Pediatric Solid Tumors

What you need to know for the Boards

Jaime Morales-Arias, MD, FAAP

Assistant Professor
Louisiana State University
Childrens Hospital New Orleans

<15

GCT

CA Other

Liver
RB

Solid

Leukemia/
Lymphoma

STS

Leuk

Bone
Renal

CNS

NB
Lymph
CNS

2-years-old boy with an

asymptomatic abdominal mass and
hypertension

Wilms Tumor
Most common renal cancer in children
Occurs in children <5 years (peak 2-3
years)
Arises within the kidney
Hematuria
Hypertension

Wilms Tumor

Work-up: CT abdomen, CXR, CT chest

Most important prognostic factor: Histology (favorable
vs. diffuse anaplasia)
Stage I-V (Stage V= Bilateral)
Treatment: Surgery, chemotherapy, radiation
Metastases: Lungs, liver
Associated with deletions in chromosome 11 (WT1
and WT2 genes)

Wilms
Pulmonary
Metastases

Wilms Tumor

Prognosis:
Favorable Histology: 80-92% 4-year EFS
Diffuse Anaplasia:
55% 4-year EFS

Associations:
Aniridia
Hemihypertrophy
GU anomalies

Wilms Tumor

Beckwith-Wiedemann Syndrome:
hepatosplenomegaly, macroglosia, omphalocele,
hemihypertrophy, mental retardation, obesity

WAGR Syndrome:
Wilms tumor, aniridia, GU malformations, mental
retardation

Infant girl with an abdominal mass,

pallor, ill-appearing

Raccoons Eye

Neuroblastoma

Most common malignancy in children <1 year

Over 50 % are <2-years-old

Most common location : Abdomen (adrenals)

Approx. 50% metastatic at diagnosis: bone marrow,

liver, spleen, bones, lymph nodes

Neuroblastoma

Periorbital ecchymoses, opsoclonus-myoclonus,

Horners (unilateral ptosis, miosis, anhidrosis)

Blueberry muffin lesions = skin mets

Excessive secretion of catecholamines: palpitations,

hypertension, flushing, diarrhea

Neuroblastoma

Work-up: MRI of primary tumor site, CBC, BM biopsy,

ferritin, LDH, bone scan, urine VMA and HVA

Prognostic Factors: Stage, age, histology, nmyc

amplification

Stage 4S: <1 year old, stage 1 or 2 with mets limited

to liver, spleen, bone marrow or skin
*Survival: 90% with limited or no therapy

Neuroblastoma

Treatment: Surgery, chemotherapy, radiation, bone

marrow transplant, retinoic acid, chimeric antibody

Prognosis worst in patients >12-18 months

Wilms vs Neuroblastoma

Infant girl with an abdominal mass

and elevated alphafetoprotein

Hepatoblastoma

Median age: 1 year

Tumor marker: AFP

Work-up: CT abdomen + chest , CXR (Mets = lungs)

Most important prognostic factor: histology

Treatment: Surgery, chemotherapy, liver transplant

(should be initial surgery if anticipated)

Alphafetoprotein in Infants
Age

premature
newborn
newborn - 2 wk
newborn - 1 mo
2 wk - 1 mo
2 mo

11
55
16
43
12
40

Mean SD
(ng/ml)
134,734 41,444
46,406 34,718
33,113 32,503
9,452 12,610
2,654 3,080
323 278

Hepatocellular Carcinoma

Malignant hepatic tumor in a teenager =

Hepatocellular Carcinoma

Fibrolamelar carcinoma

Associations: Hepatitis B and C

2-years-old boy with a white pupil,

no red reflex

Retinoblastoma

Most common presentation: Leukocoria

Peak age: 18 months
Diagnosis: Clinical, no need for biopsy
25% of cases are bilateral: Autosomal dominant
inheritance associated with germline mutation in
chromosome 13
Trilateral: Both eyes, brain
Treatment: Eliminate tumor and save vision. Local
therapy, chemotherapy, radiation. Enucleation if no
salvageable vision.

Retinoblastoma

Metastases: Uncommon

Prognosis: >93% alive at 5 years

Ophthalmologic exams until age 5-6

Risk of secondary malignancy with germline

mutation: Osteosarcoma most commonly (both within
XRT area and typical OS sites)

Pay Attention to the Red

Reflex

2-years-old boy with recurrent otitis,

seborrheic dermatitis and bone lesion

Langerhans Cell Histiocytosis

Lytic lesions in bone (skull)

Local pain, lumps
Vertebra plana
Chronic suppurative otitis
Seborrhea
Eczema-type rash
Teeth abnormalities
Diabetes insipidus

Langerhans Cell Histiocytosis

Eosinophilic Granuloma (localized): Lytic bone

lesions

Hand-Schuller-Christian (disseminated):
Classic triadbone lesions, exopthalmos and
diabetes insipidus

Letterer-Siwe (disseminated): Chronic otitis,

rash, hepatosplenomegally, organ
involvement

Bone Tumors

600-700 new cases/year

400 Osteosarcoma
200 Ewings sarcoma

6% of cancer <20 y/o

Age - adolescents with peak at age 15
(3rd most common malignancy)

 16-years-old

male with a
painless mass
in the distal
femur

13-years-old
male with pain in
his arm, fever,
paleness, fatigue

Osteosarcoma:

- Long bones
(metaphyseal)
- Painless mass
- Pathologic fracture
- Xray: Lytic or
sclerotic lesion,
sunburst

Ewings Sarcoma:

- Midshaft of long
bones (diaphyseal)
or central axis
- Painful mass
- Systemic symptoms
- Xray: Permeative
lesion, onion skin

Bone Tumors

Alk Phos elevation--Osteosarcoma

Sequelae of radiation therapy--Osteosarcoma
Secondary malignancy in hereditary
retinoblastoma--Osteosarcoma
Li-Fraumeni (p53 mutation)--Osteosarcoma
Radiation sensitive--Ewings
EWS-Fli1 t(11;22)--Ewings
Bone marrow involvement--Ewings

Bone Tumors
Mets: Lungs
Treatment: Limb salvage or
amputation, chemo, radiation (Ewings)
Survival:
*Non-metastatic: 70-75%
*Metastatic: 20-30%

Rhabdomyosarcoma

Skeletal muscle
Painless mass
Head and neck, extremities, genitourinary
tract (bladder, prostate, paratesticular,
vagina, sarcoma botryoides--grape-like)
Two types: Embryonal and alveolar (worse
prognosis)
TX: Surgery, chemo, radiation

Practice Questions

A 4-year-old presents with weight loss,

anorexia, and weakness for 2 months. Left
sided ptosis and an ipsilateral miotic pupil are
noted on PE. The most likely diagnosis is:

A. Leukemia
B. Neuroblastoma
C. Wilms tumor
D. Cerebral astrocytoma
E. Cerebellar astrocytoma

 Answer:

Practice Questions

A 5-year-old previously healthy white female

manifests progressive painless proptosis and
decreased visual acuity of the left eye during
a 2-month period. The most likely diagnosis is

A.Pseudotumor
B.Trichinosis
C.Retinoblastoma

D.Rhabdomyosarcoma
E. Orbital cellulitis

 Answer:

Practice Questions

Important prognostic factors in a child

with neuroblastoma are:

A.Size of tumor and location

B.Chromosomal abnormalities
C.Stage and Age
D.Catecholamine and ferritin levels

 Answer:

Practice Questions
1.Diarrhea
2.Vaginal bleeding
3.Headache
4.Night sweats
5.Leukocoria
6.Periorbital ecchymosis
7.Chromosome 13
8.Chromosome 11
9.Pancytopenia

A.Rhabdomyosarcoma
B.Astrocytoma
C.Lymphoma
D.Neuroblastoma
E.Leukemia
F.Retinoblastoma
G.Wilms tumor

1
2
3
4
5
6
7
8
9

D
A
B
C
F
D
F
G
E

Practice Questions
1.
2.
3.
4.
5.
6.
7.
8.
9.

Neuropathy
Coagulopathy
Hearing loss
Pneumonitis
Cardiomiopathy
Pancreatitis
Hemorrhagic cystitis
Mucositis
SIADH

A. Cyclophposphamide
B. Cisplatinum
C. Vincristine
D. Bleomycin
E. Asparaginase
F. Adriamycin
G. Methotrexate

1
2
3
4
5
6
7
8
9

C
E
B
D
F
E
A
G
C