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Wilm's Tumor and Neuroblastoma
Wilm's Tumor and Neuroblastoma
Wilm's Tumor and Neuroblastoma
Wilm’s Tumor
Wilm’s Tumor and
Neuroblastoma
Venée Tubman, MD
Children’s Hospital Boston
HEARTT
Wilm’s Tumor (WT)
• Wilm’s tumor is the most common renal
malignancy in pediatrics
• Amongst Americans, more common in African
Americans
• Slightly more common in females, bilateral
disease more common in females
WT: Associated Syndromes
• Syndrome are associated with mutations or
deletions in WT1/FWT1/FWT2/p53 genes
• Beckwith-Wiedeman syndrome: macrosomia,
macroglossia, omphalocele, large kidneys,
hemihypertrophy
• 5-10% of children will develop WT
• WAGR: WT, aniridia, GU anomalies, mental
retardation
• Denys-Drash: male pseudohermaphrodite and
renal disease
WT: Pathology
• Approximately 7% of cases have bilateral disease
• Most tumors are enclosed by renal capsule or
intrarenal pseudocapsule
• Tumor can contain a mixture of cells:
– blastemal cells
– stromal cells
– epithelial cells
• High degree of anaplasia associated with poor
outcomes
WT: CLINICAL PRESENTATION
• Many cases present with an abdominal mass
discovered by the parents during bath or
changing time
• Associated findings: abdominal pain (30%),
hematuria (12-25%), hypertension (25%)
• Firm, nontender mass which does not cross the
midline
• Examine carefully given risk of capsular rupture
• Examine for associated anomlaies
WT: EVALUATION
• KUB/US
• If available, CT (chest/abdomen)
• Labs: chemistries, LFTs, urinalysis
WT: STAGING
National Wilm’s Tumor Study (US): staging is
determined before chemotherapy is initiated
WT: STAGING
International Society of Pediatric Oncologists
(SIOP) (Europe): staging is determined after
chemotherapy is initiated for 4-6 weeks
• Stage 1: fully resected and encapsulated
• Stage 2: beyond renal capsule but fully resected
• Stage 3: extends into abdomen
• Stage 4: hematogenous spread
• Stage 5: bilateral renal disease
WT: TREATMENT
• Resection is the mainstay of treatment, but
without chemotherapy there is a high rate of
recurrence
• Stage 1 or 2: 18 weeks of vincristine and
dactinomycin
• Stage 3: 24 weeks of vincristine, doxorubicin,
and dactinomycin; and radiation to the flank
• Stage 4: 24 weeks of chemotherapy with
radiation to flank and lungs
WT: OUTCOMES
• Response rate of 90% with chemotherapy and
resection
• Best prognosis is under 2 years and stage 1
disease
• 15% recurrence rate if histology is favorable;
50% recurrence if anaplasia is present
• Complications are largely related to
chemotherapy
NEUROBLASTOMA
NEUROBLASTOMA (NB)
• Represents a variety of tumors including
neuroblastoma, ganglioneuroma,
ganglioneuroblastoma
• Tumors arise from primitive sympathetic ganglion
cells
• Characterized by variable location, histology and
behavior
• Most common extracranial solid tumor in
children,
accounting for 8% to 10% of all childhood cancers
• Prevalence is about 1 case per 7,000 live births
NB: PATHOLOGY
• Small blue round cell tumor