• Benign Bone Tumors and

Reactive Lesions
 Osteoid osteoma

• A distinctive, painful, benign osteoblastic bone

tumor.
M:F = 2:1
5 and 30 years of age.
 Genetics/etiology
Unclear
Nerve fibers play a role in producing pain.
High prostaglandin and cyclooxygenase levels
within the lesion
 Clinical presentation

a. Classic symptom is night pain relieved NSAID

b. The pain referred to an adjacent joint, .
c. femur, tibia,vertebral arch, humerus, and fingers;
the proximal femur is the most common site, and
the hip is the most common intra-articular location.
d. diaphyseal or metaphyseal regions of long bones
e. cause of a painful scoliosis, the lesion is usually at the
center of the concavity of the curve.
f. joint effusions, contractures, limp, muscle atrophy)
 Imaging appearance

a. Round, well-circumscribed intracortical lesion with

radiolucent nidus
b. <1 cm in diameter
c. periosteal reaction
d. intra-articular, subperiosteal, or medullary,
e. differential diagnosis includes osteomyelitis and
Ewing sarcoma.
f. MRI may show extensive surrounding edema
• Pathology
a. Microscopic appearance consists of uniform,
thin osteoid seams and immature trabeculae
(Figure 1, E and F).
b. Trabeculae are lined with uniform plump osteoblasts.
1- to 2-mm fibrovascular rim surrounds the
sharply demarcated nidus.
d. No pleomorphic cells, and the lesion does not
infiltrate the surrounding bone
e. Similar in appearance to osteoblastoma but
smaller in size
 Treatment/outcome
•
a. percutaneous radiofrequency ablation (RFA)
• A CTguided probe is inserted into the lesion with the temperature
raised to 90°C for 4 to 6 minutes to produce a 1-cm zone of necrosis.
i. Recurrence rates after RFA are <10%.
ii. Contraindications include lesions close to the spinal cord or nerve
roots.
b surgical resection or burring, although the lesion
c. around the hip, require internal fixation, sometimes with bone
grafting, if a large portion
d. Long-term medical NSAIDs can relieve symptoms
 Osteoblastoma

• A rare, aggressive, benign osteoblastic tumor

a. Male to female ratio = 2:1
b. Osteoblastomas are much less common
than osteoid osteomas.
• 10 and 30 years of age
 Clinical presentation
•
a. Slowly progressive dull, aching pain of long

b. Night pain is not typical, and aspirin not relieve the symptoms.
c. spine is the most common location for osteoblastoma
(posterior elements)
d. diaphysis or metaphysis of long bones (tibia and femur) and
the mandible.
e. Related swelling, muscle atrophy, and a limp
f. Osteoblastomas may be associated with oncogenic
osteomalacia.
 Imaging appearance
• a. Radiolucent lesion 2 to 10 cm
b. Cortically based (two thirds) more often than
medullary (one third)
d. 25% of the lesions have an extremely aggressive
e. Radiographic differential diagnosis includes
osteosarcoma, aneurysmal bone cyst (ABC),
osteomyelitis, and osteoid osteoma.
• CT, MRI is necessary to fully evaluate the extent of
the lesion
 Pathology

similar to an osteoid osteoma but with increased giant cells.

b. Irregular seams of osteoid separated by loose fibrovascular stroma
c. Osteoid is rimmed by prominent osteoblasts
that are occasionally large and epithelioid
d. Most commonly there is a sharp demarcation from the
surrounding bone.
e. 10% to 40% are associated with secondary ABC formation.
f. Numerous mitotic figures may be present
g. It is important to differentiate osteoblastoma
from osteosarcoma, although giant cell tumor
and ABC are also similar in appearance
 Treatment/outcome

• a. Osteoblastoma requires surgical treatment.

b. curettage and bone grafting
c. Nerve roots should be maintained when
treating spinal lesions.
• resection is required
 Parosteal osteoma

• A rare, self-limited deposition of reactive bone

on the surface of the cortex.
• 30s or 40s
• no known cause,
 Clinical presentation

• a. gradual swelling or dull pain

b. ncidental radiographic findings are present.
c. found in the craniofacial bones,
• but rare in the long bones
(tibia, femur), pelvis, and vertebrae.
 Imaging appearance

• Uniform radiodense lesion attached to the

bone cortex with a broad base ranging from 1
to 8 cm in size
• b. Well-defined with smooth, lobulated borders
c. No cortical or medullary invasion
• d. Radiographic differential diagnosis includes
parosteal osteosarcoma, healed stress fracture,
and osteoid osteoma.
 Pathology

• a. Histologic appearance is of mature,

hypocellular lamellar bone with intact
Haversian systems.
b. No atypical cells are present.
 Treatment/outcome

• a. Nonsurgical treatment
c. Local recurrence of the lesion suggests it
was initially not recognized as a parosteal
osteosarcoma.
 Bone island (enostosis)

• A small deposit of dense, compact bone

within the medullary cavity
• occur frequently in adults,
 Genetics/etiology
• Possible arrested resorption of mature bone
during endochondral ossification
 Clinical presentation
• asymptomatic
b. Any bone can be involved, but the pelvis
and femur are most common.
c. Osteopoikilosis is a hundreds of bone
islands throughout the skeleton, usually
centered about joints.
 Radiographic appearance
• Well-defined, round focus of dense bone
within the medullary cavity usually measuring
2 to 20 mm in diameter.
• No bony reaction or edema on MRI
• Radiographic differential diagnosis includes
well-differentiated osteosarcoma, osteoblastic
metastasis, and bone infarct.
 Pathology

• Bone islands appear histologically as cortical

bone with a well-defined lamellar structure
and haversian systems.
b. Border between the lesion and surrounding
medullary bone shows no endochondral
ossification.
 Treatment/outcome
• No treatment is required,
• but follow-up
 Enchondroma

• A benign tumor comprised of mature hyaline

cartilage
• located in the medullary cavity.
• 20 to 50 years.
 Genetics/etiology
• incomplete endochondral ossification,
• fragments of epiphyseal cartilage displace into
the metaphysis during skeletal growth
 Clinical presentation
a. asymptomatic

b. Lesions in the small bones of the hands and

feet can be painful, especially after pathologic

fracture.

d. If the patient has pain and the radiographic

appearance is suspicious, low-grade chondrosarcoma must be considered.

e. Half occur in the small tubular bones, hands;

f. Other common locations include the metaphysis or diaphysis of long bones (proximal humerus, distal femur, tibia);

• rare in the spine and pelvis.

h. malignant transformation is <1%.

 Imaging appearance
well-defined, lucent, central medullary lesions that
calcify overtime
more diaphyseal as the long bone grows.
b. The classic radiographic appearance involves
rings and stippled calcifications within the lesion
c. 1 to 10 cm in size.
d. Small endosteal erosion
e. The cortices in hand enchondromas may be
thinned and expanded
 Pathology
•
a. The gross appearance is of blue-gray, lobulated
hyaline cartilage with a variable amount of
calcifications throughout the tumor.
b. The low-power histologic appearance is of mature hyaline cartilage lobules separated by
normal marrow, which is key to differentiating an
enchondroma from a chondrosarcoma.
c. Endochondral ossification encases the cartilage
lobules with lamellar bone.
d. Lesions in the small tubular bones and proximal fibula are more hypercellular than lesions
in other locations.
e. Enchondromas in long bones have abundant
extracellular matrix but no myxoid component.
f. The cells are bland, with uniform, dark-stained
nuclei; they have no pleomorphism, necrosis,
mitoses, or multinucleate cells (Figure 5, C)
• Treatment/outcome
a. Asymptomatic lesions require no treatment but
should be followed with serial radiographs to
ensure inactivity.
b. Rarely, when pain due to other causes is excluded, symptomatic enchondromas
can be
treated with curettage and bone grafting.
c. Pathologic fractures through enchondromas in
small, tubular bones should be allowed to heal
prior to curettage and bone grafting.
d. If radiographs are suspicious for a chondrosarcoma, then surgery is necessary.
e. A needle biopsy is not reliable to differentiate
enchondroma from low-grade chondrosarcoma and should be used only if
confirmation
of tissue type is needed
 Periosteal chondroma

• A benign hyaline cartilage

tumor located on the surface of the bone.
1. Demographics: Periosteal chondromas occur in
patients from 10 to 30 years of age.
2. Genetics/etiology: These are rare lesions thought
to arise from pluripotential cells deep in the
periosteum that differentiate into chondroblasts
instead of osteoblasts.
 Clinical presentation

• Patients present with pain, or sometimes the

lesions are found incidentally in asymptomatic
patients.
Any bone can be involved, but the proximal
humerus, the femur, and the small bones of the
hand are the most common locations.
c. The lesions can grow slowly after patients
reach skeletal maturity but have no malignant
potential.
 Imaging appearance

• a. The classic appearance is a well-defined surface lesion that creates a

saucerized defect in
the underlying cortex (Figure 6).
b. The lesion ranges from 1 to 5 cm in size and is
metaphyseal or diaphyseal.
c. There is a rim of sclerosis in the underlying
bone.
d. The edges of the lesion often have a mature
buttress of bone.
e. The amount of calcification is variable, and
soft-tissue swelling may be present because of
the surface location.
f. The radiographic differential diagnosis includes periosteal chondrosarcoma
and periosteal osteosarcoma.
• Pathology
a. The low-power appearance is of
wellcircumscribed hyaline cartilage lobules.
b. The histologic appearance is similar to that
of
enchondroma, with mildly increased
cellularity, binucleated cells, and occasional
mild pleomorphism
• Treatment/outcome
a. No treatment is needed for asymptomatic
patients.
b. Symptomatic patients are treated with
excision
with a marginal margin.
c. Local recurrence is rare.
 Osteochondroma

• A benign cartilage tumor arising

from the surface of the bone.
1. Demographics
a. Osteochondromas are the most common benign
bone tumor.
b. The true incidence of osteochondromas is
unknown because most lesions are asymptomatic.
c. Most lesions are identified in the first two
decades of life.
 Genetics/etiology

• a. Osteochondromas are hamartomatous proliferations of

bone and cartilage.
b. They are thought to arise from trapped
growth-plate cartilage that herniates through
the cortex and grows via endochondral ossification beneath
the periosteum.
c. A defect in the perichondrial node of Ranvier
may allow the physeal growth to extend from
the surface; as the cartilage ossifies, it forms
cortical and cancellous bone that is the stalk of
the lesion.
 Clinical presentation

• a. Most lesions are solitary and asymptomatic.

b. Most are <3 cm in size, but they can be as
large as 15 cm.
c. Depending on size and location, patients can
have pain from an inflamed overlying bursa,
fracture of the stalk, or nerve compression.
. When close to the skin surface, they are firm,
immobile masses.
e. Osteochondromas continue to grow until the
patient reaches skeletal maturity.
f. The lesions most commonly occur around the
knee (distal femur, proximal tibia), proximal
humerus, and pelvis; spinal lesions (posterior
elements) are rare.
g. Subungual exostosis arises from beneath the
nail in the distal phalanx; this is a posttraumatic lesion and not a true osteochondroma.
h. Patients can present with multiple lesions
(multiple hereditary exostosis).
i. The risk of malignant degeneration of a solitary osteochondroma to a chondrosarcoma is
<1%.
j. Rarely, a dedifferentiated chondrosarcoma can
develop from a solitary osteochondroma.
 Imaging appearance

• a. Osteochondromas can be sessile or pedunculated on the bone surface

. There is a higher risk of malignant degeneration in sessile lesions.
c. Lesions arise near the growth plate and appear
to become more diaphyseal with time.
d. Pedunculated lesions grow away from the adjacent joint (Figure 7, B and C).
e. The medullary cavity of the bone is continuous
with the stalk of the lesion.
f. The cortex of the underlying bone is continuous with the cortex of the stalk.
g. The affected bony metaphysis is often flared or
widened.
h. The cartilage cap is usually radiolucent and involutes at skeletal maturity.
i. Metaplastic cartilage nodules can occur within
a bursa over the cartilage cap.
j. The radiographic differential diagnosis includes
parosteal osteosarcoma and myositis ossificans.
k. CT or MRI scans can better evaluate the cartilage cap and are useful when malignant
degeneration is a concern.
 Pathology

• The gross appearance of a pedunculated lesion

is similar to a cauliflower, with cancellous
bone beneath the cartilage cap.
b. Histologically, the cartilage cap consists of hyaline cartilage and is organized like a
growth
plate with maturation to bony trabeculae (Figure 7, F).
c. There is a well-defined perichondrium around
the cartilage cap.
d. The stalk consists of cortical and trabecular
bone with spaces between the bone filled with
marrow.
e. The chondrocytes within the lesion are uniform, without pleomorphism or
multiple nuclei.
f. Thick cartilage caps imply growth but are not
a reliable indicator of malignant degeneration.
 Treatment/outcome

• a. For asymptomatic or minimally symptomatic

patients who are still growing, nonsurgical
treatment is preferred.
b. Relative indications for surgical excision of an
osteochondroma (performed by excision at the
base of the stalk)
i. Symptoms secondary to soft-tissue inflammation (bursae, muscles, joint capsule, tendons)
ii. Symptoms secondary to frequent traumatic
injury
iii. Significant cosmetic deformity
iv. Symptoms secondary to nerve or vascular irritation
v. Concern for malignant transformation
c. The perichondrium over the cartilage cap must
be removed to decrease the likelihood of local
recurrence.
d. Delaying surgical excision until skeletal maturity allows a higher chance of local control.
e. Patients with osteochondromas extending into
the popliteal fossa can have pseudoaneurysms
and are subject to vascular injury during excision.
 Chondroblastoma

• A rare, benign bone tumor differentiated from

giant cell tumor by its chondroid
matrix.
1. Demographics
a. Male to female ratio = 2:1
b. 80% of patients are younger than 25 years.
 Genetics/etiology

• a. Chondroblastoma has been categorized as a

cartilage tumor due to its areas of chondroid
matrix, but type II collagen is not expressed by
the tumor cells.
b. It is thought to arise from the cartilaginous
epiphyseal plate
 Clinical presentation

• a. Patients present with pain that is progressive at

the site of the tumor.
b. As these tumors often occur adjacent to a
joint, there can be decreased range of motion,
a limp, muscle atrophy, and tenderness over
the affected bone.
c. Most chondroblastomas are found in the distal
femur and proximal tibia, followed by proximal humerus, proximal
femur, calcaneus, and
flat bones.
d. <1% develop benign pulmonary metastasis
from chondroblastoma.
 Imaging appearance

• a. Chondroblastomas are small, round tumors

that occur in the epiphysis or apophysis; they
often extend into the metaphysis (Figure 8, A
and B).
b. Most are 1 to 4 cm in size and have a sclerotic
rim.
c. Most are centrally located in the epiphysis,
with a small subset having a more aggressive
appearance due to secondary ABC formation.
d. Cortical expansion of the bone may be
present, but soft-tissue extension is rare.
e. 25% to 40% of chondroblastomas have stippled calcifications within the lesion.
f. The differential diagnosis includes giant cell tumor, osteomyelitis, and clear cell chondrosarcoma.
g. Three-dimensional imaging is not required, but
a CT scan defines the bony extent of the lesion.
h. MRI shows extensive edema surrounding the
lesion.
 Pathology

• a. The tumor consists of a background of mononuclear cells, scattered

multinucleated giant
cells, and focal areas of chondroid matrix.
b. The mononuclear stromal cells are distinct,
round, S100+ cells with large central nuclei
that can appear similar to histiocytes; the nuclei have a longitudinal
groove resembling a
coffee bean (Figure 8, C).
c. Chicken-wire calcifications are present in a
lace-like pattern throughout the tumor.
d. Mitotic figures are present but not atypical.
e. One third of chondroblastomas have areas of
secondary ABC.
 Treatment/outcome

• a. Curettage and bone grafting is indicated for

the treatment of chondroblastoma.
b. Adjuvants such as phenol or liquid nitrogen
are often used to decrease local recurrence.
c. The local recurrence rate is 10% to 15%.
d. Surgical resection is indicated for rare
benign
pulmonary metastasis.
 Chondromyxoid fibroma (CMF)

• A rare, benign cartilage tumor containing

chondroid, fibrous, and myxoid tissue
• 1. Demographics
a. Most CMFs occur in the second and third
decades of life, but they may be seen in patients
up to 75 years of age.
b. There is a slight male predominance
• 2. Genetics/etiology—CMF is thought to arise from remnants of
the growth plate.
• 3. Clinical presentation
a. Most patients present with pain and mild
swelling of the affected area.
b. Occasionally the lesions are noted incidentally.
c. The most common locations are the long
bones of the lower extremities (proximal tibia)
and pelvis. Small bones in the hands and feet
are also affected
 Imaging appearance

• a. CMF is a lucent, eccentric lesion found in the

metaphysis of long bones (Figure 9, A).
b. It can cause thinning and expansion of the adjacent cortical
bone (Figure 9, B).
c. It often has a sharp, scalloped sclerotic rim.
d. Radiographic calcifications within the lesion
are rare.
e. CMF ranges in size from 2 to 10 cm.
f. The radiographic differential diagnosis includes ABC,
chondroblastoma, and nonossifying fibroma.
g. There is increased tracer uptake within the lesion on bone scan.
 Pathology

• a. On low power, the lesion is lobulated with peripheral

hypercellularity.
b. Within the lobules, the cells are spindled or
stellate with hyperchromatic nuclei.
c. Between the lobules are multinucleated giant
cells and fibrovascular tissue.
d. Areas of myxoid stroma are present, but hyaline cartilage is rare
(Figure 9, C).
e. The cellular areas may resemble chondroblastoma.
f. Areas with pleomorphic cells with bizarre nuclei are common.
g. The histologic differential diagnosis includes
chondroblastoma, enchondroma, and chondrosarcoma.
 Treatment/outcome

• a. CMF is treated with curettage and bone

grafting.
b. The local recurrence rate is 10% to 20%.
• . Fibrous/Histiocytic
• Nonossifying fibroma (NOF)—A developmental abnormality
related to faulty ossification rather than a
true neoplasm.
1. Demographics
a. Very common skeletal lesions
b. Occur in children and adolescents (age 5 to 15
years)
c. 30% of children with open physes have nonossifying
fibromas.
d. Also frequently called fibrous cortical defect or
metaphyseal fibrous defect
• 2. Genetics/etiology—Possibly caused by
abnormal
subperiosteal osteoclastic resorption during
remodeling of the metaphysis
 Clinical presentation

• a. Usually an incidental finding

b. May be multifocal
i. Familial multifocal
ii. Neurofibromatosis
iii. Jaffe-Campanacci syndrome (congenital
with café au lait pigmentation, mental retardation, and
nonskeletal abnormalities involving heart, eyes, gonads)
c. Most common in long bones of lower extremity (80%)
d. Patients occasionally present with a pathologic
fracture.
 4. Radiographic appearance

• a. Eccentric, lytic, cortically based lesions with a

sclerotic rim (Figure 10, A and B)
b. Occur in the metaphysis and appear to migrate
to diaphysis as bone grows
c. May thin the overlying cortex with expansion
of the bone
d. Lesions enlarge (1 to 7 cm) as patient grows.
e. As patient reaches skeletal maturity, the lesions
ossify and become sclerotic.
f. Occasionally associated with secondary ABC
g. Plain radiographs are diagnostic.
h. A lesion similar in appearance to a nonossifying fibroma on the posteromedial aspect of
the
distal femur is an avulsive cortical irregularity,
which is the result of an avulsion injury at the
insertion of the adductor magnus muscle.
 Pathology

• a. Prominent storiform pattern of

fibrohistiocytic
cells (Figure 10, C and D)
b. Variable numbers of giant cells
c. May have areas of xanthomatous reaction
with foamy histiocytes
d. Prominent hemosiderin
e. Occasional secondary ABC component
 Treatment/outcome

• a. Most treated with observation; will spontaneously

regress
b. Monitor along with skeletal growth if they are
large lesions.
c. When symptomatic and large, curettage and
bone grafting is often necessary
d. For pathologic fractures, often allow to heal,
then observe or curettage/graft
e. Internal fixation rarely needed, depending on
anatomic location
 Fibrous dysplasia

• A common developmental abnormality

characterized by hamartomatous proliferation
of fibro-osseous tissue within the bon
1. Demographics
a. Can be found in any age, but ~75% in
patients
<30 years
b. Affects females more than males
• 2. Genetics/etiology
a. Solitary focal or generalized multifocal inability to produce mature lamellar
bone
b. Areas of the skeleton remain indefinitely as immature, poorly mineralized
trabeculae.
c. Not inherited
d. Monostotic and polyostotic forms associated
with activating mutations of GSα on chromosome 20q13, which produce a
sustained activation of adenylate cyclase cAMP.
e. Fibrous dysplasia tissue has high expression of
fibroblast growth factor (FGF)-23, which may
be the cause of hypophosphatemia in patients
with McCune-Albright syndrome or oncogenic
osteomalacia
• 3. Clinical presentation
a. Usually asymptomatic and found incidentally
b. Can be monostotic or polyostotic
c. Affects any bone, but predilection for proximal
femur, rib, maxilla, tibia
d. Fatigue fractures through lesion can cause
pain.
e. May have swelling around lesion
f. Severe cranial deformities, blindness with craniofacial involvement
g. Patients occasionally present with pathologic
fracture
h. McCune-Albright syndrome
i. Triad of polyostotic fibrous dysplasia, precocious puberty, and pigmented skin lesions
(coast of Maine)
ii. Unilateral bone lesions
iii. Skin lesions usually on same side as bone lesions
iv. 3% of patients with polyostotic fibrous dysplasia
i. Myriad endocrine abnormalities with polyostotic forms
j. Most common entity causing oncogenic osteomalacia (renal phosphate wasting due to FGF-
23)
k. Mazabraud syndrome is fibrous dysplasia
(usually polyostotic) associated with soft-tissue
intramuscular myxomas.
 4. Radiographic appearance

• a. Central lytic lesions within the medullary canal—usually

diaphysis/metaphysis
b. Sclerotic rim
c. May be expansile with cortical thinning
d. “Ground glass” or “shower-door glass” appearance (Figure 11,
A)
e. Bowing deformity in proximal femur (shepherd’s crook) or tibia
f. Can see vertebral collapse and kyphoscoliosis
g. Long lesion in a long bone (Figure 11, B)
h. Increased activity on bone scan, but plain radiographs usually
diagnostic
 5. Pathology

• a. Gross: yellow-white gritty tissue

b. Histology: poorly mineralized immature fibrous tissue surrounding
islands of irregular,
often poorly mineralized trabeculae of woven
bone (Figure 11, C)
c. “Chinese letters” or “alphabet soup” appearance
d. Metaplastic bone arises from fibrous tissue
without osteoblastic rimming.
e. Common mitoses
f. May have metaplastic cartilage present or areas
of cystic degeneration
g. Can be associated with secondary ABC
h. Differential diagnosis includes low-grade intramedullary osteosarcoma.
 Treatment/outcome

• a. Asymptomatic patients may be observed.

b. Surgical indications include painful lesions,
impending/actual pathologic fracture, severe
deformity, neurologic compromise (spine).
c. Curettage and bone-graft the lesion. (Use cortical allograft, not cancellous autograft!)
d. Cancellous autografts are replaced by dysplastic bone.
e. Usually requires internal fixation to achieve
pain control (intramedullary device more effective than plate) in the lower extremity
f. Osteotomies for deformity
g. Medical treatment with bisphosphonates provided good pain relief in small series.
h. In ~1% of lesions, malignant transformation
to osteosarcoma, fibrosarcoma, or malignant fibrous histiocytoma occurs, with
extremely
poor prognosis
 Osteofibrous dysplasia

• A nonneoplastic fibroosseous lesion affecting

the long bones of young children.
1. Demographics
a. Affects males more than females
b. Usually noted in first decade of life
2. Genetics/etiology: Trisomy 7, 8, 12, and 22
have been reported
 Clinical presentation

• a. Unique predilection for the tibia

b. Can have anterior or anterolateral bowing
deformity
c. Pseudarthrosis develops in 10% to 30% of
patients.
d. Patients usually present with painless
swelling
over anterior border of tibia.
• Radiographic appearance
a. Eccentric, well-defined anterior tibial lytic lesions
(Figure 12, A)
b. Usually diaphyseal
c. Single or multiple lucent areas surrounded by
dense sclerosis
d. Confined to the anterior cortex; may expand
e. No periosteal reaction
f. Differential diagnosis: adamantinoma (radiographic
appearance can be identical)
• . Pathology
a. Moderately cellular fibroblastic stroma
b. Islands of woven bone with prominent
osteoblastic rimming (Figure 12, B)
c. No cellular atypia
d. May have giant cells
e. Differential diagnosis: fibrous dysplasia
• 6. Treatment/outcome
a. Avoid surgery if possible; brace when necessary.
b. Lesions may spontaneously regress or stabilize
at skeletal maturity.
c. May need deformity correction
d. Controversy whether there is a continuum from
osteofibrous dysplasia to adamantinoma, but
the exact nature of this relationship is uncertain
• An extremely rare benign
bone tumor composed of dense bundles of
fibrous
connective tissue.
1. Demographics—Most common in patients
age 10
to 30 years
• 2. Genetics/etiology
a. Bone counterpart of the aggressive fibromatosis
(desmoid) in soft tissue; may originate from
myofibroblasts
b. Loss of 5q21-22 (gene location for familial
adenomatous polyposis and Gardner syndrome)
has been reported.
c. Loss of 4p and rearrangement of 12q12-13,
and trisomy 8 (0%-33%), trisomy 20 (2%-
25%), or both (0%-16%)
• 3. Clinical presentation
a. Can occur in any bone
b. Intermittent pain unrelated to activity
c. Palpable mass/swelling
• 4. Imaging appearance
a. Lytic lesion centrally located in metaphysis
b. Honeycomb/trabeculated appearance (Figure
13)
c. Usually no periosteal reaction unless a fracture
is present (12%)
d. May appear aggressive with cortical destruction and
soft-tissue extension
e. No calcification within lesion
f. Increased activity on bone scan
• 5. Pathology
a. Gross: dense, white, scarlike tissue
b. Histology: abundant collagen fibrils with intermixed
spindle cells
c. Appearance is hypocellular and similar to scar
tissue.
d. Monotonous uniform nuclei
e. Infiltrative growth pattern—trapping native
trabeculae
f. Differential diagnosis includes low-grade fibrosarcoma
• 6. Treatment/outcome
a. Surgical treatment is the standard of care.
b. Thorough curettage allows good results.
c. Wide resection for expendable bones or locally
recurrent lesions
d. Desmoplastic fibromas do not metastasize, but
they often recur locally
 E. Langerhans cell histiocytosis

• A clonal proliferation of Langerhans-type histiocytes

that can have multiple clinical presentations
1. Demographics
a. Most common in children (80% < 20 years of
age)
b. Male to female ratio = 2:1
2. Genetics/etiology: N/A
• 3. Clinical presentation
a. Previously categorized as eosinophilic granuloma, Hand-Schuller-Christian
disease (chronic,
disseminated), and Letterer-Siwe disease (infantile, acute)
b. Now believed to be three scenarios
i. Solitary disease (eosinophilic granuloma)
ii. Multiple bony sites
iii. Multiple bony sites with visceral involvement (lungs, liver, spleen, skin,
lymph nodes)
c. Rarely, bone lesions are asymptomatic; usually,
they cause localized pain/swelling/limp.
d. Can occur in any bone but most commonly
skull, ribs, clavicle, scapula, vertebrae (thoracic > lumbar > cervical), long
bones, pelvis
• 4. Radiographic appearance
a. Classic appearance is a “punched-out” lytic lesion
(Figure 14, A).
b. May have thick periosteal reaction
c. Can appear well-demarcated or permeative
(Figure 14, B)
d. Commonly causes vertebral collapse (vertebra
plana) when affecting the spine (Figure 14, C)
e. Great mimicker of other lesions (osteomyelitis,
Ewing sarcoma, leukemia)
 5. Pathology

• a. The characteristic tumor cell is the Langerhans cell or

histiocyte (Figure 14, D).
• b. Histiocytes have indented nuclei (coffee bean)
with eosinophilic cytoplasm.
c. Histiocytes stain with CD1A.
d. Giant cells are present.
e. Eosinophils are variable in number.
f. Mixed inflammatory cell infiltrate
g. Birbeck granules on electron microscopy (tennis rackets)
 6. Treatment/outcome

• a. Solitary lesions can be treated effectively with

an intralesional injection of methylprednisolone acetate.
b. Curettage/bone grafting if open biopsy is being
performed for diagnosis
c. In 90% of patients with vertebra plana due to
Langerhans cell histiocytosis, bracing alone
will correct the deformity; 10% will need corrective surgery.
d. Low-dose radiation is used in rare cases (spinal cord compression).
e. Patients with disseminated disease and visceral
involvement can die of the disease. The prognosis is improving with
more effective chemotherapy but worsens with increasing number of
extraosseous sites of disease.
• IV. Cystic