Pediatric Malignancies

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Pediatric Malignancies

• Collectively they make a relatively common
  group of diseases in children

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Pediatric Malignancies

• 1 of all cancers
• Involves tissues of
• CNS, bone, muscle, endothelial tissue
• Grows in a short period of time

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Causes

• Genetic alteration
• Environmental influences
• No known prevention
• Metastasis is seen in 80

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Response to Treatment

• Very responsive to chemotherapy
• More than 60 cure rate

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Classification of Tumors

• Embryonal tumor arises from embryonic tissue
• Lymphomas lymphatic tissue
• Leukemias blood
• Sarcoma seen in bone, cartilage, nerve and fat

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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.

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Diagnostic Tests

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

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Biopsy

• Identify cell to determine type of treatment

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Treatment Modalities

• Determined by
• Type of cancer
• Location
• Extent of disease

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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

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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.

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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.

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Goals of Chemotherapy

• Reducing the primary tumor size
• Destroying cancer cells
• Preventing metastases and microscopic spread of
  the disease

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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

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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
• Parents stem cells

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Gene Therapy

• Use of gene therapy in the treatment of childhood
  cancer is promising yet complex and still in
  early phases of clinical application.

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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.

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Pain Management

• Pain caused by disease
• Pain with procedures and treatments
• Pain associated with side effects of treatment

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Pain Management

• Pharmacologic
• Non-Pharmacologic
• Sedation or anesthetic medications
• EMLA cream
• Conscious sedation

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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.

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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.

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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

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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.

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Central Venous Access Devices

• Two decades ago, CVAD were introduced as an
  integral part of the pediatric oncology patients
  treatment plan.
• Used to deliver chemotherapy, blood components,
  antibiotics, fluids, TPN, medications and blood
  sampling.

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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.

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CNS Tumors

• 2nd most common malignancy
• 65 have 5 year survival rate
• Most common tumors
• Astrocytomas 50
• Medulloblastomas 25
• Brain stem gliomas 10

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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.

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Management

• Surgery if tumor accessible
• Chemotherapy
• Radiation Reserved for patients older that
  2-years of age
• Survival rate based on location

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Chemotherapy

• After surgery to prevent tumor from coming back
• Shrink tumor that cannot be operated on
• Shrink tumor so it can be operated on

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Brain Tumors
Astrocytoma Large right frontal lobe neoplasm
with small area of necrosis
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Hodgkin's Disease

• 3rd most common malignancy
• 15 to 30 years
• Three times higher in males
• Excellent cure rates

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Clinical Manifestations

• Night sweats
• Weight loss
• Malaise
• Painless, firm nodes

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Treatment

• Radiation to nodes
• Chemotherapy
• Combination therapy for six months
• Prednisone
• Stem cell transplant

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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

35
Neuroblastoma

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

36
Clinical Manifestations

• Depends on the site of tumor
• Diagnosis
• CT scan
• Bone scan
• 95 secrete catecholamines in the urine.

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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.

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Neuroblastoma
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Wilms Tumor

• Most common type of renal tumor in children

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Clinical Manifestations

• Firm non-tender, painless mass in abdomen
• Hematuria
• Hypertension
• Do not palpate the abdomen

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CT Scan Wilms Tumor

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Wilms Tumor
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Treatment

• Surgery
• Nephrectomy
• Prevent rupture of capsule
• Sample for pathology
• Chemotherapy and radiation are given based on the
  stage of the disease.

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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

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Diagnosis
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Osteosarcoma Tumor
Femur has a large mass involving the metaphysis
of bone. Tumor has destroyed the cortex.
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Ewing Sarcoma

• Tumor of flat bones
• Pelvis, chest, vertebrae
• Rare in children under 5 years
• 75 diagnosed by age 20

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Ewing Sarcoma
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Ewing Sarcoma Tumor
Ewing Sarcoma at distal end of the tibia. tumor
extends into the soft tissue.
Andersons Pathology
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Rhabdomyosarcoma

• Most common soft bone tissue tumor
• Head and neck 40
• GU 20
• Extremities 20
• Trunk 15

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Retinoblastoma

• Intraocular / Embryonic tumor
• 1 in 16,000
• family history
• High incidence of malignancies

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Retinoblastoma
Pupil reflex Cat Eyes

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Retinoblastoma
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Treatment

• Surgical enucleation of eye
• Genetic counseling
• Follow-up care up to 18 Years