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ADAMANTINOMA
Adamantinoma is a rare
primary malignant bone
tumor with a poorly
understood histogenesis. It
has also been referred to as
malignant angioblastoma,
dermal inclusion tumor,
ameloblastoma, primary
epidermoid carcinoma of
bone, and carcinoma
sarcomatodes.
ADAMANTINOMA
Approximately 165
cases have been
reported in the
literature, with all
but 18 located in
the tibia. Other
bones affected are
the jaw, ulna,
humerus, femur,
and fibula.
ADAMANTINOMA
The mid-diaphysis of
the long bones is the
location in 75% of
cases. The lesions
are extremely rare in
the spine. Most
patients are
between 10 and 40
years of age.
ADAMANTINOMA
Radiographically,
adamantinomas may be
difficult to differentiate from
other lesions because they can
be situated in either the cortex
or the medullary space.
Cortical lesions may assume a
lytic bone blister appearance,
may appear as sawtooth loss
of cortical bone with ragged
margins, or may present as a
multichambered, bubbly lesion.
ADAMANTINOMA
The typical intramedullary lesion
is usually one large,
circumscribed area of
radiolucency with a mottled
increase in density scattered
throughout the length of the
lesion. Less commonly, a
reticulated, honeycombed type
of presentation occurs. Long-
standing tumors produce marked
cortical thickening and spool-
shaped bulges of the outer
cortex in an eggshell fashion.
ADAMANTINOMA
Gradual expansion of
bone with eventual
cortical disruption and
the development of a
soft tissue mass occurs.
Periosteal response is
minimal and, if present,
is usually of the
laminated variety.
ADAMANTINOMA
The most difficult and
almost indistinguishable
differential diagnosis is
fibrous dysplasia, though
the presence of periosteal
response, moth-eaten
destruction, no bowing or
ground glass appearance,
and younger age should
allow correct diagnosis.

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Adamantinoma 1

  • 1. ADAMANTINOMA Adamantinoma is a rare primary malignant bone tumor with a poorly understood histogenesis. It has also been referred to as malignant angioblastoma, dermal inclusion tumor, ameloblastoma, primary epidermoid carcinoma of bone, and carcinoma sarcomatodes.
  • 2. ADAMANTINOMA Approximately 165 cases have been reported in the literature, with all but 18 located in the tibia. Other bones affected are the jaw, ulna, humerus, femur, and fibula.
  • 3. ADAMANTINOMA The mid-diaphysis of the long bones is the location in 75% of cases. The lesions are extremely rare in the spine. Most patients are between 10 and 40 years of age.
  • 4. ADAMANTINOMA Radiographically, adamantinomas may be difficult to differentiate from other lesions because they can be situated in either the cortex or the medullary space. Cortical lesions may assume a lytic bone blister appearance, may appear as sawtooth loss of cortical bone with ragged margins, or may present as a multichambered, bubbly lesion.
  • 5. ADAMANTINOMA The typical intramedullary lesion is usually one large, circumscribed area of radiolucency with a mottled increase in density scattered throughout the length of the lesion. Less commonly, a reticulated, honeycombed type of presentation occurs. Long- standing tumors produce marked cortical thickening and spool- shaped bulges of the outer cortex in an eggshell fashion.
  • 6. ADAMANTINOMA Gradual expansion of bone with eventual cortical disruption and the development of a soft tissue mass occurs. Periosteal response is minimal and, if present, is usually of the laminated variety.
  • 7. ADAMANTINOMA The most difficult and almost indistinguishable differential diagnosis is fibrous dysplasia, though the presence of periosteal response, moth-eaten destruction, no bowing or ground glass appearance, and younger age should allow correct diagnosis.