Review Article
Common Tumors and Tumor-like
Donald H. Lee, MD
Jeffrey M. Hills, MD
Martin I. Jordanov, MD
Kenneth A. Jaffe, MD, MBA
From the Department of Orthopaedics
(Dr. Lee and Dr. Hills) and the
Department of Radiology
(Dr. Jordanov), Vanderbilt University
Medical Center, Nashville, TN, and
the OrthoSports Associates
(Dr. Jaffe), Birmingham, AL.
Dr. Lee or an immediate family
member has received royalties from,
is a member of a speakers’ bureau or
has made paid presentations on
behalf of, and serves as a paid
consultant to Zimmer Biomet and
serves as a board member, owner,
officer, or committee member of the
American Academy of Orthopaedic
Surgeons, the American Shoulder and
Elbow Surgeons, the American
Society for Surgery of the Hand, and
the Association of Bone Joint
Surgeons. Dr. Jaffe or an immediate
family member serves as a paid
consultant to Zimmer Biomet and has
stock or stock options held in
Management of Motion. Neither of the
following authors nor any immediate
family member has received anything
of value from or has stock or stock
options held in a commercial company
or institution related directly or
indirectly to the subject of this article:
Dr. Hills and Dr. Jordanov.
J Am Acad Orthop Surg 2018;00:1-10
DOI: 10.5435/JAAOS-D-17-00449
Copyright 2018 by the American
Academy of Orthopaedic Surgeons.
Month 2018, Vol 00, No 00
Copyright Ó the American Academy of Orthopaedic Surgeons. Unauthorized reproduction of this article is prohibited.
Lesions of the Shoulder
Abstract
Shoulder lesions range from tumor-like lesions such as simple bone
cysts to aggressive high-grade sarcomas. The clinical presentation is
often nonspecific with shoulder pain as the primary complaint, which
may lead to a delayed or missed diagnosis. Delayed diagnosis or a
poorly planned biopsy of a malignant shoulder lesion can have a
detrimental effect on the patient’s prognosis and treatment options.
Because the initial patient assessment is crucial for successful
treatment, knowledge of the key features of common shoulder
tumors and tumor-like conditions can help determine the diagnosis
and treatment plan. This article reviews the key features and
treatment options of the more commonly encountered benign and
malignant shoulder bone and soft-tissue tumors and tumor-like
conditions.
I n 2014, the national cancer insti-
tute estimated that approximately
12,000 soft-tissue sarcomas and
Diagnostic Approach,
Staging, and Classification
3,000 bone sarcomas would be
Patients with an unknown shoulder
diagnosed in the United States.1 The
lesion commonly present for one of
shoulder accounts for approximately
four reasons: pain, a lump, an inci-
15% of primary sarcomas and is the
dental lesion noted on radiographs,
third most common site, behind hip-
or pathologic fracture.3 A detailed
pelvis and the knee.2,3 In general,
patient history including the duration
patients with musculoskeletal ma-
and characteristics of pain, presence
lignancy experience up to a 6-month
of systemic signs and symptoms,
delay before an accurate diagnosis is
smoking history, and prior medical
made.4 Misdiagnosis of benign le-
history is obtained, followed by
sions can lead to expensive, morbid,
radiographs of the shoulder and
and unnecessary testing, whereas
humerus. Critical considerations to
delayed or misdiagnosis of malig-
facilitate diagnosis include the pa-
nant and limb-threatening lesions
tient’s age, location of the lesion
may lead to devastating outcomes.5
within the bone or soft tissue (eg,
An appropriate initial evaluation is
metaphysis, epiphysis), and the char-
critical to provide the best outcome
acteristics of the lesion on plain
for the patient.
radiographs.
This article will review a diagnostic
approach of musculoskeletal tumors
and key radiographic and clinical fea- Clinical Features
tures of the more commonly encoun- A history of mild pain easily con-
tered shoulder malignant and benign trolled with over-the-counter medi-
bone and soft-tissue tumors, and cations, no systemic symptoms, or
tumor-like lesions. an incidentally found lesion is more
1
Common Shoulder Tumors
consistent with a benign condition.
Although absence of pain does not
exclude malignancy, malignant tu-
mors are more commonly symptom-
atic with severe pain, and systemic
signs and symptoms. A fracture with
worsening antecedent pain should
raise suspicion of a pathologic pro-
cess. However, pathologic fractures
are seen in benign and malignant le-
sions. In regard to soft-tissue masses,
features favoring soft-tissue sarcoma
over a benign process include a non-
tender mass, firm consistency, deep
or subfascial location, and size larger
than 5 cm. When a superficial mass
can be easily moved about the su-
perficial tissue, the mass has usually
not invaded the deep fascia and is
more likely benign.
Imaging
Imaging of primary bone and soft-
tissue masses should begin with plain
radiographs of the entire involved
bone. The four questions posed by
Enneking should always be asked
when evaluating bone or soft-tissue
lesions: Where is the lesion? What is
the lesion doing to the bone or soft
tissue? How is the normal tissue re-
acting to the lesion? Is there anything
that suggests a certain histogenic type
of lesion?3 When properly analyzed,
most of the primary bone tumors can
be accurately diagnosed based on
age, history, and plain radiographs.
Age and location in the bone (diaph-
ysis, metaphysis, or epiphysis, as well as
centrally versus eccentrically) can assist
in forming a differential diagnosis. In
addition, key radiographic features of
bone lesions that can assist in deter-
mining benign versus malignant lesions,
which will be referenced throughout
this review, are as follows:2,3,6-8
(1) Zone of transition: The margin
refers to the interface between
the lesion and normal bone, and
reflects the lesion’s growth rate.
A distinct radiographic margin
with a sclerotic rim (ie, mature
2 Journal of the American Academy of Orthopaedic Surgeons
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reactive cortical bone) indicates
that the normal bone has had
sufficient time to react to a slow
growing lesion, typically seen
with a benign lesion. In contrast,
a poorly marginated lesion in-
dicates the lesion is growing too
fast for the surrounding bone to
react. A broad zone of transition
is indicative of an aggressive
growth pattern, but is also seen
in infections and eosinophilic
granuloma.
(2) Periosteal reaction:
• A benign periosteal reaction is
due to chronic irritation and re-
sults in well-formed and unin-
terrupted periosteum. When a
benign periosteal reaction is seen
in the setting of a lesion that does
not cause periosteal reaction (eg,
enchondroma), the cause is often
a stress fracture.
• An aggressive periosteal reaction
is due to an aggressive lesion
and results in a periosteum that
is interrupted, disorganized,
multilayered, lamellated (ie,
onion-skinning), or growing per-
pendicular to the bone (sunburst
appearance).
(3) Cortical disruption: Complete
cortical destruction and endosteal
scalloping are more concerning
features for malignancy, but can
also be seen in benign processes.
Endosteal thinning or scalloping
is seen in benign processes, but
rarely cortical breakthrough.
Endosteal thinning refers to the
resorption of the inner margin of
cortical bone and reflects a slow-
growing intramedullary lesion.
Some malignancies allow perme-
ative growth through Haversian
channels and the cortex may
appear normal on plain radio-
graphs, but MRI may show an
aggressive bone lesion accom-
panied by a soft-tissue mass.
(4) Matrix: Identifying a matrix
within a lesion can be extremely
helpful in developing a differen-
tial. When present, a visible tumor
matrix can generally be described
as one of the following:
• Chondroid matrix: described as
popcorn calcifications, rings and
arcs, or punctate; these features
represent mineralization of a
cartilage matrix.
• Osteoid matrix: described as
cloud-like, wispy, opaque, or
sclerotic; these indicate forma-
tion of immature bone.
• Fibroid matrix: described as
ground-glass or hazy opacification.
For primary bone and soft-tissue le-
sions with concerning features, MRI
with contrast of the entire bone should
be obtained for complete character-
ization and preoperative staging.9
Once an intraosseous malignancy
is suspected, staging before biopsy
should be performed and should
include chest CT and a whole-body
bone scan or whole-body Positron
emmission tomography (PET)/CT.
In patients older than 40 years, CT
with contrast of the chest, abdomen,
and pelvis should be obtained for
staging.10 For soft-tissue masses,
ultrasonography can identify venous
malformations which need no addi-
tional workup, but rarely obviates
the need for MRI.7
Laboratory Evaluation
With notable exceptions, initial labo-
ratory data are not of particular assis-
tance in making the initial diagnosis.
Serum alkaline phosphatase is often
elevated in osteosarcoma or Paget dis-
ease and has been shown to be related
to prognosis.11 Patients suspected to
have multiple myeloma should have
serum calcium and serum electro-
phoresis evaluated. Basic laboratory
tests should be obtained, including
complete blood count (CBC), eryth-
rocyte sedimentation rate (ESR), and
C-reactive protein (CRP) tests, to assess
for bleeding or infection, and analysis
of serum lactate dehydrogenase (LDH)
level for its prognostic value.

Biopsy
Biopsy is a critical procedure in the
diagnosis and staging of bone and soft-
tissue tumors. Obtaining an appropriate
biopsy requires a thorough pre-biopsy
evaluation, an appropriate biopsy tech-
nique, and a qualified pathologist to
accurately diagnose the biopsied mate-
rial. A poorly planned or executed
biopsy, or an unindicated biopsy, can
have detrimental effects on the patient’s
outcome. Whenever possible, patients
with suspected malignant neoplasms
should be referred to a center capable
of definitive treatment.12
Staging
In 1980, Enneking et al13 described
a staging system for benign and
malignant lesions, which has been
adopted by the Musculoskeletal Tumor
Society. Benign lesions are staged 1
(latent), 2 (active), or 3 (aggressive),
which are based on radiographic fea-
tures, often requiring serial imaging.
Enneking staging system for malig-
nancies of bone and soft tissue assigns a
tumor grade based on histology (G),
the anatomic site of primary disease
(T), and the presence of metastases (M).
The following is a review of spe-
cific lesions in descending order
of incidence, unless otherwise indi-
cated. A wide variation exists in the
literature in regard to the incidence
of shoulder lesions, and true inci-
dence rates are often difficult to
estimate for benign, asymptomatic
lesions. Unless otherwise stated, the
incidence data are derived from Un-
ni’s series, in combination with
more recent reviews.1-3,14-16
Bone Lesions
Benign Osseous,
Cartilaginous, and Fibrous
Tumors
Osteochondroma
Osteochondromas are the most com-
mon primary benign lesion of the
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Figure 1
Radiograph of osteochondroma of
the proximal humerus demonstrating
the metaphyseal location and bony
outgrowth that is continuous with the
cortical and medullary bone.
shoulder. Typically located in the
proximal humerus, these bone-forming
tumors with a cartilage cap arise as a
growth from the peripheral physis and,
thus, most commonly develop in ado-
lescence with tumor growth ceasing in
adulthood.2,16 Most are asymptomatic
and found incidentally, or as a pal-
pable osseous mass. Radiographically,
they appear as pedunculated bony
outgrowths continuous with the cor-
tical and medullary bone (Figure 1).
The diagnosis is made on plain
radiographs alone, without additional
workup. Approximately 15% of pa-
tients presenting with osteochon-
dromas have multiple hereditary
exostoses, which can be diagnosed by
genetic testing. Patients with multiple
hereditary exostoses should be coun-
seled on the risk of malignant trans-
formation, which was estimated at
2.7% in a 2015 multicenter study.17
Surgical resection is indicated when
lesions are symptomatic and, if pos-
sible, should be delayed until adoles-
cence because of the risk of recurrence.
Chondroma (Enchondroma and
Periosteal Chondroma)
Enchondromas are the second most
common benign tumor, and most
Donald H. Lee, MD, et al
Figure 2
AP radiograph and FS T2-weighted
sagittal MRI demonstrating an
enchondroma in the humeral
metaphysis. The lesion contains
punctate calcifications on the
radiograph and has a typical
lobulated appearance on the MRI.
frequently occur in the proximal
humeral metaphysis.18 Chondromas
are benign tumors of hyaline carti-
lage (referred to as enchondromas
when present within the medullary
canal and as periosteal chondroma
when arising from within or under
the periosteum). Enchondromas are
most commonly asymptomatic and
found incidentally, often during
workup of a separate shoulder pa-
thology (incidentally found in 2.1%
of routine shoulder MRIs in one
study18). On radiographs, charac-
teristics favoring benign enchon-
droma include metaphyseal location,
well-marginated border, central areas
of mineralization or “popcorn calci-
fications,” and lack of periosteal
reaction, endosteal scalloping, corti-
cal breakthrough, or associated soft-
tissue mass (Figure 2). The differential
diagnosis includes chondrosarcoma
and bone infarct/osteonecrosis. The
diffuse calcifications distinguish a
cartilage tumor from a bone infarct,
which features peripheral calcifica-
tions surrounding a lytic lesion.
Differentiating enchondroma from
low-grade chondrosarcoma can
3
Common Shoulder Tumors
Figure 3
AP radiograph of giant cell tumor of
the proximal humerus demonstrating
a metaphyseal location with
extension into the epiphysis and up
to the subchondral bone. Cortical
destruction is noted but no periosteal
reaction.
be challenging and any aggressive
radiographic features, such as end-
osteal destruction, cortical break-
through, or an enlarging lesion,
should prompt further imaging. If
the radiographic features are con-
sistent with a benign lesion, no
additional workup is needed and the
lesion can be monitored with serial
radiographs. The risk of malignant
transformation is low for solitary
enchondromas; however, the risk
is 25% for Ollier disease (multiple
enchondromatosis) and even higher in
Maffucci syndrome (multiple enchon-
dromas and soft-tissue hemangiomas).
Giant Cell Tumors
Giant cell tumor of bone (GCTB)
accounts for an estimated 5% of pri-
mary benign bone tumors; however,
it is present in the proximal humerus
only approximately 10% of the time
(more commonly located around the
knee, distal radius, or sacrum).19 The
tumor is composed of mononucle-
ated cells and osteoclast-like multi-
nucleated giant cells. Pain and swelling
4 Journal of the American Academy of Orthopaedic Surgeons
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Figure 4
Radiograph and FS PD-weighted
oblique coronal MRI of
chondroblastoma of the proximal
humerus demonstrating a well-
defined lytic lesion in the
epiphysis. The MRI demonstrates
a large amount of surrounding
edema that is characteristic of this
lesion.
are the most common symptoms, and
patients typically present between 20
and 40 years of age. On radiographs,
GCTB appears as an aggressive
radiolucent metaphyseal lesion usually
extending into the epiphysis up to the
subchondral bone. Frequently, corti-
cal destruction and extension in to the
soft tissues is seen, but in the absence
of a prior fracture, periosteal reaction
is uncommon (Figure 3). Differen-
tial diagnoses to consider include
chondroblastoma, osteosarcoma, and
brown tumor of hyperparathyroidism
(hypercalcemia and multiple lesions
can also be expected). An MRI should
be obtained to evaluate the soft-tissue
component and extent of marrow
involvement. Despite being considered
a benign lesion, GCTB is known to
metastasize to the lung in up to 7% of
cases, and thus chest radiographs
should be obtained with referral to an
orthopaedic oncologist for biopsy to
confirm diagnosis.20 Extended intra-
lesional curettage with cavitary ad-
juvants (thermal or chemical) has
helped reduce recurrence and is now
the preferred method of treatment.21
Figure 5
Radiograph and CT scan of osteoid
osteoma of the clavicle
demonstrating a radiolucent nidus,
surrounded by exuberant solid
periosteal new bone.
Still, patients should be counseled on
the local recurrence rate of 9%,
regardless of treatment.22
Chondroblastoma
Benign chondroblastoma is a rare
cartilaginous tumor with an esti-
mated incidence one-fifth of that of
giant cell tumor.2 Chondroblastoma
is thought to arise from the epi-
physeal cartilage, and usually pres-
ents in adolescence as an epiphyseal
lesion.23 Because of the epiphyseal
location, patients typically present
with several months of pain and
tenderness, often accompanied with
joint swelling and stiffness. Typical
radiographic appearance is a small,
well-defined, eccentric radiolucent
epiphyseal lesion sometimes with a
sclerotic border (Figure 4). Infre-
quently, the lesion may penetrate
the cortical bone and extend into the
soft tissue, appearing quite aggres-
sive. 24 Calcification of the chon-
droid matrix may occur in up to
50% of chondroblastomas, which
can help in differentiating from
giant cell tumors. Differential diag-
noses include giant cell tumor, oste-
omyelitis, and chondrosarcoma.
Treatment typically consists of
intralesional curettage and grafting,
or radiofrequency ablation for
smaller lesions. Although considered
benign, patients should be counseled
on the rare possibility of pulmonary
metastasis.

Figure 6
Radiograph of nonossifying fibroma
of the proximal humerus
demonstrating an eccentrically
located lesion within the metaphysis,
with a well-defined sclerotic border.
Osteoid Osteoma
Osteoid osteomas account for
approximately 12% of benign bone
tumors; however, they are located in
the proximal humerus or glenoid less
than 10% of the time. Nearly 80% of
patients present between 5 and 25
years of age.2 This benign bone-
forming tumor arises from osteo-
blasts (producing nonmineralized
osteoid and, thus, radiolucent) sur-
rounded by a rim of reactive bone.
Patients classically present with night
pain (attributed to local prostaglan-
din E2 and COX 1/2 expression)
relieved by aspirin. Radiographs
reveal a small (,1.5 cm), radiolucent
nidus (osteoid), with a rim of reactive
bone (Figure 5). Differential diag-
noses include Brodie abscess and
stress fracture, which often require
CT scan to distinguish. Osteoid
osteoma has no malignant potential
and treatment consists of NSAIDs
for symptom control, or radio-
frequency ablation to destroy the
nidus.25 A lesion measuring .2 cm is
referred to as osteoblastoma, and the
pain is typically not relieved with
Month 2018, Vol 00, No 00
Copyright Ó the American Academy of Orthopaedic Surgeons. Unauthorized reproduction of this article is prohibited.
Donald H. Lee, MD, et al
Figure 7
Radiographs of unicameral bone cyst (A) and aneurysmal bone cyst (B) of
proximal humerus. The radiograph on the left demonstrates a centrally located
and mildly expansile lytic lesion abutting the epiphysis with symmetrical thinning
of the cortices and the pathognomonic “fallen leaf” sign, representing a UBC. The
radiograph on the right demonstrates a radiolucent markedly expansile
metadiaphyseal lesion that is expanding beyond the width of the physis,
characteristic of an ABC.
NSAIDs and always requires surgery Nearly all manifest before 20 years of
for pain relief. age. UBC is considered a reactive, non-
neoplastic, fluid-filled lesion because of
Tumor-like Lesions of Bone failure and necrosis of the medullary
bone near the physis during develop-
Nonossifying fibroma (also known as
ment. UBCs are typically asymptom-
metaphyseal fibrous defect, fibrous
atic and only become painful with
cortical defect, or fibroxanthoma) is
fracture or impending fracture. Radio-
rare around the shoulder and contro-
graphs show a centrally located lytic
versy exists as to whether these are
truly neoplastic. This asymptomatic lesion within the metaphysis, abutting
lesion is usually found incidentally and the epiphysis, with symmetrical thin-
more than 80% of patients are less ning of the cortices (Figure 7, A). The
than 20 years of age. On radiographs, “fallen-leaf” sign is pathognomonic
the lesion is typically eccentrically and results when a cortical fragment
located within the metaphyseal cortex breaks off and falls to the floor of the
with a sclerotic border and bulging cavity (and confirms the fluid-filled
of the cortical outline (Figure 6). nature of the cavity). Differential
Asymptomatic lesions can be mon- diagnoses include fibrous dysplasia,
itored and typically reossify in early eosinophilic granuloma, aneurysmal
adulthood. bone cyst (ABC), and others. These
Unicameral bone cyst (UBC) or simple lesions can be observed or treated by
bone cyst is the most common shoulder corticosteroid injection, percutane-
tumor-like lesion and is most commonly ous bone grafting, or curettage in the
located in the proximal humerus. setting of impending fracture.
5
Common Shoulder Tumors
Figure 8
Radiograph of the neuropathic shoulder
demonstrating severe joint destruction
and periarticular soft-tissue swelling.
Aneurysmal Bone Cyst
ABC is less common than UBC and
occurred approximately half as fre-
quently as giant cell tumor in Unni’s
review, with about equal distribution
between the scapula and proximal
humerus.2 Like UBCs, roughly 80%
occur in patients less than 20 years
of age. Recently, it was discovered
that ABCs are due to a translocation
and, thus, are neoplastic in nature.26
Secondary ABCs can develop in
association with another lesion and
are not attributable to a transloca-
tion. Patients may present with pain
and swelling about the shoulder but
rarely with a pathologic fracture.
Radiographs show an eccentrically
located expansive radiolucent lesion
in the metaphysis (Figure 7, B). ABCs
may expand beyond the width of the
physis, whereas UBCs typically do
not. Differentiating between UBCs
and ABCs is necessary because treat-
ment is different. After biopsy-proven
diagnosis, treatment typically involves
curettage and bone grafting.
Eosinophilic Granuloma
(Unifocal Langerhans Cell
Histiocytosis)
Eosinophilic granuloma is one of four
forms of Langerhans cell histiocytosis
(histiocytosis X) and is rare. Patients
6 Journal of the American Academy of Orthopaedic Surgeons
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Figure 9
Radiograph of metastatic prostate
cancer to the proximal humerus
demonstrating the osteoblastic
nature of prostate cancer.
may present with focal pain that is
worse at night, with or without a pal-
pable mass. Radiographs typically
show a poorly defined lytic lesion with
malignant-appearing periosteal reac-
tion. Cortical destruction may be pre-
sent, especially if seen in the clavicle.
Because of the aggressive radiographic
features, eosinophilic granuloma has
been called the “great mimicker” and
diagnoses such as Ewing sarcoma or
osteomyelitis should be ruled out by
biopsy.
Neuropathic Shoulder (Charcot
Arthropathy)
Neuropathic arthropathy of the
shoulder is most commonly due to
cervical syringomyelia.27 A neuro-
pathic joint is due to loss of joint
innervation followed by repeated
trauma, inflammation, and osteoclas-
tic bone resorption. Patients may pre-
sent with a painless, swollen shoulder,
mimicking a sarcoma, with loss of
pain and temperature sensation in a
cape-like distribution when associated
with a syrinx.28 Radiographs may
show severe joint destruction, peri-
articular soft-tissue calcifications, and
glenoid sclerosis (Figure 8). Neuro-
pathic shoulder is often considered
a diagnosis of exclusion and, thus,
Figure 10
AP radiograph of plasmacytoma of
the proximal humerus showing an
aggressive lytic lesion with a
nondisplaced pathologic fracture.
infection and neoplasm should be
ruled out. Evaluation should include
measurement of hemoglobin A1c,
ESR, and CRP levels (which will
typically be normal in neuropathic
shoulder), rapid plasma regain test,
rheumatoid factor test, antinuclear
antibody testing, and a cervical MRI.
Malignant Bone Lesions
Metastatic Carcinoma
Metastatic adenocarcinoma is the
most common musculoskeletal tumor
and most commonly occurs in the
kidneys, lungs, breasts, or prostate.3
Approximately 20% of patients with
metastatic bone disease will have
disease present in the upper extremity
and most commonly in the humerus.
Most of the patients with metastatic
disease will present with a known
primary tumor. Patients are typically
older than 40 years and may present
with deep pain. Metastatic carcinoma
has an extremely variable radio-
graphic appearance. Most are de-
structive and radiolucent; however,
breast and prostate carcinomas are
known to cause osteoblastic lesions
(Figure 9). If the primary tumor is
unknown, the patient should undergo

Figure 11
AP radiograph and coronal FS T2-
weighted MRI of osteosarcoma of
the proximal humerus showing an
aggressive mixed lytic and sclerotic
lesion in the proximal humerus.
Aggressive periosteal new bone
formation (Codman triangles) is
evident. The MRI better
demonstrates the local extent of the
neoplastic process.
biopsy and staging. Operative versus
nonoperative (immobilization and
radiation) management will depend
on shoulder stability, amount of bone
loss, and life expectancy of the
patient. When surgery is indicated,
the amount of humeral head bone
loss and status of the tuberosities will
guide surgical planning. Surgical op-
tions typically consist of curettage
with cement and plating, cemented
hemiarthroplasty, proximal humeral
endoprosthetic reconstruction, or in-
tramedullary fixation.
Multiple Myeloma
Multiple myeloma is the most com-
mon primary malignancy of bone,
with an incidence approximately
Month 2018, Vol 00, No 00
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Figure 12
AP radiograph and FS PD-weighted
sagittal MRI of chondrosarcoma of the
proximal humerus. The radiograph
demonstrates an aggressive osteolytic
lesion in the proximal humerus
containing punctate calcifications with
medial cortical destruction and
extension into the soft tissues. The
MRI shows a large mass arising from
the humerus and extending into the
soft tissues.
double that of osteosarcoma. Multi-
ple myeloma is a neoplasm of plasma
cells and appears on radiographs as
small (20 mm) focal, lytic lesions
with a narrow zone of transition,
lacking a sclerotic rim29 (Figure 10).
Patients are typically aged older than
55 years and if multiple myeloma is
suspected, a complete workup should
be performed and diagnosis con-
firmed via bone marrow aspirate.
Treatment of impending pathologic
fractures is either surgical stabiliza-
tion or irradiation, or both.
Osteosarcoma
Osteosarcoma, a malignant neoplasm
of osteoid-producing mesenchymal
cells, is the most common primary
sarcoma in the shoulder. Patients most
commonly present in the twenties or
thirties with a 3- to 6-month history of
night pain and swelling unrelated to
activity. Radiographically, osteosar-
coma usually appears as an osteo-
Donald H. Lee, MD, et al
Figure 13
AP radiograph and coronal FS T-2
weighted MRI of Ewing sarcoma of
the distal clavicle. The radiograph
shows an aggressive osteolytic
process in the lateral clavicle with an
adjacent soft-tissue mass. The MRI
shows the soft-tissue component of
the tumor better.
blastic lesion with poorly defined
margins and with characteristic fea-
tures such as sunburst appearance
(due to the tumor growing too rapid
for the periosteum to respond and
subsequent calcification of Sharpey
fibers) and Codman triangle (due to
subperiosteal bone formation raising
the periosteum away from normal
bone) (Figure 11). Patients suspected
of having osteosarcoma should have
plain radiographs and MRI of the
entire involved bone, chest CT, whole-
body bone scan, and referral to a
treatment center. Treatment consists
of multiagent chemotherapy and sur-
gery (limb salvage versus amputation).
Chondrosarcoma
Chondrosarcoma, a malignancy of
neoplastic chondrocytes which can
arise primarily or secondarily from a
preexisting chondroma, is the second
most common primary malignancy
around the shoulder and the most
common of the coracoid process.
Patients typically present between
their forties and sixties with deep pain
unrelated to activity. Because this
disease exists on a broad spectrum in
regard to aggressiveness, accurate
7
Common Shoulder Tumors
Figure 14
Axial T1-weighted MRI of deep
lipoma of the arm showing a fatty
tumor in the lateral head of the triceps
muscle. A few very thin septae and
blood vessels course through the
tumor. No thick septations or swirling
soft tissue is present.
plain radiograph interpretation is
critical for diagnosis and manage-
ment. Plain radiographs of a low-
grade chondrosarcoma will appear
similar to those of an enchondroma,
but with more aggressive endos-
teal cortical erosions and deeper
endosteal scalloping (Figure 12). Any
extra-osseous extension indicates
malignancy. Any aggressive features
on plain radiographs should prompt
an MRI in addition to CT scan,
which yields the finest bony detail.
Because of the resistant nature to
chemotherapy and radiation, treat-
ment relies on surgery alone, and the
extent of surgery depends on the
grade of the tumor.
Ewing Sarcoma
Ewing sarcoma, a small round cell
sarcoma due to 11:22 translocation,
is the third most common primary
malignancy around the shoulder. The
vast majority of patients are within
their twenties and typically present with
pain and swelling and nonspecific
systemic signs. Radiographs show
aggressive-appearing poorly mar-
ginated lytic lesions most often in
the metadiaphyseal region with a
8 Journal of the American Academy of Orthopaedic Surgeons
Copyright Ó the American Academy of Orthopaedic Surgeons. Unauthorized reproduction of this article is prohibited.
Figure 15
Axial FS T1-weighted MRI of
fibromatosis (desmoid) after IV
gadolinium administration showing
an avidly enhancing mass in the left
axilla with multiple dark bands
coursing through the mass.
laminated (“onion-skin”) periosteal
reaction, and frequently an associ-
ated large soft-tissue mass (Figure 13).
When suspected, staging studies and an
MRI of the affected bone are required.
Treatment consists of systemic che-
motherapy and surgical resection, with
or without adjuvant radiation.
Lymphoma
In the shoulder, lymphoma is slightly
less common than Ewing sarcoma.
Lymphoma includes many subtypes
and, thus, has a varied clinical and
radiographic presentation. Patients
are typically older than 50 years and
present with systemic signs such as
fevers, chills, weight loss, and lymph-
adenopathy. Patients may present
with bone pain worsened with activity
or pathologic fracture, but rarely is
shoulder pain the presenting symp-
tom. Radiographic appearance can
vary, but typically large, poorly mar-
ginated lytic lesions (with or without
mixed sclerosis), usually in the diaph-
ysis, can be observed. In contrast
to Ewing sarcoma, periosteal bone
formation is uncommon. Patients
respond well to steroids and chemo-
therapy and rarely require surgical
intervention, even in the setting of
pathologic fracture.
Figure 16
Axial T1-weighted image of
liposarcoma showing a very large
fatty tumor containing multiple very
thick septae, some of which
demonstrate nodularity and
enhancement (not shown here). This
tumor is a complex fatty mass, a well-
differentiated liposarcoma.
Soft-tissue Tumors
Benign Soft-tissue Tumors
Lipoma
Lipoma, a benign tumor of mature
adipocytes, is the most common
overall soft-tissue tumor around the
shoulder, and typically presents in
middle-aged adults.14 Superficial
lipomas are more common and pre-
sent as a slowly enlarging painless,
mobile, small mass (,5 cm), just
beneath the skin. Deep lipomas are
typically found incidentally. MRI is
diagnostic and will show the tumor
as a homogeneous mass with signal
isointense to subcutaneous fat on all
sequences (Figure 14). Marginal
resection is indicated when the lesion
is symptomatic, rapidly enlarging, or
located deep to fascia and when its
benign nature is in question.
Fibromatosis
Fibromatosis refers to a broad group
of well-differentiated fibroblastic ne-
oplasms that lack metastatic potential
(and thus considered benign), but is

the most locally invasive soft-tissue
tumor and tends to recur. The two
major groups include superficial (fas-
cial) and deep fibromatosis (extra-
abdominal desmoid). The shoulder is
the most common location of extra-
abdominal desmoid tumors, which
typically present in patients less
than 40 years of age as firm, painful,
deep lesions, often leading to re-
stricted shoulder motion.30 MRI is
the imaging modality of choice and
biopsy is required for diagnosis
(Figure 15). Traditional treatment
of symptomatic lesions consisted of
margin-negative resection; however,
because of the high recurrence rate
despite negative margins, treat-
ment has moved away from surgery
and toward the use of systemic
therapies.
Soft-tissue Sarcomas
In the largest study available, the most
common soft-tissue sarcomas around
the shoulder at age older than 26 years
are dermatofibrosarcoma protuber-
ans, malignant fibrous histiocytoma,
and liposarcoma.15 Four key clinical
findings that should raise suspicion
for a soft-tissue sarcoma are as fol-
lows: firm consistency on examina-
tion, deep to superficial muscular
fascia, size larger than 5 cm, and
typically nontenderness (Figure 16).
When suspected, plain radiographs
and an MRI should be obtained.
Staging should be performed and
referral to a treating surgeon for
biopsy is critical to avoid a con-
taminated marginal resection.
Other Tumor Simulators of
the Bone, Joint, and Soft
Tissue Around the
Shoulder
Acute and chronic infection can have
a broad presentation and should al-
ways be considered in the differential
diagnosis when a tumor is suspected.
Plain radiographs can have aggressive
Month 2018, Vol 00, No 00
Copyright Ó the American Academy of Orthopaedic Surgeons. Unauthorized reproduction of this article is prohibited.
features and, in cases of chronic infec-
tion, inflammatory markers may not
always be elevated. A gadolinium-
enhanced MRI can help differentiate
infection from tumor, but open biopsy
and débridement may be necessary.
Synovial chondromatosis is a benign
metaplastic proliferation of the syno-
vium which results in loose bodies
within the joint, or an extra-articular
disease that can mimic a soft-tissue
sarcoma.31
Myositis ossificans is a reactive
process characterized by hetero-
topic ossification that typically oc-
curs in young people after trauma.
The appearance of the lesion on
a radiograph, CT, and MRI can
mimic a malignant lesion and a
history of trauma may not always
be present, which has contributed
to misdiagnoses.32
Summary
The diagnosis and management of
shoulder tumors and tumor-like le-
sions can be challenging. With benign
lesions, accurate interpretation of plain
radiographs can often save a patient
from costly and morbid testing. When
concerning features are noted on
physical examination and radio-
graphs, advanced imaging should
be obtained. Whenever possible,
patients with suspected malignant
neoplasms should be referred to a
treatment center for biopsy and defin-
itive management.
References
Evidence-based Medicine: Levels of
evidence are described in the table of
contents. In this article, references 12
and 20 are level II studies. References
11 and 19 are level III studies. Ref-
erences 4, 17, and 22-25 are level IV
studies.
References printed in bold type are
those published within the past 5 years.
Donald H. Lee, MD, et al
1. National Cancer Institute, A Snapshot of
Sarcoma. 2017. https://www.cancer.gov/
research/progress/snapshots/
sarcoma#footnote1.
2. Unni KK, Dahlin DC: Dahlin’s Bone
Tumors: General Aspects and Data on
11,087 Cases, ed 5. Philadelphia, PA,
Lippincott-Raven, 1996, vol. 1, pp 1-58.
3. Enneking WF: Musculoskeletal Tumor
Surgery. New York, NY, Churchill
Livingstone, 1983, vol. 2, pp 1-87.
4. Brouns F, Stas M, De Wever I: Delay in
diagnosis of soft tissue sarcomas. Eur J Surg
Oncol 2003;29:440-445.
5. Cummings JE, Seidel MJ: Clinical
Presentation and Staging of Bone Tumors,
in Orthopaedic Knowledge Update:
Musculoskeletal Tumors. Rosemont, IL,
American Academy of Orthopaedic
Surgeons, 2014, pp 3-12.
6. Deyrup AT, Siegal GP: Practical
Orthopedic Pathology: A Diagnostic
Approach. Philadelphia, PA, Elsevier, 2016,
pp 23-34.
7. Ecklund K: Imaging of musculoskeletal
tumors: Updates and current practice,
in Biermann JS, ed: Orthopaedic
Knowledge Update: Musculoskeletal
Tumors. Rosemont, IL, American
Academy of Orthopaedic Surgeons,
2014, pp 13-22.
8. Weisstein JS, Conrad EU: The shoulder, in
Rockwood CA, ed: The Shoulder, ed 4.
Philadelphia, PA, Saunders/Elsevier, 2009,
vol. 2, pp 1509-1535.
9. Ritchie DA, Davies AM: MR imaging of
tumors and tumor-like lesions of the
shoulder girdle. Magn Reson Imaging
Clin N Am 2004;12:125-141.
10. Biermann JS, Adkins DR, Agulnik M, et al:
Bone cancer. J Natl Compr Canc Netw
2013;11:688-723.
11. Levine AM, Rosenberg SA: Alkaline
phosphatase levels in osteosarcoma tissue
are related to prognosis. Cancer 1979;44:
2291-2293.
12. Mankin HJ, Mankin CJ, Simon MA:
The hazards of the biopsy, revisited:
Members of the Musculoskeletal Tumor
Society. J Bone Joint Surg Am 1996;78:
656-663.
13. Enneking WF, Spanier SS, Goodman MA:
A system for the surgical staging of
musculoskeletal sarcoma. Clin Orthop
Relat Res 1980:106-120.
14. Kransdorf MJ: Benign soft-tissue tumors
in a large referral population: Distribution
of specific diagnoses by age, sex, and
location. AJR Am J Roentgenol 1995;164:
395-402.
15. Kransdorf MJ: Malignant soft-tissue
tumors in a large referral population:
Distribution of diagnoses by age, sex, and
9
Common Shoulder Tumors
location. AJR Am J Roentgenol 1995;164:
129-134.
16. Link TM, Brinkschmidt C, Lindner N,
Wortler K, Heindel W: Primary bone
tumors and “tumor-like lesions” of the
shoulder: Their histopathology and
imaging [German]. Rofo 1999;170:
507-513.
17. Czajka CM, DiCaprio MR: What is the
proportion of patients with multiple
hereditary exostoses who undergo
malignant degeneration? Clin Orthop Relat
Res 2015;473:2355-2361.
18. Hong ED, Carrino JA, Weber KL, Fayad LM:
Prevalence of shoulder enchondromas on
routine MR imaging. Clin Imaging 2011;
35:378-384.
19. Niu X, Zhang Q, Hao L, et al: Giant cell
tumor of the extremity: Retrospective analysis
of 621 Chinese patients from one institution. J
Bone Joint Surg Am 2012;94:461-467.
20. Chan CM, Adler Z, Reith JD, Gibbs CP Jr:
Risk factors for pulmonary metastases from
giant cell tumor of bone. J Bone Joint Surg
Am 2015;97:420-428.
10
Copyright Ó the American Academy of Orthopaedic Surgeons. Unauthorized reproduction of this article is prohibited.
21. Cheong D, Letson D: Giant cell tumor of
bone, in Orthopaedic Knowledge Update:
Musculoskeletal Tumors. Rosemont, IL,
American Academy of Orthopaedic
Surgeons, 2014, pp 133-146.
22. Kremen TJ Jr, Bernthal NM, Eckardt MA,
Eckardt JJ: Giant cell tumor of bone: Are
we stratifying results appropriately? Clin
Orthop Relat Res 2012;470:677-683.
23. Ramappa AJ, Lee FY, Tang P, Carlson JR,
Gebhardt MC, Mankin HJ:
Chondroblastoma of bone. J Bone Joint
Surg Am 2000;82-A:1140-1145.
24. Dahlin DC, Ivins JC: Benign
chondroblastoma: A study of 125 cases.
Cancer 1972;30:401-413.
25. Soong M, Jupiter J, Rosenthal D:
Radiofrequency ablation of osteoid
osteoma in the upper extremity. J Hand
Surg Am 2006;31:279-283.
26. Ye Y, Pringle LM, Lau AW, et al:
TRE17/USP6 oncogene translocated in
aneurysmal bone cyst induces matrix
metalloproteinase production via activation of
NF-kappaB. Oncogene 2010;29:3619-3629.
Journal of the American Academy of Orthopaedic Surgeons
27. Snoddy MC, Lee DH, Kuhn JE:
Charcot shoulder and elbow: A review
of the literature and update on treatment.
J Shoulder Elbow Surg 2017;26:
544-552.
28. Su J, Al-Delfi F, Mills G, Peddi P: Charcot’s
osteoarthropathy mimicking an
osteosarcoma of humerus. BMJ Case Rep
2016;2016.
29. Scharschmidt TJ, Lindsey JD, Becker PS,
Conrad EU: Multiple myeloma: Diagnosis
and orthopaedic implications. J Am Acad
Orthop Surg 2011;19:410-419.
30. Goldblum JR, Folpe AL, Weiss SW:
Enzinger & Weiss’s Soft Tissue Tumors.
ed Sixth Edition. Philadelphia, PA:
Saunders, 2014, pp 188-244.
31. El Rassi G, Matta J, Hijjawi A, Khair OA,
Fahs S: Extra-articular synovial
chondromatosis eroding and penetrating
the acromion. Arthrosc Tech 2015;4:
e443-e448.
32. Yamaga K, Kobayashi E, Kubota D, et al:
Pediatric myositis ossificans mimicking
osteosarcoma. Pediatr Int 2015;57:996-999.