Description

Musculoskeletal hemorrhage in a patient with hemophilia may mimic slow-growing tumor.


 

Most affected patients are adult males.

 

Clinical features:

(1) severe coagulopathy, typically Factor 8 or 9 deficiency

(2) an expanding encapsulated hematoma, Normally this is slowly growing but it may undergo a sudden change in size if there is acute hemorrhage into the mass.

(3) there may be a history of trauma at the site prior to its appearance

(4) usually painless at first

 

Locations affected include the pelvis, lower extremity and upper extremity:

(1) subperiosteal

(2) within bone (osseous) with osteolysis

(3) within skeletal muscle or soft tissue

 

Complications may include:

(1) a compartment syndrome

(2) nerve compression

(3) pathologic fracture

(4) secondarily infected

(5) rupture with massive hemorrhage

(6) significant disability

(7) a draining fistula

 

Biopsy or draining of the pseudotumor is contraindicated due to the high rate of complications.

 

Histologic exam shows:

(1) new and degenerating blood with hemosiderin

(2) a fibrous capsule

 

Imaging studies may show:

(1) nonspecific masses with patchy calcifications and/or dystrophic calcifications

(2) osseous pseudotumors can destroy the involved bone

(3) subperiosteal pseudotumors may develop an overlying "strut" if the periosteum becomes calcified

(4) avascularity

 


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