Pediatric Cancer: Definition

a group of chronic disease characterized by uncontrolled growth and spread of abnormal cells which, if not adequately treated, results in the death of the patient

What is Cancer?

genetic mutation

permanent DNA alteration

begins in single cell (clonal)

mutation results in

lack of differentiation - cell remains immature

loss of contact inhibition

unregulated growth

cellular immortality - failure of cell to recognize need to stop growing

What causes childhood cancer?

unknown (in most cases)

not anything anyone did (or failed to do)

related to peak growth

possibly related to environmental agents

electromagnetic fields

chemicals

pesticides

viruses

Children at increased risk for cancer

genetic predisposition

down syndrome

li-fraumeni syndrome

retinoblastoma gene

immunodeficiency

congenital/acquired

treatment-related

history of cancer

prior cancer treatment

Classification of childhood cancer

childhood cancer is not a single disease, but a wide range of diseases that are classified into two broad subcategories

hematological malignancies

solid tumors

Tissue classification

developing embryo has 3 germ cell layers

ectoderm

mesoderm

endoderm

Common types of childhood cancer

hematological

leukemia (blood/marrow)

lymphoma (lymph)

solid tumors

CNS tumors (brain/spine)

neuroblastoma (SNS)

rhabdomyosarcoma (muscle)

Wilms tumor (kidney)

Ewing's sarcoma, osteosarcoma (bone)

retinoblastoma (eye)

Childhood cancer: patterns of onset

infancy/early childhood

neuroblastoma

Wilms tumor

retinoblastoma

adolescence

Hodgkin's lymphoma

osteosarcoma

Impact of a cancer diagnosis in childhood

loss of control

living with uncertainty

potential loss of future

of child

of future generations

developmental issues

family issues

Leukemias: two forms

acute lymphoid leukemia (ALL)

acute nonlympoid leukemia (ANLL)

Lymphomas

Hodgkin Disease

originates in the lymphoid system and involves primarily the lymph nodes

enlarge cervical lymph nodes along collar bones, neck, under arm, etc

may not be tender to touch

low grade fever, night sweats, wt loss

diagnosis

early prognosis good; chemo and radiation

Non-Hodgkin Lymphoma

a generalized disease, rather than just involvement of nodes and specific organs

mediastinal and meningeal involvement is common

spread can occur early or rapidly

s/s - similar with hodgkins

tx - chemo/radiation

Wilms Tumor

tumor of the kidneys - 7% childhood tumors

congential anomalies usu associated

clinical manifestations

abd swelling, high BP, hematuria

management

remove tumor, part of adrenal gland, or part of kidney

chemo/radiation

avoid contact sports after

never palpate abdominal (can cause seeding which will spread throughout the body)

Rhabdomyosarcoma

most common soft-tissue sarcoma dx in children

comes from skeletal muscle (tissue, wrapped around intestines, etc)

Dx

imaging, CTs, MRIs, pet scans, CBCs

Tx

remove tumor, chemo/radiation

S/S

eyes (vision changes), renal (urinary pattern changes)

Retinoblastoma

intraocular malignancy of the retina

usu. have to remove eye

do not have red reflex in eye (white look to pupil)

Brain tumors

S/S behavior, neuro changes, n/v, dizzy, awakening at night with HA

Medulloblastoma: external layer of cerebellum, fast growing, most common in young schoolage (5-7 y/o)

Astrocytoma: arise from glial cells above or below cerbrum and cerebellum 40% of ch BT

Brain stem gliomas 6.5% of all BT

radiation is tx of choice > combo of radiation, chemo, and surgical removal

Neuroblastoma

solid tumor occuring outside the cranium

most common tumor in infants

98% survival rate in stage 1

metastisis is common - adrenal glands

S/S alteration in bowel and bladder, fatigue, wt loss, dec appetite, fever

Tx - rad, chemo, surgery

tumor of the bone

peak incidence is during rapid growth

usu located at the metaphysis of distal femur, proximal tibia, or proximal humerus

13 y/o girl, 14 y/o boy

pain swelling of affected area

try to do lymph salvage - 20% met to lung

chemo and rad not much affective