Osteosarcoma

 Osteosarcoma (also called osteogenic sarcoma) is the most common type of cancer
that starts in the bones. The cancer cells in these tumors look like early forms of
bone cells that normally help make new bone tissue, but the bone tissue in an
osteosarcoma is not as strong as that in normal bones.(American Cancer
Society,2020)
 Most osteosarcomas occur in children, teens, and young adults. Teens are the most
commonly affected age group, but osteosarcoma can develop at any age. (American
Cancer Society,2020)
 Osteosarcoma is a type of bone cancer that usually develops in the osteoblast cells
that form bone. It happens most often in children, adolescents, and young adults.
Approximately 800 new cases of osteosarcoma are reported each year in the U.S.
Of these cases, about 400 are in children and teens. It happens slightly more often
in males than in females. (Johns Hopkins, n.d.)
 Osteosarcoma most commonly happens in the long bones around the knee. Other
sites for osteosarcoma include the upper leg, or thighbone, the lower leg, upper arm
bone, or any bone in the body, including those in the pelvis, shoulder, and skull.
(Johns Hopkins, n.d.)
 Osteosarcoma may grow into nearby tissues, such as tendons or muscles. It may
also spread, or metastasize, through the bloodstream to other organs or bones in the
body. (Johns Hopkins, n.d.)
 Osteosarcoma is the most common primary pediatric bone malignancy, derived
from primitive bone-forming (osteoid producing) mesenchymal cells. (Prater and
McKeon, 2021).
 Osteosarcoma is a bone tumor and can occur in any bone, usually in the extremities
of long bones near metaphyseal growth plates. The most common sites are as
follows:
 Femur (42%, 75% of which are in the distal femur)
 Tibia (19%, 80% of which are in the proximal tibia)
 Humerus (10%, 90% of which are in the proximal humerus)
 Skull and jaw (8%)
 Pelvis (8%)
Types/ Classification/stages
Classification
1. Central (Intramedullary):
High-Grade Central
Conventional – “classic” appearance; cells are spindle-like to
polyhedral in shape; nuclei are variable in appearance, and cells
undergoing mitosis are readily identifiable. Matrix production by the
tumor cells may be osseous (“osteoblastic”), cartilaginous
(“chondroblastic”), or fibrous (“fibroblastic”) but a combination of
the three often presents. Osteoid matrix must be identified
somewhere in the lesion, even if only in a minuscule amount. In the
case of osteoblastic osteosarcoma, there may be excessive osteoid
matrix production such that the tumor is described as “sclerosing” in
appearance.Osteoblastoma-like – histologically resembles
osteoblastoma but features more cellular atypia and local
aggressiveness, and osteoid matrix production
 Chondroblastoma-like – histological resembles
chondroblastoma but features more cellular atypia, local
aggressiveness, and osteoid matrix production
Chondromyxoid fibroma-like – histological resembles
chondromyxoid fibroma but features more cellular atypia, local
aggressiveness, and osteoid matrix production
Malignant fibrous histiocytoma-like – histologically resembles
malignant fibrous histiocytoma but features more cellular
atypia, local aggressiveness, and osteoid matrix production
Epithelioid – features cells that are so poorly differentiated that
it may be hard to distinguish histologically whether the lesion
is a sarcoma (connective tissue origin) or a carcinoma
(epithelial origin)
Giant cell – features benign multinucleated osteoclast-like
giant cells
Clear cell – features numerous cells with clear or ground-glass
cytoplasm and vacuoles

Telangiectatic – comprised of numerous blood-filled sinusoids such

that the tumor mimics the histology of an aneurysmal bone cyst; the
presence of pleomorphic/atypical nuclei will distinguish the
malignancy.
Small Cell – considered to be a histological combination of Ewing
sarcoma and osteosarcoma, features numerous small round cells. A
minuscule volume of an osteoid matrix will distinguish this as a
variant of osteosarcoma.
Low-Grade Central
Fibrous dysplasia–like – features a large volume of osseous matrix
embedded in a small amount of fibrous stroma.
Desmoplastic fibroma–like – features a very small volume of osseous
matrix embedded in a large amount of fibrous stroma.
2. Surface (Periosteal/Cortical):
Low-Grade Surface
Parosteal – found on the outer periosteal surface of the bone,
features ribbons of osseous trabeculae oriented in parallel,
primarily composed of a chondroid matrix with only a
minuscule amount of osteoid matrix.
Intermediate-Grade Surface
Periosteal – found between cortex and the inner periosteal
surface of the bone, features ribbons of osseous trabeculae
oriented in parallel, primarily composed of a chondroid matrix
with only a minuscule amount of osteoid matrix, more nuclear
atypia than the parosteal variant.
High-Grade Surface
High-Grade Surface – histological identical to high
grade/conventional/central variant, varies only in location
(being confined to the surface of the bone) which is thought to
represent dedifferentiated parosteal osteosarcoma.
3. Extraskeletal:
 Low-Grade-Histologically identical to low-grade surface/parosteal
variant and low-grade central variant varies only in geography,
potentially appearing at any extraskeletal location in the body, including
the soft tissues of the thigh, buttocks, upper extremities, or
retroperitoneum.
High-Grade-Histological identical to high grade/conventional/central
variant differs only in geography, being found at any extraskeletal
location in the body.
Stages
 Musculoskeletal Tumor Society (MSTS) staging system
A system commonly used to stage osteosarcoma is the MSTS system, also known
as the Enneking system. It is based on 3 key pieces of information:
 The grade (G) of the tumor, which is a measure of how likely it is to
grow and spread, based on how it looks under the microscope. Tumors are
either low grade (G1) or high grade (G2). Low-grade tumor cells look more
like normal cells and are less likely to grow and spread quickly, while high-
grade tumor cells look more abnormal.
 The extent of the primary tumor (T), which is classified as either
intracompartmental (T1), meaning it has basically remained within the
bone, or extracompartmental (T2), meaning it has extended beyond the
bone into other nearby structures.
 If the tumor has metastasized (M), which means it has spread to other
areas, either to nearby lymph nodes (bean-sized collections of immune
system cells) or other organs. Tumors that have not spread to the lymph
nodes or other organs are considered M0, while those that have spread are
M1.
These factors are combined to give an overall stage, using Roman numerals
from I to III. Stages I and II are further divided into A for intracompartmental tumors or
B for extracompartmental tumors.

In summary:
Low-grade, localized tumors are stage I.
High-grade, localized tumors are stage II.
Metastatic tumors (regardless of grade) are stage III.
  The TNM staging system
Another system sometimes used to stage bone cancers (including osteosarcomas)
is the American Joint Commission on Cancer (AJCC) TNM system. This system is
based on 4 key pieces of information:
 T describes the size of the main (primary) tumor and if it appears in
different areas of the bone.
 N describes the extent of spread to nearby (regional) lymph nodes. Bone
tumors rarely spread to the lymph nodes.
 M indicates if the cancer has metastasized (spread) to other organs of the
body. (The most common sites of spread are to the lungs or other bones.)
 G stands for the grade of the tumor, which describes how the cells look
under a microscope. Low-grade tumor cells look more like normal cells and are
less likely to grow and spread quickly, while high-grade tumor cells look more
abnormal.

Musculoskeletal Tumor Society/Enneking System for Staging of Malignant

Musculoskeletal Tumors
Stage IA: Low grade, intracompartmental tumor location, no metastasis
Stage IB: Low grade, extracompartmental tumor location, no metastasis
Stage IIA: High grade, intracompartmental tumor location, no metastasis
Stage IIB: High grade, extracompartmental tumor location, no metastasis
Stage III: Any grade, any location, metastasis present

American Joint Committee on Cancer (AJCC) System for Staging of Primary Bone
Sarcomas (8th Edition)
Stage IA: Low grade, less than 8 cm tumor size, no spread to regional lymph nodes, no
distant metastasis
Stage IB: Low grade, greater than 8 cm tumor size or skip lesions, no spread to regional
lymph nodes, no distant metastasis
Stage IIA: High grade, greater than 8 cm tumor size, no spread to regional lymph nodes,
no distant metastasis
Stage IIB: High grade, less than 8 cm tumor size, no spread to regional lymph nodes, no
distant metastasis
Stage III: High grade, discontinuous tumor involvement/"skip" lesions, no regional
lymph nodes, no distant metastasis
Stage IVA: Any grade, any size, no regional lymph node spread, lung metastasis
Stage IVB: Any grade, any size, regional lymph node spread, lung or extrapulmonary
metastasis

Etiology/Risk Factors
The highly complex karyotypes typical of osteosarcoma tumor cytology have
created challenges regarding the thorough characterization of recurrent chromosomal
mutations. However, research has identified several genetic aberrations in cases of
primary osteosarcoma:
 Hereditary Retinoblastoma: An autosomal dominant condition caused by germline
mutations in the RB1 gene, causes bilateral retinoblastoma at an average presenting
age of one year. Retinoblastoma characteristically presents as an absence of the
"red reflex" in the eye or eyes of the affected child. This disorder imparts an
increased risk of osteosarcoma later in life.
 Li-Fraumeni Syndrome: An autosomal dominant disorder due to mutations in the
p53 tumor suppressor gene, has been found in up to 3% of children with
osteosarcoma. Patients with this disorder are also at a high risk of developing
several additional types of cancer at a very early age.
 Rothmund-Thompson Syndrome: An autosomal recessive syndrome due to a
mutation in the RECQL4 gene, conveys a predisposition to osteosarcoma as well as
a characteristic infantile rash, dysplastic osseous structures, alopecia, premature
cataracts, and chronic gastrointestinal distress.
 Bloom Syndrome: An autosomal recessive disorder caused by mutations in the
BLM gene, a gene responsible for maintaining DNA stability during replication. In
addition to a predisposition to osteosarcoma and other cancers, these patients may
also present with UV-induced rashes, short stature, and sparse subcutaneous fat.
 Werner Syndrome: An autosomal recessive disorder, also known as adult progeria,
is characterized by premature aging, bilateral cataracts, osteoporosis, short stature,
scleroderma-like skin changes, and a predilection for osteosarcoma. A faulty WRN
gene is responsible
Review of A and P
Skeletal System
The skeletal portion of the system serves as the main storage
system for calcium and phosphorus. The importance of this storage is
to help regulate mineral balance in the bloodstream. When the
fluctuation of minerals is high, these minerals are stored in bone; when
it is low, minerals are withdrawn from the bone.
The skeleton also contains critical components of the
hematopoietic (blood production) system. Located in long bones are
two distinctions of bone marrow: yellow and red. The yellow marrow
has fatty connective tissue and is found in the marrow cavity. In times
of starvation, the body uses the fat in yellow marrow for energy.
The red marrow of some bones is an important site for
hematopoeisis or blood cell production that replaces cells that have
been destroyed by the liver. Here, all erythrocytes, platelets, and most
leukocytes form in bone marrow from where they migrate to the
circulation.

The Axial Skeleton

The axial skeleton is the part of the skeleton that consists of the bones of the head
and trunk .
The axial skeleton functions to support and protect the organs of the dorsal and
ventral cavities. It also serves as a surface for the attachment of muscles and parts of the
appendicular skeleton.

The human’s axial skeleton is composed of 80 bones and is the central core of the body.
The primary divisions of the skeleton system are:
 Head, including the bones of the skull (cranium), face, auditory ossicles,
and hyoid bone.
 Thorax, including the rib cage and sternum.
 Vertebral column.
The Appendicular Skeleton
The appendicular skeleton includes the skeletal elements within the limbs, as well as
supporting pectoral and pelvic girdles.
 The appendicular skeleton comprises 126 bones and is involved in locomotion and
manipulation of objects in the environment. It is unfused, allowing for greater range of
motion.
Diagnostic Procedures
1. Clinical History and Physical Exam
Symptoms of osteosarcoma may be present for a significant amount of time,
sometimes weeks to months, before patients seek evaluation. Most commonly, the
presenting symptom is bone pain, particularly with activity. Parents are often concerned
that their child has incurred a sprain, arthritis, or growing pains. There may or may not
be a reported history of traumatic musculoskeletal injury.
Pathologic fractures are not a common mainstay of osteosarcoma, except for the
telangiectatic type of osteosarcoma, which is associated with pathologic fractures. The
resulting pain may manifest as a limp. A swelling or lump may or may not be reported,
depending on tumor size and location. Systemic symptoms, such as those seen in
lymphoma (fever, night sweats, etc.), are rare.
Respiratory symptoms are rare and, when present, indicate extensive lung
involvement. Additional symptoms are unusual because metastases to other sites are
extremely rare.
Physical examination findings are typically focused around the location of the
primary tumor and may include:
 A palpable mass may be tender and warm with or without an overlying
pulsation or bruit, though these signs are nonspecific
 Joint involvement with decreased range of motion
 Local or regional lymphadenopathy (unusual)
 Respiratory findings with metastatic forms
2. Laboratory Analysis of Lactate Dehydrogenase (LDH) and Alkaline
Phosphatase (ALP) Levels: Biochemical markers such as serum alkaline phosphatase
(ALP) and lactate dehydrogenases (LDH) are assessed in the initial workup because
they provide evidence for diagnosis and prognosis. ALP levels will be high due to the
increased osteoblastic activity associated with osteosarcoma. Extremely high levels
have been linked to heavy tumor burden and are generally considered a poor prognostic
indicator. It is important to evaluate the levels of the biomarkers later in the treatment
process as well, as levels may decrease with success therapy or rise with residual
disease or recurrence.
3. Diagnostic Imaging of Primary Tumor Site
 Radiographs - although MRI is the gold standard for diagnostic imaging of
osteosarcoma, radiographs are generally the first study obtained when a potential
bone mass is identified on the physical exam. A conventional radiograph of
osteosarcoma may demonstrate- medullary and cortical bone destruction,
permeative or moth-eaten cortex, "Sunburst" configuration (due to aggressive
periostitis), "Codman triangle" configuration (due to elevation of the periosteum
away from the bone), ill-defined "fluffy" or "cloud-like" osseous lesion, soft-tissue
mass, calcification of osteoid matrix produced by the tumor.
 Magnetic Resonance Imaging - after identifying a suspicious lesion on a
radiograph, MRI may be necessary for further characterization. MRI is an
indispensable tool for defining the extent of a tumor inside and outside the bone.
The entirety of the involved bone, as well as one joint above and one joint below
the tumor, should be included in the study so that “skip” lesions are not missed.
MRI can accurately and precisely delineate the degree of tumor in the adjacent soft
tissues, joint involvement, whether or not the tumor crosses the physis, proximity to
 the nearest neurovascular bundle. Nearly every aspect of treatment is assessable
with MRI, from pre-surgical assessment for limb-sparing resection to the degree of
chemotherapy response in the form of tumor necrosis, shrinkage, and improved
capsulation. Traditional sequences acquired in MRI of osteosarcoma may
demonstrate the following:
 T1 Weighted Images
 Non-ossified soft tissue component: intermediate signal intensity
 Osteoid components: low signal intensity
 Peritumoral edema: intermediate signal intensity
 Scattered foci of hemorrhage: variable signal intensity based on
chronicity
 T2 Weighted Images
 Non-ossified soft tissue component: high signal intensity
 Osteoid components: low signal intensity
 Peritumoral edema: high signal intensity
Computed Tomography - the role of CT is primarily to assist with biopsy planning
and disease staging. CT may not significantly contribute to direct assessment of the
tumor after radiography and MRI unless the osseous lesion in question is
predominantly lytic. In the case of lytic lesions, small amounts of mineralized
material may be unobservable on both plain film and MRI. CT of the chest,
however, is the modality of choice for the evaluation of metastasis.
4. Nuclear Imaging
Positron Emission Tomography – PET is a nuclear medicine imaging modality that
detects highly metabolic lesions. It is an essential tool that is useful for determining
tumor extent and searching for subtle lesions after identifying a suspicious mass on
initial diagnostic imaging. Later in the treatment process, PET is valuable for the
detection of recurrence.
Radionuclide Bone Scan - Technetium 99 methylenediphosphonate (Tc99 MDP) bone
scan is an effective and readily available imaging modality for detecting bony
metastasis. It is a less expensive but less specific alternative to PET imaging

Medical management
National Comprehensive Cancer Network's 2020 Guidelines for Management of
Osteosarcoma (Version 1.2020)

OSTEO-1 (Low-Grade Osteosarcoma, No Metastasis)

 Intramedullary and surface
 Wide excision alone (no neoadjuvant chemotherapy)
 If postsurgical pathology demonstrates low-grade features, then no
adjuvant chemotherapy
 If postsurgical pathology demonstrates high-grade features, consider
adjuvant chemotherapy
Periosteal
 Neoadjuvant chemotherapy then perform a wide excision
If postsurgical pathology demonstrates is consistent with biopsy (low grade features
only) then no adjuvant chemotherapy
If postsurgical pathology demonstrates high-grade features, consider adjuvant
chemotherapy
OSTEO-2 (High-Grade Intramedullary or Surface Osteosarcoma, No Metastasis)
Neoadjuvant chemotherapy then restage the lesion
  If restaging suggests the lesion is resectable, then perform a wide excision
 Positive margins
 If there was a good response to preoperative neoadjuvant chemotherapy
(less than10% viable tumor on postsurgical pathology), then continue the
same neoadjuvant chemotherapy regimen and consider additional surgical
resection +/- radiation therapy
 If there was an inadequate response to preoperative neoadjuvant
chemotherapy (greater than 10% viable tumor on postsurgical pathology),
then continue the same neoadjuvant chemotherapy regimen or consider a new
regimen and consider additional surgical resection +/- radiation therapy
 Negative margins
 If there was a good response to preoperative neoadjuvant chemotherapy
(less than 10% viable tumor on postsurgical pathology), then continue the
same neoadjuvant chemotherapy regimen. No further resection is required.
 If there was an inadequate response to preoperative neoadjuvant
chemotherapy (greater than 10% viable tumor on postsurgical pathology),
then continue the same neoadjuvant chemotherapy regimen or consider a new
regimen. No further resection is required.
 If restaging suggests the lesion is unresectable, then continue
chemotherapy and consider radiation therapy.
OSTEO-3 (Any Grade With Metastasis at Presentation)
 If metastases are resectable (pulmonary, visceral, or skeletal), then
perform metastasectomy and follow OSTEO-2 guidelines.
 If metastases are unresectable, then consider chemotherapy and radiation
therapy, after which the primary site requires reassessment for local control.
OSTEO-4 (Follow-up & Surveillance)
Surveillance schedule
 Every three months for post-op years 1 and 2
 Every four months in post-op year 3
 Every six months in post-op years 4 and 5
 Yearly for post-op years six and beyond
Surveillance visit should include
 Physical exam with assessment of function
 Imaging of post-op site and chest
Consider PET/CT or bone scan
CBC +/- additional laboratory tests as clinically indicated (e.g., alkaline phosphatase
levels)
If a relapse is detected, the following are the guidelines to follow:
 Chemotherapy +/- resection (if possible)
 Response to these treatments should have an evaluation via:
 Radiographs of the original tumor site
 CT or MRI (both with contrast) of the site of relapse
 CT of the chest to assess for pulmonary lesions
 Good response to treatment:
 Surveillance (restart OSTEO-4 guidelines)
 Poor response/progression of the disease:
 Resection (if possible)
 Clinical trial
 Palliative radiation
 Best supportive care
Surgical
Limb Salvage

 The vast majority of patients (about 85 to 90%) with osteosarcoma

undergo limb salvage. Limb salvage involves the removal of a tumor from a
limb without the removal of the entire limb itself. This process occurs in two
main steps: initial resection and subsequent reconstruction. Resection is
essential for the elimination of the disease. As previously mentioned, the
surgical excision of the mass should also include the biopsy site/tract, with a
minimum margin of 2 cm, to avoid recurrence of disease from tumor cells,
which may have escaped during tissue sampling. Radiological imaging
performed during the initial evaluation should be reviewed preoperatively to
determine the volume of bone that requires removal. Ideally, the mass and
reactive zone surrounding it should not be disturbed, such that the entirety of
dissection occurs through normal healthy tissues, which typically includes an
additional 6 to 7 cm of adjacent normal bone. Computer-generated anatomical
reconstructions are very useful for this purpose, particularly in tumors of the
flat bone of the pelvis and sacrum, where excessive resection of bone can create
postoperative problems with structural stability.
 Because osteosarcoma is the most common primary osseous malignancy
in the pediatric population, surgery presents a unique set of challenges. To
achieve clear margins, excision may necessitate physeal resection, which can
lead to growth disturbances as the child matures. In the past, a tumor that
traversed the growth plate was considered to be an indication for amputation
because there was no available means to restore function. With the advent of
options that “grow” or expand with the patient , masses that cross the growth
plate are no longer considered a contraindication to limb salvage.
 Another difficulty that surgeons encounter in the quest for limb salvage
is a mass that encompasses a joint; this is a common challenge due to the
predilection of osteosarcoma for the knee. However, a combination of resection
and tissue regeneration have helped to over this obstacle such that joint
involvement is no longer considered a contraindication to limb salvage.
 After resection, reconstruction can begin. The purpose of reconstruction
is the restoration of function to the affected limb. In the case of non-weight-
bearing bones like the fibula or clavicle, reconstruction is unnecessary because
the excision of these structures does not impart functional deficit.
Reconstruction of weight-bearing bones, as one might imagine, is an arduous
task. One of the greatest challenges for surgeons is a recapitulation of large
swaths of missing bone. In recent years, several options have become available.
These options fall into three main classes: allograft/autograft bone
reconstruction, metallic (endoprosthesis) reconstruction, and tissue regeneration
reconstruction.
 Allograft/Autograft Bone Reconstruction
 Allograft bone replacement utilizes bones collected in the
postmortem period from organ donors. As with organ donation,
potential donors undergo screening for communicable diseases.
Once surgically grafted into the osteosarcoma patient, the native
bone will grow into the allograft bone and heal. Rejection is rare
because very few donor cells remain within the donated bone, and
the bone itself is a relatively inert material. As one might imagine,
 the most serious complication that may arise with allograft
reconstruction is the failure of fusion between patient bone and
allograft bone. Infection and fracture are also important
complications that require internal fixation or removal,
respectively. A hybrid reconstructive device is available in the
form of an allograft prosthetic composite (APC), which combines
an allograft bone fragment with a metallic prosthesis. APC
arthroplasty is useful for the reconstruction of weight-bearing joints
such as the knee or hip. This device combines the easier reinsertion
of a biological graft with the instant weight-bearing ability of a
prosthetic.
 In centers without access to a donor bone bank, resected
malignant bone can be irradiated (or less frequently, pasteurized or
treated with liquid nitrogen) and reimplanted, resulting in a perfect
match for the osseous defect at the surgical site. This process is
known as autografting, and it can be very cost-effective. However,
there are only limited indications for autografting, as donor bone
for allograft is relatively easy to procure
 Metallic Prosthetics
 Metallic prosthetics have revolutionized surgical
reconstruction. So-called “mega prostheses” provide for the
replacement of large segments of the bone and the joint that
connects them. A decade ago, most of these devices had to be
custom made but today, “off the shelf” options are available for
immediate implantation. Some of these prostheses are expandable
“growing” implants that permit interval lengthening. These are
particularly efficacious in skeletally immature individuals. Because
the growth plates of the affected segment of bone often get
resected, the prosthesis can be elongated by 1 to 2 cm at a time, so
the length of the previously diseased limb correlates with the
contralateral healthy limb.
 Tissue Regeneration
 Tissue regeneration for reconstruction following resection
of osteosarcoma is a relatively new field. In general, this process
utilizes a combination of a patient’s own cells, purified intrinsic
growth factors, and synthetic, scaffold-like matrix materials to
induce autologous tissue regeneration. Until this emerging
technology is more widely available, procedures such as the
Ilizarov technique or spatial frame method utilize external fixation
devices to promote the growth of long bones up to 1 millimeter per
day (about 1 inch per month).
Amputation

 Amputation, previously considered the gold standard for surgical management

of osteosarcoma, is reserved only for non-resectable masses with contamination of
myotendinous and neurovascular that make limb salvage impossible. Amputation
may be performed as a standalone treatment or in conjunction with rotationplasty.
 Rotationplasty is a procedure that involves resection of the lower extremity to
the level of the distal femur. Resection is followed by a 180-degree rotation of the
lower extremity with subsequent reattachment at the distal femur, essentially
 transforming the ankle into a “knee” joint such that the plantar flexors (soleus and
gastrocnemius) get converted to knee extensors. The procedure has been shown to
provide surprisingly favorable functionality. However, its unusual appearance has
been known to cause psychological distress in some patients.

Outcomes of Limb Salvage vs. Amputation

 A few studies have demonstrated a slight increase in the rate of recurrence in
patients with limb salvage when compared to amputees. Still, the overall rate of
survival in patients who recur is comparable. Interestingly, though, several studies
report higher survival rates in patients who have undergone limb salvage versus
amputation. The vast majority of providers who treat osteosarcoma now favor limb
salvage over amputation.
Radiation Therapy
National Comprehensive Cancer Network's 2020 Guidelines of Radiation Therapy for
Osteosarcoma (Version 1.2020)

Post-Operative Radiation Treatment for Primary Tumors

Resectable Tumors- Post-operative radiation therapy with 55 Gy plus 9 to 13 Gy
boost for residual microscopic or gross disease (64-68 total dose)
Unresectable Tumors- 60 to 70 Gy (the total dose is dependent on tolerance on normal
tissue)
Radiation Treatment for Metastatic Disease
Samarium 153-EDTMP
Stereotactic radiosurgery (SRS)

Medical
Before the advent of chemotherapy, the survival rates for patients with high-grade
osteosarcoma were abysmal despite the removal of all visible disease via amputation;
this indicated the presence of undetectable micrometastases, typically to the lungs.
Chemotherapy, in conjunction with surgical resection, has addressed the presence of
micrometastases and significantly improved survival rates.
National Comprehensive Cancer Network's 2020 Recommendations for Chemotherapy
Agents & Regimens for Treatment of Osteosarcoma (Version 1.2020)

1. Recommended Neoadjuvant/Adjuvant Chemotherapy Regimens for Initial-

Occurrence
 Cisplatin and doxorubicin (Category 1)
 MAP (high-dose methotrexate, cisplatin, and doxorubicin) (Category 1)
 Doxorubicin, cisplatin, ifosfamide, and high-dose methotrexate
2. Recommended Chemotherapy Regimens for Relapsed, Refractory or Metastatic
Disease
 Regorafenib (Category 1)
 Ifosfamide (high dose) +/- etoposide
 Sorafenib
 Sorafenib and everolimus
“Category 1” recommendations are those based upon high-level evidence, with uniform
NCCN consensus that the intervention is appropriate. This designation represents the
highest level of clinical confidence in efficacy.

Summarize Diagnostic procedure and medical management

 Diagnostic
 x-ray- This picture does an excellent job of showing osteosarcoma in the tibia
 CT scan can help evaluate the extent of soft tissue damage.
 MRI could also be ordered for difficult to visualize areas.
 needle biopsy allows for the cancer to be staged for size and degree of spread.
 Physical Assessment
patients will have pain that usually is described as severe or dull localized acute pain.
It’s usually relieved by a flexed position – so bending the extremity usually makes the
pain better. With the generally vague pain complaints, the pain is commonly
attributed to trauma or growing pains.
There could be some swelling and may be able to actually palpate the mass.
There could also be some warmth that could feel to the affected area as well.
Patients will also have musculoskeletal changes, so assessing mobility and ability to
perform ADLs is important.
Pain can cause limping if a weight bearing limb is affected as well as limited range of
motion. Increased pain could indicate pathological fractures at the tumor site, so this
is important to consider as well.

Medical Management
 Radiation is the treatment of choice in early osteosarcoma as it reduces both the size of
tumor and pain.
 Chemo can be used before and after surgery and is used to kill the rapidly growing
cancer cells
 If chemo is administered, make sure to monitor for potential complications that can
occur with treatment.
 Surgical resection reduces the size of tumors or can remove them entirely, such as in a
limb salvage procedure where the goal is to save the limb and remove the affected
tissue. Usually surgery is combined with radiation or chemo for the most effective
results.
 Depending on the size of resection, an implant or allograft may be needed. (an allograft
is a tissue graft from a donor – typically from cadavers.)
 Depending on the extent of tumor invasion, amputation may be indicated. Osteosarcoma
is one of the most common reasons for amputation in children.
 Pathologic fractures are caused by metastatic bone and are very painful so pain control
is a treatment priority.
 Wound care could be indicated if surgery is performed or if there are wounds from
infection. Make sure to monitor for infection and perform neurovascular checks just like
with other surgical procedures.
 Depending on disease progression, patients may choose palliative treatment vs curative
treatment if the prognosis is poor or if there is metastasis.

Nursing management
 Provide quiet environment and calm activities to prevent or lessen pain.
 Provide comfort measure such as back rub, change position and use of heat or cold
application.
 Encourage diversional activities
 Administer analgesics as indicated to maximal dose as needed.
 Encourage the patient to increase fluid intake.
 Encourage rest periods to prevent fatigue.
 Provide accurate information about the situation, medication and treatment. It can
help patient and family feels empowered
 Assess muscle strength, gross and fine motor coordination.
 Provide pillows for cushion and support.
 Keep side rails up all the time.

Nursing Diagnosis
Impaired oral mucous membrane related to chemotherapy as manifested by
inflamed mouth, oral discomfort and oral plaque.

Nursing inference:
Chemotherapy can damage cells in the mucous membranes so they become inflamed.
Thus impaired oral mucous membrane.

Nursing goal:
After_____________ of nursing interventions, the patient will be free of oral
mucous membrane irritation as manifested by uninflamed mouth, reduce oral
discomfort, and absence of oral plaque.

Nursing Interventions Rationale

Allow the patient to choose foods they
prefer from the list given.
Teach the patient in performing oral
hygiene using a soft bristle toothbrush or
sponge-tipped swab.
Encourage the patient to eat foods high
in protein and vitamins.
Encourage the patient to suck sugarless
candy or gum every 2 hours.
Provide an antiseptic mouth rinse 30
minutes before meals.
Apply topical analgesic as ordered
before meals and encourage cool, bland,
and, smooth foods that are not spicy.
Educate parents on mouth rinsing and
topical application of medications.
Educate parents regarding chemotherapy
effect on the oral mucosa and
its treatment.
Promotes independence and allows
control over the situation to decrease
helplessness and enhanced nutritional
status.
Avoids damage and irritation to the oral
mucosa.
Promotes wound healing process and
enhance recovery.
Stimulates salivation and avoid dry
mouth.
Promotes comfort of oral mucosa and
maintains integrity.
Prevents trauma and discomfort.
Promotes sufficient oral care to relieve
discomfort and prevent mucosa irritation
and increased inflammation.
Allows better knowledge of the
unwanted effects that may occur during
chemotherapy.

Nursing Evaluation:
After of nursing interventions, the patient is free of oral mucous
membrane irritation as manifested by uninflamed mouth, reduce oral discomfort, and
absence of oral plaque.
Nursing Diagnosis:
Anxiety related to Change in health status as manifested by Expressed concern
and worry about procedures.

Nursing Interventions Rationale

Assess possible need for Supports the child’s ability to deal
special counseling services for the with illness and promotes adjustment
child. to lifestyle changes.
Encourage parents to stay with child;
Enhances care and promotes
Leave a telephone number in case of
emotional comfort to the child.
a need for more information.
Communicate with the child based
on developmental age level and
Allows better understanding and
answer questions calmly and
promotes trust.
honestly; use pictures, models, and
drawings for explanations.
Allow open expression of concerns
about Provides an opportunity to
illness, procedures, management, vent feelings and fears to
and possible consequences of reduce anxiety.
surgery.
Provide child with structure and input
in decisions about care and routines Empower the child and promotes
as independence.
possible.
Provide consistent
Promotes trust and comfort
care nurse assignment with the same
and familiarity with staff giving care.
personnel.
Orient child to surgical and ICU unit, Lessens the anxiety incident brought
equipment, noises, and staff. about by the fear of unknown.
Teach parents and child about the
disease process, surgical procedure,
Provides information to promote
what to expect preoperatively and
understanding that will relieve fear
postoperatively including
and anxiety; understanding
chemotherapy and its benefits and
of preoperative and
side effects (nausea,
postoperative treatments and effect
vomiting, diarrhea, stomatitis,
on body image.
alopecia, and others are possibilities
but are temporary; phantom pain).
Explain all procedures and care Supplies information about
in simple, direct, honest terms all diagnostic procedures and
and repeat as often as
tests such as CBC, platelets
necessary; reinforce physician
with chemotherapy and scans, and X-
information if needed and provide
rays for diagnosis.
specific information as requested.
Inform parents and child of
the extent of surgery planned with
the removal of a limb (that a
Reduces anxiety when knowledge
temporary prosthesis will be
and  support is given, and child
fitted immediately following surgery,
and parents will not feel betrayed
and a
by inadequate preparation
permanent one will be fitted in 6 to 8
of procedures and treatments.
weeks; that recreational and physical
therapy will be undertaken following
amputation).
Provides information and
Introduce the child to another who support from a peer with the same
has same disease and amputation. condition and who would have
empathy.

American Cancer Society, (2020). What Is Osteosarcoma? Retrieved from:

https://www.cancer.org/cancer/osteosarcoma/about/what-is-osteosarcoma.html
Antipuesto, D., (2010). Osteosarcoma. Retrieved from: https://nursingcrib.com/nursing-
notes-reviewer/medical-surgical-nursing/osteosarcoma/
Johns Hopkins, (n.d). Osteosarcoma. Retrieved from:
https://www.hopkinsmedicine.org/health/conditions-and-
diseases/sarcoma/osteosarcoma
Prater S, McKeon B. , (2021). Osteosarcoma. Retrieved from:
https://www.ncbi.nlm.nih.gov/books/NBK549868/