Professional Documents
Culture Documents
Pediatric Bone Imaging Differentiating Benign Lesions From Malignant
Pediatric Bone Imaging Differentiating Benign Lesions From Malignant
SA–CME Information
Description and Crystal May, DO, is a Radiologist at UC Davis Medical
Pediatric Bone Imaging: Differentiating Benign Lesions Center, Sacramento, CA. The authors declare no conflicts of
From Malignant interest. This material has not been previously published. A
Bone tumors are one of the most common lesions encoun- few examples were used at a meeting titled “Pediatric Imag-
tered by radiologists. Fortunately, most pediatric bone tumors ing: A comprehensive Review and Innovations,” held March
are benign. Although cross-sectional imaging such as CT or 9-11, 2017 in Scottsdale, AZ.
MRI can be useful, the most important imaging modality in
the initial workup of a bone tumor is the plain radiograph. Target Audience
Differentiating between benign and malignant bone tu- • Radiologists
mors is not always straightforward; however, it is possible • Radiologic Technologists
to distinguish between them by carefully evaluating charac- • Related Imaging Professionals
teristics such as the lesion’s type of margin, pattern of bone
destruction, type of periosteal reaction and presence of an as- System Requirements
sociated soft tissue mass. In addition, matrix type and tumor In order to complete this program, you must have a
location can help narrow the differential diagnosis. computer with a recently updated browser and a printer. For
assistance accessing this course online or printing a certifi-
Learning Objectives cate, email CustomerService@AppliedRadiology.org
After completing this activity, the participant should be
able to: Instructions
• D escribe the common imaging features of This activity is designed to be completed within the
benign and malignant pediatric bone lesions. designated time period. To successfully earn credit,
• E xplain the differences between benign and participants must complete the activity during the valid
malignant pediatric bone lesions. credit period. To receive SA–CME credit, you must:
• N arrow the differential diagnosis of a bone
lesion based on location (epiphysis, metaphysis, 1. Review this article in its entirety.
and diaphysis), and other characteristics. 2. Visit www.appliedradiology.org/SAM2.
3. Login to your account or (new users) create
Accreditation/Designation Statement an account.
The Institute for Advanced Medical Education is accred- 4. Complete the post test and review the discussion
ited by the Accreditation Council for Continuing Medical and references.
Education (ACCME) to provide continuing medical educa- 5. Complete the evaluation.
tion for physicians. 6. Print your certificate.
The Institute for Advanced Medical Education designates
this journal-based CME activity for a maximum of 1 AMA PRA Estimated time for completion: 1 hour
Category 1 Credit™. Physicians should only claim credit com- Date of release and review: July 1, 2018
mensurate with the extent of their participation in the activity. Expiration date: June 30, 2020
These credits qualify as SA-CME credits.
Disclosures
Authors No authors, faculty, or any individuals at IAME or Applied
Alysha Vartevan, DO, and Craig E. Barnes, MD, are Ra- Radiology who had control over the content of this program
diologists at Phoenix Children’s Hospital, Phoenix, AZ; have any relationships with commercial supporters.
July 2018 7
©
B
one lesions are commonly en-
countered in pediatric patients, A B C
with primary bone tumors rep-
resenting the 6th most common neo-
plasm. 1 Fortunately, most pediatric
bone tumors are benign. 2 Although
cross-sectional imaging such as CT
or MRI can be useful, plain film con-
tinues to be the primary modality in
the initial evaluation of osseous ab-
normalities. The most common pe-
diatric benign bone tumors include
nonossifying fibroma (NOF), osteo-
chondroma, Langerhans cell histio-
cytosis (LCH), unicameral bone cyst
(UBC), aneurysmal bone cyst (ABC), FIGURE 1. Bone tumor margins of geographic lesions. (A) sharply sclerotic; (B) sharply lytic;
and osteoid osteoma.3,4 (C) ill-defined.
The most common malignant pediat- Peritumoral edema can be misleading,
Dr. Vartevan and Dr. Barnes are Radiol- ric bone tumors include osteosarcoma as it can be seen in both benign and
ogists at Phoenix Children’s Hospital, and Ewing sarcoma.1 Differentiating malignant lesions. In addition, matrix
Phoenix, AZ; and Dr. May is a Radiol- between benign versus malignant bone type and tumor location can help nar-
ogist at UC Davis Medical Center, Sac-
ramento, CA. The authors declare no tumors is not always straightforward. row the differential diagnosis.
conflicts of interest. This material has not There are, however, radiographic char- The main teaching point of this ar-
been previously published. A few exam- acteristics that can help differentiate ticle is to help the reader understand,
ples were used at a meeting titled “Pedi- between the two, including tumor mar- recognize, describe and differenti-
atric Imaging: A comprehensive Review gin, periosteal reaction, and bone de- ate the characteristics and features
and Innovations,” held March 9-11,
2017 in Scottsdale, AZ. struction. The presence of a soft-tissue of common benign and malignant
mass is uncommon in benign tumors. bone lesions.
8 May
July 2018
©
Periosteal reaction
Periosteal reaction results from corti-
cal insult.7 It is not specific to bone tu-
mors; however, it can help distinguish
benignity versus malignancy (Figure
5). Smooth, uninterrupted periosteal re-
FIGURE 2. Nonossifying fibroma. Coronal FIGURE 3. Aneurysmal bone cyst. Radio- action is seen in non-aggressive, benign
reformatted CT of the proximal tibia shows an graph of the proximal humerus shows a cen- tumors (Figure 6). In contrast, irregu-
eccentrically located, metaphyseal radiolu- trally located, metadiaphyseal radiolucent lar or interrupted periosteal reaction is
cent lesion with sharply sclerotic 1A margins. expansile lesion with sharply lytic 1B margins.
seen in aggressive or malignant tumors.
“Sunburst” or perpendicular periosteal
reaction (Figure 7) and “onion-skin”
or multi-lamellated (Figure 8) perios-
teal reaction are commonly described
in malignant tumors. Codman trian-
gle (Figure 9), where the periosteum is
lifted off the cortex by the tumor with
central interruption, is also associated
with malignancy.7
Bone destruction
The pattern of bone destruction is
related to the growth of a tumor (Fig-
ure 10). Benign tumors, which are
typically slow growing, display geo-
graphic bone destruction. Geographic
bone destruction can be seen in UBCs
(Figure 11), LCHs, enchondromas, and
GCTs.3 Malignant tumors demonstrate
rapid growth and infiltration causing
more aggressive bone destruction.
Moth-eaten (Figure 4) and permeative
FIGURE 4. Ewing sarcoma. Radiograph of the proximal humerus shows a radiolucent lesion (Figures 7, 12) bone destruction are de-
with ill-defined 1C margins (upper box). Aggressive “moth-eaten” bone destruction is also
scribed in malignant bone tumors such
noted (lower box).
as Ewing sarcoma and osteosarcoma,
Lesion characteristics A sharp border indicates a tumor is but can be seen with osteomyelitis.
Margins slow growing, which suggests benig-
Tumor margins are an important nity.3 1A margins are sharply defined Soft-tissue mass
factor in differentiating between a be- with a sclerotic border, which is typ- The presence of a soft-tissue mass
nign versus malignant process (Figure ical of NOF (Figure 2) and UBC. 1B almost always suggests a malignant
1). Sharply demarcated margins (1A margins are sharply defined without a process.5 The rare exceptions in which
and 1B) are described as those that you sclerotic border. 1B margins are also a benign tumor may have soft tissue in-
can easily draw around with a pencil. referred to as sharply lytic, which can volvement include ABC and GCT. A
July 2018 9
©
FIGURE 5. Periosteal reaction. Smooth, uninterrupted appearance represents a FIGURE 6. Osteoid osteoma. Radiograph of the distal
benign process. Perpendicular, codman triangle, and multi-lamellated represent an radius and ulna show smooth, uninterrupted periosteal
aggressive or malignant process. reaction of the ulna.
FIGURE 7. Osteosarcoma. Radiograph FIGURE 8. Ewing sarcoma. Radiograph of FIGURE 9. Ewing sarcoma. Radiograph
of the distal femur shows aggressive, per- the distal tibia and fibula shows mulit-lamel- of the distal femur shows Codman triangle
pendicular, spiculated appearing periosteal lated periosteal reaction of the posterior tibia. periosteal reaction on the lateral aspect.
reaction (white arrow). In addition, the yel-
low arrow shows Codman triangle. Tumor sarcoma, which may aid in differentiat- when trying to decide if a tumor is be-
also shows permeative bone destruction ing between them.6 nign or malignant. Although osteosar-
and osteoid, “cloud-like” matrix coma and Ewing sarcoma demonstrate
soft tissue component is frequently seen Peritumoral edema bone marrow edema, so do benign
with both osteosarcoma and Ewing sar- Peritumoral edema, best evaluated tumors such as osteoid osteoma, chon-
coma, but it is much more pronounced on MRI with fat-suppressed fluid-sensi- droblastoma, and LCH.4 Another draw-
than the osseous involvement in Ewing tive sequences, can be very misleading back of peritumoral edema is that the
10 July 2018
©
FIGURE 10. Bone destruction. Geographic pattern is more often related to benign bone
destruction indicating slow growth. Moth-eaten and permeative patterns represents aggres-
sive bone destruction as can be see with rapidly growing tumors and infection.
increased T2 signal can obscure the true diagnosis. Matrix type depends on the Location
margins of the lesion, necessitating the material produced by the mesenchymal Bone tumors often have a propensity
use of T1 sequences. It is for this rea- cells of the tumor. The most common for certain locations within the bone,
son that plain film should always be in- types of matrix are chondroid and oste- which can help with the differential di-
cluded in a bone tumor work-up rather oid.3 Chondroid matrix calcifications are agnosis (Figure 14). Epiphyseal tumors
than relying on MRI alone.8 described as “ring-and-arcs,” which can are uncommon overall, and only a cou-
be seen with enchondromas (Figure 13) ple tumors tend to occur in this location.
Matrix and chondroblastomas. Osteoid matrix is If the physes are open, the most likely
The internal composition of a bone described as “cloud-like,” which can be diagnosis is chondroblastoma (Fig-
tumor can help narrow the differential seen in osteosarcomas (Figure 7). ure 15). If the physes have closed, the
July 2018 11
©
FIGURE 14. Site of lesion. Long bone distribution of various lesions in the pediatric patient.
leading differential diagnosis is GCT benign bone tumors, and they are most
(Figure 16), which nearly always ex- common in the first two decades. 2,9
tends to the articular margin of a bone.3 They are often asymptomatic and dis-
Differentiating between metaphyseal covered incidentally, unless there is
and diaphyseal origin of a lesion is not an associated pathological fracture. A
always easy. Some tumors begin in the “fallen fragment” sign, which is de-
metaphysis, but end up in the diaphy- scribed as a piece of cortex falling into
sis from skeletal growth. UBCs, ABCs the cystic cavity, is pathognomonic for
and enchondromas can occur in either a UBC.3, 4 Radiographically, a UBC is
the metaphysis or diaphysis. Whereas a well-defined radiolucent lesion with
NOF tends to occur only in the metaph- 1A or 1B margins, centrally located
ysis, and osteosarcoma favors the meta- within the metaphysis and/or diaphy-
diaphysis. Ewing sarcoma typically sis. They are commonly located within
occurs in the diaphysis. the proximal humerus and femur. On
The location of the tumor in relation MRI, UBCs have fluid signal intensity,
to the central axis of the bone can also and typically demonstrate a thin rim of
help narrow the differential diagnosis. enhancement on post contrast images.4
For example, UBC, LCH, and enchon- UBCs are typically uniloculated, but FIGURE 16. Giant cell tumor. Radiograph of
dromas are typically located centrally in may show fluid-fluid levels, which sug- the distal tibia and fibula shows an expan-
the bone. ABCs are typically eccentric, gests prior hemorrhage.4,8 sile, geographic, radiolucent lesion with
but can be central when in a small long sharply lytic 1B margin in the distal tibia
bone such as the fibula. Eccentric le- Aneurysmal bone cyst extending to the articular surface of the bone
in this skeletally mature patient.
sions include NOF, GCT, and osteosar- Aneurysmal bone cysts (ABCs, Fig-
coma. Osteoid osteoma is a cortically ure 3) represent approximately 8% of may exhibit benign periosteal reaction
based lesion. benign bone tumors and are most com- and are commonly located in the me-
mon the first two decades.3,9 They may taphysis. If the periosteum is bulging, a
Benign tumor arise de novo in bone, or may occur soft tissue “mass” can be produced.3 An
Unicameral bone cyst secondary to trauma or in association ABC can be a difficult benign tumor to
UBCs (Figure 11), also known as with another lesion such as a chon- assess because of the similarity to the
simple bone cysts, are tumor-like le- droblastoma or fibrous dysplasia. 3,4 rare telangiectatic osteosarcoma.8 The
sions of unknown origin, thought to Radiographically, an ABC is an eccen- best plain film or CT finding to help dis-
be developmental rather than true neo- tric, multi-cystic, expansile lesion with tinguish benignity is a narrow zone of
plasms.3 UBCs represent up to 8% of well-defined 1A or 1B margins. They transition with well-defined borders. On
12 July 2018
©
A B
July 2018 13
©
FIGURE 20. Chondroblastoma. (A) Sagittal reformatted CT of the proximal tibia in bone win-
dow shows focal, well-defined, lytic lesion with surrounding sclerosis arising in the epiphysis
FIGURE 19. Langerhans cell histiocytosis. extending into the metaphysis without internal calcified matrix. (B) Sagittal T1 fat-suppressed
Plain film of the right iliac bone shows geo- MRI post-gadolinium contrast demonstrates a focal lesion within the epiphysis with scle-
graphic bone destruction with 1B margins rotic margination, dense local enhancement and local reactive change within the epiphysis,
and surround sclerosis, which is more typi- metaphysis, dorsal and peri-cruciate soft tissues, a finding which is suggestive of a more
cal of a slow-growing bone lesion. aggressive lesion in this benign entity.
margin. They are usually asymptomatic lesion with surrounding bone marrow They occur almost exclusively after
and found incidentally, unless there is edema.3 physeal closure. Radiographically,
an associated pathological fracture. On GCTs are eccentrically located radio-
MRI, NOF demonstrates T2 hyperin- Chondroblastoma lucent lesions with well-defined lytic
tensity during the development phase, Chondroblastoma (Figures 15, 20) is 1B margins and geographic bone de-
and progresses to low signal intensity a benign tumor of immature cartilage, struction. In skeletally mature patients,
on T1 and T2 sequences as it matures. which accounts for 3% of all benign GCTs begin in the metaphysics and ex-
Contrast enhancement is not uncom- bone tumors.9 They are most common tend deep to the subchondral bone plate
mon and tends to be greater in immature in the 2nd decade.3 It is characteristi- of the articular surface. On MRI, GCTs
or developing lesions than longstanding cally located in the epiphysis or in an exhibit inhomogeneous increased T2
NOFs.8 apophysis, but may extend into the signal and inhomogeneous enhance-
metaphysis if it develops after physeal ment on postcontrast images.
Langerhans cell histiocytosis closure.8 Radiographically, it is an ec-
Langerhans cell histiocytosis (LCH, centrically located, radiolucent lesion Malignant
Figure 19), also known as eosinophic with a thin well-defined sclerotic 1A Osteosarcoma
granuloma, represents less than 1% of margin and geographic pattern of bone Osteosarcoma (Figure 7) is the most
biopsy-proven primary bone lesions.3 destruction. MRI can be misleading be- common primary malignant bone
They can be seen in the first few months cause of local reaction including peritu- tumor, representing 20% of all primary
of life through the 8th decade, but the moral marrow edema, soft tissue edema malignant bone tumors.3,4 There is
mean age is 5-10 years.3 These are lytic and inflammation (Figure 20), and joint a bimodal distribution with most oc-
lesions that may have either a wide or effusion, which suggests a more aggres- curring in the 1st and 2nd decade and
narrow zone of transition, and may sive lesion, unlike the plain film exam. a second peak after the 7th decade. 3
even demonstrate a more permeative Chondroblastomas have a peripheral The most common subtype is an intra-
pattern of bone destruction.4 There is a thin hypointense rim, which corre- medullary osteoid producing tumor,
propensity for location in the calvarium, sponds to the sclerotic margin seen on which occurs at the metadiaphysis
pelvis, ribs, and long bones such as the plain film or CT.4 of long bones, frequently around the
femur. Within long bones, LCH is typ- knee. 4,6 Radiographically, osteosar-
ically metaphyseal or diaphyseal and Giant cell tumor comas are destructive, eccentrically
may be associated with periosteal reac- GCTs (Figure 17) make up 14-22 % located lesions with poorly defined 1C
tion. MRI appearance is variable, but of all benign bone tumors and are most margins and an associated soft-tissue
the most common presentation is a focal common in the 3 rd to 5 th decades. 3,9 mass. They frequently have osteoid or
14 July 2018
©
the 1st and 2nd decade.3 They can have type of matrix, and patient age can help
7. Rana RS, Wu JS, Eisenberg RL. Periosteal
reaction. AJR Am J Radiol. 2009; 193(4)
a variable radiographic appearance. narrow the differential diagnosis. The : W259-W272.
Ewing sarcoma typically occurs in the most important modality in the initial 8. Dumitriu DI, Menten R, Clapuyt P. Pitfalls in the
diaphysis of the long bones with moth- workup of a bone tumor is the plain ra- diagnosis of common benign bone tumours in chil-
dren. Insights Imaging. 2014; 5(6): 645-655.
eaten or permeative bone destruction diograph. CT and MRI are important to 9. Niu X, Xu H, Inwards CY, et al. Primary bone
and an aggressive periosteal reaction, fully evaluate the extent of malignant tumors. Epidemiologic comparison of 9200
classically known as the “onion-skin” bone tumor invasion for staging and patients treated at Beijing Ji Shui Tan Hospital,
Beijing, China, with 10165 patients at Mayo Clinic,
pattern. It does not produce an osteoid treatment planning. Although bone tu- Rochester, Minnesota. Arch Pathol Lab Med.
matrix, which is a key differentiation mors will be encountered frequently in 2015; 139(9): 1149-1155.
July 2018 15
©