Volume 8

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, rib, 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

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 allographic

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 #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 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

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 #900.5

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 IM nailing

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

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 #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


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


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


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

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


L-4

Photomic with Reed-Sternberg cell

eosinophil

Case #936.1

28 year old male with hip pain for past 3 months


Bone scan

Cor T-1 T-2 Gad

Axial T-1 T-2

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-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 lymphoblastic

leukemia hand

Case #944

40 year female

chronic monocytic

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


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


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


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


proximal femur

Coronal T-1 MRI

Coronal T-2 MRI

Case #954

65 year female with


femur

Post op x-ray with

blade-plate fixation

Case #955

41 year male with


femur

Case #956

40 year female with


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 #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


proximal humerus and

path fracture

Bone scan showing

primary tumor in kidney

and met to shoulder

Photomic from humeral biopsy

Case #978

9 year male with


proximal humerus

Another view

Case #979

1 year old female with


proximal humerus

Case #980

5 year female with


proximal femur

Case #981

11 year female with


femur treated with wide

resection and fibular strut

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


distal femur

Case #983

7 month male with large

calcific renal mass which

on biopsy proved to be

neuroblastoma

Lateral view

Bone scan

Sag PD T-2

Volume 8

Health & Medicine

Transcript of Volume 8