Fibrous Xanthomas and Paget's Flashcards
What conditions make up the group of fibrous xanthomas in order from smallest to largest?
Fibrous cortical defect, nonossifying fibroma, fibrous dysplasia
What is unique about the specific placement of a fibrous cortical defect in bone?
Loves cortex
What other lesion loves the cortex of bone like a fibrous cortical defect?
Osteoid osteoma
What is the treatment for a fibrous cortical defect or nonossifying fibroma?
Nothing because they usually regress over a 2-5 year period
How does a fibrous cortical defect look different than an osteoid osteoma?
No nidus or cortical thickening with FCD
What is the age range for a nonossifying fibroma?
8-20 years old (most occur in second decade aka teens)
How common are nonossifying fibromas?
30-40% of children
What is the specific part of bone where we find nonossifying fibromas?
Eccentric and metadiaphyseal
What are the common locations for nonossifying fibromas?
Long bones of lower extremity like distal tibia and distal femur
Why are fractures more possible with a nonossifying fibroma compared to a FCD?
Larger than a FCD (same with fibrous dysplasia)
What is the age range for fibrous dysplasia?
1-2 decades of life
Is fibrous dysplasia most commonly monostotic or polyostotic?
Monostotic (involving one bone)
Polyostotic fibrous dysplasia is seen with what type of condition that is also associated with Coast of Maine cafe au lai spots and precocious puberty in females?
McCune-Albright Syndrome
What radiographic finding is associated with fibrous dysplasia?
Rind sign
What is the term for the area of thickened sclerosis along the rim of a lesion of fibrous dysplasia?
Rind sign
What appearance occurs with the internal matrix involving fibrous dysplasia?
“Ground glass” appearance
What deformities can arise in the femur/tibia from fibrous dysplasia?
Saber shin and/or shepard’s crook deformities
How common is Paget’s disease?
3% of population over 40 years old
In what area of the world is Paget’s more common?
Norther latitudes and specifically UK, Australia, New Zealand
What is the age range for Paget’s?
Over 60
What is thought to be the cause of Paget’s?
Virus
How does bone change overall from Paget’s disease?
Increased resorption followed by increased bone formation which has a disorganized trabecular pattern lending to bone softening (bone increases in size and gets very white)
What are some of the more common areas for Paget’s?
Pelvis, lumbar/thoracic spine, proximal femur, calvarium..
What are the lab findings seen with Paget’s?
Increased hydroxyproline and alkaline phosphatase with normal serum calcium and phosphorus levels
“Cotton wool” appearance is associated with what lesion?
Paget’s (in path we called it the “fuzzy pelvis”)
What are the 4 stages of Paget’s disease?
1 lytic/destructive 2 mixed 3 sclerotic/ivory 4 malignant degeneration and possible change to osteosarcoma
Paget’s disease can lead to what serious change in the skull?
Basilar invagination with foraminal encroachment (due to bone softening)
What is the term for the well defined lysis of the frontal and occipital regions?
Osteoporosis circumscripta
What are the initial patient symptoms with Paget’s?
None really…except maybe hats are feeling tighter (due to cortical thickening)
At what specific part of bone does Paget’s begin in in long bones?
Subarticular end of bone (diff dx with GCT and chondroblastoma)
What is the radiographic sign is a lucent leading edge in a long bone seen during the lytic phase of Paget disease of bone?
Candle flame or blade of grass sign
2-9% of the time, Paget’s can become malignant into what type of lesion?
Osteosarcoma
What is the radiographic sign when there is thickening of the iliopectineal line and cortical lines of the pubic bones?
Rim sign
Paget’s disease can also cause what kind of issue with the femur heads?
Acetabular protrusion
What radiographic signs can be seen in a vertebral body from Paget’s?
Picture frame vertebra and ivory vertebra
What are the 3 differentials for an ivory vertebra?
Hodgkins (20-40 YOA), blastic mets (over 40 YOA), Paget’s (over 60 YOA)
What are some possible complication of Paget’s?
Peripheral nerve or brainstem compression with spinal stenosis, hearing loss, blindness, facial palsy, hyperemia of overlying skin, high output cardiac failure, DJD
What is the name of the horizontal jagged fracture sometimes seen in the long bones with Paget’s?
Banana fractures
