Running Head: OSTEOSARCOMA 1

Osteosarcoma

Alexa Pascucci

The University of Alabama

HD 426 - 001

Dr. Sherwood Burns-Nader

April 14, 2022

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Osteosarcoma

Osteosarcoma is a bone cancer that predominantly affects children and adolescents who

are experiencing growth spurts. There are many different physiological and psychological needs

of patients with this condition, depending on their developmental age. Family aspects of the

condition should also be considered, as parents, siblings, and other family members will require

support after an osteosarcoma diagnosis as well. In order to best treat osteosarcoma,

multidisciplinary care is necessary, as it involves specialists from several different departments

within the hospital setting.

Definition, Symptoms, and Causes of Osteosarcoma

Osteosarcoma– also referred to as osteogenic sarcoma– is a type of bone cancer that

usually begins in the osteoblast cells, which are the cells that form the bone matrix (Johns

Hopkins, 2022). It is most often found in children, adolescents, and young adults– with the

average age at diagnosis being 15– but is also seen in older adults. Osteosarcoma is most often

found in the femur, tibia, humerus, pelvis, and shoulder, specifically the metaphyseal areas where

the fastest growth is taking place, however, it can be found in any bone in the body (Cleveland

Clinic, 2021). Osteosarcoma may also be found in the tissues surrounding bones, and it may

metastasize through the bloodstream or lymphatic system to other parts of the body (Johns

Hopkins, 2022). Between 400 and 1,000 new cases of osteosarcoma are diagnosed in the United

States each year and it is the most common primary bone tumor found in children and the third

most common cancer in adolescents (Cleveland Clinic, 2021). Osteosarcoma accounts for

approximately 20% of all bone cancers (Kundu, 2014), and is slightly more common in males

than females and more common in African Americans than Caucasians (St. Jude Children’s

Research Hospital, 2022).

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There are several symptoms of osteosarcoma. The most common symptoms include bone

pain or tenderness, a mass or lump that is warm and may be felt through the skin, swelling or

redness at the tumor site, increased pain when lifting or limping (depends on affected bones),

limited movement, fever, bone injury for no clear reason (Cleveland Clinic, 2021). These are the

most common symptoms of this disease, however, every individual may experience symptoms

differently.

Osteosarcoma begins when a healthy bone cell, or osteoblast, develops changes in its

DNA. These changes tell the cell to make new bone when it is not needed, resulting in a mass, or

tumor, of poorly formed bone cells that invade and destroy healthy tissues surrounding it. It is

not clear what exactly causes the osteoblasts’ DNA to mutate and for osteosarcoma to be

developed (Mayo Clinic, 2022). Despite this, there are still several risk factors that may make an

individual more likely to develop osteosarcoma including rapid bone growth (teenage growth

spurts); previous exposure to or treatment with radiation, especially at a young age or in high

doses; bone infarction; and certain inherited or genetic conditions, such as Li-Fraumeni

syndrome, Rothmund-Thompson syndrome, and hereditary retinoblastoma (Johns Hopkins,

2022). At this time, there are no known ways to prevent osteosarcoma.

Diagnosis, Treatments, and Potential Outcomes of Osteosarcoma

The doctor will begin with a physical examination and thorough medical history to best

understand the patient’s symptoms. Then, multiple imaging tests may be done to look for the

presence of cancer, including x-rays, a computerized tomography (CT) scan, magnetic resonance

imaging (MRI), a positron emission tomography (PET) scan, and a bone scan (Mayo Clinic,

2022). CT scans use an x-ray and a computer to make detailed images of the body, showing the

bones, muscles, fat, and organs of the body (Johns Hopkins, 2022). MRI uses a large magnet,
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radio waves, and a computer to create clearer visualizations of certain body parts. They may be

ordered if an x-ray image is not clear enough to get an accurate view (Cleveland Clinic, 2021).

PET scans are when tagged radioactive glucose is injected into the bloodstream. Tumors use

glucose more than other tissues, so they will be easily detected by the scanning machine. PET

scans can be used to locate small tumors that have spread, and check to see if treatment for a

known tumor is working (Johns Hopkins, 2022). Complete blood counts (CBC) are a

measurement of the size, number, and maturity of the different blood cells in a specific volume

of blood. These blood tests are done to provide information about blood counts and how organs,

such as the kidneys and liver, are working (Cleveland Clinic, 2021). There is no blood test to

detect the presence of a bone tumor. If a mass or tumor is located, a biopsy procedure is done to

collect a sample of suspicious cells for laboratory testing. Lab tests can show whether the cells

are cancerous and can determine the type and the grade of cancer. Types of biopsy procedures

used to diagnose osteosarcoma include needle biopsies and surgical biopsies. A needle biopsy is

performed by a doctor inserting a thin needle through the skin and guiding it into the tumor. The

needle is used to remove small pieces of tissue from the tumor. A surgical biopsy is done when

the doctor makes an incision through the skin and removes either the entire tumor (excisional

biopsy) or a portion of the tumor (incisional biopsy). Doctors perform the biopsy in a way that

will not interfere with future surgery to remove cancer (Mayo Clinic, 2022).

If the aforementioned tests result in a diagnosis of osteosarcoma, the next step is to find

out if the cancer cells have spread or if they have remained localized, which is called “staging”

(Cleveland Clinic, 2021). The staging classification used in osteosarcoma is the Musculoskeletal

Tumor Society staging scheme, also known as the Enneking system. This system is based on the

histological grade of the tumor, its local extent, and the presence or absence of metastasis
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(Kundu, 2014). The Enneking system establishes whether a tumor is a low or high grade (I or II),

whether the tumor is intra- or extra-compartmental (A or B), and whether any metastases are

present (III). Osteosarcoma patients are most commonly diagnosed at stage IIB (Misaghi et al.,

2018). The World Health Organization divides osteosarcoma based on its histology into central,

intramedullary, and surface tumors, with subtypes under each group (Misaghi et al., 2018).

Osteosarcomas are classified as primary or secondary based on the tumor’s histology

(microscopic structure of the tissues) and where it is located within the body (Kundu, 2014).

Osteosarcoma tumors can also be categorized as low-grade, intermediate-grade, or high-grade.

Low-grade means that cancer grows slowly and remains where it began (localized), while

high-grade indicates that it will spread quickly and metastasize. There are nine types of

high-grade osteosarcomas, one type of immediate-grade osteosarcoma, and two types of

low-grade osteosarcomas (Cleveland Clinic, 2021).

Treatment for osteosarcoma varies based on the patient’s age, medical history, severity

and subtype of cancer, and expectations for the course of the disease (Johns Hopkins, 2022).

Typically, treatment for osteosarcoma involves chemotherapy and surgery. Chemotherapy is the

use of drugs to kill cancer cells. Neoadjuvant chemotherapy is given before surgery to shrink the

tumor and kill any cancer cells in the bloodstream and typically takes about 10 weeks. Adjuvant

chemotherapy is given for an additional 18 weeks after surgery to kill any cancer cells that might

still remain in the body (Cleveland Clinic, 2021). During surgery, the tumor and some of the

healthy tissue around it will be cut out of the affected bone. In most cases involving the arm or

leg, a limb-salvage surgery can be done, which is when a tumor is removed without amputation

of the affected limb. Limb-salvage procedures may be possible in those whose cancer has not

spread beyond its original site. Occasionally, artificial implants or bone is taken from another
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part of your body can be used to replace the bone that was removed during surgery (Cleveland

Clinic, 2021). There are some cases where a section or all of an arm or leg must be amputated.

Rotationplasty procedures can be done on tumors of the distal femur, which is the area of the leg

just above the knee. The leg is partially amputated, the tumor is removed, and then the lower leg

is rotated 180 degrees and reattached, using the ankle as a new knee. This converts the

above-knee to a below-knee amputation that is more functional and helps fit a prosthesis

(Cleveland Clinic, 2021). Radiation therapy can also be used to treat osteosarcoma. Radiation

therapy is particularly useful in areas where surgery is not possible. There are two types: external

radiation therapy and internal radiation therapy. External radiation therapy uses a machine that

points radiation at the area of the body that is cancerous. Internal radiation therapy is where the

substance is inserted via needle or catheter. For osteosarcoma, some patients are treated with

bone-seeking radiopharmaceuticals (samarium or radium) that are taken up by the bone-forming

osteosarcoma cancer cells, delivering radiation specifically to the cancer site (Cleveland Clinic,

2021). Many patients, especially those with higher-grade tumors, will receive a combination of

treatments. Over the last decade, there have been no significant advances in the treatment of

osteosarcoma, however, advancements are slowly being made in the treatment of the disease as

more is being understood about the pathophysiology of the disease (Misaghi et al., 2018).

The prognosis of a patient with osteosarcoma greatly depends on the extent of the

disease, the size and location of the tumor, the pathologic grade of cancer, and the tumor’s

response to therapy (Johns Hopkins, 2022). Cells from the tumor may metastasize, or break away

from the original tumor site and spread throughout the body, making treatment and recovery

more difficult (Mayo Clinic, 2022). Osteosarcoma often spreads to the lungs or other bones.

Once metastasized, survival rates for osteosarcoma can lower significantly, from around 70% to
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30% (Cleveland Clinic, 2021). The aggressive chemotherapy needed to control osteosarcoma can

cause substantial side effects, both in the short and long term. Another potential outcome are the

late effects of cancer and its treatment. A person who was treated for bone cancer as a child or

adolescent may develop effects months or years after treatment ends. The kind of late effects one

develops depends on the location of the tumor and the way it was treated. Some types of

treatment may later affect fertility for both men and women, causing the inability to have

children (Johns Hopkins, 2022). Recurrence of the disease is possible and typically happens

within the 18 months following the completion of treatment. Prognosis and long-term survival

vary greatly from person to person, but because of improvements in chemotherapy, surgery, and

radiation, more people diagnosed with osteosarcoma are achieving better results than in years

prior (Cleveland Clinic, 2021).

Fears, Anxieties, and Potential Stressors Related to Osteosarcoma

There are many fears, anxieties, and potential stressors that are related to an

osteosarcoma diagnosis. The fear of death brought on by the disease is evident, as is the fear of

losing the affected limb if the damage brought on by the disease is extensive enough.

Post-traumatic stress disorder (PTSD) related to the osteosarcoma diagnosis and invasive

treatments has been reported in long-term survivors of pediatric sarcomas (Wiener et al., 2006).

As previously stated, the possibility of long-term side-effects from the original cancer or brought

on by intense treatments is a concern for patients with osteosarcoma, such as neurological

problems, secondary cancers, problems with the heart, lungs, kidneys, ears, and fertility, and

changes in thinking, learning, or memory (Cleveland Clinic, 2021). The possibility that the

cancer may return in the years following treatment is another concern for osteosarcoma patients.

One-third of osteosarcoma cases are recurrent, and 95% of relapses occur within the first five
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years after diagnosis (Halldorsson et al., 2009). With that, there is a constant potential for future

illness, hospitalization, and procedures.

Osteosarcoma is most commonly seen in adolescents and young adults, and their

perceptions of and reactions to hospitalizations are different from other age groups. Adolescents

fall into Erik Erikson’s fifth stage of psychosocial development, which is identity versus role

confusion. During this stage, which spans between the ages of 12 and 18, adolescents fear the

loss of identity, struggle with independence and relying on adults, have a heightened dependence

on peers and social groups, and increased focus on appearance and sexual identity (including

concerns about body changes, such as loss of hair), and have concerns about peers and life

moving on without them (Burns-Nader, 2022). Adolescents experience formal operational

thinking, which may cause them to have fears about the reality of their illness. For this age

group, self-worth is based on egocentric qualities, such as appearance and acceptance from their

peers (Burns-Nader, 2022). Something like an amputation or hair loss as a result of their cancer

and treatment could cause distress in an adolescent because they are constantly trying to please

the “imaginary audience”. In addition to the typical anxieties, fears, and stressors associated with

a diagnosis of osteosarcoma, the developmental age group of the patient and their specific

thought processes play a role in how they process and cope with the disease.

Family Variables

An osteosarcoma diagnosis does not only affect the patient, but it affects their family as

well. How a family handles the stressors brought on by hospitalization is based on their past

experiences in healthcare, coping abilities, and the circumstances of an illness (Burns-Nader,

2022). Sources of stress that may arise for parents after their child is diagnosed with

osteosarcoma include the management, decisions, and perceived severity of the illness; the
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hospital environment, including unfamiliar items and personnel, and the uncertainty of their

parental role; the type of hospital admission (expected, unexpected, emergency) and the length of

the stay; changes in the child’s behavior as a result of illness and psychosocial effects; and

changes in the routine of daily life (Burns-Nader, 2022). Parents may develop guilt because they

worry that they did something to cause the hospitalization, such as waiting too long to act on the

illness. The priorities of parents are typically focused on the needs of the hospitalized child, the

needs of the siblings, work responsibilities, and home responsibilities. At first, parents may

passively cope with their child’s hospitalization by listening to but not hearing the information

and following any direction given by the medical team. After some time, parents often become

active, gathering information and resources to better understand and advocate for their child’s

needs. Effective coping by parents is related to their satisfaction with the hospitalization

(Burns-Nader, 2013). Parents of patients with osteosarcoma often have to make very difficult

decisions regarding their child’s care, such as how to proceed with treatment and when to stop

treatment if it is not working. The outcomes of their child’s diagnosis may affect family life in

several ways, as parents who have lost a child or have a sick child bear additional stress and have

a higher rate of divorce than couples who have healthy children (Behrman et al., 2003).

Siblings are also affected by the patient’s diagnosis and hospitalization. Siblings may

experience a wide range of emotions, and their reactions are often underestimated. Siblings

experience healthcare indirectly, but may have many of the same reactions and emotions to the

healthcare environment as patients do, depending on their developmental stage (Burns-Nader,

2022). Stress that siblings are experiencing may be expressed through: changes in sleeping or

eating, concentration problems, behavior problems, aggression with peers, or acting nervous,

withdrawn, angry, or clinging to their parents. Siblings’ reactions to the outcome of their
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sibling’s diagnosis may vary and are influenced by: their developmental stage (younger children

may feel blame themselves for their sibling’s illness and fear that they themselves may get sick);

fears of sibling death; their relationship with the sibling; and separation from their parents and

siblings, as well as subsequent changes in their caregivers, routines, and environment

(Burns-Nader, 2022).

Family-Centered Care

Family-centered care is “a way of providing services that assures the health and

well-being of children and their families through respectful family/professional partnerships. It

honors the strengths, cultures, traditions, and expertise that families and professionals bring to

this relationship” (Family Voices, 2021). Family-centered care improves both the patient’s and

family’s experience with healthcare, reduces stress, improves communication between the

healthcare team and the family, and reduces conflict. Family-centered care develops policies,

practices, and systems that are family-friendly and family-centered in all settings and

acknowledges the family as the constant in a child’s life. It builds on the family’s strengths,

honors their cultural diversity and family traditions, and supports the child in learning about and

participating in their care and decision-making while promoting an individual and developmental

approach. The foundation of family-centered care is the partnership between families and

professionals (Family Voices, 2021).

Family-centered care can be offered to a patient with osteosarcoma by organizing

meetings between the family and the healthcare team so that questions can be asked and

answered, helping families by making informed healthcare decisions on behalf of their child, and

by initiating palliative care. It is important that siblings are included in the discussions of the

patient’s care as well, and that the diagnosis, hospitalization, and outcomes are explained to them
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in developmentally appropriate language. Family-centered care benefits both the patient and their

family by helping to decrease anxiety, accelerate recovery time, decrease emotional distress by

teaching better coping prior to procedures, hospitalization, and recovery, and increasing the

parents’ confidence in the healthcare team (Family Voices, 2021).

Multidisciplinary Care

Pediatric patients with osteosarcoma have a team of healthcare providers to help treat the

disease. The team is led by a pediatric oncologist, which is a doctor who specializes in pediatric

cancer, and may consist of several other pediatric specialists (Cleveland Clinic, 2021). An

orthopedic surgeon will be a part of the patient’s care to recommend surgical options for

treatment, including limb-salvage surgery, amputation, and rotationplasty. Pediatric

subspecialists (such as a pediatric cardiologist– the subspecialists utilized will depend on the

affected parts of the body) play a role in the healthcare team to offer their services and expertise

in a certain specialized field to best monitor and treat certain areas of the child’s body. A

radiation oncologist may be a part of the team if radiation therapy on the tumor is required.

Several nurses will tend to the patient’s care throughout treatment. Depending on whether or not

an amputation is required as part of the patient’s treatment, a prosthetist may be a part of the

patient’s multidisciplinary care to fit them for a custom prosthetic leg or arm. Physical therapists

and occupational therapists will help the patient rehabilitate and gain strength and physical,

cognitive, and sensory skills. A child life specialist helps children and their families adapt to the

hospital environment and cope with the challenges that come along with illness and disability.

Child life specialists are trained to ease anxieties associated with medical care, as well as comfort

and educate patients and families by breaking down confusing medical jargon, and the use of

therapeutic play and coping techniques. Child life helps to promote normalcy within the
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unknown and potentially stressful hospital environment, as well as serve as the liaison between

the family and the medical team. Psychologists can help the patient and family overcome trauma

and address the family’s mental health needs during and after cancer treatment. Palliative care

addresses the psychological, social, and/or spiritual problems of children and their families. The

goal of palliative care is to promote the best quality of life, especially with pain and end-of-life

care. Multidisciplinary care is important for a child who has been diagnosed with osteosarcoma

because it is a complicated disease to treat, therefore it is imperative to have a healthcare team

made up of professionals from many different specialties.

Conclusion

Osteosarcoma is a bone cancer that is commonly found in children and young adults that

requires an aggressive treatment plan involving chemotherapy, surgery, and radiation therapy.

Treatment for osteosarcoma may have long-lasting effects on the patient, such as PTSD,

neurological problems, secondary cancers, and problems with various body parts, as well as the

possibility of relapse. Osteosarcoma has an approximate 70% survival rate if localized, and an

approximate 30% survival rate if it has metastasized to other parts of the body. The patient’s

developmental age will determine their psychological understanding of the disease and

treatments, as well as how they will cope. An osteosarcoma diagnosis affects many members of

the family differently, so family-centered care involving a child life specialist and members of

the healthcare team is imperative. Many avenues of care intersect to influence the care and

treatment of osteosarcoma, and all are very important to ensure that the patient has the best

outcome.
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References

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Burns-Nader, S. (2022, February). Emotional Responses to Pediatric Healthcare Experiences

[Lecture]. HD 426 Hospitalized Children and Youth Course, The University of Alabama.

Burns-Nader, S., Hernandez-Reif, M., & Porter, M. (2013). The relationship between mothers’

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