Osteosarcoma

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Osteosarcoma (osteogenic sarcoma)

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MOST COMMON BONE CANCER IN CHILDREN

PEAK INCIDENCE BETWEEN 10-25 YEARS OF AGE

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Osteosarcoma presumably arises from

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bone-forming mesenchyme, which gives rise to MALIGNANT OSTEOID TISSUE

most PRIMARY tumor sites are in the METAPHYSIS (wider part of the shaft, adjacent to the epiphyseal growth plate) of long bones.....ESPECIALLY LOWER EXTREMITIES

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More than half of osteosarcoma cases occur in the

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FEMUR (particularly the DISTAL portion) with the rest involving the:

1) humerus
2) tibia
3) pelvis
4) jaw
5) phalanges

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Optimum treatment for osteosarcoma is

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1) surgery
2) chemotherapy

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Surgical approach to osteosarcoma involves

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1) surgical biopsy followed by
2) limb salvage or amputation

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Dependent on the site, surgery includes

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1) amputation at least 3 inches above the proximal tumor margin
2) 3 inches above the join proximal to the involved bone

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Tumors of the DISTAL femur, preservation of the

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hip joint may be possible

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Other amputations are

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1) above-the-knee for tumors of the tibia or fibula
2) hemipelvectomy for tumors of the innominate (hip) bone
3) forequarter (removal of arm, scapula, portion of the clavicle) for tumors of the upper humerus

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Limb salvage procedure which involves

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1) en bloc resection of the primary tumor
2) prosthetic replacement of the involved bone

Ex: osteosarcoma of the distal femur (a total femur and joint replacement is performed)

Pre-operatively: CHEMO (to decrease tumor size)

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Chemotherapy - Osteosarcom

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Medications:

1) Antineoplastic (high-dose methotrexate with citrovorum factor rescue
2) Doxorubicin
3) Bleomycin
4) Actinomycin D
5) Cyclophosphamide
6) Ifosfamide
7) Cisplatin

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Pulmonary metastasis (Osteosarcoma)

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Thoracotomy
Chemotherapy

prolonged survival and POTENTIAL CURE

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Nursing Education - Diagnosis of Cancer

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1) Should not disguise with falsehoods such as "infection"
2) Child should be aware of lack of alternatives for treatment
3) Responsibility of telling the child lies with the physician but the nurse should be present or aware of the exact conversation
4) Child should be told a few days before the surgery to think about the diagnosis, treatment and prepare questions

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Sometimes, children have many questions for osteosarcoma surgery, such as

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1) Prosthesis
2) Limitations on physical ability
3) Prognosis in terms of cure

Sometimes,
1) Silence or calm belies the fear

ACCEPT EITHER RESPONSE (GRIEVING PROCESS)

Answer questions without offering additional information to not overwhelm the child

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Chemotherapy, as well, shoud be discussed without offering too much information,

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1) Hair loss, focus on the positive aspects, such as wearing a wig.
2) Adolescent may become angry over all the radical body alterations

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If amputation is performed, the child

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1) is fitted with a temporary prosthesis immediately after surgery
2) permits early functioning
3) psychological adjustment
4) PERMANENT PROSTHESIS IS USUALLY FITTED WITHIN 6-8 WEEKS

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Phantom limb pain, signs and symptoms

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1) tingling
2) itching
3) pain

REAL NOT IMAGINED

Administer: Amitriptyline (Elavil)

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Juveline Idiopathic Arthritis is a

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chronic, autoimmune inflammatory disease causing inflammation of joints and other tissue with an unknown cause

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JIA - two factors hypothesized

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1) Immunogenic susceptibility
2) Environmental
3) External trigger (virus)

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Pathophysiology - JIA

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1) Chronic inflammation of the synovium
2) Joint effusion
3) Eventual erosion, destruction and fibrosis of the articular cartilage

IF PERSISTENT INFLAMMATION:
1) Adhesions between joint surfaces
2) Ankylosis of joints

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JIA waxes and wanes .and eventually

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becomes inactive in approximately 70% of the cases....HOWEVER....these children may have severe or minimal joint damage remaining when active arthritis abates

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JIA, approximately 30% of children

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will have progressive arthritis into adulthood.

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JIA can cause

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chronic and acute UVEITIS and can cause PERMANENT vision loss if undiagnosed and not aggressively treated

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Durban classification of JIA (heterogenous group of diseases):

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Systemic: 1 or more joints w/2 weeks fever, rash, lymphadenopathy, hepatosplenomegaly and serositis.

Oligoarthritis - 1 to 4 joints for first 6 months.
Subdivided into
Persistent Oligoarthritis if remains 4 joints
Extended Oligoarthritis if 4+

Polyarthritis rheumatoid factor (-) 5 or more joints but have NEGATIVE rheumatoid factor

Polyarthritis rheumatoid factor (+) AFFECTS 5 or more joints but have POSITIVE rheumatoid factor

Psoriatic with psoriasis or dactylitis, nail pitting, or onycholysis or psoriasis in first degree relative

Enthesitis -
1) inflammation at the tendon insertions site with sacroiliac or lumbosacral pain
2) HLA-B27 antigen
3) arthritis in male older than 6 years
4) acute anterior uveitis
5) inflammatory bowel disease
6) Reiter syndrome
7) acute anterior uveitis in first-degree relative

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JIA

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no definitive tests

Classifications based on:
1) age of onset before 16
2) one or more joints for 6 weeks

ESR may or may not be elevated

Leukocytosis frequently present

Antinuclear bodies are common - may identify children at greater risk for uveitis

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JIA best imaging studies

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plain radiographs

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Slit lamp

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eye examination is necessary to diagnose uveitis (inflammation of the anterior chamber of the eye)

COMMON IN: Antinuclear antibody-positive young girls with oligoarthritis

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Major goals therapy JIA

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1) control pain
2) preserve joint ROM and function
3) minimize effects of inflammation
4) promote normal growth and development

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Medications JIA

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FIRST DRUGS:
NSAIDs - Naproxen (take with food), ibuprofen, and tolmetin are approved for children - NO ASPIRIN

SECOND LINE:
Methotrexate - started in combination with NSAID
Monitor - CBC and LIVER
TEENAGERS: Birth defects/avoid alcohol

CORTICOSTEROIDS used for
1) life-threatening complications
2) incapacitating arthritis
3) uveitis
LOW DOSE/BRIEF PERIOD/DISCONTINUED TAPERED SCHEDULED
4) Oral, Intraarticular joint injections, IV infusions, eye drop form for uveitis

PROLONGED USE CORTICOSTEROIDS:
1) Cushing syndrome
2) Osteoporosis
3) Infection Risk
4) Glucose intolerance
5) Cataracts
6) Growth suppresssion

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Tumor Necrosis Factor Inhibition (Children unresponsive to methotrexate)

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Etanercept....once/twice week subcutaneous

S/E: allergic reaction
increased infection risk
demyelinating disease
pancytopenia

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SAARDS

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may take MONTHS to be effective and typically work in combination with NSAIDs - used less often

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JIA - Physical Therapy

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1) Hot pack or warm bath before
2) Nighttime splinting - knees, wrists, hands
3) Firm mattress
4) No pillow or very low one
5) No support under knee

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JIA is linked with

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maternal depression
parental distress

NOT with physical disability

NO OPIOID NO NO NO NO

Behavioral therapy
Relaxation

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JIA Refractory Pain

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short-term opioids

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JIA Bedtime Routine

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1) Firm mattress
2) Heated water bed
3) Electric blanket
4) Sleeping bag

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JIA physician check ups required when

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1) URTI
2) relatively minor illnesses

to prevent flare-up

MOIST HEAT IS BENEFICIAL!!!!!
BATHTUB WARM WATER
WHIRLPOOL BATH
PARAFFIN BATH
HOT PACKS
WET HOT BATH TOWEL/COVER PLASTIC/20 MINUTES
PAN WARM WATER 2-3 TIMES DAILY IN ADDITION TO TUB BATHS

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Electric blanket set timer

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1 hour before awakening