Pediatric Malignancies

Collectively they make a relatively common group of diseases in children

Pediatric Malignancies 1% of all cancers

Involves tissues of:

CNS, bone, muscle, endothelial tissue

Grows in a short period of time

Causes Genetic alteration

Environmental influences

No known prevention

Metastasis is seen in 80%

Response to Treatment Very responsive to chemotherapy

More than 60% cure rate

Classification of Tumors Embryonal tumor arises from embryonic tissue

Lymphomas = lymphatic tissue

Leukemias = blood

Sarcoma = seen in bone, cartilage, nerve and fat

Cardinal Signs of Cancer Unusual mass or swelling Unexplained paleness and loss of energy Spontaneous bruising Prolonged, unexplained fever Headaches in morning Sudden eye or vision changes Excessive – rapid weight loss.

Diagnostic Tests X-ray Skeletal survey CT scan Ultrasound MRI Bone marrow aspiration FNA

Biopsy Identify cell to determine type of treatment

Treatment Modalities Determined by:

Type of cancer

Location

Extent of disease

Surgery The oldest form of cancer treatment Surgery plays important role in initial

diagnosis: biopsy of primary tumor. Excision of tumor when possible Facilitating treatment: insertion of catheters

for long-term treatment

Radiation Therapy The use of ionizing radiation to break apart bonds

within a cell causing cell damage and death. External beam therapy accounts for the majority of

radiation treatments in children. Problems: radiation beams cannot distinguish

between malignant cells and healthy cells.

Chemotherapy Can be given in addition to another form of

therapy such as radiation or surgery. Drugs may be administered before surgery to

reduce size of tumor. Adjuvant chemotherapy is used after surgery

or radiation therapy to prevent relapse.

Goals of Chemotherapy Reducing the primary tumor size Destroying cancer cells Preventing metastases and microscopic

spread of the disease

Chemotherapy Drugs Alkylating drug: attack DNA Antimetabolites: interfere with DNA production Antitumor antibiotics: interferes with DNA production Plant alkaloids: prevent cells from dividing Steroid hormones: slow growth of some cancers

Bone Marrow Transplant HSCT: Hematopoietic Stem Cell Transplant: The option of HSCT depends on the patients

disease, disease status, and general physical condition.

Involves: Umbilical cord blood Parent’s stem cells

Gene Therapy Use of gene therapy in the treatment of

childhood cancer is promising yet complex and still in early phases of clinical application.

Management of Cancer Patient / family education

Begins at time of diagnosis Continues through treatment phases Maintained in post-survival years Support if death of the child occurs.

Emotional aspects of leukemia.

Pain Management Pain caused by disease

Pain with procedures and treatments

Pain associated with side effects of treatment

Pain Management Pharmacologic

Non-Pharmacologic

Sedation or anesthetic medications EMLA cream Conscious sedation

Immunosuppression and Infection Children with cancer become immune impaired

from a number of causes: Lymphocyte production is altered Splenic dysfunction can prevent maturation of blood

cells and alteration is inflammatory response. Cancer therapy can decrease immunoglobulin

concentrations.

Neutropenia Significant neutropenia can develop during

chemotherapy creating an increased risk of infection in the child with cancer.

Neutropenia occurs when the absolute neutrophil count decreases below 500.

Treatment of Neutropenia Granulocyte colony stimulating factor

decreases the duration of neutropenia by stimulating the proliferation of the progenitor cells of the granulocytes, specifically the neutophils.

G-CSF: 5mcg/kg/day given subcutaneously

Varicella If an immunosuppressed child with no history

of varicella infection or varicella immunization has direct contact with an individual with chickenpox or shingles, varicella zoster immune globulin should be administered.

Acyclovir IV is used in some cases.

Central Venous Access Devices Two decades ago, CVAD were introduced as

an integral part of the pediatric oncology patient’s treatment plan.

Used to deliver chemotherapy, blood components, antibiotics, fluids, TPN, medications and blood sampling.

Chemotherapy Side Effect Drugs affect not only the cancer cells but also

healthy cells. Cells most affected are rapidly growing cells

such as hair follicles, reproductive system, bone marrow and gastrointestinal tract.

CNS Tumors• 2nd most common malignancy• 65% have 5 year survival rate• Most common tumors:

• Astrocytomas 50%• Medulloblastomas 25%• Brain stem gliomas 10%

Clinical Manifestations Classic signs and symptoms are indicative of

increased intracranial pressure. Pressure is due to tumor mass compressing vital

structure, blockage of cerebrospinal fluid flow or tumor associated edema. Gait changes / ataxia Headache with or without vomiting Blurred vision, or diplopia Forceful vomiting upon rising in the morning or

papilledema.

Management Surgery if tumor accessible Chemotherapy Radiation = Reserved for patients older that

2-years of age Survival rate based on location

Chemotherapy After surgery to prevent tumor from coming

back Shrink tumor that cannot be operated on Shrink tumor so it can be operated on

Brain Tumors

Astrocytoma

Large right frontal lobe

neoplasm with small

area of necrosis

Hodgkin's Disease

3rd most common malignancy

15 to 30 years

Three times higher in males

Excellent cure rates

Clinical Manifestations Night sweats

Weight loss

Malaise

Painless, firm nodes

Treatment Radiation to nodes Chemotherapy

Combination therapy for six months Prednisone

Stem cell transplant

Long Term Side Effects Infertility: drugs can damage ovaries or testicles Second cancers: small risk for leukemia in future Heart disease: some drugs can cause heart

problems or radiation to middle of chest Lung damage: pneumonitis from bleomycin

Neuroblastoma

Embryonic tumor Average age of diagnosis is 2 years. Poorest survival rate 50 to 60% have metastases at time of

diagnosis.

Clinical Manifestations Depends on the site of tumor

Diagnosis CT scan Bone scan 95% secrete catecholamines in the urine.

Treatment Determined by the stage of disease and age

of child. Children who have localized disease and

complete response to treatment are more likely to achieve a disease free state and long-term survival.

Neuroblastoma

Wilm’s TumorMost common type of renal tumor in children

Clinical Manifestations Firm non-tender, painless mass in abdomen

Hematuria

Hypertension

Do not palpate the abdomen

CT Scan Wilm’s Tumor

Wilm’s Tumor

Treatment Surgery

Nephrectomy Prevent rupture of capsule Sample for pathology

Chemotherapy and radiation are given based on the stage of the disease.

Osteogenic Sarcoma Malignant tumor of bone

Peak incidence is in the second decade of life, when adolescents are gaining vertical height rapidly.

Approximately 20% have metastases at diagnosis

High rate of metastasis to lungs

Diagnosis

Osteosarcoma Tumor

Femur has a largemass involvingthe metaphysis of bone. Tumor has destroyed the cortex.

Ewing Sarcoma Tumor of flat bones

Pelvis, chest, vertebrae

Rare in children under 5 years

75% diagnosed by age 20

Ewing Sarcoma

Ewing Sarcoma Tumor

Ewing Sarcoma atdistal end of the tibia.tumor extends intothe soft tissue.

Anderson’s Pathology

Rhabdomyosarcoma Most common soft bone tissue tumor

Head and neck 40% GU 20% Extremities 20% Trunk 15%

Retinoblastoma Intraocular / Embryonic tumor

1 in 16,000

+ family history

High incidence of malignancies

Retinoblastoma

Pupil reflex

“Cat Eyes”

Retinoblastoma

Treatment Surgical enucleation of eye

Genetic counseling

Follow-up care up to 18 Years