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Volume 8
Round Cell Tumors of Bone
Ewing’s sarcoma-----------------Case 169-175 & 861-900
Large cell lymphoma------------Case 176-178 & 901-933
Hodgkin’s lymphoma-----------Case 179-180 & 934-936
Leukemia-------------------------Case 181 & 937-944
Plasma cell tumors
Solitary plasmacytoma-------Case 182-185 & 946-957
Multiple myeloma------------Case 186-188 & 958-976
Metastatic neuroblastoma------Case 189 & 977-983
Round Cell
Tumors Of Bone
Ewing’s Sarcoma
Ewing’s Sarcoma
The Ewing’s sarcoma is the second most common primary
malignant bone tumor seen in children and is the fourth most
common malignant tumor overall. This tumor is a very primitive
mesenchymal sarcoma that has a mysterious etiology. However,
with the advent of the electron microscope and immunohisto-
chemical studies, most experts today feel that this tumor probably
represents a poorly differentiated member of a larger family of
neural tumors, distinct from the neuroblastoma. In 90% of cases,
cytogeneticists will find reciprocal translocation in chromosome
11 and 22. That also is found in patients with the diagnosis of
primative neuroectodermal tumor (PNET) and Askin’s tumor.
Other round cell tumors that have a similar histological appearance
include the embryonal rhabdomyosarcoma, the mesenchymal
chondrosarcoma, and the metastatic neuroblastoma. It is very
important to separate out the large group of histiocytic lymphomas
seen in an older age group that have a similar appearance with
H & E staining. With special staining techniques, most lymphomas
will be positive for leukocyte antigen. Likewise with metastatic
embryonal rhabdomyosarcoma, specific immunohistochemical
studies will reveal muscle markers such as actin, desmin, and
myoglobin not found in the Ewing’s sarcoma.
Ninety percent of patients with Ewing’s sarcoma will be from 5
to 25 years of age, with males being affected slightly more than
females. The two most common locations for Ewing’s sarcoma are
the femur and pelvis, followed by the tibia, humerus and scapula,
but it can be found in any location in the body that includes
myelogenous tissue, including the spine, ribs, foot and hand. Radio-
graphically, the Ewing’s tumor will be found typically in a meta-
diaphyseal location in the medullary canal with very diffuse,
permeative lytic destruction of the surrounding cortical structures
that looks like hematogenous osteomyelitis. Likewise, the clinical
appearance of Ewing’s sarcoma can mimic infectious disease
with elevated temperature, white count and sed rate. Because of the
high incidence of necrosis with the Ewing’s sarcoma, it is common
to find liquefied necrotic debris in the tumor site that gives the
clinical appearance of osteomyelitis. With early breakthrough into
the subperiosteal tissues, the radiographic finding of a reactive
periostitis is quite common, creating a multilaminated “onionskin”
appearance on a routine radiograph. Another characteristic radio-
graphic finding is the “hair-on-end” appearance that is created
by reactive new bone formation along the perpendicular periosteal
blood vessels running between the periosteum and the subadjacent
cortex.
Prior to 1970, the prognosis for survival in Ewing’s sarcoma was
extremely poor with approximately 10% of patients surviving their
disease. Currently with the use of adjuvant systemic chemotherapy
in non-metastatic Ewing’s sarcoma, the survival prognosis now runs
approximately 70%. However, in about 20% of patients with
Ewing’s sarcoma that present with metastatic disease to other bones
or to the lung, the survival rate drops to about 30%. Whenever
possible, the orthopedic oncologist will attempt a wide resection of
the primary tumor site, a technique similar to that used for osteo-
sarcoma. If wide surgical margins are obtained, the chances for
survival is probably better than if radiation therapy and chemo-
therapy had been used without surgery. However, in cases where
the surgical margins are positive at the time of surgical resection,
postoperative radiation therapy is indicated. The chance for local
recurrence with chemotherapy and radiation therapy alone is 20%
or higher. A devastating complication of radiation therapy is path-
ological fracture that frequently results in intramedullary fixation
that may fail and can result in amputation. Secondary sarcomas can
occur with the use of radiation therapy for Ewing’s sarcoma in 10%
of cases. The primative neuroectodermal tumor accounts for 10%
of all Ewing’s-like tumors and carries the same prognosis for
survival. The clinical management for this entity is essentially the
same as for Ewing’s sarcoma.
CLASSIC
Case #169
13 year female
Ewing’s sarcoma
distal femur
onion skin
periostitis
Bone scan
Coronal proton
density MRI
Coronal T-2 MRI
Coronal T-2 MRI
showing multifocal
disease
Macro section Ewing’s
sarcoma distal femur
necrotic
viable
Photomic showing pseudo-rosette formations
Same patient with
multifocal involvement
proximal humerus
Case #170
15 year female
Ewing’s sarcoma
proximal tibia
Lateral view
Sagittal T-1 MRI
Axial T-1 MRI
Photo of resected proximal tibia & prosthesis
Compress
system
Placement of rotating
hinge Compress prosthesis
Attachment of
patellar ligament with
double tooth washers
X-ray 2 mos post op
anchor plug
5.5 years post op
with excellent
osseointegration at
bone-prosthetic
interface
11 years post op
Case #171
19 year female
Ewing’ sarcoma
proximal femur
Bone scan
1 year later with
path fracture thru
radiated bone
Persistent non union
after IM nailing
X-ray 1 year post op
placement of cemented
long stem custom
bipolar prosthesis
Case #172
13 year male with
“hair-on-end”reactive
subperiosteal new
bone formation
permeative
lysis
Macro section resected
proximal femur
Closeup macro section
showing “hair-on-end”
reactive subperiosteal
new bone
necrosis
periostium
permeation
Closeup macro section
“hair-on-end” subperiosteal
reactive bone formation
periostium
Photomic Ewing’s sarcoma
Case #173
15 year male Ewing’s
sarcoma femur
hair-on-end
onion skin
Case #174
28 year male with Ewing’s sarcoma pelvis
CT scan
tumor
Case #175
14 year male with Ewing’s sarcoma pelvis
Macro section from
autopsy specimen
acetabulum
ilium
tumor
Photomic
Case #861
34 year female with Ewing’s sarcoma pelvis
Close up hip
Coronal Gad contrast MRI
Coronal T-2 MRI
Axial T-1 MRI
Axial proton density MRI
Photomic
Case #862
28 year male with Ewing’s sarcoma pubis
tumor
Coronal T-1 MRI
Coronal T-2 MRI
tumor
T-2 MRI
tumor
Coronal T-2 MRI
tumor
Axial PD MRI
tumor
Photomic
Axial PD MRI following successful chemotherapy
Coronal T-2 MRI post chemo
Case #863
10 year female with Ewing’s sarcoma pelvis
6 months later
Coronal T-1 MRI
Coronal T-2 MRI
Case #864
19 year male with Ewing’s sarcoma SI area
Axial gad contrast MRI
Axial T-2 MRI
Photomic
Post op cementation
Case #865
19 year male
Ewing’s sarcoma
sacrum
Close up
Myelogram showing
nerve root pressure
CT scan
tumor
Photomic
Case #866
12 year male
Ewing’s sarcoma
L-5
Oblique view
Case #867
37 year male
Ewing’s sarcoma
proximal femur
Coronal T-1 MRI
Photomic
X-ray allograft
prior to implantation
AP lateral
Post op alloprosthetic
reconstruction
Case #868
14 year male
Ewing’s sarcoma
mid femur
Bone scan
Pre chemo
Coronal proton
density MRI
Axial PD MRI pre chemo
Photomic
Axial PD MRI post chemo
Amputation following good chemo response
Post op x-ray with modified bipolar reconstruction
cement
Case #869
21 year male with Ewing’s sarcoma pelvis and femur
Coronal T-1 MRI
Coronal T-2 MRI
tumor
Coronal T-2 MRI
Case #870
7 year male
Ewing’s sarcoma
distal femur
Bone scan
Sagittal proton
density MRI
Axial T-1 MRI
tumor
Photomic
Axial T-2 MRI post chemo
Coronal T-2 MRI post chemo
Case #871
13 year male
Ewing’s sarcoma
femur
5 years after
radiation & chemo
with recurrence &
path fracture
Post op x-ray with
IM nail for path
fracture
Total femur replacement specimen
tumor bulge
Custom total femur replacement prosthesis
Placement of custom prosthesis
Post op X-ray
Case #872
16 year male
Ewing’s sarcoma
proximal femur
Lateral view
Coronal T-1 MRI
Coronal T-2 MRI
Axial proton density MRI
Case #873
9 year male with Ewing’s sarcoma tibia
Bone scan
Sagittal T-1 MRI T-2 MRI
Post op reconstruction
allograft over IM nail
8 years later
Case #875
2.5 year male
Ewing’s sarcoma
distal tibia
onion skin laminated periostitis
Lateral view entire tibia
Coronal T-1 MRI
Axial T-1 Gad contrast MRI
Case #876
17 year male with
Ewing’s sarcoma tibia
Different view
Post op alloprosthetic
reconstruction allograft
Multifocal lesion prox
femur 6 mos later
Case #877
19 year female with
Ewing’s sarcoma
proximal humerus
tumor
hair on
end
Post op alloprosthetic
reconstruction
Case #878
39 year male
Ewing’s sarcoma
proximal humerus
X-ray one year later
without treatment
Photomic
Case #879
10 year male with Ewing’s sarcoma scapula
Scapular view
Bone scan
CT scan
Special CT scan scapula
Case #880
9 year male with Ewing’s sarcoma scapula
Case #881
20 year male with Ewing’s of scapula
Special soft tissue technique x-ray
Soft tumor
Case #882
16 year male with Ewing’s sarcoma clavicle
Case #883
16 year female with Ewing’s sarcoma clavicle
Case #884
7 year female with Ewing’s sarcoma 3rd posterior rib
Case #885
17 year female with Ewing’s sarcoma post 7th rib
Case #886
11 year male with Ewing’s sarcoma rib
Case #887
12 year female
Ewing’s sarcoma
fibula
Macro section from
resected specimen
Scanning lens
photomic
Higher power
Case #888
20 year female
Ewing’s sarcoma
distal fibula
Axial T-2 MRI
tumor
Case #888.1 Ewing’s Fibula
15 yr male with pain and swelling of leg for 3 months
Cor T-1 T-2 Gad
Axial T-1 T-2
Gad
Case #889
15 year female
Ewing’s sarcoma
proximal fibula
Case #890
18 year male
Ewing’s sarcoma
distal fibula
Bone scan
Case #891
17 year male with Ewing’s sarcoma 4th metatarsal
Coronal T-1 MRI
Axial T-1 MRI
tumor
Axial T-2 MRI
tumor
Photomic with pseudo-rosettes
CT scan thoracic spine shows multi focal lesion
Coronal CT scan
Case #892
35 year female with Ewing’s sarcoma os calcis
Several months later
tumor
T-1 MRI
tumor
Gad contrast MRI
tumor
Case #893
21 year male
Ewing’s sarcoma
mid tarsal area
Case #894
20 year male
Ewing’s sarcoma
2nd toe
Case #895
5 year female
Ewing’s sarcoma
proximal ulna
Lateral view
Axial T-1 MRI
Sagittal T-1 MRI
Coronal T-2 MRI
Photomic
Case #896
50 year female with Ewing’s sarcoma proximal ulna
Case #897
2 year old with Ewing’s
sarcoma middle finger
Lateral view
Sagittal T-1 MRI
Photomic
Case #898
15 year male with Ewing’s sarcoma thumb
Case #898.1
18 year male with lateral knee knee for 3 months
Parosteal Ewing’s sarcoma
Bone scan
Axial T-1 Axial Gad C+
Coronal STIR Gad C+ Sagittal Gad C+
Case #898.2
13 year male with painful mass in forearm for 3 months
Parosteal Ewing’s sarcoma
Bone scan
Cor T-1 T-2 FS
Sag T-1 Gad
Axial T-2 Gad
Post chemo T-2 Gad
Case #898.3
27 yr female with firm tender mass in upper medial arm for 2 mos.
Parosteal Ewing’s Sarcoma Humerus
Axial T-2 Gad
Cor T-1 T-2 Gad
Ewing’s Sarcoma
Pseudotumors
Case #899
11 year female
osteomyelitis distal femur
looking like Ewing’s
sarcoma
onion skin
periostitis
sclerosis
Lateral view
Bone scan
Coronal T-2 MRI
Axial T-2 MRI
Photomic of reactive periostitis
Photomic showing inflammatory cells
polys
lymphs
Case #899.1
3/06 7/07 7/07
14 year old male with pain right arm for 17 months
Osteomyelitis Ewing’s pseudotumor
Cor T-1 T-2 Gad
Axial T-1 T-2 Gad
Case #900
8 year male with acute
fracture thru prior
femoral stress fracture
Day of fracture
stress
periostitis
3 days after acute fracture
Stress
periostitis
At 3 weeks looking
like Ewing’s sarcoma
6 mos later with solid
union unlike Ewing’s
sarcoma
Case #900.1
16 yr male with forearm pain
for 6 weeks
Axial T-2
Gad
Stress Periostitis Radius
Case #900.2 Stress Periostitis Femur
10 yr male with pain in thigh for 2 months
May June
Cor T-1 T-2 Sag T-2
Axial T-2
Axial CT
Case #900.5
Eosinophillic granuloma
fibula looking like
Ewing’s sarcoma in a
5 year male
Lymphoma of
Bone
Lymphoma of Bone
Lymphoma of bone accounts for approximately 7% of all primary
malignant tumors of bone and can be divided roughly into two
clinical groups: Hodgkin’s lymphoma of bone and non-Hodgkin’s
lymphoma of bone. Hodgkin’s lymphoma carries a much better
prognosis for survival. It tends to be localized and presents with a
radiographic appearance that frequently includes a dense, sclerotic
response. The non-Hodgkin’s lymphomas can be divided into
two categories. The systemic form, the most common form with
generalized involvement of the entire lymphatic system including
soft tissue and bone, carries a poor prognosis. The less common
form that is found in bone and does not have systemic manifestations
carries a better prognosis for survival. To meet the strict criteria for
a primary lymphoma of bone, the disease must be contained within
the skeletal system for at least six months before becoming
disseminated to other lymphatic organs such as lymph nodes and
spleen, at which time the prognosis for survival becomes much
worse. The age group for primary lymphoma of bone is between
25 and 50 years. The most common bones involved are the spine
and pelvis in 50% of cases. In the extremities, the most common
location is the femur followed next by the humerus and tibia with
multiple skeletal sites involved in approximately 25% of cases.
Radiographically, the primary lymphoma takes on a permeative
lytic appearance in cortical bone in a metadiaphyseal location,
but can also involve epiphyseal bone as well. Because of the
permeative nature of the bone destruction, pathologic fractures are
common, similar to the situation with Ewing’s sarcoma. In both
primary lymphoma and Ewing’s sarcoma, large soft tissue masses
can be found. With H&E staining, the histological appearance
of lymphoma and Ewing’s sarcoma can be quite similar but with
specific immunohistochemical staining techniques, B cell and T cell
subtypes of lymphoma can be identified and clearly separated
from Ewing’s sarcoma. As in the case of Ewing’s sarcoma, the
advent of chemotherapy dramatically improved the prognosis for
survival that, for a solitary primary lymphoma of bone, is similar to
Ewing’s sarcoma with a 70% five year survival. However, with more
disseminated involvement of multiple bones or other lymphatic
organs, the prognosis drops dramatically to 10-25%. As with Ewing’s
sarcoma, the lymphoma of bone is quite sensitive to external beam
radiation therapy. However, the complications (including path-
ological fracture) are a problem and the orthopedic oncologist will
attempt a wide resection of the tumor if possible or, in more extensive
involvement, an intralesional approach with a long stem cemented
prosthesis with adjuvant bone cement may be indicated to avoid
pathological fracture. In cases of systemic involvement with extensive
metastatic disease, patients can be considered for bone marrow
transplantation. The same holds true for Ewing’s sarcoma.
CLASSIC
Case #176
45 year male
lymphoma proximal
femur
path fracture
Frog leg lateral
Bone scan
signal void
Coronal T-1 MRI
tumor
Coronal T-2 MRI
tumor
Axial T-2 MRI
High power photomic showing folded nuclear forms
Case #176.1
47 yr old male smoker with 4 mo history of left hip pain
Large cell lymphoma
Bone scan
Coronal
T-1 T-2
Axial
T-1 T-2
Post op recon nailing
Case #176.2
69 yr female with painful hip for 3 months
Large cell lymphoma
Cor CT scan Axial
Case #177
83 year female with lymphoma right pelvis
6 months later with pathologic fracture
Another 6 mos later
Bone scan
Photomic
Case #178
40 year female
lymphoma lower
spine
Sagittal T-2 MRI
Sagittal T-2 MRI
upper spine
Sagittal T-2 MRI
mid spine
Case #178.1
72 year male with LBP for many years
Lymphoma sacrum
Bone scan
Axial T-1 MRI
T-2 Gad
Sagittal
T-1
T-2
STIR
Gad
Case #901
17 year male with lymphoma acetabulum
Coronal T-1 MRI
tumor
Axial T-1 MRI
tumor
Axial T-2 MRI
tumor
Photomic
Good response from
chemotherapy
Case #902
49 year male with lymphoma pelvis
CT scan
tumor
Case #902.1
69 year male with 2 month history of left hip pain
Lymphoma of pelvis
Coronal T-1 T-2 Gad C+
tumor
Sagittal T-1 T-2 Gad
Axial
T-1
T-2
Gad
Case #903
44 year male with
lymphoma pelvis
Case #904
31 year male with lymphoma pelvis
Bone scan
Case #905
40 year male
lymphoma pelvis
Case #906
47 year male
lymphoma
distal femur
Lateral view
Bone scan
6 months later
Sagittal T-1 MRI
Sagittal T-1 MRI thru notch
Sagittal T-2 MRI
tumor
Axial T-2 MRI
tumor
Photomic
Case #906.5
49 year male with lymphoma distal femur
Lateral view
Bone scan
Sagittal T-1 MRI
Coronal T-1 MRI
Coronal STIR MRI
tumor
Case # 906.6
48 year female with medial knee pain 1 year and history of
torn medial meniscus & 18000 WBC & elevated cholesterol
Large cell lymphoma knee
Bone scan
Sag T-1 Cor T-2
Axial PD
Femoral cut Tibial cut
Case #906.7 Bone scan
58 year male with painful swelling above knee for 3 months
Lymphoma
Cor T-1 T-2 FS
Axial PD T-2
Sag PD T-2
Case #906.8 Lymphoma Path Fracture Distal Femur
55 yr female with recent severe knee pain & prior breast CA
Axial CT scan
Bone scan
Cor T-1 T-2
Case #907
23 year female
lymphoma
distal femur
Lateral view
Bone scan
Sagittal T-1 MRI
Coronal T-1 MRI tumor
Coronal STIR MRI
tumor
tumor
Positive silver stain for reticulum fiber
Case #908
28 year male
lymphoma femur
Macro section
resected specimen
Photomic
Case #909
34 year male with
lymphoma prox femur
Frog lateral
Case #910
38 year male
pathologic fracture
lymphoma prox femur
Case #911
77 year male with parosteal lymphoma femur
tumor
Axial T-1 MRI
Axial T-2 MRI
tumor
Case #912
17 year male
lymphoma distal
femur
AP view
Coronal T-1 MRI
tumor
Sagittal T-1 MRI
tumor
Case #913
54 year male with HIV lymphoma proximal femur
Coronal T-1 MRI
Axial proton density MRI
tumor
Case #914
65 year female
ORIF hip fracture
prior history of
lymphoma 5 yrs ago
5 years later with
OGS at healed
fracture site
Bone scan shows multifocal OGS
Case #915
41 year male
lymphoma
proximal tibia
Lateral view
Oblique view
3 months later
Pathologic fracture
following radiation
therapy at 6 months
Photomic
Custom total knee
prosthesis for
reconstruction
Post op x-ray with
prosthetic reconstruction
Case #916
34 year female
lymphoma prox tibia
Sagittal T-1 MRI
Sagittal T-2 MRI
Case #917
21 year female with lymphoma proximal tibia
Coronal T-1 MRI
Sagittal T-1 MRI
tumor
Case #918
26 year female
lymphoma distal
tibia
Coronal T-1 MRI
tumor
Sagittal T-1 MRI
tumor
Axial T-2 MRI
tumor
Case #919
29 year female
lymphoma proximal
humerus
Bone scan
Coronal T-1 MRI
Axial proton density MRI
tumor
Photomic
Case #920
Pathologic fracture
lymphoma proximal
humerus in a 64 year
female
Bone scan
Coronal T-2 MRI tumor
Case #921
38 year female with
lymphoma humerus
Bone scan
Coronal T-2 MRI
Case #922
27 year male
lymphoma distal
humerus
Sagittal T-1 MRI
Axial T-2 MRI
tumor
Photomic
Case #923
28 year male with lymphoma proximal ulna
Axial T-1 MRI
Axial T-2 MRI
tumor
Case #924 Laminogram x-ray
50 year female with lymphoma proximal ulna
Case #925
64 year female with soft tissue lymphoma forearm
Sagittal T-1 MRI
tumor tumor
Axial proton density MRI
tumor
Axial T-2 MRI
Case #926
70 year female with
lymphoma distal radius
Case #927
Tumor defect
20 year male with lymphoma sacrum
1 year post resection and radiation therapy
2.5 years post op
Case #928
43 year male with lymphoma 11th posterior rib
Resection specimen cut in path lab
Photomic
Lymphoma
Pseudotumors
Case #929
73 year female with radiation osteitis 2nd to radiation
therapy for ovarian carcinoma 19 years ago
pain
9 days later with path fracture looking like lymphoma; biopsy negative for sarcoma
2 weeks later with progressive collapse
6 weeks later and more collapse
Coronal T-1 MRI with low signal like a lymphoma
Coronal proton density MRI
25 year male alcoholic and smoker with R hip pain 4 mos
Case #929.1 Lymphoma pseudotumor
transient osteoporosis
Cor T-1
Cor T-2
Axial T1
Axial T-2
Case #930
52 year male
radiation osteitis
humerus 2nd to
radiation for soft
tissue sarcoma
years ago now looking
like a lymphoma
Pathologic fracture
later requiring
IM nail
Case #931
54 year male tennis player with stress fracture sacrum
R L
Bone scan
CT scan showing fracture callus
Coronal T-1 MRI showing low signal like lymphoma
pseudotumor
Coronal T-2 MRI showing high signal from fracture
Case #932
22 year male
stress fracture thru
lateral plateau looking
like a lymphoma
Bone scan hot like lymphoma
Sagittal T-1 MRI
showing fracture
edema looking
like lymphoma
Case #933
32 year female
mastocytosis spine &
pelvis looking like
large cell lymphoma
Femoral mastocytosis
looking like lymphoma
Similar lesions
distal femur
Same changes
in humerus
Changes in ribs &
humerus
Hodgkin’s
Lymphoma
CLASSIC
Case #179
40 year male
blastic form of
Hodgkin’s lymphoma
L-4
Photomic showing Reed-Sternberg cell
Case #180
33 year female with Hodgkin’s disease SI area
Photomic with Reed-Sternberg cells
Case #934
58 year male with blastic form Hodgkin’s lymphoma pelvis
Left iliac lesions
Right iliac lesion
Photomic with Reed-Sternberg cells & eosinophils
Case #935
38 year male with
blastic form of lymphoma
L-3
Case #936
51 year male
lytic form of
Hodgkin’s lymphoma
L-4
Photomic with Reed-Sternberg cell
eosinophil
Case #936.1
28 year old male with hip pain for past 3 months
Hodgkin’s lymphoma
Bone scan
Cor T-1 T-2 Gad
Axial T-1 T-2
gad
Case #936.2
25 yr male with pain in shoulder 1 year
Hodgkin’s of Scapula
Cor T-2 Gad
Axial T-2 Axial Gad
Sag T-2 Sag Gad
Leukemia
CLASSIC Case #181
10 year female with hemorrhagic purpura second to
acute lymphoblastic leukemia
Transverse radioleucent metaphyseal bands seen in
acute lymphoblastic leukemia
Lymphoblasts in peripheral blood smear
Case #937
59 year male
chronic lymphocytic
leukemia
Bone scan
Coronal T-1 MRI
Sagittal T-1 MRI
Coronal T-1 MRI
Coronal T-1 MRI
Coronal T-2 MRI
Lymphoblasts seen in marrow smears
4.5 year male
acute lymphoblastic
leukemia
Case #938
Bone scan
Axial T-2 MRI
Lymphoblasts in peripheral blood smeer
Case #939
40 year male
5 year history of
chronic lymphocytic
leukemia with chronic
reactive periostitis
Lateral view
Case #940
14 month female with
acute lymphoblastic
leukemia femur showing
laminated periostitis
Casswe #941
43 year male with
chronic granulocytic
leukemia with focal
lesion in femur
tumor
Another view
tumor
Case #941.1
3/06 4/06 6/06
12 year female with wrist pain and elevated sed rate 3 mos
Acute lymphocytic leukemia
Coronal T-1 T-2 Sagittal T-2
6/06
Case #942
4 year female
acute lymphocytic
leukemia with
laminated periostitis
fibula
Case #943
4 year male
acute lymphocytic
leukemia hand
Case #944
40 year female
chronic lymphocytic
leukemia with
osteoporotic codfish
shaped vertebral bodies
AP view
Plasma Cell
Tumors
Solitary
Plasmacytoma
Solitatary Plasmacytoma
To fit the strict criteria for the diagnosis of a solitary plasmacytoma,
the patient should present with a single bony involvement, as
demonstrated by a bone skeletal survey, and remain free of other
bony site involvement for at least six months after the initial
diagnosis. Unfortunately, in approximately 70% of cases, the
solitary form of the disease will disseminate into the more common
form of multiple myeloma. Likewise, in the case of pure solitary
plasmacytoma, the serum protein electrophoresis study is
completely normal in 75% of cases with the remaining 25%
showing a mild abnormality. The age group for solitary plasma-
cytoma is slightly younger than multiple myeloma, typically before
the age of 40 years. The most common sites of involvement
include the spine, pelvis and proximal femur with radiographic
evidence of a solitary lytic destruction of bone that may take on the
appearance of a benign lesion such as a solitary form of fibrous
dysplasia. Because the disease is purely lytic in nature, the bone
isotope scan may be negative.
Since the disease is considered localized at first, the treatment is
localized with a wide surgical resection and prosthetic replacement,
with or without bone cement, followed by local radiation therapy
if the margins are positive. No systemic chemotherapy is used until
the disease becomes more disseminated as demonstrated by increased
levels of abnormal myeloma protein found by serum electrophoresis
studies. The prognosis for survival is quite good until the disease
becomes more disseminated which is usually within three years
after the initial diagnosis.
CLASSIC Case #182
40 year male with solitary plasmacytoma pelvis
Bone scan showing
signal void
Axial T-1 MRI
Axial T-2 MRI
Photomic showing plasma cells
High power
Case #183
42 year male with solitary plasmacytoma pelvis
Axial T-1 MRI
Axial T-2 MRI
Post op x-ray after
radiation therapy &
total hip replacement
Case #184
53 year male
solitary plasmacytoma
proximal femur
Pathologic fracture one year later
Post op x-ray with
cemented long stem
biopolar prosthesis
Case #185
55 year male
solitary plasmacytoma
proximal femur
Case #185.1
40 year male with
increasing pain in
thigh for 6 mos
Solitary plasmacytoma
Bone scan
Sag T-1 PD FS Cor STIR
Axial T-1
T-2
Gad
PO interlocking nail
and cementation
Case #946
49 year male
solitary plasmacytoma
acetabulum
CT scan
tumor
Bone scan
Photomic
Reconstruction with
metal cage
Steinman pin
Cementation over metal
and completed THA
inside cage
Post op X-ray
Case #947
32 year male with solitary plasmacytoma acetabulum
tumor
Coronal T-1 MRI
Case #948
53 year male with
solitary plasmacytoma
sacrum
R L
Bone scan shows signal void in tumor
CT scan
tumor
Sagittal T-2 MRI
tumor
Case #949
52 year male with large solitary plasmacytoma pelvis
CT scan at sacral level
CT scan at lumbar level
tumor
Case #950
45 year male with solitary plasmacytoma sacrum
Lateral view
tumor
Bone scan shows signal void in tumor site
Bone scan
tumor
Axial T-2 MRI
tumor
Sagittal PD MRI
tumor
Sagittal T-2 MRI
tumor
Photomic
Case #951
56 year male with solitary plasmacytoma sacrum
Axial T-1 MRI
tumor
Sagittal T-1 MRI
tumor
Case #952
28 year female with path fracture thru supra acetabular Solitary plasmacytoma
Case #953
46 year male with
solitary plasmacytoma
proximal femur
Coronal T-1 MRI
Coronal T-2 MRI
Case #954
65 year female with
solitary plasmacytoma
femur
Post op x-ray with
blade-plate fixation
Case #955
41 year male with
solitary plasmacytoma
femur
Case #956
40 year female with
solitary plasmacytoma
proximal fibula
Case #957
24 year male with solitary plasmacytoma scapula
tumor
Bone scan showing signal void in center of tumor
CT scan
tumor
Multiple Myeloma
Multiple Myeloma
Multiple myeloma is considered to be the most common primary
tumor of bone, accounting for approximately 45% of all malignant
bone tumors. It is usually seen in patients over 40 years of age and
is two times more common in blacks than whites. Radiographically,
the lytic lesions seen in multiple myeloma are typically punched-out
with fairly sharp margins but no sclerotic response at the periphery
and thus are not frequently picked up on a total body bone isotope
study. The diagnosis is usually made by a combination of a bone
marrow biopsy and a serum protein electrophoresis that reveals
the elevated monoclonal immuninoglobulin at either the alpha or
gamma spike. Bence-Jones protein is found in the urine examination
secondary to a light chain immuninoglobulin spillover.
Three per cent of patients with myeloma have a sclerotic form
(seen on the radiographic examination) associated with a peripheral
neuropathy. This type of multiple myeloma has a better prognosis
for survival and is referred to as the Poems syndrome. Skeletal
lesions are more typically seen in the spine and pelvic area and
proximal long bones, but rarely seen distal to the elbow or knee. In
aggressive forms of myeloma with extensive bony destruction by
osteoclastic erosion, the patients will develop hypercalcemia that
can result in a semi-comatose state and sometimes is associated
with nephrocalcinosis. Renal damage also results from excessive
proteins plugging the renal tubules. Pathological fractures are
common because of excessive osteoclastic activity (osteoclysis)
that can be inhibited by drugs such as Aredia. Even though large
concentrations of imminoglobulin are produced by the malignant
plasma cells, the patient’s resistance to infection is markedly
inhibited and surgical complications resulting from infection should
be anticipated when operating on patients with this disease.
Systemic chemotherapy has greatly improved the prognosis
for survival in this disease. The drugs used include Malphalan and
cortisone which have increased the chance for survival to three years.
Local treatment consists of external beam radiation therapy and
intramedullary devices, such as long stem prostheses and inter-
locking nails supplemented with bone cement, for pathological
fractures. Excessive bleeding at the time of surgery is typical with
myeloma patients, similar to the problem faced with patients with
metastatic renal cell disease and thyroid carcinoma. It is important
to radiate the entire long bone involved when considering intra-
medullary device fixation because of the potential for newer lesions
arising distal to the fixation device at a later date.
CLASSIC Case #186
65 year male with multiple myeloma pelvis and hips
fracture
Photomic showing plasma cells
Post op x-ray with THR right & ORIF left
R L
Case #187
72 year male with multiple myeloma skull
Punched out
lesions femur
Case #188
55 year male with multiple myeloma spine
Sagittal T-2 MRI
Sagittal T-2 MRI
lumbar spine with
evidence of cord
compression
Case #958
37 year male with
multiple myeloma
dorsal spine
Lumbar spine
Autopsy specimens
Photomic
Case #959
55 year male with multiple
myeloma dorsal spine
Lateral view
CT scan showing tumor in vertebra and vertebral canal
CT of adjacent vertebra
Anterior vertebrectomy specimen
Photomic
Case #960
48 year male with
multiple myeloma
dorsal spine with
cord involvement
Sagittal T-2 MRI
tumor
Axial T-2 MRI showing tumor in pedicle & costovertebral joint
Another axial T-2 cut
tumor
Biopsy photomic
Case #961
55 year male with
multiple myeloma
cervical spine and
compression fracture
Post op posterior
spinal fusion and
radiation therapy
Case #962
38 year male with
multiple myeloma
lumbar spine
Post op anterior
curettement and
cementation
AP view
Case #963
41 year male with
multiple myeloma
with collapsed vertebral
body and paraplegia
Lateral view
Case #964
16 year male with multiple myeloma lumbar spine
Case #965
59 year female with multiple myeloma pelvis
tumor
CT scan showing large aneuysmal lesion
tumor
Another CT cut
tumor
Post op x-ray with THA
with cement and
Steinman pins
Skull x-ray showing multiple punched out lesions
Case #966
72 year female with severe multiple myeloma pelvis
Case #967
59 year female with multiple myeloma skull
Same patient with scapular and humeral lesions
Thumb lesion
Case #967.1
55 yr male with a painful swollen wrist for 6 months
Multiple Myeloma
Case #968
55 year female with
multiple myeloma
with path fracture
femur
Post op x-ray with cemented
long stem THA
Case #969
75 year male with
multiple myeloma
and path fracture
femur
Case #970
68 year female with multiple myeloma shoulder area
Same patient with
tibial lesions
Punched out
femoral lesions
Punched out humeral
lesions as well
Path fracture later on
with IM nail fixation
Photomic
Case #971
62 year male with
multiple myeloma
humerus
Post op cemented Neer
prosthesis
Case #972
37 year male with
multiple myeloma
upper extremity
with punched out lesions
Case #973
48 year female with multiple myeloma shoulder
Bone scan with
scapular and
rib lesions
Coronal T-1 MRI
Axial T-2 MRI
tumor
Case #974
72 year male with
multiple myeloma
lumbar spine
Same patient with
punched out lesions
in femur and ischium
Also punched out lesions
in tibia and fibula
Case #975
29 year male with blastic form of multiple myeloma
Close up
Case #976
40 year male with a
variant of multiple
myeloma known as
fibrogenesis imperfecta
ossium
Multiple collapsed
lumbar vertebra as in
multiple myeloma
X-ray at a later date
with progressive disease
X-ray of pelvis shows diffuse lytic changes
Bone scan
Metastatic
Neuroblastoma
Metastatic Neuroblastoma
Neuroblastoma is a primitive tumor of childhood taking its origin
from the medullary portion of the adrenal gland or in other parts of
the sympathetic nervous system and is considered to be the third
most common malignancy in childhood. This tumor is usually seen
under the age of five years and typically metastasizes to bones
including the vertebra, ribs, skull, femur, pelvis, humerus, tibia, and
radius. These patients usually have systemic symptoms including
weight loss, fever, generalized pain and anemia. Radiographically,
the lesions in bone have a permeative destructive pattern typically
in the metaphyseal portion of long bones. Histologically, the
characteristic feature of the neuroblastoma is a rosette formation
with neurofibrils. However, on routine H&E stains the histology is
quite similar to that of rhabdomyosarcoma, non-Hodgkin’s
lymphoma and Ewing’s sarcoma. Diagnostic studies include
cytogenetics that will reveal a chromosomal abnormality in the
number 1 chromosome. A CT scan of the abdomen will frequently
pick up a neoplastic abnormality in the kidneys. Chemotherapy and
sometimes bone marrow transplantation is used in the treatment
protocol for this aggressive, extensive metastatic disease but with
only a a 20-25% chance for survival at five years after diagnosis.
CLASSIC
Case #189
4 year male
metastatic neuroblastoma
distal femur with
pathologic fracture
Lateral view showing
hair-on-end reactive
subperiosteal bone
formation
Bone scan
Bone scan shows abnormal
collection in kidney where
the primary tumor was
found
Sagittal T-2 MRI
showing metastatic
tumor in tibia
Abdominal CT scan showing primary neuroblastoma in the kidney area
tumor
Chest x-ray showing multiple pulmonary mets
Femoral biopsy photomic showing rosette pattern
Higher power showing rosette pattern
Case #977
7 year male with
metastatic neuroblastoma
proximal humerus and
path fracture
Bone scan showing
primary tumor in kidney
and met to shoulder &
base of skull
Photomic from humeral biopsy
Case #978
9 year male with
metastatic neuroblastoma
proximal humerus
Another view
Case #979
1 year old female with
metastatic neuroblastoma
proximal humerus
Case #980
5 year female with
metastatic neuroblastoma
proximal femur
Case #981
11 year female with
metastatic neuroblastoma
femur treated with wide
resection and fibular strut
& IM nail reconstruction
Macro section from
resected specimen
showing tumor inside
and outside the femur
Scanning lens photomic
Higher power photomic
Case #982
7 year male with
metastatic neuroblastoma
distal femur
Case #983
7 month male with large
calcific renal mass which
on biopsy proved to be
neuroblastoma
Lateral view