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Peds Exam 3

Oncology/Childhood Cancers pt 2

QuestionAnswer
sarcomas are Malignancy in bone and soft tissue
bone tumors in adolescence generally occur in 2nd decade of life
bone tumors in adolescence Osteosarcoma Ewing sarcoma- bone and soft tissue
Soft tissue- younger children Rhabdomyosarcoma
osteosarcoma occurs most commonly in the long bones- distal femur, proximal tibia (near the knee), proximal humerus (near shoulder) AKA where bones are growing rapidly
osteosarcomas are associated with pubertal growth spurt –peak 15 yrs. Boys >girls
osteosarcomas commonly metastasize to the lungs which causes a lower prognosis
initial pain in sarcomas dull and pain symptoms initially, so often mistaken for growing pains or injury so have advanced disease upon diagnosis
the initial dull pain progresses and limits movement
what can happen with osteosarcomas Swelling or lump around affected bone
risk for osteosarcoma genetic conditions, radiation, rapid bone growth
prognosis for osteosarcoma 60-70%
Ewing Sarcoma involves the bone or soft tissue
Ewing sarcoma is most commonly in femur, pelvis, ribs (diaphysis of bone) - long part of flat bones, not the ends
ewing sarcoma is classified as localized or systemic
localized or systemic ewing sarcoma commonly metastasizes to lungs, marrow, other bones
if ewing sarcoma does or does not metastasize, 75% survival if no meta, decreased survival if metastasizes
ewing sarcoma is rare and typically affects children between ages 10-20, a little younger than osteosarcoma Males> Females
ewing sarcoma causes Intermittent pain progressively worsens Swelling/lump around site, (possibly warm to touch) Unexplained fracture Systemic symptoms
Systemic symptoms of ewing sarcoma Unexplained fever Fatigue, weight loss – concern for metastasis Anemia
why are there systemic symptoms of ewing sarcoma because of metastasizes
Diagnosis of bone tumors Chest x-ray and chest CT, MRI primary site, PET or bone scans, bone marrow biopsy
treatment of bone tumors Surgery - clean margin, optimum function Chemo Radiation for Ewing sarcoma, not osteosarcoma Stem cell rescue transplant for Ewing
Surgery - clean margin, optimum function for ewing Treatment of choice limb-salvage surgery –prosthesis amputation possible - there are now limb surgeries which can take long to recover
chemotherapy for ewing Pre-operative (neo-adjuvant) Post-operative- resume ASAP following wound healing
Radiation- for Ewing sarcoma, not osteosarcoma Preoperative- shrinks tumor, makes surgery easier Post operative- kills remaining cancer cells Used if unable to operate
Rhabdomyosarcoma is highly malignant due to metastasize to lungs
Rhabdomyosarcoma is a Soft tissue tumor primarily, develops in skeletal muscle cells
Rhabdomyosarcoma is most common in Head (especially orbit), neck, GU tract, or extremities
Risks for Rhabdomyosarcoma High birth weight Genetic conditions
genetic conditions leading to higher risk for Rhabdomyosarcoma Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Neurofibromatosis type 1, Costello syndrome, Noonan syndrome
Diagnosis of Rhabdomyosarcoma CT, MRI, biopsy - primary site CT of chest, bone scans, bone marrow biopsy, and LP to evaluate metastasis
Management of Rhabdomyosarcoma Chemo, plus surgery and/or radiation
the younger the patient with Rhabdomyosarcoma, the better the prognosis
Neuroblastoma is a Peripheral nervous system tumor arising along the sympathetic chain (embryonic neural crest cells)
Neuroblastoma is most commonly in adrenal glands- mass in abdomen, but also cervical, thoracic, and pelvic chains
Neuroblastoma is classified as low, intermediate or high risk
Neuroblastoma best prognosis is Under 12-18 months of age
Neuroblastoma Symptoms based on Location: spine may stop crawling or walking or have weakness or paralysis
Neuroblastoma Symptoms based on Location: chest may have trouble breathing
Neuroblastoma Symptoms based on Location: abdomen they may have pain, enlarged belly, constipation, vomiting, diarrhea, difficulty urinating, high BP
Neuroblastoma Symptoms based on Location: metastasize to bone or other areas may have black eyes, bone pain, bruises, fever, or paleness and may limp or stop crawling/walking, have unequal pupils
Diagnosis of Neuroblastoma CT scan or MRI Chest x-ray, bone scan, skeletal survey Bone marrow aspiration and biopsy 24- hour urine collection: elevated catecholamine metabolites
Treatment of Neuroblastoma is dependent on age and risk factors
Treatment of Neuroblastoma Multi-modal therapy Low risk- observation, surgery Intermediate risk- surgery chemo High risk – surgery, chemo, high dose chemo with stem cell transplant rescue, immunotherapy, radiation
Low risk neuroblastoma prognosis >95%
Intermediate risk neuroblastoma prognosis >90%
High risk neuroblastoma prognosis <60%
stem cell transplant for oncology so they can get increased dose of radiation/chemo to fight because medication already cleared everything
Nephroblastoma (Wilms Tumor) is a Primary malignant renal tumor
Nephroblastoma (Wilms Tumor) peak age 2 - 3 years, parents will notice mass in abdomen during bath
small percentage of Nephroblastoma (Wilms Tumor) is associated with congenital anomalies, familial predisposition
Nephroblastoma (Wilms Tumor) most commonly presents as Asymptomatic abdominal mass: does not cross midline Hematuria Malaise Hypertension Fever
Treatment of Wilms Tumor Surgery Chemo Radiation
Surgery for Wilms Tumor resect tumor without rupturing capsule Kidney on affected side will be removed
What not to do with Wilms Tumor DO NOT PALPATE- very thin and easily torn which will spread cancer cells
Chemo for Wilms tumor intensity and duration dependent on stage
Radiation for Wilms tumor reserved for stage III & IV patients
retinoblastoma (cancer in retina) is Congenital, most common intraocular cancer in childhood- in retina
gene mutation in retinoblastoma RB1 tumor suppressor gene Hereditary - autosomal dominant (often bilateral) Nonhereditary forms (advanced age- unilateral)
risks for retinoblastoma < 3 yrs., family history
early signs and symptoms of retinoblastoma leukocoria (white pupillary reflex- “cat’s eye reflex”), strabismus (eye misalignment)
late signs and symptoms of retinoblastoma Pain, decreased visual acuity, orbital edema, redness of eye
Diagnosis of retinoblastoma ultrasound, CT, MRI, ophthalmic exam
Treatment of retinoblastoma brachytherapy, photocoagulation, cryotherapy (get rid of tumor but maintain vision)
if advanced retinoblastoma, complete enucleation (eye removal) with prosthesis (3 wk till fitting and 6 wks till get it)
chemo for retinoblastoma if spread outside eye, metastasis
prognosis of retinoblastoma 90-95% if hasnt metastasized, and if has needs chemo
Enucleation of retinoblastoma Prosthetic eye Protect vision in remaining eye
Retinoblastoma Nursing care Follow up Genetic counseling - screening other children Increased lifetime other cancer risk- osteosarcoma Assess adaptation to possible vision loss
Genetic counseling for retinoblastomas at birth, 1m and 3m and then every 4-6m until 7 yrs
Treatment Modalities for cancer Surgery Chemotherapy Radiation Biologic Therapy Hematopoietic stem cell (bone marrow) transplantation (HSCT) *** treatment is typically multimodal
Treatment terms Primary treatment- main treatment Neoadjuvant- given before primary treatment Adjuvant – given after primary treatment
Neoadjuvant- given before primary treatment Reduces tumor size Kills cancers cells that have spread
Adjuvant – given after primary treatment Destroys remaining cancer cells
what kind of treatment do children normally get the trifecta - primary, neoadjuvant and adjuvant
Surgery for children Seldom used alone Primary goal -diagnostic vs. therapeutic Definitive surgery may be delayed Specific to type of cancer
Primary goal -diagnostic vs. therapeutic obtain biopsy document extent of disease
chemo kills all cells not just cancer cells
overview of chemo Antineoplastic agents Takes advantage - rapid rates of cellular division Given to cure, control cancer, or ease symptoms Primary treatment or adjuvant to surgery, radiation, or HSCT: Neoadjuvant – shrink tumor
Dose-limiting toxicities in chemo: myelosuppression Neutropenia, thrombocytopenia, anemia
Dose-limiting toxicities in chemo: GI n/v
Dose-limiting toxicities in chemo: integumentary Stomatitis/mucositis
Dose-limiting toxicities in chemo: heart, liver, lungs End-organ toxicities
Nursing Management for Patient on Chemotherapy pt 1 Pre-treatment assessment Dose calculation & verification Safe handling & administration Monitoring for acute and delayed onset toxicities
Nursing Management for Patient on Chemotherapy pt 2 Patient & family education: monitor temperatures, NO rectal temps, axillary best (especially with mouth sores) When to call the treatment team: first sign of fever
Myelosuppression Decreased bone marrow activity Caused by chemotherapy, radiation, infiltration of marrow with cancer cells Nadir Support during Nadir
Decreased bone marrow activity with myelosuppression WBCs- neutropenia RBCs - anemia Platelets -thrombocytopenia
Nadir when blood counts are at their lowest timing and duration dependent on agent(s), dose, intensity
Support during nadir reduce risk of life-threatening complications; neutropenia, anemia, and thrombocytopenia
when does Nadir occur 7-10 days after chemo and counts must recover before next treatment
Prevention of infection due to neutropenia pt 1 Handwashing, good mouth care, private room Precautions based on ANC – absolute neutrophil count Prophylaxis against Pneumocystis pneumonia- Trimethoprim-sulfamethoxazole (Bactrim)
Prevention of infection due to neutropenia pt 2 Colony stimulating factors- G-CSF, Neupogen, Neulasta No flowers No fresh fruits and vegetables Avoid sick contacts
Management of infection due to neutropenia Monitor for subtle signs/symptoms Fever may be only symptom – Call MD for all fevers 1.) Cultures- blood, urine, nasopharyngeal, stool for fever 2.) Initiate broad-spectrum antibiotics for fever during period of neutropenia
how long do you give BSA until blood cultures allow to narrow down
when drawing cultures, use first aspirate!!! never flush first incase line is infected
thrombocytopenia Platelet transfusions as needed Bleeding risk Avoid skin puncture (even avoid BP cuff) Meticulous mouth care- prevent bleeding and mucositis (use soft toothbrush) Avoid rectal temperatures, suppositories Use acetaminophen for pain
anemia PRBC transfusions as needed (plt only last 1-3 days) Not as severe as neutropenia May be delayed effect Regulate child’s activity – manage fatigue
an increased number of transfusions means increased number of transfusion reactions
Nausea/Vomiting is the most distressing side effects of cancer treatment
Nausea/Vomiting types acute – during or immediately following chemo delayed – starting or persisting beyond 24 hrs. anticipatory – conditioned response when n/v poorly controlled with prior courses
Nausea/Vomiting goal Prevention is goal – achievable for many children
Nausea/Vomiting Emetogenic potential likelihood that specific agents will cause vomiting (low, moderate, high) - treatment for nausea before any chemo and continue at least 24 hrs after chemo
For agents with moderate or high potential, administer anti-emetics before, during, and beyond chemotherapy regimen (time dependent on med)
Tailor approach based on individual preferences, past experiences
important to do this during n/v Keep patient well hydrated- may need to start IV fluids
radiation Ionization causes single-strand or double-strand breaks in DNA
radiation causes inability of cells to divide inability to repair DNA damage cell death
side effects of radiation dependent on location and dose
goals for radiation cure, long term control, and palliation
radiation can be used alone or adjuvant therapy with surgery, chemotherapy or both
Radiation therapy
Nursing Management Assist with positioning child Monitor for side effects (acute or delayed)
Assist with positioning child for radiation Reproducibility of treatment field essential
Monitor for side effects (acute or delayed) in radiation Depends on area irradiated Myelosuppression- bone marrow Skin breakdown, alopecia- skin, head Nausea/ vomiting, anorexia, mucosal ulceration – GI tract, head Fatigue/post-radiation somnolence syndrome
late complications of radiation diminished organ function, cognitive limitations, impaired growth, second cancers (osteosarcoma)
cognitive issues are worse the younger the child Benefit vs Risk: Try to avoid brain radiation under 3-year-old
Hematopoietic Stem Cell Transplantation (HSCT) goal rescue child after high dose of treatment to treat child after cancer exceed myelosuppressive threshold of standard cancer therapies
HSCT is Maximum doses of chemo and/or radiation to maximize chance of killing cancer cells
HSCT has a profound risk of toxicity, life-threatening complications, GVHD
types of HSCT Autologous: own stem cells so won't reject but might not fight so increased risk of getting cancer back Allogeneic: match related donor, risk for rejection, devel graft vs host Syngeneic: identical twin Umbilical cord blood: have baby to get cord blood
Nursing Management for HSCT pt 1 Coordinate administration of high-dose chemo and possible full body radiation Administer donor stem cells via IV infusion (blood transfusion) Implement positive isolation No dried, fresh flowers, potted plants
Nursing Management for HSCT pt 2 Monitor for infection: fever Monitor for graft vs host disease (GVHD) Administer blood products as needed until engraftment Reposition patient
graft vs host disease (GVHD) symptoms Diarrhea, maculopapular rash on hands, bottom of feet
Created by: leh195
 

 



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