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Muscoloskeletal Diseases #8 Prof Scotti Infections and Tumors

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Muscoloskeletal Diseases #8

Infections and Tumors


Professor Scotti 21 January 2014 Author Francesca Rossetti Reviewer Luigi Bonini

INFECTIONS
Infections in orthopedics are osteomyelitis, arthritis and infected arthroplasty.

OSTEOMYELITIS
Osteomyelitis is an infection of bone and bone marrow; it may be caused by
direct inoculation of bacteria through an open fracture or via the hematogenous
route by blood-borne organisms. Osteomyelitis is typically caused by bacteria,
very rarely by fungi. It may be acute or chronic.
ACUTE HEMATOGENOUS OSTEOMYELITIS is typical of children and is
diagnosed within 2 weeks from the onset of symptoms; typically patients seek
for medical advice within 48 hrs since symptoms are very severe. It shows meta-epiphyseal localization (lower extremity more frequent) and it is typically
monosthotic, i.e. it affects only one bone.
The metaphysis is the typical localization in children because it is highly
vascularized. This area is very metabolically active because of the presence of
the growth plate allowing for limb lengthening during childhood and adolescence; this area is extremely vascularized and that's why typically hematogenous bacteria stop here. Moreover, the growth plate, which is avascular and
cartilaginous, is a natural barrier to the diffusion of the abscess to the epiphysis (you can appreciate it also in the MRI below). So, generally the onset of
acute hematogenous osteomyelitis is located here in the metaphysis with no spread to the epiphysis; this may,
however, occur in more advanced cases.
As you can see here in this drawing (left), the abscess diffuses prevalently through the bone and tends
to elevate the periosteum. This causes abnormal ossification around the bone: the cambial layer of the periosteum in fact is rich in mesenchymal progenitor cells
which typically participate in bone healing; in this case
these cells are stimulated by the inflammatory microenvironment rich in cytokines, so the periosteum tends to
ossify during osteomyelitis.
Here (below) you can see how the infectious process may also erode the periosteum and go through
the soft tissue and the skin, forming a draining sinus.
Q: How long does this take to develop?
A: It's variable, but this doesn't happen in the first days. Typically it's difficult
to see this nowadays. If it's superficial e.g. in the distal metaphysis of the tibia, it
may take a few days. The progression of osteomyelitis is quite fast also because
the immune system of children is not completely mature so these kinds of infection tend to be more aggressive in kids.
Symptoms include pain, loss of limb function, limp (they cannot walk), swelling (can be very important), tenderness, soft tissue abscess (in case of diffusion
of the infection to the soft tissues), and fever (up to 39-40 C). Typical presentation is within 48 hrs.
The most sensitive monitor for diagnosis and follow-up is C-reactive protein,
while MRI is the most sensitive and specific imaging technique for diagnosis and
differential diagnosis. Differential diagnosis is crucial in children presenting with
typical symptoms; acute hematogenous osteomyelitis' symptoms are in fact very similar to those of sarcoma
(Ewing Sarcoma), that's why it's vital to perform an adequate diagnosis in both cases. Bone scan may also be very
useful when diagnosis is unclear.

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The two following imaging features are characteristic of osteomyelitis and may help you in the DD with
e.g. sarcoma: the sequestrum (ita: sequestro) and the
involucrum (ita: sarcofago). Sequestra are areas of
dead bone with surrounding granulation (see on the
MRI on the left) while the involucrum is the periosteal
new bone formation (appears later wrt sequestra) (in
the MRI the periosteal membrane appears enhanced
and detached by the infection and ossified because of
the activation of cells).

Clinical case: 9 yo female after 7 days of pain; so


quite a delayed diagnosis. Look at the X-ray pictures
and this patchy bone resorption, there is not a clear area of resorption, but it's rather patchy. You can see
here how the growth plate has been interrupted and,
since the diagnosis has been delayed, also the epiphysis has been involved. So these are the consequences
of a delayed diagnosis and that's why it's crucial to do it
ASAP. What could be the consequences of diffusion to
the epiphysis? Possible complications can be osteoarthritis and infection to the joint with very severe consequences, e.g. disruption of the cartilage, necrosis, and
early osteoarthritis. Since this was a young child, this
pathology may affect the normal growth pattern. You may have both necrosis, so a growth arrest, or you may have
a stimulation of cells, so excessive growth compared to the controlateral side. But this is difficult to predict and it
also depends on how soon you treat the osteomyelitis.
Treatment is based on identification of pathogen involved, by performing blood culture and with local aspiration
if possible. Often also surgical debridement is required, especially in case of massive infection. However, while
you're waiting for cultures (typically you wait 5 days) you can establish an empirical therapy with the most common
antibiotics vs common bacteria, as you know that most of these infections in children are due to S. Aureus (most
important), but also gram-bacilli and group B Streptococci in the newborn, while in children >4yo group A streptococci. In adults there is a wide variety of bacteria, but still S.Aureus is the one most commonly involved.
ACUTE POST TRAUMATIC OSTEOMYELITIS is typical of adults. Usually it is secondary to open fractures,
that's why prevention, adequate prophylaxis of open fractures is very important and its usually done with a common antibiotic; open fractures with wide soft tissue damage (e.g. type III of Gustilo Anderson class.) require a multiple antibiotic therapy in order to cover gram-, gram+ and anaerobes. Clinical findings are very similar to those of
the hematogenous, as this is again an acute osteomyelitis. The most common infecting pathogens are S. Aureus
(because it resides on the skin), P. Aeruginosa (quite aggressive, infections can be quite troublesome), and grambacteria. Empirical therapy again should be started immediately while waiting for cultures.
In case of fractures which became infected after implantation of any kind of hardware (plates or nails), you have
to remove the hardware because this may be a site where bacteria attach and in which it is difficult to kill them with
the antibiotic. So we always have to use an external fixator to minimize the implants at the site of infection.
This drawing shows the osteomyelitis with all the bacteria growing across the bone; you perform surgical debridement to clean it up, remove the necrotic bone and use an external fixator as a stabilization device: as you can
see, it does not include any kind of implant through
the infection site. Finally, you properly cover with
soft tissue, so you have to perform a muscle flap if
needed. This should be accompanied by a proper
use of antibiotics, i.e. intravenous antibiotics at high
doses.
CHRONIC OSTEOMYELITIS is typically the result of a poorly treated acute osteomyelitis, usually
post-traumatic. We don't see chronic osteomyelitis
in children, because symptoms are so severe that
they have diagnosis soon and you can establish a
proper and effective therapy in time. In adults this
is different: especially after a trauma, onset of os-

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teomyelitis may be very subtle, not as severe as in children and that's why it may become chronic.
Risk factors include local/mild systemic deficiency, smoke, major nutritional systemic disorders (e.g. diabetes),
diabetic foot (skin ulcers if not treated may involve the bone). Typical patients are old, diabetic or immunosuppressed for any reason (e.g. systemic disease, age...) or IV drug abusers (because they have a continuous source
of bacteria from the skin).
Typical infecting organisms are S.Aureus and P.Aeruginosa, but now you have to be very careful to the
methicillin-resistant S.Aureus (MRSA): this is an emerging clinical problem because it can be treated with only very
few antibiotics, vancomycin for example.
This is the Cyernys Anatomic classification of chronic osteomyelitis, very academic:

Medullary
Superficial
Localized
Diffuse

The natural history is quite typical: periods of quiescence (with no big symptoms) and periods of exacerbation. This is why they seek medical advice quite late,
because they have long periods of quiescence, they may take some NSAIDs during the exacerbation periods, so they can only get worse.
There is no rationale for empirical therapy in chronic osteomyelitis: they are often not given any antibiotic at all for months, so it doesn't make any sense to establish an empirical antibiotic therapy. Therapy is only based on deep cultures; even
blood cultures are not often diagnostic in these patients. You have to go in the infection site, perform a biopsy, do a
debridement of the lesion and ask for cultures.

ARTHRITIS
This is another very important topic which can have terrible consequences if not treated.
Here you can see the typical presentation: a knee
like this should always warn you: here there is something wrong and whatever it is, it must be serious.
CHILDREN: causes are hematogenous spread from
metaphyseal osteomyelitis or from other infection sites,
or it can be a complication of therapeutic/diagnostic
procedures. So, every time you perform an intraarticular procedure (like steroid injections) you have to be
very careful with abscesses. Even arthroscopy, a very
minimally invasive surgical procedure, may have arthritis as a consequence.
An important organism you have to consider is haemophilus influenzae (30% of < 5 yo patients), plus
S.Aureus and type A streptococci. The most common site is the hip; then proximal humerus, distant fibula, radial
neck. Usually it involves one single joint.
Treatment: surgical decompression and drainage: you have to decompress the intraarticular swelling, because
it may result in necrosis. Also, you have to immediately establish an empirical therapy while waiting for definitive
cultures.
Terrible consequences can ensue if not treated properly. Here you can see an intraarticular abscess which can
cause damage to the vascular supply especially at sites like the hip where vascularization is not particularly well
represented: the femoral epiphysis has been completely disrupted because of the intraarticular pressure. In these
cases antibiotic treatment alone is not
enough and surgery to decompress the joint
is mandatory.
In ADULTS arthritis is more commonly a
complication of therapeutic/diagnostic
procedures (iatrogenic), or less commonly
form hematogenous spread. The most
common infecting organisms are S.Aureus,
N. Gonorrhoeae and streptococci.
Treatment is the same: first empirical
therapy plus definitive cultures. It is im-

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portant to perform decompression which can be arthroscopic: basically you do an arthroscopy, wash the joint (you
do arthroscopy under fluid, so you can wash it) you can also leave one tube going into the joint, one tube going
out in order to continuously wash the joint even in the ward for a couple of days and decompress.
What I want you to know is that the sequelae may be terrible. An arthritic joint may develop, rapidly evolving to
osteoarthritis and also muscle contraction complicates the situation. So every time you see a knee like this in an
adult or in a child, it's crucial to start treatment ASAP, even when you
are in the ER: give immediately an antibiotic after performing an arthrocentesis, a synovial fluid aspiration, not to have false negatives. If
you are seeking for the bacteria responsible for the infection you want
to harvest the fluid or the tissue when the patient is not taking any antibiotic, otherwise the bacterial count would be too low.
These are the consequences of an improper or of a proper but late
treatment. Look at the disruption: the patient had to receive prosthesis
of the good hip because of arthritis.

INFECTED ARTHROPLASTY
As I said we are implanting every year more and more arthroplasties. Metallic implants, as in the case of heart
valves, are very prone to infection, and when they get infected, this is really a devastating complication for the
patient. It causes severe disability requiring long term IV antibiotic therapy and a two stage surgical procedure: you
have to first remove the implant (which is infected, it is impossible to clean it from the bacteria with antibiotics), implant a cemented spacer (a kind of prosthesis made of cement capable of releasing locally antibiotics in order to
sterilize the area), and then after months during which the patient cannot walk on his limb (the cement spacer
doesn't work like a prosthesis), you can implant a new prosthesis.
Infection to an arthroplasty can occur at any time after surgery. That's why, even if the risk is higher in close
st
proximity to surgery (1 six weeks), sometimes infections in the mouth, teeth, lungs, urinary tract, or skin, or just
after regular dental procedures that involve gum bleeding can force bacteria in the bloodstream, causing infected
arthroplasty. Therefore you always have to recommend antibiotic prophylaxis to any patient carrying a prosthesis before any (even dental) surgery. Every time we operate a patient, in case we do a medium/demanding
surgery, we always establish a prophylactic therapy, which is very effective in minimizing the risk. However, 1-1.2%
of patients receiving an arthroplasty get infected, despite the prophylaxis. This is a complication you are going to
see if you are attending an orthopedic department. In our department we do maybe 120 prostheses per year, so we
see at least one infection in these patients, whatever we do to prevent it. These are part of the complications which
are not caused by malpractice, but by the procedure itself. What you can do is to recommend to patient to establish
prophylaxis and of course never operate on patients who have an ongoing UTI or dental infection.
The most frequent pathogens are Staphylococcus Epidermidis and S.Aureus,
which are on the skin, group B streptococci and of course MRSA; MRSA infection to
an arthroplasty is a really severe condition because it requires long-term therapy with
multiple antibiotics with frequently lots of complications.
This is the early presentation of an infected total knee (here in a recently operated
patient). The joint is typically swollen, red and warm. The joint is typically flexed in a position as motion is usually very painful, so we have loss of function, and also drainage
may be present.
CRP is typically very elevated before starting the antibiotic therapy and is the best
monitor to assess the effectiveness of your therapy. If your antibiotic is the right one and
it is effective, CRP decreases fast once you have started the treatment.
Q: How do you distinguish if CRP is elevated due to surgery or due to infection?
A: CRP is increased after surgery, but not that much compared to how it can be increased in such cases; so
with such infections CRP is much more increased. Also, it generally decreases in the first days after surgery, so
within the first six weeks you have time to establish the proper diagnosis based on CRP elevation, fever and symptoms. Moreover patients usually have fever after surgery, because surgery is a trauma, patients are reabsorbing
the hematoma which is rich in cytokines and this causes fever. Fever in the first days after surgery is a very unspecific sign, it is not a sign of infection. It has to be correlated with elevated CRP and it has to be prolonged for days
after surgery. The value depends on the unit used by the lab: in our lab, after surgery we may have a value of CRP
of 10/20/50 while with an infection is really much higher like 100.

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Infection of prosthesis causes its mobilization. There are some very specific radiological signs that can help
you in performing diagnosis (image on the right). Look at this tibial plate that has been mobilized. All the bone
around here has been resorbed because infection activates macrophages and osteoclasts which resorb the bone
around the prosthesis; it is very painful because
this is moving, it is not stable anymore. This patient had to be treated with revision implant, bone
grafting...a very long treatment.
Q: What is the resolution rate of these infections?
A: I don't really have statistical values, but in
the normal population it is close to 100%. Chronic
infections are rare. Typically they occur in immunocompromised patients.

TUMORS
Musculoskeletal oncology is a very complex topic which is 100% managed by orthopedic surgeons specialized
in musculoskeletal oncology.
Tumors to the bones are very rare and account for ~2% of all malignant tumors, while metastatic bone diseases are probably the most common form of metastasis. Bone metastases are an emerging clinical problem
which requires proper treatment.
Grading means "how bad is the tumor". Grading is based on histopathological features, so it's only given on the
basis of biopsy, not on imaging. Sarcoma is the general name for mesenchymal tumors.

Grade 1: very well differentiated tumors resembling the original tissue, with very low risk of metastasis
Grade 2
Grade 3: poorly differentiated, very high risk of metastasis.

However, grading of sarcomas is typically very difficult for the pathologist. So, they tend to classify them into
high grade and low grade, i.e. poorly vs highly differentiated.
The TNM classification of tumors is a bit different here. The tumor
size is not calculated in cm, but is instead based on the diffusion of
the tumor through a compartment; the T of sarcomas is defined as
intracompartmental (T1) vs extracompartmental (T2), i.e. whether the primary tumor of the bone extends out of the bone or a soft
tissue sarcoma extends out of the fascia. This is based on imaging
studies not on biopsy like the M, so whether it is metastatic or not.
That's why every time you suspect a malignant tumor, always ask
for a total body CT scan and a bone CT scan.
Enneking's staging system is based on grade (G), tumor site (T)
and metastatic status (M). It identifies three stages, 1, 2 and 3, which correspond to low grade, high grade without
metastasis and high grade with metastasis. Also, stages 1 and 2 are further divided into a and b according to their
site, whether they are compartmental or not. Typically a low grade sarcoma does not determine metastasis at distant sites but it may.
Q: What about the N staging?
A: Lymph node involvement is extremely UNfrequent in sarcomas. Only some subtypes, e.g. synovial sarcomas, give metastasis to lymphnodes.
Localization is crucial and follows typical patterns according
to age of patient and tumor type. You don't have to memorize
this, but it's to give you an idea. Of course you have to memorize
the sites of tumors, but we'll talk about it later; I want you to understand how difficult it may be to perform diagnosis on the basis
of an X-ray when most of them look the same on the X-ray. But if
you know the features, then diagnosis is easier. Here you can
see the localization <30 yo and >30 yo. Metastasis and myeloma
or lymphoma typically can involve the diaphysis, myeloma being
a tumor of adults, while Edwing sarcoma is a tumor of children,

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but still localized in the diaphysis. Epiphysis is typically spared by tumors because of the barrier represented by the
growth plate; however, chondroblastoma, for example, or infections can affect it; the growth plate is also typically
disrupted by malignant tumors, like osteosarcomas. The metaphyseal area can be a site of many tumors. Giant cell
tumor is typically epiphyseal, while cartilage tumors, chondrosarcomas, are metaphyseal.
Benign tumors of bone are also very common but we don't know the epidemiology because, being asymptomatic, their diagnosis is typically incidental. So anyone may have a bone cyst. Lots of patients are diagnosed
when they fall down and they may have fractures for a minimal trauma because the bone was weakened by the
cyst or the enchondroma for example; this is typical of enchondromas of finger. Benign tumors are innocuous, but
have this feature, they cause pathologic fractures.
Typical symptoms of malignant tumors are persistent pain and progressive swelling, so the symptoms typically should drive the patient to seek medical advice immediately because they are quite scaring, but especially
soft tissue sarcomas tend to grow very slowly and especially old patients don't really care about it. They affect
mainly young patients.
Surgical resection is the mainstay of treatment; you always have to remove the tumor. These tumors are
never treated with CT/RT only, you have to perform resection. Neoadjuvant/adjuvant CT and/or RT depending on
the tumor type and grade have to be performed.
Resection can be performed in different fashions:

Intralesional resection: improper treatment for a malignant tumor.


Marginal resection: you only enucleate the tumor. This is not a proper treatment for a malignant tumor while
it is a proper treatment for benign tumors.
Wide resection: with wide margins in healthy tissue around the tumor. It is a proper treatment for some malignant tumors
Compartmental/radical resection: we take out the entire compartment surrounding the tumor. It is a proper
treatment for high grade malignant tumors.
Compartmental resection is very important because you often have skip-metastases: these
are metastases to the same tissue, but distantly from the primary tumor. Skip metastases
may be microscopic and that's why you should
perform a wide resection. Also, these tumors
diffuse through the vessels, not through the
lymphatics, that's why diffusion is typically locally, or to the lungs or to the bone.
Now we start our overview of tumors, starting form conditions which are not tumors.

TUMOR-LIKE CONDITIONS
NON-OSSIFYING FIBROMA: it is not a tumor. This is the typical presentation in the metaphysis. Typically you see a radiolucent
lesion like a cyst surrounded by a sclerotic rim. The sclerotic rim is
always a sign of a benign lesion; malignant tumors do not show
this sclerotic rim.
They often resolve spontaneously; so often no treatment is required when the risk of pathological fracture is low, whereas it is
required if the lesion is big and there's risk of pathologic fracture.
In this case the patient needs no treatment, as it is not that big.
Fibroma typically does not disrupt the cortices of bone, so they make the bone weaker
compared to other kinds of lesion. This is the
typical incidental finding of patients that may
come to the ER: they will be very scared, but you
can reassure them because it is not serious,
nothing really pathologic.
Another tumor-like condition is the SIMPLE
BONE CYST. The difference with fibroma is that,
as you can see here in the MRI (central), cysts
are filled with fluid.
Simple bone cysts are characterized by symmetric expansion with thinning of the cortices. So, pathologic frac-

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tures may occur more frequently in cysts wrt fibromas.


Very common presentation with a fracture, otherwise they are totally asymptomatic. They show metaphyseal
localization (proximal femur, humerus and distant fibula): it may extend to the epiphysis or the diaphysis, but it
starts in the metaphysis.
Treatment is mandatory, since the risk of pathologic fractures is very high. Treatment is curettage (i.e. intralesional resection) and bone grafting (not always performed, only for big cysts). Bone grafting may be autologous
from the iliac crest, or you can use synthetic bone substitutes hydroxyapatite or allografts from multiorgan donors.
Musculoskeletal transplants are the most frequently preformed transplants. We very often use allografts taken
from multiorgan donors for ligament reconstruction, bone grafting procedures. And we have no rejection, since these allografts are decellularized, so there are no allogeneic cells, it is exclusively extra cellular matrix.
ANEURYSMAL BONE CYST (ABC): this is not a tumor, but it is typically really disrupting. Patients are typically
young (<20 yo), localization is typically to the metaphysis or to the spine, where it can be very severe. It has an excentric growth and it can lead to massive
bone disruption. Look here in this proximal humerus of a very young child.
What is really diagnostic and helps in
differentiating this from a simple bone
cyst are fluid level and MRI. Patient here
is lying in supine position and the cyst is
full of blood. There is a lot of blood here
and when the patient is lying down, you
can see the fluid levels at the MRI, which
is typically diagnostic.
Treatment, as for normal cysts, is
curettage and bone grafting (always). It is very important to perform an adequate bone grafting. Normal cysts
may not require bone grafting, because when you curette them, you make the surrounding bone bleed and it regenerates; so, in simple cysts you do bone grafting when they are very big, while in aneurysmal bone cysts is better to do it anyway.
A very important differential diagnosis for this kind of lesions is with a subtype of osteosarcoma, the teleangectatic osteosarcoma. You don't need to know the subtypes of osteosarcoma, which are many, but I want you to
know that ABC is a DD with teleangectatic osteosarcoma. That's really important for your future carrier. That's why
when you see a lesion like this you at least perform a bone scan to check if there are distant localizations and when
you treat these you perform a biopsy. Every time you are dealing with a bone lesion, always perform a biopsy with
histopathology.

BENIGN BONE TUMORS


OSTEOID OSTEOMA: it is a rather strange condition: often you
don't see it, you just see the edema around, but the clinical presentation is characteristic; it is a self-limiting benign intracortical bone lesion producing pain (typically at night and responding to NSAIDs) in
young patients (<30 yo).
Imaging typically shows a small nidus located in the cortices of
bone and surrounded by intense cortical sclerosis and bone edema.
You always have to perform a bone scan and a CT scan because it's
more sensitive for bone lesions. This is a big osteoid osteoma, but
typically it is not big. It is very important that you consider osteoid osteoma when you see a patient with a strange
pain in absence of trauma, responding well to anti-inflammatory drugs and getting worse at night.
CT-guided radiofrequency ablation in the treatment of choice: with a radiofrequency ablator you burn all the
tissue, under CT control. If you did well, the pain usually immediately stops, so you can understand immediately
after surgery if you did it well or not. You can also do an open surgery in case this procedure is not feasible.
GIANT CELL TUMOR: classified as benign, but has some malignant features (borderline tumor): first of all it is
the only benign type which can give metastasis to the lung in 2% patients, which is a feature of malignant tumors.
Also, it can be extremely aggressive. Look at this distal radius: it has been totally disrupted and since it involves the
epiphysis, all the cartilage has been destroyed by the giant cell tumor. This was also called osteoclastoma as probably the cells of origin are osteoclasts. This is a purely destructive lesion and it extends to the subchondral bone

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affecting also the cartilage.


It is uncommon in children and adolescents, it is more
typical of adults 30 - 50 yo. It involves typically epiphysis
and metaphysis of long bones (50% around the knee and
10% distal radius).
Treatment is very difficult and has to be planned correctly. Treatment is curettage (removal of all the tissue
inside which is brownish and soft) in combination with local adjuvants (cement, phenol), to kill all the possible remaining cells. However, depending on the patient, you
may also decide to do a bone grafting or even a massive allograft, i.e. implanting a whole piece of bone from a cadaver or to implant a prosthesis. So treatment is very challenging and depends on the site. I remember a patient
with giant cell tumor to the distal tibia with the involvement of the ankle joint with subchondral bone, so all the cartilage. Even if we were very conservative with the treatment, he developed late osteoarthritis. There was no functional cartilage in the joint anymore.
Q: Is it fast or slowly progressing?
A: This is very difficult to say, because, since it is asymptomatic patients come to you only when pain and disruption come up, so you never know when it started, you only know when it's like this.
ENCHONDROMA: this is a very common cartilaginous bone tumor. They are persisting islands of cartilage in
bone developed through endochondral ossification,
like the tibia. Hyaline cartilage has some degree of
calcification. This is only cartilage not completely calcified and this is a pathological fracture; typical fracture
of the proximal phalanx after a minimal trauma.
They're seen at any age especially because they are
totally asymptomatic, so diagnosis is typically incidental because of pathologic fractures mostly or during routine X-rays maybe for minor trauma.
Treatment is curettage, so you just take it out.
OSTEOCHONDROMA (EXOSTOSIS): it is
another typical cartilaginous tumor. They are
small overgrowth of cartilage at the edge of a
growth plate that develops into a bony protuberance with a cartilaginous cap. This was the
growth plate and because of an anomaly, there
is an overgrowth. They can have a pedicle-like
appearance or the can be sessile, so with a very
wide basis. Of course at this site it may have
some complications, like compression of the
neuromuscular bundle. Here is a patient with
brachial artery pseudoaneurysm because of an osteochondroma. Here the bony protuberance has a cartilaginous
cap: this is calcified cartilage and this grew like a continuation of the growth plate.
There is a small risk of malignant transformation, especially when it's isolated. However, with multiple lesions
the risk of malignant transformation increases up to 6-fold: in those cases you always have to perform strict follow
up to check for malignant transformation and to possibly remove these lesions even if they are not symptomatic.
They may be symptomatic when they compress neurovascular structures or muscles, when they create swelling.
They affect typically teenagers. They grow together with the growth plate, so they stop growing together with
the bone: if the growth continues after the closure of the growth plate, it is a sign of malignancy. So when there is
no growth plate like here, if the osteochondroma continues to grow, that is a sign of malignancy. Treatment is
marginal resection. It is quite common in the distal phalanges.

MALIGNANT BONE TUMORS


CHONDROSARCOMA: this is a malignant tumor of cartilaginous origin. Low grade chondrosarcoma may resemble an enchondroma, that's why you have to perform proper imaging when you see something like this. In general, if you don't have an orthopedic oncology background you always consider them enchondromas; indeed most
of them are just enchondromas but still in some cases, especially when the borders are not well defined like here,
these lesions may be malignant. And that's why you always have to be very careful.

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Chondrosarcoma is the most common primary malignant tumor of the bone. They are typically lowgrade, so not particularly aggressive. It affects the
proximal long bones (proximal humerus, proximal femur) and it's frequently found around the knee (distal
femur, proximal tibia), pelvis and shoulder. Localization
is typically central and metaphyseal. It is painful and it
can present as an enlarging mass, like this one. This is
a high grade osteosarcoma: massive destruction of
normal anatomy, huge mass requiring wide/radical resection.
Surgical treatment of this tumor may be difficult sometimes because they are in close proximity with vascular
structures: you want to remove them properly, but preserving motion. We'll see afterwards that most malignant tumors were once treated with amputation. Of course this is not true anymore; we always try to perform limb-saving
procedures by using allografts and prostheses. Since chondrosarcomas are mainly or typically low grade, they do
not require neoadjuvant or adjuvant chemotherapy, only surgery or radiation therapy, in case you did not have
clean margins. If you did not an adequate surgery, you have to perform a radiation therapy.
OSTEOSARCOMA: it is the second most common primary malignancy of bone. There are many subtypes (that
you don't need to know); teleangectatic was one, the DD with bone cyst.
Differently from chondrosarcoma, osteosarcoma is mainly high-grade; so this is an extremely aggressive condition. It is typical of children, that's why you have to perform an adequate, ASAP diagnosis. Usually localized about
the knee (=distal femur or proximal tibia) (50%) in children with a second
peak in late adulthood.
This (right) is the typical appearance of osteosarcoma: patchy osteolytic lesions, very wide alterations to normal anatomy. It can be barely visible,
but one sign that may help you in the
diagnosis is the Codman triangle: it is
a periosteal reaction to the tumor. It is
typically a sign of malignant bone tumor. Always perform an MRI, since it is
usually diagnostic (above you see that the MRI could detect the edema and bone involvement that was not visible
on x-ray, in same patient). Also adults may be affected but much less frequently.
These tumors are treated with neoadjuvant CT + wide/radical resection and reconstructive procedure +
adjuvant CT, a very aggressive treatment. Chemo is not required in low grade, but low grade osteosarcomas are
rather rare. This is one of the most important successes of polytherapy with 70% of long term survival. All of these
patients used to undergo amputation decades ago. Now 70% of these patients are long term cured with a limb
sparing procedure. Metastases are typically to the lung and to the bone.
EWING SARCOMA: this is a terrible
condition, typically affecting young children >5 yo. Its origin is unknown, we don't
know from which cells it originates. It's
mainly located around the knee, proximal
humerus, femoral diaphysis and symptoms are pain, mass, swelling, fever, elevated erythrocytes sedimentation rate,
anemia, leukocytosis; the differential diagnosis is with osteomyelitis, and since this
is an extremely aggressive malignant tumor, DD has to be performed immediately, because the consequences of a delayed diagnosis both of osteomyelitis
or of Ewing sarcoma may really compromise the success of treatment.
You often see a large disrupting mass. Disruption can be enormous or minimal, but in a patient with these
symptoms always ask for an X-ray and full blood count. Treatment is the same of osteosarcoma, plus radiation. So
treatment is even more aggressive.
Limb sparing surgery: standard of care. One of the most relevant advances in surgery in the last decades,
which has a strong impact on the quality of life. We do not perform primary amputation anymore in bone tumors,

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especially in children (like picture on the right, left image). Nowadays we do use modular prostheses (also taken from cadavers); this is a total femur prosthesis (picture on
the right, right image). Function will not be optimal, but it is better than the loss of a
limb.
Allograft-prosthesis composite is a type of salvage surgery: plastic surgeons
can reproduce the extensor mechanism of the knee, the quadriceps, by using a latissimus dorsi flap. It can be performed both in children and in adults.
I'll show you some examples. This
(left) was a malignant tumor we treated
with a wide resection implanting a modular
reversed prosthesis; after this procedure
the functionality of the limb was not identical to a normal limb, he won't play volleyball, but still perfectly compatible with
normal life.
This is another example of limbsparing surgery, tumor to the proximal tibial metaphysis: what we did was an allograft prosthesis composite. So we implanted a prosthesis but since we had to
resect all the proximal tibia, we cut the tibia in the diaphysis, we put here a
massive allograft from a cadaver and then put the prosthesis on the allograft.
The benefit here of having an allograft was that we had the possibility to attach the patellar tendon to the tendon of the allograft allowing for some function. Without this allograft we would have to attach the patellar tendon to the
prosthesis which is metallic and does not allow for the integration of the tendon. So, no extension is possible without an allograft. Again function here
was very good: patient could walk without crutches after wide resection.
SOFT TISSUE SARCOMA: sarcomas may arise also from soft tissues. This is the typical presentation, a huge
mass growing within thigh. They don't feel it, especially because the patients are usually old, with poor care of
themselves.
Soft tissue sarcomas may arise from fibrous, fatty, neural, muscular, synovial, vascular tissues. Typical presentation is a painful, or even painless, frequently enlarging mass.
By definition, every mass >5cm deep to fascia
has to be considered a sarcomas; even lipomas
which are benign tumors of fat may be larger than 5
cm. They have to be removed properly.
In children the most common is rhabdomyosarcoma, which is an extremely aggressive tumor; otherwise they are typical of adults.
Metastases are to the lungs or lymphatics in 5%
of cases (only in synovial sarcoma and rhabdomyosarcoma); so lymphatic metastases are really uncommon in sarcomas.
Treatment is wide/radical resection in combination with adjuvant therapy depending on the grade. Typically the
very large masses are low grade, because they develop
slowly and they are painless.

METASTASES
They are more common in adults. Metastases typically
come from breast, lung, prostate, kidney, thyroid carcinomas. Common localizations are to the pelvis and to the
spine, ribs and proximal limbs (proximal femur, proximal
humerus). The most important problem with metastases is
the risk of pathologic fractures because they impair quality of life. I don't want to go into the details of metastatic
mechanism of osteolysis; however I want you to know that
osteolysis is not caused by the cancer cells directly. Instead, tumor cells release PTH-related peptide that stimu-

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lates cells towards an osteolytic phenotype; also, osteoclasts are activated and secrete TGF- and IGF1, which further enhance the release of PTH-rp. So this is basically a vicious circle of metastatic bone disease and osteolysis
continues and can only get worse without treatment.

PATHOLOGIC FRACTURES
These are a very important clinical entity for you, since they will be more and more common because cancer is
becoming a chronic disease. Most treatments of cancer result not in a cure, but they make cancer chronic, increasing the risk of bone metastasis.
The best treatment is prevention, so you have to be able to evaluate impending fractures to understand
whether a bone metastasis may determine a fracture with minimal trauma. Typically we perform a prophylactic internal fixation of prosthetic replacement when more than 50% of cortical destruction occurs or 50-75% of metaphyseal destruction. The goal of this treatment is not to cure the patient from cancer, but to maintain independence,
avoid fractures and control pain. Treatment has to be as
definitive as possible.
Commonly we use cement (PMMA) as an adjuvant.
Here (first image of the picture on the right) the metastasis was treated with resection, cement and plate; we want
this humerus to be strong to allow the patient to live normally. Here (second image) the metastasis was in the
femoral neck with disruption of structures, so very high
risk of pathologic fractures with implantation of prosthesis.
Look at this (right) pathologic fracture: there is no
bone here. And look at this skip metastasis, very problematic to treat: we have to perform a resection, implantation of a modular prosthesis with a long stem and cement
in order to allow for immediate weight bearing and avoid
relapse.
This was an improper treatment: you cannot use a plate
without cement for a metastasis, otherwise the metastasis
continues to grow. What they should have done was to
resect the whole piece and implantation of a modular
prosthesis.
I want to show you another important concept that is very
up to date: isolated lesions 2 years after diagnosis of primary
(possibly with a good prognosis) disease have to be treated
radically with wide resection. You have to consider this kind of
metastases like a new primary to be treated radically because
these patients have a very long life expectancy. We made a
massive allograft from a cadaver donor so that we could attach
the glutei here to the greater trochanter and this was synthesized with a plate to the normal bone. These patients live usually 7-8 yrs after such a treatment so a long survival after the
metastasis.
*hip reconstruction video*