Pediatric Bone Tumors

Timothy G. Sanders, MD
 Fibrous Dysplasia
• Hamartomatous fibro-osseous metaplasia
• Age: 10-70; common in 2nd - 3rd decade
• Common locations:
– femur, tibia, pelvis, ribs, skull
• 70% monostotic
 Fibrous Dysplasia
• Radiography:
– Variable: fibrous matrix- ground glass
osteoid matrix- sclerotic
– Zone of transition narrow (sclerotic rim)
-Sclerotic lesion in femur
secondary to presence of
increased woven bone
-Pathologic fracture
 Polyostotic Fibrous Dysplasia
• 90% unilateral
• 3% Albright’s Syndrome
– precocious puberty, café-au-lait spots,
polyostotic bone lesions
• Femur- Sheppard’s crook varus deformity
• Cherubism- expanding facial bones, sinuses
• Pregnancy- lesions may enlarge
 Fibrous dysplasia
• Monostotic: UBC
• Polyostotic: Ollier’s disease, mets
• Rib lesions: EG, Ewing’s, mets
• Skull base: meningioma- skull base
hyperostosis, Paget’s
-Differential Dx: UBC- FD may appear very
cystic on MR
-UBC cold on bone scan; FD- warm
 Osteofibrous Dysplasia
• Occurs only in the tibia and fibula
• Infants and children
• X-ray: mixed lucent/sclerotic, thickened
cortex (anterolateral), anterior bowing,
synchronous tibia/fibula
• DDX: adamantinoma (epithelial nature)
– slightly older age >20
 Chondroblastoma
• Epiphyseal lesion
• Age: <30 (growth plate usually open)
• X-ray: epiphysis, lytic destructive lesion,
centrally located, usually extends into
metaphysis,
• DDX: CIGG – chondroblastoma, infection,
GCT, intraosseous ganglion,
• DDX: EG, ABC, mets, myeloma
 T1

T1 GAD

T2
 Soft Tissue Hemangioma
• PF: S.T. mass with vascular calcifications
• MRI: T1:
– Lacy high signal within lesion represents fat
within lesion
– T2 and T1 post Gad: tortuous vascular channels
possible phleboliths
– Most common S.T. mass of childhood;
intramuscular; size may vary throughout the day
 Ewing’s Sarcoma
• Primary small cell bone neoplasm
• Derived from reticulocyte- occurs in areas
of red marrow; long bones, axial skeleton
• Diaphyseal- older; Metaphyseal younger
• Age: 1st –2nd decade; 96% Caucasian
 Ewing’s Sarcoma
• Radiography
– Highly aggressive tumor; permeative pattern
– Medullary tumor; infiltrative; periosteal
reaction- spiculated, lamellated- many layers
present
– No mineralized matrix
– S.T. mass common
– DDX: EG, lymphoma, OS, infection
 Leukemia
• Typically metastatic to bone; common tumor
in childhood
• In childhood- acute leukemia,
– lymphocytic or undifferentiated
• 50% of children have osseous involvement at
time of DX, and 70-80% during disease
 Leukemia
• Childhood radiographic changes
– Radiolucent metaphyseal bands- growth arrest
disturbance; not leukemic infiltrate- involve
areas of rapid growth
– Diffuse osteopenia- often the only finding
– Periosteal reaction- lamellated
– Osteolytic lesions- permeative/ moth eaten
– Sclerotic lesions
 Nonossifying fibroma
• One of the most common bone tumors
• Almost always in long bones (femur, tibia)
• Age: Usually > 2 y.o. and < 20 y.o.
• Histo: fibroblasts and giant cells
• Usually regress with age; rarely seen in 30’s
• Usually an incidental finding
 Nonossifying Fibroma
• Radiography
– Unilateral or bilateral
– Osteolytic lesion; well defined; sclerotic margin;
oval, scalloped, lobulated, eccentric,
– Metadiaphyseal, cortically based,
– Periosteal reaction absent- unless path fracture
– Femur: posteromedial; healing begins in
diaphyseal region
 Nonossifying Fibroma
• Jaffe-Campanacci Syndrome
– Multiple NOFs
– Café-au-lait spots
– Mental retardation
• precocious puberty, hypogonadism, cryptorchidism,
ocular anomalies, cardiovascular anamolies
T1

T2

T1 GAD
 Aneurysmal Bone Cyst
• Uncommon: long bones, spine, hands, feet,
pelvis, shoulder
• Age: 5- 20 y.o.
• Usually metaphyseal
• Highly vascular expansile lesion; multiple
cavities- filled with blood
• Primary or secondary lesion; solid component
 Aneurysmal Bone Cyst
• Radiography
– Expansile, geographic lytic lesion; eccentric
– Usually metaphyseal
– Margin- sharp, nonsclerotic; outer margin
thinned, may be imperceptible radiographically
– MRI/ CT fluid-fluid levels
– DDX: UBC, NOF, FD, Osteoblastoma- spine,
Telangiectatic osteosarcoma
 Unicameral Bone Cyst
• Long bones- (prox humerus/ femur)
metaphyseal- move toward diaphysis with
bone growth
• Age: 1st two decades of life
• Bone cyst- serosanguineous fluid
• Bony septations
• Pathologic fractures 2/3’s of cases
 Unicameral Bone Cyst
• Radiography
– Well-defined lytic lesion- central in medullary
space; bone expansion, sharp margin
– Periosteal reaction only in presence of path
fracture- “fallen fragment sign”
– Radiolucent with trabeculation
– DDX: FD, ABC, NOF
 Eosinophilic Granuloma
• Nonneoplastic lesion of bone
(reticulohistiocytosis)
• Usually solitary- EG
– Multiple- Hand-Schuller-Christian
– Systemic- Letterer-Siwe
– Long bones, skull, spine, ribs, pelvis
– Age: 1st two decades (5 – 10 y.o.)
 Eosinophilic Granuloma
• Radiography
– Variable: osteolytic oval, slow growing lesion
occasionally aggressive appearing
– Medullary space; centrally located
– Periosteal reaction – 10% of cases
– Nucs scan – negative 1/3 of cases
 Giant Cell Tumor
• Metaphyseal- epiphyseal lesion; long bones
• Age: 20 - 50 y.o. (growth plate closed) rare
under age 15 y.o.
• Progressive potentially malignant lesion
• Histo: Giant cells and mononuclear spindle
shaped cells
 Giant Cell Tumor
• Radiography
– Lytic lesion; arises in metaphysis and extends
into epiphysis; growth plate closed
– Well defined, nonsclerotic margin, abuts
articular surface, eccentric, usually large
(> 4-6cm)
– Radiographic appearance does not help in
determining if benign or malignant
 Osteochondroma
• Exostosis; bony projection with a cap of hyaline
cartilage; aberrant cartilage
• Metaphyseal, points away from joint; long bones,
around knee
• Age: 2nd decade; growth stops when growth plate
closes
• Single or multiple; sessile, pedunculated
• Malignant potential; increase with multiple lesions
 Osteochondroma
• Radiography
– Bony projection; contiguous medullary space
and cortex; overlying cartilage cap
– Pedunculated/ sessile
– Pain- malignant transformation/ mechanical
problems, bursa formation
– Cartilage cap; normally < 1-2 cm
 Osteochondroma
• DDX:
– Single lesion: Parosteal osteosarcoma (no
continuity of medullary space and cortex)
– Multiple: Enchondromatosis; FD
– Malignant Transformation: Chondrosarcoma;
• Pain; Growth after growth plate closes
• Cartilaginous cap > 2cm thick
T1 T1 GAD

T2
 Osteoid Osteoma
• Similar to osteoblastoma; < 2 cm in size
• Femur/ tibia/ bones of the hands/ feet/ spine
• Usually cortical;Fibrovascular nidus (< 1cm);
adjacent reactive bony sclerosis
• Night pain relieved by aspirin
• Age: < 25 y.o.
 Osteoid Osteoma
• Radiography
– Round/ oval radiolucency (nidus <1-2cm)
– Cortically located
– Thick adjacent bony sclerosis
• Nucs: “Target” sign; MR: Nidus- bright on
T2 with marked enhancement with Gad;
CT used to identify the nidus
• DDX: stress fracture, osteomyelitis
 Osteogenic Sarcoma
• Malignant bone tumor that forms osteoid
tissue
• Most common primary malignant bone tumor
other than multiple myeloma
• Involves areas of active growth; Metaphysis;
femur, humerus, tibia, pelvis
• Age: 2nd decade
 Osteogenic Sarcoma
• Radiography
– Aggressive tumor; lytic (permeative)/ sclerotic
(amorphous, cloudlike)/ mixed
– Periosteal reaction: lamellated, spiculated
– Large S.T. mass; skip lesions (MRI)
 Osteogenic Sarcoma
• Subtypes
– Telangiectatic: (<20y.o.) Lytic, expansile,
aggressive, may mimic ABC radiographically
– Parosteal: (2nd-5th decades) low grade, better
prognosis, stuck on appearance post femur
– Periosteal: (20 y.o.) Surface OS, aggressive,
better prognosis than conventional OS; tibia
– Multicentric: synchronous lesions, poor
prognosis; sclerotic lesions; children
 Synovial Chondromatosis
• Synovial metaplasia; multiple osseous/
cartilaginous intraarticular loose bodies
• Nourished by synovial fluid
• Limited ROM/ pain
• DDX: PVNS, loose bodies DJD
• Monoarticular; knee, hip, shoulder, elbow
 Synovial Chondromatosis
• Radiography
– Effusion present; abnormal calcifications
– Ossified loose bodies seen on plain film
– Cartilaginous loose bodies seen on MR
– Secondary OA
– Pressure erosive changes