TUMORS

564 Section III / Subspecialty Surgery

What are the best indicators

Stage and histologic subtype of tumor; 85%

of survival?

of patients have favorable histology (FH);

15% have unfavorable histology (UH);

overall survival for FH is 85% for all stages

What is the treatment?

Radical resection of affected kidney

with evaluation for staging, followed by

chemotherapy (low stages) and radiation

(higher stages)

What is the neoadjuvant

Large tumors may be shrunk with

treatment?

chemotherapy/XRT to allow for surgical

resection

What are the associated

Aniridia, hemihypertrophy,

abnormalities?

Beckwith-Wiedemann syndrome,

neurofibromatosis, horseshoe kidney

What is the Beckwith-

Syndrome of:

Wiedemann syndrome?

1.

Umbilical defect

2.

Macroglossia (big tongue)

3.

Gigantism

4. Visceromegaly (big organs) (Think:

Wilms’ Beckwith-Wiedemann)

NEUROBLASTOMA

What is it?

Embryonal tumor of neural crest origin

What are the anatomic

Adrenal medulla—50%

locations?

Paraaortic abdominal paraspinal

ganglia—25%

Posterior mediastinum—20%

Chapter 67 / Pediatric Surgery 565

What are the symptoms?

Vary by tumor location—anemia, failure

to thrive, weight loss, and poor nutritional

status with advanced disease

What are the signs?

Asymptomatic abdominal mass (palpable

in 50% of cases), respiratory distress

(mediastinal tumors), Horner’s syndrome

(upper chest or neck tumors), proptosis

(with orbital metastases), subcutaneous

tumor nodules, HTN (20%–35%)

LABS?

24-hour urine to measure VMA, HVA,

and metanephrines (elevated in 85%);

neuron-specific enolase, N-myc oncogene,

DNA ploidy

566 Section III / Subspecialty Surgery

Define the stages:

Stage I

Tumor is confined to organ of origin

Stage II

Tumor extends beyond organ of origin

but not across the midline

Stage III

Tumor extends across the midline

Stage IV

Metastatic disease

Stage IVS

Infants: Localized primary tumor does

not cross the midline, but remote

disease is confined to the liver,

subcutaneous/skin, and bone marrow

Chapter 67 / Pediatric Surgery 567

What are the laboratory

Aneuploidy is favorable! The lower the

prognosticators?

number of N-myc oncogene copies, the

better the prognosis

Which oncogene is associated

N-myc oncogene

with neuroblastoma?

Think: N-myc Neuroblastoma

RHABDOMYOSARCOMA

What is it?

Highly malignant striated muscle sarcoma

What is its claim to fame?

Most common sarcoma in children

What is the age distribution?

Bimodal:

1.

2–5 years

2.

15–19 years