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Volume 6
             Fibrous Tumors of Bone
Fibrous cortical defects------------Case 130-131 & 700-702
Non-ossifying fibroma-------------Case 132-133 & 703-713
Fibrous dysplasia
  Monostotic------------------------Case 134-135 & 714-748
  Polyostotic------------------------Case 136 & 749-757
  Mazabraud syndrome-----------Case 758-759
  Albright’s disease---------------Case 137 & 760-764
Desmoplastic fibroma-------------Case 138-139 & 765-771
Fibrosarcoma-----------------------Case 140-141 & 772-783
Malignant fibrous histiocytoma--Case 142-143 & 784-800
Leiomyosarcoma-------------------Case 144-45
Fibrous Bone
   Tumors
Fibrous Cortical
    Defects
Fibrous Cortical Defects
  The fibrous cortical defect is a very small metaphyseal lesion seen
in long bones, typically around the knee joint in children. They are
usually picked up on a routine radiograph as an incidental finding.
They are considered dysplastic or hamartomatous lesions and are
very common, with about a 25% incidence in normal children
around the age of four to five years. As the child matures, the
lesions will disappear by a process of skeletal remodeling and,
in most cases, the diagnosis is obvious based on a simple radio-
graphic study. Microscopically, the lesions are filled with benign
fibroblastic cells forming collagen and usually demonstrate no
macrophage or inflammatory activity.
CLASSIC
Case #130




16 year female
fibrous cortical
defect distal femur
Photomic
Case #131




18 year male
fibrous cortical defect
distal femur
Lateral view
Case #700




18 year male
fibrous cortical defect
distal femur
Lateral view
Bone scan
Case #701




 15 year male with fibrous cortical defect distal femur
Case #702




42 year female with fibrous cortical defect femoral neck
Frog leg lateral
Bone scan
CT scan shows bilateral lesions
Non-ossifying
  Fibroma
Non-ossifying Fibroma
  The non-ossifying fibroma is considered to be a larger form of the
fibrous cortical defect seen in the lower extremities of children.
The lesions are usually eccentrically located but the larger ones can
become more central. Radiographically, they have a soap-bubbly
appearance with slight thinning and dilitation of the adjacent
cortical structure. The diagnosis can usually be established by x-ray
alone without biopsy. These lesions are a common cause for path-
ologic fracture in children. They are usually asymptomatic until
the day of injury. There can be multiple lesions in the same patient,
similar to fibrous dysplasia, and can even be associated with café-
au-laite skin lesions.
   Histologically, the lesion consists of benign fibrous tissue
speckled with giant cells, histiocytes, and occasional foam cells
and even cholesterol deposits. Treatment usually consists of curette-
ment and bone grafting in high risk athletic males.
CLASSIC     Case #132




22 year female with non-ossifying fibroma proximal tibia
Close up lateral
Photomic showing giant cells
Post op x-ray after
bone grafting


                      fibular
                      strut
Case #133




12 year male
non-ossifying fibroma
distal tibia with
pathologic fracture
Case #703




16 year male
non-ossifying fibroma
distal tibia
Coronal T-2 MRI
giant cells




foam cells

    Photomic with foam cells and giant cells
Case #704




                                     stress fracture




    9 year female with non-ossifying proximal tibia
stress fracture




                  Lateral view
Coronal GRE T-2* MRI showing stress fracture
Coronal GRE T-2 MRI showing stress fracture
Case #705




11 year female
non-ossifying fibroma
tibia with path fracture
Case #706




13 year male
non-ossifying fibroma
distal tibia
Case #707




  18 year male with non-ossifying fibroma distal tibia
Case #708




9 year male
non-ossifying fibroma
distal fibula
Case #709




12 year male
non-ossifying fibroma
distal fibula
Case #710
                    xanthofibroma




34 year female
xanthofibroma and
ABC prox fibula


                                    ABC
Lateral view
Case #711




  17 year male with non-ossifying fibroma distal femur
Bone scan
Photomic
Case #712




5 year male
non-ossifying fibroma
femur with path
fracture
Case #713




16 year female
non-ossifying fibroma
femur with path fracture
Fibrous Dysplasia
Fibrous Dysplasia
    Fibrous dysplasia of bone is a relatively common hamartomatous
developmental process in the skeletal system that can be either
monostotic or polyostotic. In the case of polyostotic, there can also
be endrocrine abnormalities such as precocious puberty in females
with the McCune-Albright syndrome. This condition occurs
primarily in females and can be associated with café-au-laite spots
on the skin which have a rough serrated edge compared to the
smooth edges in neurofibromatosis. The monostotic form has an
appearance similar to non-ossifying fibroma except that the lytic
component has a typical smokey appearance caused by metaplastic
bone formation in the fibrous tissue. In the more severe polyostotic
form, representing only about 10% of fibrous dysplasia, the disease
tends to amplify on one side of the body more than the other
causing leg length discrepancy, especially when there is a character-
istic chronic deformity of the proximal femur; referred to in the
literature as the shepherd’s crook deformity; that frequently
requires surgical correction.
    The most common locations for fibrous dysplasia are the
proximal femur, followed by the tibia, humerus, ribs and,
occasionally, caranio-facial anatomy. Other endrocrinopathies
associated with polyostotic fibrous dysplasia include hyperpara-
thyroidism, acromegaly, Cushing’s disease and, occasionally,
hypophosphatemic osteomalacia. Occasionally, one will see soft
tissue myxomas with fibrous dysplasia which is referred to as the
Mazabraud’s syndrome. Radiographically, there will be diffuse
dysplastic changes throughout the involved bones with gradual
dilation and thinning of the cortices, and a soap-bubbly appear-
ance but with a smoky look compared to non-ossifing fibroma.
Histologically, there is evidence of benign fibrous tissue matrix
studded with islands of metaplastic bone with a so-called
alphabet soup appearance but without osteoblastic rimming of the
trabeculae. Occasionally one will see macrophages, osteoclasts,
foam cells and even cholesterol deposits similar to what is found
in non-ossifying fibroma. In approximately 1% of cases, there will
be a conversion to a secondary sarcoma in adult life that could
present as an osteosarcoma, or malignant fibrous histiocytoma or
even a chondrosarcoma.
   Treatment usually consists of corrective osteotomies for secondary
developmental deformities, along with bone grafting, including
fibular strut grafts and occasionally intramedullary devices to prevent
repeated stress fractures. Recently there has been enthusiasm for
the use of biphosphonates to inhibit the osteoclastic activity in
growing children that produces weakening of the bone. It is best
not to perform bone grafting procedures in children because of the
high recurrence rate in children under 16 years of age.
Monostotic
Fibrous Dysplasia
CLASSIC
Case #134




45 year female
monostotic
fibrous dysplasia
proximal tibia
Bone scan
Coronal T-1 MRI
Sagittal T-2 MRI
Photomic showing alphabet soup metaplastic bone
metaplastic     osteoclasts
bone




                              fibrous
                              tissue



              Close up photomic
Case #135




59 year female
monostotic fibrous
dysplasia humerus
Bone scan
Sagittal T-1 MRI
Case #135.1




24 year female with monostotic fibrous dysplasia humerus
Case #714




30 year male
monostotic fibrous
dysplasia femur
Lateral view
Coronal T-1 MRI
Photomic with metaplastic bone
Monostotic fibrous dysplasia
Case #714.1




                                                      Bone scan


       46 year male with mild stress pain right hip
Cor T-1   STIR
Sag T-1   PD   Gad
P.O. DHS and cancellous
allograph
Case #715




18 year female
monostotic fibrous
dysplasia prox femur
Bone scan
metaplastic bone




 Photomic
Case #716




21 year female
monostotic fibrous
dysplasia prox femur
Case #717




69 year female
monostotic fibrous
dysplasia femur
Lateral view
Bone scan
Coronal T-1 MRI
Coronal T-2 MRI
Photomic
Case #717.1




25 year male
monostotic fibrous
dysplasia femur
Post op x-ray
following surgical
correction of varus
deformity
Case #718




27 year male
monostotic fibrous
dysplasia femur
Lateral view
Case #719




47 year male
monostotic fibrous
dysplasia femur



Lateral view
Bone scan
Case #720




10 year female
monostotic fibrous
dysplasia proximal
femur with pathologic
fracture
Case #721




16 year female
solitary bone cyst
femoral neck
X-ray picture one
year following bone
grafting procedure
Then developes tibial pain because of fibrous dysplasia
Macro cyst seen in fibrous dysplasia beneath cortical window
cortical window




                              macro cyst
 Surgical specimen following curettement
Photomic with alphabet soup bone
Appearance following curettement
Placement of ipsilateral fibular bone strut
fibular strut


X-ray a year later
Case #722




38 year female
monostotic fibrous
dysplasia tibia
Close up view
Bone scan
Case #723




39 year female
monostotic fibrous
dysplasia tibia
CT scan
Case #724




 20 year female with monostotic fibrous dysplasia tibia
Coronal T-2 MRI
Axial T-2 MRI
osteoclasts



metaplastic bone




            Photomic
Case #725




 27 year male
 monostotic fibrous
 dysplasia tibia
Another view
Case #726




37 year male
monostotic fibrous
dysplasia fibula
Bone scan
Sagittal T-1 MRI
Sagittal T-2 MRI
Case #727




8 year female
monostotic fibrous
dysplasia humerus
with path fracture
X-ray one year later
with 2nd path fracture
CT scan
Case #728




37 year female
monostotic fibrous
dysplasia humerus
Lateral view
Bone scan
Coronal T-1 MRI
Post op x-ray    fibular
following bone   strut
graft
Case #729




45 year female with
monstotic fibrous dysplasia
humerus
Lateral view
distal humerus
Surgical debriedment of fibrous tissue
Photomic
Placement of IM nail prior to cementation
Cementation completed
nail



Post op x-ray
2.5 years later
Case #730




28 year female
monostotic fibrous
dysplasia humerus
Case #731




 36 year male with monostotic fibrous dysplasia scapula
Case #732




19 year female
monostotic fibrous
dysplasia prox radius
Sagittal T-1 MRI
Sagittal T-2 MRI
Case 733




14 year male
monostotic fibrous
dysplasia radius
Case #734                          Lateral view




 13 year female with monostotic fibrous dysplasia ulna
AP view
Case #735




 16 year female with monstotic fibrous dysplasia ulna
Case #736




11 year female
monostotic fibrous
dysplasia 1st
metatarsal
Case #737




 26 year female with monstotic fibrous dysplasia 3rd rib
Macro section of resected rib
Photomic
Case #738




39 year female
monostotic fibrous
dysplasia 12th rib

                     gall stones
Case #739




Monostotic fibrous
dysplasia 10th rib
in a 25 year female
Case #740




 46 year female with monostotic fibrous dysplasia pelvis
CT scan
3-D CT anterior oblique reconstruction
3-D CT posterior oblique reconstruction
Case #741




 14 year male with monostotic fibrous dysplasia pelvis
CT scan
Lower cut
Case #741.1




37 year female with
incidental finding on
X-ray of pelvis
Axial T-1         T-2 FS




            Gad
Cor T-1         T-2 FS




          Gad
Sag T-2 FS   Gad
Case #742




 22 year male with fibrocartilaginous dysplasia sacrum
Bone scan
Axial T-1 MRI
tumor




Axial T-2 MRI
Coronal T-1 MRI
tumor




Coronal T-2 MRI
cartilage              fibrous




                       bone



            Photomic
cartilage


fibrous




 Photomic
Photomic showing chondroid matrix
Case #743




 CT scan




23 year female with monostotic fibrous dysplasia L-spine
Another cut
Another cut
Case #744




 17 year female with monostotic fibrous dysplasia skull
Case #745




16 year male
monostotic fibrous
dysplasia maxilla
Skull x-ray
Photomic
Case #746




      Monostotic fibrous dysplasia frontal bone
Case #747




   22 year male monostotic fibrous dysplasia mandible
Case #748




  23 yr female Monostotic fibrous dysplasia mandible
Polyostotic
Fibrous dysplasia
CLASSIC
Case #136




25 year male
polyostotic fibrous
dysplasia
Photo of left face
involvement including
eye displacement from
orbital disease
Skull x-ray showing
changes left side of
skull and face
Shepherd’s crook
deformity proximal
femur
Macro section of
shepherd’s crook
deformity
Photomic showing alphabet soup metaplastic bone
Soap-bubbly
humeral defect
R                              L

Major changes left and minor changes right hand
Case #749                          Pelvis & hip x-ray




     25 year male with polyostotic fibrous dysplasia
tibia


X-ray femur and   femur   femur
tibia
Autopsy specimen of
femur cut in path lab
Macro section of
proximal femur
Photomic with punctate metaplastic bone
Case #750




32 year male with
polyostotic fibrous
dysplasia
Views front and back
Shepherd’s crook
deformity femur
Skull deformities
feet
Hand and foot
X-rays




                hands
Case #751




20 year female
polyostotic fibrous
dysplasia
Side view
path fracture




X-rays hand and humerus
AP x-ray pelvis and hips with path fracture femoral neck
Leg x-ray with
varus deformity
Case # 752

                  large café-au-lait spot




     29 year male with polyostotic fibrous dysplasia
AP x-ray showing
extensive thinning
and dilatation of
tibial cortex
Lateral view
Amputation specimen cut in path lab
Photomic
Case #753




58 year male with
polyostotic fibrous
dysplasia of femori
And feet
Femoral head specimen from total hip surgery
Photomic
Post op x-ray with
long stem femoral
component
Lateral view showing
old healed path
fracture
Opposite hip with
similar defects




                    L
Involvement of both feet
Case #753.1




     31 year female with mid thigh pain for 1 year
Bone scan
Coronal T-1   Coronal T-2
Coronal T-1   Coronal T-2
Axial T-1   Axial T-2
Case #753.2                 Polyostotic fibrous dysplasia




 16 year male with deformity of proximal thumb for years
X-rays of right upper extremity
Post op x-ray following curettement and bone grafting
Case #754




    34 year female with polyostotic fibrous dysplasia
Case #755




19 year male
polyostotic fibrous
dysplasia with
shepherd’s crook
deformity femur
Skull involvement
L                           R
    Changes in right hand more than left
Case #756




26 year male
polyostotic fibrous
dysplasia left hip
Case #756.1




 40 yr male with fibrous dysplasia humerus and prox ulna
 with prior surg 20 yrs ago and now aneurysmal changes
Sagittal T-1 MRI   Sagittal T-2 MRI
Coronal T-1   Coronal STIR   Coronal Gad
                 MRI
Case #757




28 year female
polyostotic fibrous
dysplasia LS spine
Oblique views LS spine
Lateral view
Sagittal T-1 MRI
Axial T-1 MRI sacrum
Sagittal T-2 MRI with cystic changes
Axial T-2 MRI with fluid-fluid level in sacrum
cyst




Axial gad contrast MRI with rim enhancement
Sagittal gad contrast MRI with lumbar cysts
Photomic from sacral biopsy
Mazabraud
Syndrome
Case #758



39 year female
Mazabraud syndrome
polyostotic fibrous
dysplasia with soft
tissue myxomas


CT skull
fibrous dysplasia




                             myxoma


myxoma

           Coronal T-1 MRI
Coronal T-2 MRI showing high signal myxomas
Case #759




     fibrous dysplasia




       62 year female with Mazabraud’s syndrome
AP shoulder
Bone scan
Bone scan skull
Bone scan femur
fibrous




Coronal T-1 MRI showing multiple myxomas
fibrous
Coronal T-2 MRI
showing high signal
myxomas
fibrous




Axial T-1 MRI with low signal myxomas
fibrous




Axial T-2 MRI with high signal myxomas
Coronal T-2 MRI with high signal myxomas
Albright’s Disease
CLASSIC
Case #137




7 year female
Albright’s disease
X-ray at 13 years
minimal changes of
fibrous dysplasia
More changes at 18 years
Stress symptoms in both hips at age 24
Close up right hip
age 24
cancellous graft




Post op x-ray with fibular struts in both femoral necks
Cibagram showing the
difference between the
rough serrated edges of
the café-au-lait spots of
fibrous dysplasia on top
compared to the smooth
edges of neurofibromatosis
seen on the bottom
Case #760




            35 year female with Albright’s disease
Close up left hip
Frog lateral left hip
R                                             L


    CT scan shows more disease on left side
Café-au-lait spot in left axilla
Photomic from proximal femoral biopsy
fibrous




Higher power showing metaplastic bone
Case #760.1




       40 year female a short left leg and early menarchy
Case #761




27 year female
Albright’s disease
humerus
Shepherd’s crook
deformity proximal
femur
Post op x-ray showing
attempt to correct the
varus deformity
X-ray 1 year later with
failure of fixation due
to nail cut out
Side plate on left tibia
to reduce stress pain
One year later with new DHS fixation to replace failed nail
Case #762




   10 year female with Albright’s of pelvis and spine
Coronal CT of LS spine
Sagittal CT scan
Axial CT scan
Sagittal T-1 MRI LS spine with multi level disease
Axial T-2 MRI
of LD spine
Case #763




                                      mandibular lesion


            37 year female with Albright’s disease
View of opposite side of mandible
AP pelvis
IM nail for stress
pain
Case #764




64 year female
Albright’s disease
with stress pain
proximal femur
Bone scan
Post op bipolar
prosthesis
Desmoplastic
Fibroma of Bone
Desmoplastic Fibroma of Bone
    Desmoplastic fibroma of bone is an extremely aggressive, but
benign, fibrous lesion seen in long bones as well as the mandible.
It represents 1% of all bone tumors and occurs typically in children
and young adults with equal sex distribution. The long bones
involved include the distal femur, proximal tibia, proximal
humerus and the lower half of the pelvis. Radiographically, these
lesions have an aggressive and moth-eaten appearance, eroding
into cortical bone and breaking out into the adjacent soft tissue,
much like the low grade fibrosarcoma. Histologically, the tumor
appears identical to aggressive fibromatosis seen in soft tissue with
intense collagen formation by aggressive-appearing fibroblasts with
an occasional mitotic figure. With curettement alone, the recurr-
ence rate is very high, in the neighborhood of 40%, and for this
reason most authorities suggest a wide resection and possible
prosthetic replacement. This tumor will not metastasize to the lung.
CLASSIC
Case #138




27 year female
desmoplastic fibroma
distal femur
Lateral view
Photomic
Case #139




13 year female
desmoplastic fibroma
proximal fibula
Lateral view
Coronal T-1 MRI
Coronal T-2 MRI
Proximal fibular resection specimen
growth plate




               Cut specimen in path lab
Case #765




   25 year female with desmoplastic fibroma prox tibia
Lateral view
Bone scan
CT scan
Another CT scan
Coronal T-1 MRI
Axial PD MRI
Post op x-ray following
curettement and
cementation
Lateral view
Case #766




25 year female
desmoplastic fibroma
proximal femur
Post op x-ray following
curettage, IM nail and
bone graft
X-ray 3 years later
and nail removed
Case #767




28 year male
desmoplastic fibroma
distal femur
6 months later with
progression of disease
requiring distal femoral
resection and TKA
Lateral view
Case #768




19 year female
desmoplastic fibroma
distal femur
Case #769




  30 year male with desmoplastic fibroma distal radius
Lateral view
Photomic
Case #770




  6 year male with desmoplastic fibroma distal radius
Case #771




  22 year female with desmoplastic fibroma prox ulna
Fibrosarcoma of
     Bone
Fibrosarcoma of Bone
   Fibrosarcoma of bone is a malignant spindle cell sarcoma seen in
an older age group with a peak incidence in the fourth decade of
life. It is ten times less frequent than osteosarcoma but involves
similar bones including the distal femur, proximal tibia, pelvis,
proximal femur and proximal humerus. It is rare in the spine, hand
or foot. Radiographically, the fibrosarcoma has a permeative lytic
appearance in the metaphyseal ends of long bones, similar to that
seen in lymphomas or malignant fibrous histiocytoma. Histo-
logically, the tumor demonstrates aggressive fibroblastic activity
with collagen formation similar to what is seen in desmoplastic
fibromas but with a much higher mitotic index. The lesions can be
low grade or high grade. The low grade lesions carry a good
prognosis with a low incidence of metastasis to the lung, whereas
the high grade lesions, just like with osteosarcoma, are more
aggressive with a worse prognosis and a higher potential for
metastasis. Treatment consists of wide local resection of the tumor,
if possible. If the lesion is high grade, adjuvant therapies including
local radiation therapy and chemotherapy are frequently implemented
to reduce the chance of pulmonary metastasis. Limb salvage
prosthetic reconstructions are frequently utilized as in the case of
osteosarcoma.
CLASSIC
Case #140




61 year male
pathologic fracture thru
fibrosarcoma distal femur
Lateral view
3 months later after
ORIF not knowing
this was a sarcoma     tumor
Another 3 months
go by with continued
tumor growth
Photomic from biopsy and amputation 6 months out
Case #141




     29 year female with fibrosarcoma 5th metatarsal
Case #772




26 year male
fibrosarcoma
distal femur
Cut specimen in path
lab after amputation
Macro section


                tumor
Photomic
Case #773




     44 year female with fibrosarcoma femoral neck
Cut specimen in path lab after internal hemipelvectomy
Macro section   tumor
Photomic
Case #774




     43 year female with fibrosarcoma femoral neck
Laminogram cut
Marginal resection of head and neck cut in path lab
Photomic
1 year post op with
bipolar hip recon
Case #775




62 year female
path fracture prox femur
thru primary fibrosacoma
thought to be metastatic
breast disease
Post op x-ray following
ORIF at which time the
unexpected biopsy
diagnosis of primary
sarcoma was made
Surgical specimen
after wide resection
entire proximal femur
with fixation devise
Specimen cut in
path lab

                  tumor
Photomic
Proximal femoral recon with custom bipolar arthroplasty
Post op x-ray
Case #776




      48 year female with fibrosarcoma distal femur
Resected distal
femur cut in path lab




                        tumor
Photomic
Post op x-ray with
custom prosthesis
Case #777




42 year female
fibrosarcoma prox tibia
2 years later
4 more years with
final diagnosis and
amputation
Lateral view
Photomic
Pulmonary met 4 years after amputation
met




met



      Autopsy photo of multiple lung mets
Case #778




64 year male
fibrosarcoma
proximal humerus
Case #779




73 year male
fibrosarcoma
distal humerus
Lateral view
Case #780




   69 year female with fibrosarcoma proximal ulna
AP view
tumor




 CT scan
tumor

radial
head




Proximal radius and ulna resection with custom implant
Photomic
Prosthesis in place ready for skin closure
median nerve




               radial nerve



   Another view of implant prior to closure
Post op x-ray
Case #781




                     tumor




       20 year female with fibrosarcoma sacrum
Case #782




21 year male
fibrosarcoma
mandible
Case #783




38 year female
fibrosarcoma
arising from monostotic
fibrous dysplasia
proximal tibia
Previous fibrous
 dysplasia




new fibrosarcoma
Lateral view
Malignant Fibrous
 Histiocytoma of
       Bone
Malignant Fibrous Histiocytoma of Bone
   Malignant fibrous histiocytoma of bone is a relatively new patho-
logical entity in bone tumors, compared to soft tissue tumors
where malignant fibrous histiocytoma is a more common tumor.
Malignant fibrous histiocytoma (MFH) of bone occurs in a middle
or older age group, more commonly in males than females. It is
found typically in the same locations as fibrosarcoma or osteo-
sarcoma, including the distal femur, proximal tibia, and proximal
humerus. Radiographically, it is an aggressive permeative lesion
in metaphyseal bone with significant cortical destruction that can
break out into the adjacent soft tissue. Histologically, there is
evidence of malignant fibroblasts as well as atypical histiocytes
and giant cells, exactly as we see in soft tissue MFH. Because this
is usually a high grade tumor, the prognosis is quite guarded with
a high potential for metastasis to the lung, similar to fibrosarcoma
and osteosarcoma. Treatment consists of a wide surgical resection
with prosthetic reconstruction when possible, along with
adjuvant chemotherapy to reduce the chance of pulmonary
metastases.
CLASSIC
 Case #142




26 year male with path
fracture thru MFH
femoral neck
Coronal T-1 MRI
Coronal T-2 MRI
Axial T-1 MRI
Cut femoral head in path lab
Photomic
Case # 143




 50 year female
 MFH distal femur
Sagittal T-1 MRI
tumor



  tumor




Axial proton density MRI
Photomic
Post op x-ray
custom TKA
Lateral view prox
end of implant
Case #143.1                        Multifocal MFH




     37 year male with painful lump behind knee for 3 months
Cor T-1         T-2




          Gad
Axial T-1   T-2




Gad
1906




Cor T-2          Axial T-2
tumor




Wide surgical resection and rotating hinge recon
Post op X-ray
Case #784




 27 year female
 MFH proximal femur
Surgical incision to include biopsy site over trochanter
biopsy




Proximal femoral resection with biopsy site
Proximal femoral resection cut in path lab
Photomic
Custom proximal femoral resection bipolar prosthesis
Ready for abductor attachment
Closure soft tissue flaps
Abductor tendon sutured to implant
Post op x-ray
10 years later with
stem loosening due to
stress shielding
X-ray 2 years after
revision with the
Compress system to
avoid further stress
shielding
5 years out showing
hypertrophic bony
response at the bone
spindle interphase
Case #785




27 year female
MFH distal femur
Lateral view
X-ray of cemented
stem of custom
implant soon after
surgery
11 years later with
stem loosening from
stress shielding
4 years after revision
surgery with stem
fracture which was
revised to a Compress
system which became
infected resulting in
amputation
Case #786




30 year female
MFH distal femur
Bone Scan
Coronal T-1 MRI
Coronal gad contrast MRI
Another coronal gad contrast MRI
Case #787




        35 year male with MFH femoral head
Frog lateral
CT scan
Bone scan
Case #788




37 year male
path fracture thru
MFH proximal femur
Bone scan
Biopsy site at time of surgical resection
biopsy site




Proximal femoral resection specimen
Tumor site uncovered on back table
Photomic
Custom proximal femoral bipolar prosthesis ready for implant
Post op x-ray
Case #789




  49 year male with path fracture thru MFH prox femur
Bone scan
Post op x-ray after
ORIF for what the
surgeon thought was
a metastatic tumor
Surgical specimen
after wide resection
proximal femur with
entire fixation device
for primary sarcoma
Photomic
Reconstruction with
custom proximal
femoral bipolar
prosthesis
Case #790




     36 year male with myxoid MFH distal femur
Lateral view
Oblique view
CT scan
Bone scan
Coronal T-1 MRI
Sagittal T-1 MRI
Sagittal T-2 MRI
Case #791




75 year male
MFH patella
CT scan
tumor




Soft tissue window CT scan
Axial T-1 MRI
Sagittal T-1 MRI
Case #792




34 year male
MFH proximal tibia
biopsy
                                site




Wide resection proximal tibia
Custom long stem
spherocentric TKA
components
Post op x-ray after
autoclaving the surgical
specimen and replacing
it over a long stem
tibial component
Case #793




                        infarct
36 year female
MFH at site of a
previous bone infarct
infarct




     Different rotation
glenoid


Wide resection
proximal humerus


                   biopsy
Resected surgical
specimen            biopsy
Photomic
Close up
Autoclaved specimen
cemented over long
stem Neer prosthesis
Reimplanted
Post op x-ray
11 years later
with proximal migration
and loss of autoclaved
bone
Case #795




               tumor




            41 year female with MFH pelvis
tumor




Coronal T-1 MRI
tumor




        Axial T-1 MRI
Case #795.1




         54 year old male with right hip pain for 3 months
CT scan
Bone scan
Cor T-1         STIR




          Gad
Axial T-1   T-2
Chest CT
Case #796




43 year female
MFH pelvis
Case #797




75 year female
MFH sacrum
                 tumor
Case #798




   43 year female with multifocal MFH tibia and radius
Sagittal T-1 MRI
Coronal T-2 MRI
Distal radius
Case #799




55 year male
metal associated
MFH with path
fracture at tip of
femoral component
Close up x-ray
showing permeative
lysis at fracture site
OR x-ray showing cortical lysis at fracture site
Biopsy photomic at time of surgery
Immediate post op
X-ray
X-ray several weeks
later


                      tumor
Case #800




84 year male
metal associated MFH
around femoral
component THA          loose stem
X-ray 3 months after
revision surgery
3 months later we see
cortical lysis medially
5 months after another
revision surgery with
extensive cortical
lysis 2nd to MFH
More lysis at tip
of stem from MFH



Hemipelvectomy
was carried out
and died soon after
with pulmonary mets
Leiomyosarcoma
    of Bone
Leiomyosarcoma of Bone
    Leiomyosarcoma of bone is an extremely rare diagnosis with
fewer than 50 cases in the world literature. It occurs typically in
middle to older age adults in the same location in the skeleton as
fibrosarcoma, MFH, and osteosarcoma are found in; distal femur,
proximal tibia and upper humerus. Radiographically, these lesions
have a permeative lytic appearance, similar to a lymphoma,with
extensive cortical breakdown and extension into the surrounding
soft tissue. Histologically, the malignant spindle cells with collagen
formation look like a fibrosarcoma or MFH so that the only way
a diagnosis can be established is by using the specific immuno-
histochemical staining for smooth muscle protein. Treatment usually
consists of wide surgical resection and prosthetic implantation.
Because of the poor prognosis with high grade lesions, they are
frequently treated with adjuvant systemic chemotherapy and
radiation which is not very effective, especially in old patients.
CLASSIC Case #144




 69 year female with leiomysarcoma proximal humerus
Rotational view
Bone scan
Surgical photo ready for wide resection including biopsy site
tumor bulge

                       humeral
cut
                       head




                              glenoid

      Wide resection proximal humerus
cut




Surgical defect ready for reconstruction
Resected specimen
Specimen cut
in path lab
Photomic
Custom long stem
Neer prosthesis in
position
Cement reconstruction
around proximal stem
Immediate post op
X-ray


                    cement
Case #145




46 year female
leiomyosarcoma
proximal humerus
Coronal proton density MRI

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Volume 6