Pediatric Tumors

Pamela Simon R.N., C.P.N.P,

MSN
Lucile Packard Childrens Hospital
Distribution of Childhood Cancer
CNS
18%
Liver
1%
Other
8%
Leukemia
33%
Retina
3%
Germ Cell
3%
Bone
5%
Wilm's
Tumor
6%
Hodgkin's
5%
NHL
3%
NBL
8%
Soft Tissue
Sarcoma
7%
Abdominal masses
History
Symptoms
abdominal discomfort, increased abdominal
size or assymptomatic
Presence of systemic symptoms
bone pain, limping, malaise, fever
Other symptoms
hematuria

Abdominal masses
Physical findings
Presence of abdominal mass
upper abdomen or lower abdomen
Other associated physical findings
other masses: orbital
bruises
hypertension
pain


Abdominal Mass
Differential diagnosis
depends on location
Upper abdominal
mass
Neuroblastoma
Wilms tumor
Hepatoblastoma
Rhabdomyosarcoma
Germ cell tumor
Lymphoma

Abdominal Mass
Differential diagnosis
(cont)
Lower
abdominal/pelvic
mass
Neuroblastoma
Rhabdomyosarcom
a
Germ cell tumor
Lymphoma
Ewings sarcoma

Abdominal Masses
Laboratory evaluation
Bone marrow aspirate
neuroblastoma, lymphoma,
rhabdomyosarcoma or
Ewings sarcoma
CSF tap
if lymphoma is a
consideration
Abdominal masses
Radiologic work-up
Abdominal/pelvic ultrasound
useful screening test: helps define location and
quality (solid or cystic)
not useful to assess for retroperitoneal adenopathy
Computed tomography
location of mass
presence of calcification
obstructive signs
Wilms Tumor
Accounts for 6% childhood malignancies
Median age at diagnosis 3 years
Metastasizes to lung and lymph nodes
70% patients present with localized disease

Wilms Tumor: Signs & Symptoms
Most commonly
presents as painless
abdominal mass
Hypertension (20-
30%)
Pain (20-30%)
Hematuria (25%)

Wilms Tumor: Staging System
Stage Definition
I Tumor limited to kidney, completely excised

II Tumor extends beyond the kidney, completely resected;
no residual tumor beyond resection margins
III Residual non-hematogenous tumor confined to abdomen
IV Hematogenous metastases
V Bilateral renal involvement
Bilateral Wilms
Survival by Stage and Histology
Histology/stage # pts 2 yr-s
*
4 yr-s
*
FH/I 546 98 97
II 281 96 94
III 290 91 88
IV 126 88 82
UH/I 20 89 89
UH/II-IV 40 56 54
*
survival
Wilms Tumor: Prognosis & Future Directions
Prognostic factors: stage and histology
Future directions
Minimize therapy for favorable histology
patients
Identify biologic factors predictive of outcome
Intensify therapy for patients with unfavorable
histology

Osteosarcoma: Distribution
by Age and Site
Clinical presentation
Pain and swelling usually lasting 3-4
months
Duration: sometimes > 6 months
Occurs around metaphysis of long
bones
Most common primary sites: distal
femur, proximal tibia and proximal
humerus
50-70% around the knee joint
Metastases: ~15-20% patients
Sites: lung and other bones
Most Common Metastatic Sites
Diagnostic workup

Imaging Work-up:
Plain X ray, MRI of
primary
Metastatic workup
Chest CT
Bone scan
PET scan
Biopsy
Imaging Evaluation
Metastatic Work-Up: Chest X-Ray
Metastatic Work-Up: Chest CT
Metastatic Work-Up: Bone Scan
Pathology
Intramedullary high-
grade sarcoma
Malignant mesenchymal
cells producing osteoid
The World Health
Organization (WHO)
recognizes three major
subtypes: based on
matrix:
Osteoblastic
Chondroblastic
fibroblastic
Osteosarcoma: Pathology
Osteosarcoma: Therapy
Historically, 2-year
survival < 20% for
patients treated with
surgery and/or
radiotherapy
Uncontrolled trials of
adjuvant
chemotherapy resulted
in EFS of 45-60%
suggesting
chemotherapy
improved outcome
Multi-Institutional Osteosarcoma
Study: Design
B
I
O
P
S
Y
S
U
R
G
E
R
Y
R
A
N
D
O
M
I
Z
E
Adjuvant Chemotherapy
No Adjuvant Chemotherapy
Osteosarcoma: Treatment
Current therapy: multi-
agent chemotherapy
usually including
cisplatin, doxorubicin
and methotrexate.
Complete surgical
resection for local
control.


Osteosarcoma Treatment
Types of surgical techniques

Amputation
Rotationplasty
growing prosthesis
Total knee titanium replacement
Amputation
Rotationplasty
Rotationplasty
Total knee titanium
replacement
(endoprosthesis)
Prognostic Factors: Metastases
20
40
60
80
100
1 2 3 4 5
pre-chemo
SURVIVAL OF PATIENTS WITH LOCALIZED OSTEOSARCOMA
years
1980s- combination chemo
1960s single agent
chemo
2004 - chemo regimens
multiple intensified
%

s
u
r
v
i
v
a
l

Osteosarcoma: Outcome Modern
Trials
Osteosarcoma: Outcome
Reached a plateau in outcome for
osteosarcoma patients
Further improvement will require large
cooperative studies: International
collaboration
EURAMOS:
North American Childrens Oncology Group
(COG)
German Austrian Swiss Cooperative
Osteosarcoma Study Group (COSS)
European Osteosarcoma Intergroup (EOI)
Scandinavian Sarcoma Group (SSG)


Osteosarcoma: Conclusions
We have made significant progress in the
treatment of osteosarcoma
Therapy has reached a plateau and
further improvements will require large
number of patients
International collaboration: significant
barriers but offers the best chance of
increasing the number of patients available
Biologic studies: essential for continued
progress
CNS TUMORS
CNS TUMORS
Most common solid tumors in children
2
nd
most frequent (16.6% of all childhood
malignancies)
Incidence has increased over the past 2
decades
Males > females, white> African American
CNS TUMORS
Signs & Symptoms
(related to the location, histologic grade of
tumor & age of child)
General
-Headache
-Seizures
-Mental status changes
-Increased intracranial Pressure (ICP)

CNS TUMORS

Signs & Symptoms-Posterior Fossa
-Cerebellum-
-nausea, vomiting, headache, papilledema,
clumsy walk, double vision, dizzyness

-Brainstem-
-vomiting, cranial nerve palsies, headache,
head tilt, personality changes, hearing loss



CNS TUMORS
S&S-Cerebral Hemisphere
Frontal lobe-one-sided paralysis, memory loss,
mental changes, urinary changes
Occipital lobe-visual changes, seizures
Parietal lobe-Language disturbances, seizures,
loss of reading, math
Temporal lobe-seizures, unable to recognize
sounds, visual impairments




CNS TUMORS
S&S-Midline Tumors
Headache, nausea/vomiting, papilledema,
visual loss or abnormal eye movements,
precocious puberty, diabetes insipidus
(impairment of hypothalmic/pituitary fx)
CNS TUMORS
S&S- Spinal Cord-depends on location
Thoracic-chest pain
Cervical or lumbar-neck, arm, back, leg
weakness, muscle spasms & wasting, altered
bowel, bladder function
Progression of symptoms can result in paralysis

CNS TUMORS

Age & developmental stage are important factors
in re: to symptoms
-infants-delay or loss of dev. Milestones
-school age-personality changes, decline in
school performance, change in handwriting
CNS TUMORS
Diagnostic Evaluation
MRI head-preferred
Add spine if required
CT head
Lumbar puncture
Lab work for tumor markers for germ cell
tumors-AFP & B-hCG
Pet Scan
CNS TUMORS
TREATMENT
Surgery
Most extensive resection feasible


--Radiation Therapy


--Chemotherapy










CNS TUMORS
Prognosis
Varies greatly depending on type of tumor,
resectability,
CNS TUMORS
Astrocytoma
Occur at any age & various areas of brain
Graded according to anaplasia
Low grade cerebellar-pilocytic-85-95 % 5 year
survival rate
Low grade cerebral-90% 5 year survival rate
-High grade astrocytoma-29% for anaplastic
astrocytoma & 18% -glioblastoma multiforme

CNS TUMORS
Medulloblastoma (PNET)
20-25%of all brain tumors
Small, round blue cell tumor-fast growing
30% metastasis
Surgery, radiation & chemotherapy
5 yr survival rates 80%