Wilms, Osteo, CNS

8/12/2019 Wilms, Osteo, CNS

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Pediatric Tumors

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

Lucile Packard Childrens Hospital


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Distribution of Childhood Cancer

CNS

18%

Liver1%

Other8%

Leukemia33%Retina

3%GermCell3%

Bone5%

Wilm's

Tumor6%

Hodgkin's5% NHL

3%

NBL8%

Soft TissueSarcoma7%


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Abdominal masses

History

Symptoms

abdominal discomfort, increased abdominalsize or assymptomatic

Presence of systemic symptoms

bone pain, limping, malaise, fever

Other symptoms hematuria


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Abdominal masses

Physical findings

Presence of abdominal mass

upper abdomen or lower abdomen Other associated physical findings

other masses: orbital

bruises hypertension

pain


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Abdominal Mass

Differential diagnosis

depends on location

Upper abdominal

mass

Neuroblastoma

Wilms tumor

Hepatoblastoma Rhabdomyosarcoma

Germ cell tumor

Lymphoma


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Abdominal Mass

Differential diagnosis

(cont)

Lower

abdominal/pelvicmass

Neuroblastoma

Rhabdomyosarcom

a

Germ cell tumor

Lymphoma

Ewings sarcoma


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Abdominal MassesLaboratory evaluation

Bone marrow aspirate

neuroblastoma, lymphoma,

rhabdomyosarcoma orEwings sarcoma

CSF tap

if lymphoma is a

consideration


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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


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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


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Wilms Tumor: Signs & Symptoms

Most commonly

presents as painless

abdominal mass

Hypertension (20-30%)

Pain (20-30%)

Hematuria (25%)


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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


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Bilateral Wilms


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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


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Wilms Tumor: Prognosis & Future Directions

Prognostic factors: stage and histology

Future directions

Minimize therapy for favorable histologypatients

Identify biologic factors predictive of outcome

Intensify therapy for patients with unfavorable

histology


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Osteosarcoma: Distribution

by Age and Site


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Clinical presentation Pain and swelling usually lasting 3-4

months Duration: sometimes > 6 months

Occurs around metaphysis of longbones 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


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Most Common Metastatic Sites


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Diagnostic workup

Imaging Work-up:

Plain X ray, MRI ofprimary

Metastatic workup

Chest CT Bone scan

PET scan

Biopsy


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Imaging Evaluation


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Metastatic Work-Up: Chest X-Ray


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Metastatic Work-Up: Chest CT


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Metastatic Work-Up: Bone Scan


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Pathology

Intramedullary high-

grade sarcoma

Malignant mesenchymal

cells producing osteoid The World Health

Organization (WHO)

recognizes three major

subtypes: based onmatrix:

Osteoblastic

Chondroblastic fibroblastic


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Osteosarcoma: Pathology


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Osteosarcoma: Therapy

Historically, 2-yearsurvival < 20% forpatients treated with

surgery and/orradiotherapy

Uncontrolled trials ofadjuvant

chemotherapy resultedin EFS of 45-60%suggestingchemotherapy

improved outcome


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Multi-Institutional Osteosarcoma

Study:Design

B

I

O

P

SY

S

U

R

G

E

R

Y

RA

N

D

O

M

IZ

E

Adjuvant Chemotherapy

No Adjuvant Chemotherapy


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Osteosarcoma: Treatment

Current therapy: multi-

agent chemotherapy

usually including

cisplatin, doxorubicinand methotrexate.

Complete surgical

resection for local

control.


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Osteosarcoma Treatment

Types of surgical techniques

Amputation

Rotationplasty

growing prosthesis

Total knee titanium replacement


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Amputation


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Rotationplasty


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Rotationplasty


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Total knee titanium

replacement

(endoprosthesis)


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Prognostic Factors: Metastases


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20

40

60

80

100

1 2 3 4 5

pre-chemo

SURVIVAL OF PATIENTS WITH LOCALIZED OSTEOSARCOMA

years

1980s- combinationchemo

1960s single agentchemo

2004 - chemo regimensmultiple intensified

%s

urvival


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Osteosarcoma: Outcome Modern

Trials


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Osteosarcoma: Outcome

Reached a plateau in outcome for

osteosarcoma patients

Further improvement will require largecooperative studies: International

collaboration

EURAMOS:

North American Childrens Oncology Group(COG)

German Austrian Swiss Cooperative

Osteosarcoma Study Group (COSS)

European Osteosarcoma Intergroup (EOI)


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Osteosarcoma: Conclusions

We have made significant progress in the

treatment of osteosarcoma

Therapy has reached a plateau andfurther 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


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CNS TUMORS


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CNS TUMORS

Most common solid tumors in children

2ndmost frequent (16.6% of all childhood

malignancies)

Incidence has increased over the past 2

decades

Males > females, white> African American


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CNS TUMORS

Signs & Symptoms

(related to the location, histologic grade oftumor & age of child)

General

-Headache

-Seizures

-Mental status changes

-Increased intracranial Pressure (ICP)


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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


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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 recognizesounds, visual impairments


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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)


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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


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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


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CNS TUMORS

TREATMENT

Surgery

Most extensive resection feasible

--Radiation Therapy

--Chemotherapy


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CNS TUMORS

Prognosis

Varies greatly depending on type of tumor,

resectability,


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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 anaplasticastrocytoma & 18% -glioblastoma multiforme


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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%

Documents

Wilms, Osteo, CNS