AJR 2004; 183:55-61
© American Roentgen Ray Society
Hemophilic Pseudotumor Involving the Musculoskeletal System: Spectrum of Radiologic Findings
Ji Seon Park1 and
Kyung Nam Ryu
1 Both authors: Department of Diagnostic Radiology, Kyung Hee University
Hospital, Heokidong Dongdaemun-ku, Seoul 130-702, South Korea.
Received October 11, 2003;
accepted after revision December 28, 2003.
Address correspondence to K. N. Ryu
(t2star{at}naver.com).
Introduction
Hemophilic pseudotumor is a rare complication occurring in 1–2% of
patients with severe cases of hemophilia (clotting factor level, < 1% of
normal) [1]. The pseudotumor
grows as a chronic, slowly expanding, encapsulated cystic mass as a result of
recurrent hemorrhage in extraarticular musculoskeletal systems. Pseudotumors
are categorized as osseous and soft-tissue lesions, on the basis of anatomic
location [2]. Occasionally,
however, they cross the anatomic boundaries. The radiologic characteristics as
well as clinical symptoms depend on the initial site and extent of hemorrhage.
Conventional radiography, sonography, CT, and MRI each play an important role
in diagnosis, characterization, and management
[3–5].
Since 1994, 23 patients with hemophilia have been treated for pseudotumors (15
osseous lesions, 14 soft-tissue lesions) at our institution. This article
illustrates the variable radiologic appearance of hemophilic pseudotumors in
musculoskeletal systems as shown by radiography, sonography, CT, and MRI.
Osseous Pseudotumor
Repetitive bleeding into the bones results in osseous pseudotumor, whereas
repetitive bleeding into the joints leads to hemophilic arthropathy. The bones
most frequently implicated, in order of descending frequency, are the femur,
pelvis, tibia, and small bones of the hand
[2]. Radiographic findings vary
greatly with the extent, location, and different stages of hemorrhages and
reflect the presence of medullary bone destruction, cortical changes, internal
opacities, various types of periosteal reaction, and surrounding soft-tissue
abnormalities. With these variable radiographic appearances, osseous
hemophilic pseudotumors can be confused with other tumorous or infectious
conditions. Hemophilic arthropathy near the pseudotumor is frequently found on
conventional radiography.
Management for hemophilic pseudotumor aims at preserving function and
includes conservative methods (immobilization, substitution), radical
extirpation, and irradiation
[6].
Intraosseous Lesions
On radiography, intraosseous pseudotumors produce a well-defined,
unilobular or multilobular, expansile lytic lesion of variable size. They
occur in any portion of the tubular bones, including metadiaphysis or
epiphysis, and have ventral or eccentric epicenters. They may show endosteal
scalloping, cortical thinning or thickening, as well as peripheral sclerosis
(Figs. 1 and
2A). Trabeculae or septalike
structures frequently extend across the osteolytic lesions. Dystrophic
calcification may be present (Fig.
3A,
3B). Progressive expansion may
lead to deformity or pathologic fracture. Intraosseous pseudotumors simulate
primary and secondary neoplasms (giant cell tumor, desmoplastic fibroma,
plasmacytoma, metastasis), tumorlike lesions (solitary bone cyst, aneurysmal
bone cyst, brown tumor), and even infection (echinococcosis)
[7]. CT is particularly useful
for the evaluation of crossing trabeculae, cortical change, and periosteal
reaction. MRI has a remarkable ability for assessing intramedullary portions
and nearby soft tissue (neurovascular bundle) as well as monitoring the
therapeutic response. The characteristic MRI appearance is an intramedullary
cystic lesion containing fluid components, which have complex signal
intensities reflecting the effects of remote and recurrent hemorrhage and clot
organization [5] (Figs.
2B and
4A,
4B,
4C).
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Fig. 1. —23-year-old man with hemophilia with osseous pseudotumor of
right calcaneus. Lateral radiograph of calcaneus shows well-defined,
expansile, osteolytic lesion occupying entire calcaneus and resulting in
scalloped appearance and contour deformity. Thick trabeculae (thin
arrows) are also seen crossing osteolytic area. Cortical thickening
(thick arrow) attests to chronic nature of this process. Note
hemophilic arthropathy of talocrural joint.
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Fig. 2A. —6-year-old boy with hemophilia and osseous pseudotumor.
Anteroposterior view of conventional radiograph of forearm reveals expansile,
osteolytic lesion within proximal ulna. Lesion extends to subchondral bone.
Cortex shows diffuse thinning, but focal cortical thickening and peripheral
sclerosis (arrow) are seen distally.
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Fig. 3A. —27-year-old man with hemophilia and two osseous pseudotumors
in right lower extremity. Initial radiograph of right tibia reveals
well-defined, multilobulated, osteolytic lesion in proximal metadiaphysis of
tibia. Cortical thickening with surrounding sclerosis (arrows), some
septalike structures crossing osteolytic zone, and hemophilic arthropathy
involving knee joint are also noted.
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Fig. 3B. —27-year-old man with hemophilia and two osseous pseudotumors
in right lower extremity. Follow-up radiograph obtained 3 years 6 months after
A reveals enlargement of previous lesion as well as new lesion at
distal femur (arrowheads). In tibial lesion, septa have thinned or
disappeared, although new irregular calcifications (arrow) have
developed within distal region.
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Fig. 2B. —6-year-old boy with hemophilia and osseous pseudotumor. On
sagittal T2-weighted spin-echo image (TR/TE, 2,000/70), extensive hemorrhagic
mass is shown to have replaced ulna. Thickened cortex (arrowheads) at
its caudal part shows isointense signal.
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Fig. 4A. —16-year-old boy with hemophilia and shoulder pain.
Anteroposterior radiograph of left shoulder shows well-defined, slightly
expansile, osteolytic lesion in humeral head. Lesion is surrounded by thin
sclerotic rim and displays incomplete septalike structures. Smaller daughter
cysts (arrows) are also seen.
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Fig. 4B. —16-year-old boy with hemophilia and shoulder pain. Sagittal
T2-weighted spin-echo (TR/TE, 3,500/80) (B) and axial T1-weighted
spin-echo (350/9) (C) images show osseous pseudotumor surrounded by
low-signal rim, indicative of fibrous wall with hemosiderin deposits.
Heterogeneous signal intensity of interior fluid reflects different stages of
hemorrhage. Note hemosiderin deposits (arrowheads) along synovial
lining of joint and bursa. Smaller daughter cysts (arrow, C)
are also seen.
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Fig. 4C. —16-year-old boy with hemophilia and shoulder pain. Sagittal
T2-weighted spin-echo (TR/TE, 3,500/80) (B) and axial T1-weighted
spin-echo (350/9) (C) images show osseous pseudotumor surrounded by
low-signal rim, indicative of fibrous wall with hemosiderin deposits.
Heterogeneous signal intensity of interior fluid reflects different stages of
hemorrhage. Note hemosiderin deposits (arrowheads) along synovial
lining of joint and bursa. Smaller daughter cysts (arrow, C)
are also seen.
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Subperiosteal Lesions
Subperiosteal pseudotumors occur secondary to hemorrhage that has caused
elevation of the periosteum along the cortex and pressure necrosis of the
bone. Immature skeleton is particularly easy to lift. Radiographic findings
suggestive of these pseudotumors include cortical erosion due to pressure,
subperiosteal bone formation, and soft-tissue extension. Pseudotumors may also
produce an aggressive periosteal reaction and curvilinear calcific strut that
projects into soft tissue (Fig.
5A,
5B,
5C). Differential diagnosis
includes Ewing's sarcoma, metastasis, or infectious conditions
[2]. On CT or MRI, hemorrhagic
lesions may be confined to the subperiosteal space or extended to the
intramedullary portion or soft tissue. The elevated periosteum is well
visualized (Fig. 6A,
6B).
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Fig. 5A. —44-year-old man with hemophilia and extensive subperiosteal
pseudotumor. Coronal T1-weighted spin-echo image (TR/TE, 800/12) discloses
massive subperiosteal bleeding involving femoral shaft with intraosseous
extension in its cranial part.
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Fig. 5B. —44-year-old man with hemophilia and extensive subperiosteal
pseudotumor. On axial T2-weighted spin-echo image (3,000/90), nodularity along
capsular wall and fluid–fluid levels (arrow) within pseudotumor
are visible.
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Fig. 5C. —44-year-old man with hemophilia and extensive subperiosteal
pseudotumor. Conventional radiograph shows extensive soft-tissue mass,
scalloped extrinsic erosion of adjacent femur, and radiating trabeculae
(arrows) mimicking malignant periosteal lesions or infection.
Pathologic fracture (not shown) was induced at distal metaphysis. Note small
flecks of calcium (arrowheads).
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Fig. 6A. —14-year-old boy with hemophilia and subperiosteal
pseudotumor. Shown on sagittal double-echo steady-state spin-echo image
(TR/TE, 25/9; flip angle, 35°), subperiosteal hemophilic pseudotumor
causes stripping of periosteum (arrowheads) along distal femoral
metadiaphysis.
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Fig. 6B. —14-year-old boy with hemophilia and subperiosteal
pseudotumor. Axial T1-weighted spin-echo image (400/12) sectioned at distal
part of lesion visualizes cortex eroded by hematoma and elevated periosteum
(arrowheads). Intramedullary portion (IM) is preserved.
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Soft-Tissue Pseudotumor
Repetitive bleeding into soft tissue that is not resolved and replaced by
fibrous tissue causes joint contractures and soft tissue pseudotumors. The
latter are most common in the thigh, gluteal region, and iliopsoas muscle.
Soft-tissue pseudotumors can be further classified as intramuscular or
extramuscular (interfascial, subcutaneous)
[8] (Figs.
7A,
7B,
7C and
8A,
8B). Osseous lesions may
coexist in some cases (Fig.
9A,
9B,
9C). Intramuscular hematomas
generally remain localized, although they may dissect along the muscle bundles
to reach the bone and thereby produce remarkable pressure deformity or
subperiosteal bone formation. Pressure to adjacent soft tissue may lead to
skin necrosis, pain, neurologic deficits, and impaired mobility. Secondary
infection is a rare phenomenon that can result from perforation into viscus or
from an iatrogenic cause (Figs.
10 and
11A,
11B). In the case of iliopsoas
pseudotumors, the retroperitoneal and intraperitoneal hemorrhage may be linked
(Fig. 12A,
12B,
12C,
12D).
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Fig. 7A. —45-year-old man with hemophilia and soft-tissue pseudotumor.
Sagittal T1-weighted spin-echo (TR/TE, 800/12) (A) and T2-weighted
spin-echo (4,291/99) (B) images show at least three sharply defined
masses within biceps femoris muscle. One of these has variable-sized nodules
(arrowheads) attached to capsular wall. These intramuscular
pseudotumors are in contact with two subcutaneous pseudotumors
(asterisks). Different combinations of signal intensity within these
lesions indicate complex processes of variable stages of hemorrhage.
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Fig. 7B. —45-year-old man with hemophilia and soft-tissue pseudotumor.
Sagittal T1-weighted spin-echo (TR/TE, 800/12) (A) and T2-weighted
spin-echo (4,291/99) (B) images show at least three sharply defined
masses within biceps femoris muscle. One of these has variable-sized nodules
(arrowheads) attached to capsular wall. These intramuscular
pseudotumors are in contact with two subcutaneous pseudotumors
(asterisks). Different combinations of signal intensity within these
lesions indicate complex processes of variable stages of hemorrhage.
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Fig. 8A. —29-year-old man with hemophilia and interfascial
pseudotumors. Coronal T1-weighted spin-echo (TR/TE, 500/12) (A) and
axial T2-weighted spin-echo (3,000/90) (B) images exhibit two
pseudotumors in interfascial locations (arrows). Larger pseudotumor
measures 16.5 x 4.3 x 4 cm and lies between fascial planes of
tensor fascia lata and vastus lateralis muscles. Axial image (B) shows
capsular wall has focal high signal intensity with slight bulging
(arrowheads) that suggests daughter cyst.
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Fig. 8B. —29-year-old man with hemophilia and interfascial
pseudotumors. Coronal T1-weighted spin-echo (TR/TE, 500/12) (A) and
axial T2-weighted spin-echo (3,000/90) (B) images exhibit two
pseudotumors in interfascial locations (arrows). Larger pseudotumor
measures 16.5 x 4.3 x 4 cm and lies between fascial planes of
tensor fascia lata and vastus lateralis muscles. Axial image (B) shows
capsular wall has focal high signal intensity with slight bulging
(arrowheads) that suggests daughter cyst.
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Fig. 9A. —36-year-old man with severe hemophilia. Conventional
radiograph reveals two osteolytic lesions involving both ilia (I). Both hip
joints are severely destroyed and show evidence of associated head
collapse.
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Fig. 9B. —36-year-old man with severe hemophilia. Coronal T2-weighted
spin-echo (TR/TE, 3,766/81) (B) and axial T1-weighted spin-echo (600/9)
(C) MR images show both bone and soft-tissue abnormalities of hip
joints, ilia (I), and gluteus muscles (G). MR signal characteristics of these
lesions indicate hemorrhage in different stages; left iliac lesion with low
signal intensity on T2-weighted image is more recent. Medial cortex
(arrowhead, B and C) of right ilium is focally
disrupted. Both focal subperiosteal bone formation (curved arrows,
C) and extrinsic erosion (straight arrows, B) are
caused by intramuscular pseudotumor.
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Fig. 9C. —36-year-old man with severe hemophilia. Coronal T2-weighted
spin-echo (TR/TE, 3,766/81) (B) and axial T1-weighted spin-echo (600/9)
(C) MR images show both bone and soft-tissue abnormalities of hip
joints, ilia (I), and gluteus muscles (G). MR signal characteristics of these
lesions indicate hemorrhage in different stages; left iliac lesion with low
signal intensity on T2-weighted image is more recent. Medial cortex
(arrowhead, B and C) of right ilium is focally
disrupted. Both focal subperiosteal bone formation (curved arrows,
C) and extrinsic erosion (straight arrows, B) are
caused by intramuscular pseudotumor.
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Fig. 10. —32-year-old man with hemophilia and soft-tissue pseudotumor
with secondary infection. Anteroposterior view shows extensive soft-tissue
mass in right thigh, resulting in extrinsic cortical erosion
(arrowheads) of femoral shaft. Skin defect and air bubbles reflect
secondary infection and skin necrosis. Note hemophilic involvement of knee
joint.
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Fig. 11A. —72-year-old man with hemophilia and two intramuscular
pseudotumors and secondary infection. Sagittal T1-weighted spin-echo image
(TR/TE, 875/12) shows intramuscular pseudotumor with skin defect and air
bubbles (arrow), representing secondary infection.
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Fig. 11B. —72-year-old man with hemophilia and two intramuscular
pseudotumors and secondary infection. Fat-suppressed gadolinium-enhanced
proton density–weighted spin-echo axial image (1,393/12) reveals two
intramuscular hemophilic pseudotumors in vastus lateralis and biceps femoris
muscles. Posterior pseudotumor shows heterogeneous enhancement with
infiltration to nearby soft tissue. Both hemosiderin deposits and air bubbles
contribute to dark signal intensities. Neurovascular bundle
(asterisk) is preserved.
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Fig. 12A. —39-year-old man with hemophilia and iliopsoas pseudotumor and
perinephric hematoma. Abdominal sonogram (A) and unenhanced CT scans
(B and C) reveal hemorrhage at right psoas muscle
(arrows, A and C) and perinephric space
(arrowheads, B). Intramuscular pseudotumor involving right
psoas muscle appears as low echoic lesion on sonography and area of high
attenuation (73 H) surrounded by thin, low-attenuated rim on CT. Mesenteric
fat and ipsilateral abdominal wall are also involved.
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Fig. 12B. —39-year-old man with hemophilia and iliopsoas pseudotumor and
perinephric hematoma. Abdominal sonogram (A) and unenhanced CT scans
(B and C) reveal hemorrhage at right psoas muscle
(arrows, A and C) and perinephric space
(arrowheads, B). Intramuscular pseudotumor involving right
psoas muscle appears as low echoic lesion on sonography and area of high
attenuation (73 H) surrounded by thin, low-attenuated rim on CT. Mesenteric
fat and ipsilateral abdominal wall are also involved.
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Fig. 12C. —39-year-old man with hemophilia and iliopsoas pseudotumor and
perinephric hematoma. Abdominal sonogram (A) and unenhanced CT scans
(B and C) reveal hemorrhage at right psoas muscle
(arrows, A and C) and perinephric space
(arrowheads, B). Intramuscular pseudotumor involving right
psoas muscle appears as low echoic lesion on sonography and area of high
attenuation (73 H) surrounded by thin, low-attenuated rim on CT. Mesenteric
fat and ipsilateral abdominal wall are also involved.
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Fig. 12D. —39-year-old man with hemophilia and iliopsoas pseudotumor and
perinephric hematoma. Radiograph of abdomen shows diffusely increased density
of soft tissue in right abdomen and buttock.
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Soft-tissue pseudotumors manifest on radiography as increased soft-tissue
density, with or without internal calcification. Adjacent bony structures may
be normal or show focal bone formation, extrinsic erosion, periosteal
reaction, or medullary destruction (Fig.
10). Gas in the pseudotumor suggests secondary infection.
Sonography is economically useful to follow the progression or regression of
hematoma after therapy. Enhanced CT can define the outlines and wall thickness
of the peripheral enhancing capsule. Variable patterns of both the
echogenicity on sonography (anechoic or heterogeneously low) and attenuation
on CT (high, isodense, or low) represent different stages of the hemorrhagic
event [4] (Fig.
12A,
12B,
12C,
12D). A heterogeneous signal
in pseudotumors (as well as those in other locations) on both T1- and
T2-weighted MR images reflects blood products in various stages of evolution.
Acute hemorrhage (1–6 days) with intracellular deoxyhemoglobin appears
isointense on T1-weighted images and hypointense on T2-weighted images. As the
T1 relaxation time shortens (by methemoglobin) and RBCs break (> 7 days),
the signal intensity progressively increases, from the periphery to the
center, on both sequences [3].
A peripheral rim of dark signal intensity on all sequences is consistent with
the fibrous capsule or hemosiderin (Fig.
13A,
13B). Intramuscular
pseudotumor may show mural nodules at the capsular wall on MRI
[8] (Fig.
7A,
7B,
7C).
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Fig. 13A. —19-year-old man with hemophilia and iliopsoas pseudotumor.
Gadolinium-enhanced T1-weighted spinecho coronal image (TR/TE, 780/12) shows
intramuscular pseudotumor involving right psoas muscle, mimicking abscess.
Pseudotumor has double capsule lining of enhancing internal wall
(arrows) and dark signal outer wall (arrowheads).
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Fig. 13B. —19-year-old man with hemophilia and iliopsoas pseudotumor.
Fat-suppressed T2-weighted spin-echo axial image (3,500/120) shows
heterogeneous signal intensity surrounded by dark hemosiderin rim
(arrowheads).
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Conclusion
Hemophilic pseudotumors may develop in soft tissue, in the bone, or under
the periosteum, in descending order of frequency. These pseudotumors have
radiographically variable appearances that make some differential diagnoses.
Adjacent articular abnormality visualized on radiography and hemorrhagic
contents of variable stages as confirmed on CT and MRI are helpful in
diagnosing and differentiating from other conditions. Accurate knowledge of
the extent and character of pseudotumors, gained through sonography, CT, and
MRI, can be extremely useful in determination of proper management and in
follow-up assessment.
References
- Brant EE, Jordan HH. Radiologic aspects of hemophilic pseudotumor
in bone. AJR1972; 115:525
–539[Abstract]
- Resnick D. Diagnosis of bone and joint
disorders, 4th ed. Philadelphia: WB Saunders,2002
:2346–2373
- Hermann G, Gilbert MS, Abdelwahab IF. Hemophilia: evaluation of
musculoskeletal involvement with CT, sonography and MR imaging.
AJR 1992;158:119
–123[Abstract/Free Full Text]
- Hermann G, Yeh HC, Gilbert MS. Computed tomography and
ultrasonography of the hemophilic pseudotumor and their use in surgical
planning. Skeletal Radiol1986; 15:123
–128[Medline]
- Wilson DA, Prince JR. MR imaging of hemophilic pseudotumor.
AJR 1988;150:349
–350[Free Full Text]
- Magallon M, Monteagudo J, Altisent C, et al. Hemophilic
pseudotumor: multicenter experience over a 25-year period. Am J
Hematol 1994;45:103
–108[Medline]
- Stafford JM, James TT, Allen AM, Dixon LR. Hemophilic pseudotumor:
radiologic-pathologic correlation. RadioGraphics2003; 23:852
–856[Free Full Text]
- Jaovisidha S, Ryu KN, Schweitzer ME, Sartoris DJ, Resnick D.
Hemophilic pseudotumor: spectrum of MR findings. Skeletal
Radiol 1997;26:468
–474[Medline]
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Introduction
Osseous Pseudotumor
