CHAPTER 29: THE CHILD WITH CANCER DIAGNOSTIC EVALUATION

ETIOLOGY • Blood chemistry yields important

information with regard to kidney, liver,
• Apoptosis – programmed cell death
and bone function and electrolyte
• Oncogenes – genes that activate tumor
balance
growth
• Lumbar puncture is a routine test
• Tumor suppressor genes – genes that
employed in leukemia, brain tumors, and
keep tumor growth in check
other cancers that may metastasize to
• Epigenetics – changes in gene the CNS
expression without alteration
• Bone marrow test is performed by
• Germ-line mutations – chromosome aspirating marrow with a large or fine
abnormalities present in all cells bore needle
• Chromosome abnormalities can be due • Bone marrow biopsy is performed by
to: obtaining a piece of bone through a
- translocations special type of needle
- abnormal numbers of
• Metaiodobenzylguanidine scan is
chromosomes
used in certain conditions such as
• Li-Fraumeni syndrome – most well- neuroblastoma & soft tissue tumors
known inherited cancer predisposition
• Targeted therapy therapies that target
Risk Factors or blocks abnormalities
Cancer Type Risk Factor
TREATMENT MODALITIES
Acute lymphoblastic • Ionizing radiation
leukemia • Race Surgery
• Birth weight
• Main goal is to remove the tumor and
Acute myeloid • Chemotherapeutic
leukemia agents restore normal body functioning
• Genetic • Becomes successful when the tumor
conditions becomes encapsulated and localized
Brain tumors • Therapeutic Radiation Therapy
radiation
Hodgkin disease • Family history • Ionizing radiation is cytotoxic in different
• Infections ways:
Non-Hodgkin • Immunodeficiency o damages the pyrimidine bases
lymphoma need for synthesis of nucleic
Osteosarcoma • Chemotherapy acids
• Ionizing radiation o causes single strand breaks in
Ewing sarcoma • Race DNA & RNA
Wilms tumor • Congenital o causes double helical-strand
anomalies breaks in these molecules
Rhabdomyosarcoma • Congenital • Lethal damage refers to the death of
anomalies the cell
Hepatoblastoma • Genetic • Sublethal damage refers to injured
conditions cells that may be repaired
Malignant germ cell • Cryptorchidism
tumors
Chemotherapy b. those that use antibiotics or
segments of genetic material
• Works by interfering with the function or to target cancer cells
production of nucleic acids, DNA, RNA c. therapies that interfere with
• Combining drugs allows for optimum cell specific molecule involved in
cycle destruction tumor growth and progression
• Chemotherapeutic agents can be (targeted therapies)
classified according to their primary • Immunotherapy
mechanism of action o Works by inhibiting the activity of
Alkylating agents replace a hydrogen the immune system against
atom of a molecule cancer cells
by an alkyl group o Includes the use of monoclonal
Antimetabolites resemble essential antibodies that attach to proteins
metabolic elements on cancer cells
Plant alkaloids arrest cells in o Checkpoint inhibitors – blocks
metaphase by the pathways which allows
binding to cancer cells to escape
microtubular protein o Two types of cytokines:
needed for spindle interferons & interleukins
formation o CAR T-cell is being studied in
Antitumor are natural products
child with leukemia that does not
antibiotics that interfere with cell
respond to traditional therapy
division
• Adrenal and gonadal hormones have • Targeted therapies
antineoplastic properties o Substances that interfere with
specific molecules involved in
• L-asparaginase is an enzyme isolated
cancer
from extracts of bacterial cultures of
o Why are they different?
Escherichia coli or Erwinia carotovora
a. They act on molecular
• Many chemotherapeutic agents are
targets
vesicants (sclerosing agents) that can
b. Selected or designed to
cause severe cellular damage
affect their target
• A potentially fatal complication is
anaphylaxis Hematopoietic Stem Cell Transplant
• Chemotherapeutic agents must be given
• SCT restores stem cells in children who
through a free flowing IV line
have disease that require high doses of
• STOP INFUSION IF INFILTRATION
chemotherapy
OCCURS
• Types of SCT:
Biologic Therapy a. Allogenic, where cells are
obtained from a family
• Also called biotherapy member or a donor
• Uses substances made from living b. Autologous, where cells
organisms or laboratory produced previously stored from the
versions of these substances patient is given back
• Three main types: • The importance of HLA matching is to
a. those that don’t target the prevent the serious complication of graft
cells directly (immunotherapy versus host disease
or biologic response modifier
therapy)
 • Umbilical cord blood or haploidentical Superior Vena Cava Syndrome
family donors are additional sources of
• Caused by space-occupying lesions in
stem cells
the chest
Autologous Transplantation • Can also be a cause of thrombotic
complications of implantable IV devices
• Uses the patient’s own marrow that was
collected from a disease-free tissue Spinal Cord Compression
• Peripheral Blood Stem Cell
• Malignancies can invade or impinge on
Transplantation can be also used
the spinal cord
• Colony-stimulating factor is first given
• Back pain is a common initial
to stimulate the production of many cells
manifestation
COMPLICATON OF THERAPY • Treatment includes high-dose steroids
Tumor Lysis Syndrome Disseminated Intravascular Coagulation
• Direct result of rapid release of • DIC forms excessive microthrombi
intracellular contents throughout the vascular system
• Metabolic abnormalities: • Life-threatening hemorrhage can occur
o hyperuricemia from DIC with thrombocytopenia
o hypocalcemia
SIGNS AND SYMPTOMS OF CANCER
o hyperphosphatemia
o hyperkalemia • Unusual mass or swelling
• Crystallization of uric acid that occur • Unexplained paleness
with hyperuricemia can lead to acute • Easy bruising
renal failure • Unexplained fever
• Risk Factors: • Leukocoria or white eye reflex can be
o high WBC count seen as a yellow “glow” in the pupil
o large tumor burden
o cancer cell sensitivity MANAGING SIDE EFFECTS OF
• Management: TREATMENT
o monitor serum chemistries and Infection
urine pH
o allopurinol • ANC lower than 500/mm3 is at risk for:
o overwhelming infection
Hyperleukocytosis o malaise
• Defined as a peripheral white blood cell o dehydration
count greater than 100,000/mm3 o seizures
• Can lead to capillary obstruction, o invasion of organism
microinfarction, and organ dysfunction • Once infection is suspected, broad-
• Children experience respiratory distress spectrum IV antibiotic therapy should be
and cyanosis administered
• Management: • Organisms most lethal
o cytoreduction a. Viruses
o hydration b. Protozoan
o urinary alkalinization c. Fungi
d. Gram-negative bacteria
e. Gram-positive bacteria
 • CSFs promote stem cell proliferation Neurologic Problems
and stimulate rapid maturation of cells
• Footdrop and weakness and numbness
Hemorrhage may cause difficulty in walking or fine
hand movement
• Can be controlled by administration of
platelet concentrates or platelet-rich Hemorrhagic Cystitis
plasma
• Sterile hemorrhagic cystitis is a side
• Avoid rectal temperatures and
effect of chemical irritation to the bladder
suppositories
• Prevention:
• Platelet transfusions are generally
o oral or parenteral fluid intake
reserved for active bleeding episodes
o frequent voiding
that do not respond to local treatment
o administer drug early in the day
Anemia o administer mesna, a drug that
inhibits the urotoxicity of
• Consequence of drug-induced cyclophosphamide and
myelosuppression ifosfamide
• Regulate the child’s activity
Alopecia
Nausea and Vomiting
• Hair loss is a common side effect of
• Serotonin receptor blocker has greatly chemotherapeutic drugs and cranial
improved the management of nausea irradiation
and vomiting
• In mild to moderate vomiting, Steroid Effects
phenothiazine drugs remain the • Short-term steroid therapy produces
mainstay for treatment physical changes and alterations
• Synthetic cannabinoids are now being
used NURSING CARE DURING HEMATOPOIETIC
• Dronabinol is also effective in STEM CELL TRANSPLANTATION
stimulating the appetite • Child is susceptible to sepsis
Altered Nutrition • The most common complication in
allogenic transplants is acute GVHD,
• High calorie and protein foods which can affect the skin, GI tract, and
• Enteral feeding may be necessary liver
• Parenteral hyperalimentation is used to • Skin breakdowns and delayed wound
those who have digestive problems healing frequently occurs
Mucosal Ulceration HEALTH PROMOTION
• Oral ulcers (stomatitis) are red, eroded, A. DENTAL CARE
painful areas in the mouth or pharynx • Delayed or absent development
• Bland and soft diet of permanent teeth can occur
• Difficulty eating is major problem • Daily toothbrush and flossing are
• If rectal ulcers develop, warm sitz bath required
after bowel movement and applying B. IMMUNIZATIONS
occlusive ointment promotes healing • Should NEVER RECEIVE live,
attenuated vaccines
 • Antiviral agents may be given if Diagnostic Evaluation
the child develops varicella
• CBC
CANCERS OF BLOOD AND LYMPH • Bone marrow aspiration
SYSTEMS • Lumbar puncture is performed to
determine whether there is CNS
ACUTE LEUKEMIAS
involvement
• Group of malignant diseases of the bone
Therapeutic Management
marrow, blood, and lymphatic system
• Myeloid cells differentiate into RBC, • Children with higher risk or
WBC, and platelets relapse are treated with the most
• Lymphoid stem cells become intensive therapy
lymphoblast that differentiate into B and • Corticosteroids
T lymphocytes, and natural killer cells
Acute Myeloid Leukemia
• In cases of acute leukemias, immature
cells predominate that cannot function • Associated with variety of predisposition
properly syndromes
Acute Lymphoblastic Leukemia Clinical Staging and Prognosis
• Most common form of childhood cancer • Favorable prognosis is
• Peak age of onset is between 2 to 5 associated with down syndrome
years old • Unfavorable prognosis is
associated with white blood cell
Clinical Staging and Prognosis
count greater than 100,000/mm3,
• Immunophenotyping – panels presence of MRD, and
of monoclonal antibodies are cytogenetic abnormalities
used to determine T-lineage, B-
Clinical Manifestations
lineage, and myeloid antigens
• Chromosomal numbers (ploidy) • Fever, night sweats, SOB,
and structural rearrangements weakness or fatigue,
are evaluated using molecular bruising/bleeding, petechia, joint
genetic analyses pain, eczema-like skin rash
Clinical Manifestation Therapeutic Management
• Pale, listless, irritable, febrile, and • Two phases of chemotherapy:
anorexic induction and intensification
• Three main consequences of • Induction consists of three drug
bone marrow infiltration: regimens
▪ anemia from decreased • Intensification consists of high
erythrocytes dose cytarabine
▪ infection
▪ bleeding from decreased LYMPHOMAS
platelets • Group of neoplastic diseases that arise
• Sites for extramedullary disease from the lymphoid and hematopoietic
are the CNS and testes systems
• Divided into Hodgkin disease and NHL
Hodgkin Lymphoma Diagnostic Evaluation
• It has a childhood form, young adult • Lymph node biopsy is essential to
form, and older adult form establish histologic diagnosis
• Metastasizes to the nonnodal or extra • There is a presence of Hodgkin
lymphatic sites and Reed-Sternberg cells
• Classified into four histologic sites
Therapeutic Management
a. Lymphocytic predominance
b. Nodular sclerosis • Chemotherapy and irradiation
c. Mixed cellularity • MOPP (methotrexate, oncovin,
d. Lymphocytic depletion procarbazine, prednisone)
Clinical Staging and Prognosis • ABVD (adriamycin, bleomycin,
vinblastine, dacarbazine)
Nursing Care Management
• Common side effect of extensive
irradiation is malaise, which may
result from damage to the thyroid
gland
• There is a high risk of sterility
from irradiation and
chemotherapy
• Each stage is further subdivided NON-HODGKIN LYMPHOMA
into A, B, E, S, or X
• Stage A denotes absence of • Risk factors: post cancer treatment,
associated symptoms infection with Epstein-Barr virus or HIV
and AIDS
• Stage B indicates presence of
symptoms Staging and Prognosis
• Stage E represents extra
lymphatic disease • The use of aggressive
combination chemotherapy has
• Stage S indicates splenic
had a major impact on NHL
involvement
survival rates
Clinical Manifestations
• Characterized by painless
enlargement of lymph nodes
• Most common finding is enlarged,
firm, nontender, movable nodes
• Mediastinal lymphadenopathy
may cause a persistent,
nonproductive cough
• Enlarged retroperitoneal nodes
may produce unexplained fever
• Pel-Ebstein disease – low grade
or intermittent fever
 Clinical Manifestations • Diffusion-weighted imaging,
spectroscopy, and perfusion
• Manifestations depend on the
imaging are MRI tools used to
anatomic site and extent of
diagnose tumor types
involvement
• CT scan permits direct
• Lymphoid tumors compressing
visualization
various organs may cause
intestinal or airway obstruction, Therapeutic Management
cranial nerve palsies, or spinal
• Treatment involves surgery,
paralyses
chemotherapy, and radiation
• Burkitt lymphoma is a type of
therapy
cancer that is a rapidly growing
• Stereotactic surgery involves the
neoplasm
use of CT and MRI
Therapeutic Management • Radiation therapy is used to treat
most tumors and to shrink its size
• Lymphoblastic lymphoma therapy
before attempting surgical
is similar to leukemia therapy
removal
NERVOUS SYSTEM TUMORS • Consequences of radiation
therapy include tissue necrosis,
BRAIN TUMORS
subsequent malignancies,
• Infratentorial (below the tentorium endocrine dysfunction, and
cerebelli) occurs in the posterior part of behavioral deficits
the brain, primarily the cerebellum or
NEUROBLASTOMA
brainstem
• Supratentorial tumors that lies in the • It is the most common extracranial
midbrain structure childhood tumor
• Occurs in a hereditary form
Clinical Manifestations
• A hallmark is amplification of an
• Bulging fontanel indicates oncogene, MYCN
hydrocephalus
Clinical Manifestations
• Most common symptom of
infratentorial brain tumors is • Most common primary site is
headache within the abdomen
• Tumors of the cerebellum often
Location Manifestations
cause nystagmus, ataxia,
dysarthria, and dysmetria
Abdomen • firm, nontender
mass
• Supratentorial symptoms include
• discomfort
seizures, personality changes,
• anorexia
visual changes, and hemiparesis
Thoracic • dyspnea
• Tumors involving the structures in • Horner syndrome
the midbrain may cause • Neck mass
endocrinopathies Spinal Cord & • Neurologic deficits
Diagnostic Evaluation Brain • Difficulty breathing
• Paraplegia
• Standard diagnostic procedure is Bone Marrow • Anemia
MRI • Thrombocytopenia
• Neutropenia
Optic Nerves • Exophthalmos
• Periorbital
Clinical Manifestations
ecchymosis
Bones • Limping • Localized pain in the affected site
• Pain is relieved by flexed position
Diagnostic Evaluation • Palpable mass is also common

• Tumor imaging is used to locate Diagnostic Evaluation

the primary tumor • Needle or surgical biopsy
• Neuroblastomas that arise in the • Ewing sarcoma reveals
adrenal glands excrete involvement of the diaphysis with
catecholamines detachment of the periosteum
Staging and Prognosis from the bone (Codman triangle)
• In osteosarcoma, lesions are
• “silent” tumor most commonly located in the
• Factors that influence prognosis: metaphyseal region of the bone
age, site of primary tumor, tumor • Radial ossification gives the
histology, regional lymph node, tumor a “sunburst” appearance
response to treatment, and
biologic feature OSTEOSARCOMA
• Arises from bone-forming mesenchyme,
which gives rise to malignant osteoid
tissue
• Most primary tumor sites are in the
diametaphyseal region of long bones
• Risk factors: ionizing radiation exposure,
genetic predisposition syndromes,
history of retinoblastoma
Therapeutic Management
• Surgery and chemotherapy
• Surgical approach consists of
surgical biopsy followed by either
limb salvage or amputation
• Limb salvage involves resection
of primary tumor with prosthetic
replacement
Therapeutic Management
EWING SARCOMA (PRIMITIVE
• Accurate clinical staging is
NEUROECTODERMAL TUMOR OF THE
important for establishing initial
BONE)
treatment
• Second most malignant tumor in
BONE TUMORS
childhood
• Osteosarcoma and Ewing sarcoma are • Arises in the marrow space of the bone
the primary bone tumors rather than from osseous tissue
• Osteosarcoma is the most common in
adolescents and young adults
 Therapeutic Management Staging and Prognosis
• Limb salvage procedures may be • Two staging systems:
feasible in extremity lesions a. Surgicopathologic staging
• Field radiation therapy and system
chemotherapy b. TMN
Nursing Care Management RETINOBLASTOMA

• Drug regimen results in hair loss, • Arise from the retina

severe nausea and vomiting, and • Common intraocular malignancy
possible cardiotoxicity • Hereditary form of retinoblastoma,
OTHER SOLID TUMOR germline mutation of the RB1 gene is
present
WILMS TUMOR
Clinical Manifestations
• Most common kidney tumor in children
• Associated syndromes: • Whitish “glow” in the pupil
- WAGR (leukocoria)
- Beckwith-Wiedemann • Strabismus
Syndrome • Cellulitis and glaucoma
Clinical Manifestations
• Swelling or mass within the GERM CELL TUMORS
abdomen
• Arise from gonadal and extragonadal
• Hematuria
sites
• Anemia
• Classified as teratomas
• Hypertension
LIVER TUMORS
Staging and Prognosis
• Two main histologic subtypes:
a. Hepatocellular carcinoma
b. Hepatoblastoma
• Surgical resection is the treatment of
choice for liver tumors

RHABDOMYOSARCOMA
• Tumors that arise from the
mesenchymal cells
• Originates from undifferentiated
mesenchymal cells
• Common primary sites are the head and
neck, GU tract, and the extremities
• Classified into histologic subtypes:
embryonal, alveolar, and pleomorphic