Problem statement 4
Osteosarcoma Case Study
DL is a 17-year-old young adult who presented with 3 months of
worsening left knee
pain. This began insidiously without any injury and progressed in
intensity over time. By
the time he saw his doctor, it had gotten to the point where he
was unable to bear any
weight on the leg due to severe pain, and had started using
crutches. He does not have
any other medical conditions and is otherwise very healthy. He is
active in sports, and
likes to play basketball.
X-ray
Imaging was done, starting with Xrays, which showed a large,
cloud-like calcified area
around the left distal femur. This imaging demonstrates some
typical features of an
osteosarcoma: a bony matrix being produced by the tumor,
periosteal elevation with
areas of “onion-skinning”, which represents layer upon layer of
ossifying periosteum,
and areas of “hair on end”, which represents radially outward
growth of the tumor being
visualized as linear ossification. The native cortex of the bone is
poorly visualized in
areas of the tumor, suggesting that it has been dissolved by the
tumor, a clear indication
of the malignant nature of the mass.
MRI
Further cross-sectional imaging with MRI demonstrates the
relationship to the joint, and
the nerves and blood vessel of the popliteal fossa.
A biopsy is performed demonstrating a classic osteosarcoma.
Bone scan of the entire
skeleton, and a CT scan of the chest complete the staging process
to understand the
full extent of the tumor. Laboratory values such as alkaline
phosphatase provide
prognostic value prior to initiating treatment. The first step in
management is a neoadjuvant
chemotherapy regimen. During this time, the patient is kept on
crutches and
care is taken not to fracture the delicate tumor.
Endoprosthesis
Once the initial chemotherapy is completed, the tumor is
removed and the leg is
reconstructed in a single surgical procedure. In this case, this is
accomplished with a
limb-sparing surgical excision, maintaining the native vessels and
nerves. The resection
resulted in negative margins, which is the intended result. Once
the tumor is removed, a
metallic internal femoral implant, called and “endoprosthesis” is
used to rebuild the
defect. This is mated to a hinged total knee replacement to
provide knee function. This
will take approximately two months to incorporate into the host
bone, at which point the
patient can put his full weight on the leg.
Once the operation is completed, the resected specimen is
studied for various
prognostic factors, including and most importantly the Huvos
Grade, which is a
pathological assessment of the percent of necrosis in an
osteosarcoma resected
specimen. The desired finding is to have a Huvos Grade of greater
than 95% necrosis
of the tumor from the initiation chemotherapy. This portends the
best prognosis for the
patient. In this case, the Huvos Grade was greater than 99%
necrosis, which was
welcome news for DL and his family.
The remainder of the chemotherapy regimen is carried out after
surgery, which a short
break incorporated for healing time. The total treatment time is
lengthy, and we usually
tell patients to expect about a year of treatment overall. This can
vary somewhat, but it
is important that the patient have a good understanding that it is
a long process, but one
that can be highly effective. DL is now getting close to the end of
this regimen, is
walking well, and is looking forward to resuming his life as a
teenager.