Pediatric Solid Tumors

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

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Pediatric Solid Tumors

What you need to know for the Boards

Jaime Morales-Arias, MD, FAAP

2-years-old boy with an

Work-up: CT abdomen, CXR, CT chest

Infant girl with an abdominal mass,

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,

Periorbital ecchymoses, opsoclonus-myoclonus,

Blueberry muffin lesions = skin mets

Excessive secretion of catecholamines: palpitations,

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

Prognostic Factors: Stage, age, histology, nmyc

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

Treatment: Surgery, chemotherapy, radiation, bone

Prognosis worst in patients >12-18 months

Infant girl with an abdominal mass

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

Malignant hepatic tumor in a teenager =

Associations: Hepatitis B and C

2-years-old boy with a white pupil,

Most common presentation: Leukocoria

Prognosis: >93% alive at 5 years

Ophthalmologic exams until age 5-6

Risk of secondary malignancy with germline

Pay Attention to the Red

2-years-old boy with recurrent otitis,

Langerhans Cell Histiocytosis

Lytic lesions in bone (skull)

Langerhans Cell Histiocytosis

Eosinophilic Granuloma (localized): Lytic bone

Letterer-Siwe (disseminated): Chronic otitis,

600-700 new cases/year

6% of cancer <20 y/o

Alk Phos elevation--Osteosarcoma

A 4-year-old presents with weight loss,

A 5-year-old previously healthy white female

Important prognostic factors in a child

A.Size of tumor and location

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