1. Wilm’s tumor

2. Introduction: also known as nephroblastoma
❏ arises from abnormal proliferation of metanephric blastoma ❏ 5% of all childhood cancers, 5% bilateral ❏ average age of incidence is 3 years ❏ 1/3 hereditary (autosomal dominant) and 2/3 sporadic • familial form associated with other congenital abnormalities and gene defects (WAGR syndrome)

3. Spread Direct invasion: major type of spread of this tumor
Lymphatic spread: very rare
Blood spread: not frequent

4. Clinical staging Stage I: tumor is restricted to the kidney
Stage II: tumor extends beyond kidney but within perinephric tissue & it is resectable
Stage III: tumor spreads beyond perinephric tissue & is not completely resectable
Stage IV: with hematogenous spread
Stage V: bilateral tumor

5. Presentation
• abdominal mass : large, firm, unilateral(most common presentation – 80%), smooth, typically doesn’t cross the midline • hypertension (60%) • abdominal pain • microscopic hematuria • pyrexia • nausea/vomiting

6. Investigations Blood: polycythemia X- ray KUB Excretory urograms USG
CT scan: nephroblastoma replaces renal parenchyma on CT scans whereas Neuroblastoma displaces it.

7. Treatment
• always investigate contralateral kidney • treatment of choice is simple nephrectomy +/– radiation +/– chemotherapy

8. Prognosis
• generally good; overall 5-year survival about 80% • metastatic disease may respond well

9. Neuroblastoma

10. Introduction
A 2 year old boy complains of belly pain and lack of appetite. Physical reveals a large abdominal mass.
Most common malignant solid tumor in children.
Derived from neural crest tissue
May arise anywhere along sympathetic ganglia.
Most common in adrenal medulla (50%)

11. Neuroblastoma Age 1-2 years Extends across midline
Ocular involvement may present as raccoon eyes.
Calcifications on chest x-ray whereas no calcifications in nephroblastoma
Elevated catecholamines, metanephrines.
Due to production of hormones, children may present with flushing, HTN, watery diarrhea, periorbital ecchymosis.

12. Neuroblastoma Age at presentation is major prognostic factor.
Less than 1 year >70% survival
Older than 1 year <35% survival
Good prognostic features:
Tumors with <10 copies of N- myc gene
Aneuploid tumors
Low mitosis index
Normal LDH and catecholamine levels.

13. Neuroblastoma
If able, surgical excision is treatment of choice, chemo may be beneficial.
The N- myc gene is associated with neuroblastoma!

14. Mass Noted by Mother
A two year old is brought by her mother who noted a mass protruding from under the left costal margin. On physical, you find a well nourished, normotensive child. There is no macroglossia, aniridia, skin abnormalities or bruising. You inquire about blood in urine, hx of bruising.
A. What is your initial differential?
B. What are your immediate diagnostic plans?

15. What is the most likely diagnosis in the differential?
X Neuroblastoma
most common, often painful, racoon eyes, blueberry muffin skin, often weight loss
Wilm’s Tumor
painless, aniridia, macroglossia, hypertension, hematuria
X Hepatoblastoma
X Lymphoma
X Germ Cell/Ovarian Mass

16. What are your diagnostic plans?
CBC, UA, LDH, urine VMA metabolites, ßHcG, FP
Sonogram
regional venous anatomy/tumor thrombus CT
Metastatic work-up as indicated (bone marrows, other cavity scans)

17. Pearls
Accessory signs are helpful when present but most tumors present without
Neuroblastoma demonstrates calcifications on plain film, Wilm’s does not
Work up should be thorough but logical--head to toe scanning is not the approach
Diagnosis should await tissue confirmation
Try to minimize palpation

18. Pearls Neuroblastoma: Wilms’ Tumor: irritable child, tender
skin: blueberry muffin
eyes: raccoon eyes
some degree of wasting
urinary metabolites
calcs on film
Wilms’ Tumor:
asymptomatic
macroglossia
aniridia
Hemihypertrophy
hypertension
hematuria

19. Pearls Biopsy and access followed by neoadjuvant therapy
Complete Resection
Surgical staging v. pathologic staging
TEAM approach: radiology, pathology, oncology, surgery, pediatrician