies according to the site of origin, but 65% of primary
neuroblastomas occur in the abdomen—40% in the adrenal gland—so most children present
with abdominal symptoms, such as fullness or distension.
Most common cancer in babies
Nearly 1 case/8000-10000 children
More common in whites
Age: typical age of diagnosis is younger than that for Wilm’s tumor
(rare in children >5years)
Etiology
Embryologically, tumors of the sympathetic nervous system differentiate along two distinct
pathways, either the pheochromocytoma line or the sympathoblastoma line.
The sympatoblastomas, also called neurocristopathies, include the well-differentiated
ganglioneuroma, the moderately differentiated ganglioneuroblastoma, and the
malignant neuroblastoma.
All of these tumors arise from primordial neural crest cells, which ultimately populate the
sympathetic chain and the adrenal medulla
Various karyotypic abnormalities occur, but a deletion of the short arm of chromosome 1 is
found in 70-80% of all patients with neuroblastomas.
Malignant transformation and maintenance of the dedifferentiated state of neural crest cells
may result from failure of those cells to respond to normal signals that are responsible for
normal morphologic differentiation
• systemic manifestation: sick, unexplained fever, wt loss, anorexia, failure to thrive, general malaise,
irritability, bone pain.
• hepatomegaly; blanching SC nodules; abdominal mass
• C/E: non tender, firm, irregular abdominal mass that cross the midline.
• 50% of patients presenting with NB have metastatic disease:
• Pepper syndrome
• Blueberry muffin
• Hutchinson
• Horner’s syndrome
• NB arise from para-spinal: subsequently cause paralysis
• NB invade retrobulbar: causing ‘Panda’s Eye”
• Paraneoplastic syndrome
Investigations
Laboratory Studies
1. CBC count and differential (Anemia or other cytopenias suggest bone marrow involvement.)
2. Urine collection for catecholamines (VMA/HVA)
1. Chest and abdominal radiographs to evaluate for the presence of a posterior mediastinal
mass or calcifications.
2. A CT scan of the primary site is essential to determine tumor extent
3. A technetium-99 bone scan can also be used to evaluate bone metastases
4. Skeletal surveys may also be useful
Diagnostic procedures
1. Biopsy findings are usually required to diagnose neuroblastoma
2. Can be classified as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma, depending
on the degree of maturation and differentiation of the tumor.
3. Even without a biopsy, the presence of elevated urinary catecholamines and a bone marrow
aspirate or biopsy with unequivocal neuroblastoma cells is diagnostic.
4. Undifferentiated neuroblastomas histologically present as small, round, blue cell tumors with
dense nests of cells + pseudorosettes
Consist of surgical , chemotherapy & radiotherapy
In low stage favorable NB , surgery is mainstay of treatment

1. Completely remove the entire primary tumour

2. Provide accurate surgical staging
3. Offer adjuvant therapy for delayed 1ry surgery
4. Remove residual disease with 2nd look surgery (second-look surgery postchemotherapy is
used to attempt a complete resection)
Chemotherapy
1. Multiple agent chemotherapy is conventional treatment for patient more advanced stages of
NB
2. Individual chemotherapy drugs are discussed below. These agents are almost invariably given
in combination. Commonly used combinations include the following:
Vincristine, cyclophosphamide, and doxorubicin
Carboplatin and etoposide
Cisplatin and etoposide
Ifosfamide and etoposide
Cyclophosphamide and topotecan
Radiotherapy
1. It is a radio-sensitive tumor
2. It improves the survival rates when used as an adjuvant therapy after partial resection (
second-look surgery post-chemotherapy )
3. It did not improve survival rate when added to chemotherapy
Prognostic
Shimada Index