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Defect
Clinical Features
Osteogenesis imperfecta
Osteopetrosis
Osteoporosis (OP)
Mosaic bone.
Anterior bowing of femur/tibia
Secondary OA
Chalk stick fractures, compression fractures
Hypervascularity cardiac failure
Tumors can occur
Defect
Vit D deficiency or abnormal metabolism of vit D
excessive unmineralized matrix soft bone
Clinical Features/Other
-Craniotabes (flat back of head), frontal bossing, rachitic
rosary, pigeon breast deformity, Harrison groove,
increased lumbar lordosis, leg bowing
Hyperparathyroidism
Fractures
Osteonecrosis
Bone Tumors
Disease
Bone Forming Tumors
Location
Patient Population
Other
Osteoma, benign
-grows slowly
-Gardner syndrome:
multiple osteomas, GI
Osteoid osteoma:
appendicular skeleton
(femur/tibia)
Osteoblastoma: central
skeleton
Osteosarcoma
Radio: destructive.
Permeative
margins/sunburst
appearance. Codman
triangle: raised periosteum,
reactive periosteal bone
formation.
Gross: bulky, gray-white,
hemorrhage. See cortical
destruction with a soft
tissue mass. Can invade
the joint/epiphyseal plate.
Histo: Pleiomorphic
nuclei, mitotic figures,
random bone formation
errywhere
Cartilage Forming
Tumors
Osteochondroma, benign
Enchondroma, benign
Chondroblastoma, benign
Metaphysis of tubular
bones (fingers, toes);
usually solitary
-in enchondral bones and
is intraosseus (cartilage
tumor inside the bones)
Teens
M:F:2:1
-rare
-painful joint effusion,
restricted movement
-weirdly enough, can cause
metastases to the lungs
Chondromyxoid fibroma,
benign
necrosis, mitoses
Radio: eccentric lucency,
surrounding rim of
sclerosis, expanded cortex
M 2x>F
Radio: endosteal
scalloping (due to
resorption of inner
marginal of cortical bones
recall that Pathoma says
cartilage tumors occurs in
the medullary space),
flocculent calcification
Gross: malignant
hyaline/myxoid cartilage,
bulky, gray-white, expands
into adjacent cortex and
soft tissues
Histo: pleiomorphic large
chondrocytes
40s or older
M 2x>F
Nonossifying fibroma,
benign
Common, in 30-50% of
children >2 yrs
Developmental defect,
resolves on its own,
asymptomatic
Detected in adolescence
Developmental defect, if
larger can get fracture but
otherwise asymptomatic
Developmental arrest of
OBsbone is replaced by
fibrous stroma and woven
bone. Immature elements.
Varieties:
-monostotic (1 bone,
Miscellaneous Tumors
Ewing Sarcoma &
Primitive Neuroectodermal
Tumor (PNET), malignant
humerus 50-100% of
patients will have
craniofacial involvement.
Also get shoulder/pelvic
girdle deformities,
fractures
Histo: curvilinear
trabeculae of woven bone
with surrounding
fibroblasts= Chinese
characters
asymptomatic, deformity if
craniofacial)
- polyostotic (multiple
bones, get fractures and
deformities)
- polyostotic with
endocrinopathy (McCune
Albright Syndrome): in
addition to polyostotic
features, cafe au lait spots,
endocrine abnormalities
(sexual precocity,
hyperthyroidism, pit.
Adenoma, adrenal
hyperplasia)
Age: 10-15 years, whites
11:22 transolcation
Second most common
bone sarcoma in children
after osteosarcoma
Ewing: cells are
undifferentiated
PNET: cells have neural
differentiation
Painful, enlarging mass
with tenderness, warmth,
swelling; systemic signs
with fever, anemia,
leukocytosis, etc
In the KNEE
Metaphysis in adolescents
Metaphyis or epiphysis in
adults
Metastatic disease
Adolescents adults,
20s-40s
<30 yrs