Volume 13

Adamantinoma----------------Case 214-218 & 1094-1098

Chordoma----------------------Case 219-224 & 1099-1106

Histiocytoses

Eosinophillic granuloma--Case 417-444

Hand-Schiller-Christian --Case 445-446

Letter Siwe disease--------Case 447-450

Sinus histiocytosis---------Case 450.1

Rosai-Dorfman’s disease

Adamantinoma

Adamantinoma

The adamantinoma of bone is an extremely rare primary sarcoma

of bone, accounting for about .3% of all malignant tumors of bone,

and in 90% of the cases it will be seen in the diaphyseal portion of

the tibia, especially in the anterior cortex. It occurs equally in males

and females, typically in the second and third decade of life. The

tissue of origin still remains a mystery but immunohistochemical

stains suggest an epithelial origin that might account for why they

are more common just beneath the skin in the anterior cortex of the

tibia. Radiographically, the adamantinoma takes on the appearance

of a fibrous dysplastic lesion or perhaps osteofibrous dysplasia of

the tibia. The lesion appears benign with a lytic core surrounded by

dense, reactive fibro-osseous bone that frequently dilates the anterior

cortex and may be multiloculated in appearance. The tumor is quite

slow growing and is usually painful, whereas in fibrous dysplasia

and osteofibrous dysplasia, the patients remain asymptomatic. If a

so-called fibrous dysplastic lesion continues to grow past maturity,

a physician should suspect adamantinoma, especially if the lesion is

painful, and look for the characteristic nests and cords of epithelial-

looking cells surrounded by fairly benign-appearing fibro-osseous

tissue on histological specimens. It is very rare for this tumor to

metastasize to different sites but occasionally it will metastasize to

regional lymph nodes and the lung.

Treatment for this low grade lesion is purely surgical, consisting

of a wide local resection, frequently a segmental resection of the

mid portion of the tibia, and reconstruction with a large bone

allograft over an intramedullary nail. There have been a few rare

cases in the literature where adamantinoma has arisen out of a pre-

existing osteofibrous dysplastic lesion.

CLASSIC

Case #214

25 year male with

adamantinoma tibia

Lateral view

Wide resection specimen

opened on back table

Photomic

Surgical specimen after

3 mins in autoclave

Autoclaved tibia

replaced over IM

nail and rotated 180

degrees

Cancellous autogenous

iliac chips placed

between tibia and

roughed up fibular

1 year later showing

union of prox tib-fib

step ladder synostosis

3 years post op with solid

union to autoclaved bone

7 years post op with

symptoms of ankle pain

Turns HIV positive

One year later with

osteopenia about

painful and swollen

ankle

Sagittal T-1 MRI

shows recurrent tumor

above ankle

Cut amputation specimen

showing excellent

osseointegration between

fibula & autoclaved tibia

tumor

Photomic at tibia-fibula interphase

autoclaved tibia

reactive live bone

Case #215

22 year female

adamatinoma

distal tibia

Photomic

Another photomic

Wide resection

distal tibia biopsy

site

Autoclaved specimen

cemented over IM nail

including total ankle

Composite reconstruction

completed including

total ankle

Post op lateral x-ray

AP x-ray showing

fibular recurrence

resulting in BK amp

2 years later

Case #216

11 year female with

adamantinoma arising

from osteofibrous dysplasia

tibia

AP view

Bone scan

Low power photomic with osteofibrous dysplasia

to right and adamantinoma to left

Photomic with cords and nests of epithelioid cells

Higher power of cords and nests of epithelioid cells

4 years following

segmental resection

and allograft recon

over IM nail

Case #217

25 year female

adamantinoma tibia

Close up AP view

Low power photomic

Case #218

58 year female

adamantinoma

proximal tibia

Lateral view

Bone scan

Coronal Gad

Contrast MRI

Sagittal STIR MRI

Axial T-2 MRI

Another axial T-2 MRI showing cystic fluid-fluid levels

Photomic

Another photomic

Case #1094

15 year female with

adamantinoma arising

from osteofibrous dysplasia

AP view several years later

showing slow progression and

fibular involvement as well

Lateral view showing

path fracture

Photomic showing combined fibro-osseous and adamantinoma histology

Photomic showing mostly adamantinoma histology

Another photomic

6 months following

curettement and cancellous

allograft placement

9 months later

AP view same time

3.5 years later showing

good healing and no

progression of disease

Lateral view at same time

Case #1095

58 year female with

adamantinoma tibia

Sagittal T-1 MRI

Sagittal T-2 MRI

Case #1096

18 year male with

adamantinoma tibia

Bone scan

Sagittal T-2 MRI

Axial T-2 MRI

Case #1096.1

78 year male with chronic osteomyelitis tibia since age 2 years

with recent fungating lesion anterior tibia

Pseudo adamantinoma

Case #1097

14 year male with

adamantinoma distal

tibia and fibula

Coronal T-1 MRI

Photomic

Case #1098

16 year male with

adamantinoma mandible

AP x-ray

Oblique view

Surgical exposure

Resected specimen

Photomic

Another photomic

Chordoma

Chordoma

The chordoma is a very rare malignant tumor of bone that

accounts for only 4% of all malignant bone tumors. It arises from

the primitive notochord of the axial skeleton and is most commonly

seen in the lower portion of the sacrum, accounting for 50% of the

chordomas. 37% arise in the spheno-occipital area and a small

number occur in the cervical and lumbar spine. The more common

sacral lesions are seen in an older age group between the ages of

40 and 80 years, compared to the spheno-occipital chordomas that

occur in a younger adult age group. The later carry a much worse

prognosis because of the location at the base of the skull. The

chordoma is clinically similar to a mucinous type of chondrosarcoma.

In the sacral area, chordomas are usually attached to the anterior

portion of the distal three segments of the sacrum and grow in the

retroperitoneal space, pressing up against the rectum where

eventually they will present with clinical symptoms related to

constipation and can be picked up on a rectal examination. Because

the tumor is very slow growing, it rarely causes significant pain

symptoms. The radiographic findings are frequently not very obvious,

even with large tumors that are better evaluated by soft tissue

technique, such as CT scan or, better yet, MRI. Microscopically, the

chordoma has a mucinous appearance similar to a low grade myxoid

chondrosarcoma, but the diagnostic feature is the “signet ring”

appearance of the physaliferous cells that have a peripheral nucleus,

a large cytoplasmic inclusion of physaliferous mucinous material that

can look a bit like a liposarcoma.

Treatment for the chordoma consists primarily of a wide surgical

resection, which sometimes is very difficult, especially with lesions

extending into the upper sacral segments where the nerve roots

become a problem and may result in significant neurogenic bowel

and bladder complications. Even with surgical treatment, the local

recurrence rate is very high so that post op radiation therapy is

recommended. It has cut the local recurrence rate to about 30%

compared to 65% without RT. Recurrences can occur locally up to

fifteen years after the original surgery. Pulmonary metastases are

extremely rare and systemic chemotherapy is not indicated for this

tumor.

CLASSIC Case #219

45 year male with chordoma sacrum

Lateral view

Sagittal T-1 MRI

Coronal T-1 MRI

Axial T-1 MRI

Axial T-2 MRI

Resected specimen cut in path lab

Photomic showing physaliferous cells

Case #220 Sagittal T-1 MRI

74 year male with chordoma sacrum

Sagittal T-2 MRI

Case #220.1 CT scan

48 year male with 4 mo history of sacral pain

Sacral chordoma

Bone scan

Axial MRI

T-1

T-2

Sag T-2

Cor T-2 Gad

Post op x-ray

Case #221

82 year male with chordoma lower sacrum

Lateral view

Sagittal T-1 MRI

Sagittal T-2 MRI

tumor

Axial T-1 MRI

tumor

Case #222 CT scan

65 year female with chordoma sacrum and buttock

Axial T-2 MRI

tumor

Axial T-2 MRI at higher level

Sagittal T-2 MRI

tumor

femur

Photomic

T-2 MRI

Sacral chordoma

65 yr male

Case #222.1

Rebar and cement reconstruction

Case #223

49 year male with recurrent chordoma sacrum

CT scan

tumor

Sagittal T-2 MRI

tumor

Axial T-2 MRI

tumor

Wide resection entire

sacrum and recon with

CD rods and Steinman

pins prior to cementation

CD rod

Steinman pins

L-5

After cementation

CD rods

cement sacrum

Post op x-ray

cement

Lateral view

cement coccyx

L-4

L-5

cement

Case #224 Sagittal T-1 MRI

55 year male with chordoma lumbar spine

L-3

S-1

Resection L-4, L-5 and part of S-1

Resected vertebrae

Photomic

Anterior recon with large bone allograft and plate

sacrum

Post op lateral x-ray

CD rods

allograft

Case #1099

53 year female with chordoma sacrum

Bone scan

Oblique bone scan

CT scan one year later

tumor

Sagittal T-2 MRI

Sagittal T-2 MRI

tumor

tumor

Axial T-2 MRI

Photomic

Case #1100 CT scan

61 year male with chordoma distal sacrum

Bone scan

bladder

Sagittal T-1 MRI

tumor

Sagittal T-2 MRI

tumor

Axial T-1 MRI

Photomic

Case #1101 Axial T-1 MRI

60 year female with chordoma sacrum

Axial T-2 MRI

Sagittal T-2 MRI

Case #1102

Axial and coronal CT scan

37 year male with chordoma in body of L-4

CT scan myelogram with block at L-4

Myelogram showing block

at the L-4 level

Oblique myelogram views

Photomic

Post op x-ray following

removal of L-4 body

and recon with fibular

strut from L-3 to L-5

Lateral view of fibular

strut reconstruction

8 mos later with

collapse of L-3 on L-5

10 more months

and further collapse

of L-3 on L-5

Sagittal T-1 MRI showing collapse and kinked dural sac

X-ray following restoration

of collapsed vertebral space

with iliac strut graft in

front and CD rods behind

AP view

Case #1103

34 year male with

chordoma L-5

CT scan at L-5 shows chondroid like tumor arising from

the postero-lateral elements of L-5

tumor

Another CT cut

tumor

Another

Axial T-1 MRI

tumor

Axial T-2 MRI

Another axial T-2 MRI

Sagittal T-2 MRI

Another sagittal T-2 cut

Photomic

Case #1104

65 year male with a

chordoma of L-2

Myelographic study

showing complete

block at L-2 level

Surgical exposure of paraspinous mass at L-2 level

Post op x-ray showing

recon with cement

and pins in tumor defect

Lateral view

Case #1104.1

49 year male with back

pain for 6 mos and recent

paraparesis

Chordoma L-1

Axial CT L-1 level

Axial T-1 T-2

Gad

Sag T-1 T-2

PO x-ray

PO Cor CT Sag

Case #1105

54 year male with

chordoma C-spine

Case #1106

42 year female with chordoma base of skull

Autopsy specimen

showing lobulated

chondroid looking

mass in post fossa

Macro section

Photomic

Photomic

Histiocytoses Of

Bone

Eosinophilic

Granuloma

Eosinophilic Granuloma (Langerhans Histiocytosis)

The so-called benign histiocytoses, sometimes referred to as

histiocytosis X, include various disease conditions such as eosino-

phillic granuloma, Hand-Schuller-Christian disease and Letterer-

Siwe disease. Eosinophillic granuloma is the most benign of the

histiocytic disorders, followed next by Hand-Schuller-Christian

disease that presents with an intermediate diffuse process of both

bone and soft tissue that can be fatal. Letterer-Siwe disease is the

most aggressive and fatal form of the histiocytoses, presenting like

leukemia with a very poor prognosis for survival.

Eosinophillic granuloma, now referred to as Langerhans histio-

cytosis, is a benign histiocytic disorder that frequently presents in

children between the ages of 5 and 15 years with a clinical picture

that can masquerade as a malignant neoplasm such as Ewing’s

sarcoma. It occurs twice as often in males than females. It is

usually a monostotic disorder of the skeletal system, however, in

10% of cases it will be seen in two or three separate sites. The

etiology of this histiocytic process is still unknown but some have

postulated a viral etiology. Patients present with inflammatory pain,

more severe at night, that may be associated with a low grade fever

or elevated sed rate. The most common location is in the skull,

followed next by the ribs, pelvis, maxilla, vertebral body, clavicle

and scapula, in that order. Besides flat bone involvement, it is

commonly seen in the diaphyses of long bones where it can

masquerade as Ewing’s sarcoma, but can also occur in metaphyseal

bone and is found least commonly in epiphyseal bone. In young

children, the condition can be extremely permeative and destructive

in nature, taking on the appearance of Ewing’s sarcoma, metastatic

neuroblastoma, or acute osteomyelitis.

On x-ray, eosinophillic granuloma has an onion-skin appearance

similar to a Ewing’s sarcoma. In an older age group, the condition

tends to be more focal and more granulomatous in appearance with

less permeative change. Microscopically, there are large, pale-

staining histiocytes speckled with small, bright-staining eosinophils

and an occasional giant cell. Eosinophillic granuloma tends to

involute spontaneously without treatment and therefore symptomatic

treatment should be conservative--simple curettement for diagnostic

purposes and perhaps cortical steroid injections can be beneficial to

inhibit the inflammatory process. In more difficult parts of the body,

such as the spine or pelvis, very low grade radiation therapy can be

considered, realizing that this could convert the process to a malignant

sarcoma at a later date. In more aggressive forms with multi-focal

involvement, especially if there is soft tissue involvement of the skin,

lymph nodes or lung, a low dosage chemotherapy program can be

considered. Sometimes the low grade eosinophillic granuloma

histiocytosis can upgrade to a more aggressive and dangerous form

such as Hand-Schiller-Christian disease or even Letterer-Siwe disease.

With spinal lesions, spinal cord compression can result in paraparesis

requiring laminectomy decompression. However, kyphotic deform-

ities in younger patients tend to correct spontaneously without surgery.

CLASSIC

Case #417

17 year male

EG distal femur

Lateral view

Coronal T-1 MRI

Sagittal T-2 MRI

Coronal STIR MRI

Biopsy photomic

eosinophil histiocyte

Case #417.1

6 yr male with pain in left thigh for 6 months

CT scan

Axial T-1 T-2

Gad

Cor Gad Sag Gad

Case #417.2

6 year male with pain in thigh for 1 mo.

10/08 11/08 12/08

11/08 1/09

Cor T-1 T-2 T-1 T-2

Case #418

2 year male

EG mid femur

Case #419

8 year male

EG mid femur

Case #420

3 year female

path fracture

EG femur

Lateral view

Case #421

3 year male

EG mid femur

Lateral view

Case #422

7 year female with

EG femur

Case # 423

14 year male with EG clavicle

Case #424

16 year male with EG mid clavicle

Macro section of resection specimen

Photomic

Case #425

6 year male with EG proximal humerus

Case # 426

12 year male with

EG mid humerus

Arteriogram

Bone Scan

Case #427

2.5 year male

EG distal humerus

Lateral view

Case #428

2.5 female with EG proximal ulna

AP view

CT scan showing reactive involucrum formation

More proximal CT cut

Axial T-2 MRI

edema

Case #429

23 year female with EG right ilium

Bone scan

CT scan

Axial STIR MRI

Case #429.1

51 year female with 6 mos pelvic pain second to EG

Bone scan

Axial T-2 MRI

Case #429.2

39 year male with dull aching pain left hip for 9 mos.

EG pelvis

Axial CT scan

Cor and Sag CT

Axial

T-1

T-1

T-2

T-2

Cor T-1

Sag T-2

Case #430

12 year male

EG ilium

Case #431

20 year male with EG scapula

CT scan

Coronal T-2 MRI

Axial T-2 MRI

Case #432

4 year male

EG mid fibula

Case #433

12 year male with EG posterior rib

24 year male with EG skull

x-ray resection specimen

Case #434

CT scan of EG skull

Photomic

Case #435

4 year female

EG right mandible

Oral photo of submucosal mass

Mandibular view

Full mouth dental x-ray view

Axial CT scan

Photomic

Case #436

3 year male

vertebra plana from

EG thoracic vertebra

Sagittal CT scan

Sagittal T-1 MRI

Sagittal T-2 MRI

Sagittal gad contrast MRI

Case #438

7 year male with EG T-11

Sagittal gad contrast MRI

Sagittal T-2 MRI showing protrusion into vertebral canal

Case #439

19 year male with EG collapse

at 3 different levels but at

different times

The lower levels show height

restoration as a spontaneous

healing process seen in children

new collapse

old

old

new collapse

old

old

Case # 440

7 year female with

healing collapsed

lumbar vertebra

Case # 441

15 year male with

EG lumbar vertebra

Case #442

5 year female

EG C-3

Case #443

10 year female

EG C-7

Case #444

8 year male

EG skull with

EG dermatitis

skull defect

Severe EG dermatitis back

Case #444.1

5 year old male with pain

in heel and knee for 6 mos

Multifocal osteomyelitis

EG pseudotumor

Sag T-1 T-2 Gad

Cor T-1 Sag T-2

Axial T-2

Case #444.2

2 year old male with painful

foot for 2 months

EG

Sag T-1 T-2

Gad

Axial T-1 T-2

Gad

Hand-Schiller-

Christian Disease

Hand-Schiller-Christian Disease

Hand-Schiller-Christian disease is the intermediate form of

histiocytosis-X that involves predominantly children, two-thirds of

the cases being younger than five years of age. The classic triad

for this syndrome is diabetes insipitus, exophthalmos, and single

geographic lesions involving mostly the skull and pelvic bones.

The initial lesions appear like eosinophillic granuloma and, in fact,

eosinophillic granuloma can progress into a Hand-Schiller-Christian

type syndrome as the disease advances. It is common to have soft

tissue involvement of lymph nodes, liver, spleen, lung, brain and

kidney as well as skin changes that can be seen in eosinophillic

granuloma. Histologically, the same histiocytic cells as are seen

with eosinophillic granuloma are present, along with eosinophils. In

the later stages, foam cells and cholesterol deposits are typical.

as the disease progresses and more and more soft tissue organs are

affected, the prognosis worsens with an overall fatally rate of

10-30%. Treatment consists of local surgical treatment plus systemic

treatment consisting of therapeutic protocols similar to those used in

leukemic patients.

CLASSIC Case # 445

5 year male with HSC disease skull

Photomic from edge of skull lesion

reactive bone

granuloma

Photomic showing foam cells

Higher power of foam cells

Photomic showing cholesterol deposits

Case # 446

6 year female with HSC disease skull, spine and pelvis

Pelvis

Spinal lesions

Biopsy photomic

Photomic showing cholesterol deposits

Letterer-Siwe

Disease

Letterer-Siwe Disease Letterer-Siwe disease is the least common of the histiocyoses

comprising about 10% of all histiocytic disorders. It is a pro-

gressive, acute syndrome in children under three years of age,

involving multiple visceral organ systems, such as the spleen,

lymph nodes and skin, associated with purpura, bleeding gums,

and multiple lesions similar to those seen in Hand-Schiller-

Christian disease. The skull and pelvis are frequently involved.

The skeletel lesions tend to be more diffuse than with the other

histiocytoses and take on a picture similar to that of leukemia or

diffuse lymphoma. These patients usually die of bacterial infections

within one or two years of their acute clinical onset because of bone

marrow suppression. Histologically, the lesions look very similar

to eosinophillic granuloma or Hand-Schuller-Christian disease,

although it is unusual to see foam cells in this form of histiocytosis.

Treatment consists of chemotherapeutic agents similar to those

used in leukemia.

CLASSIC Case #447

3 year female with LS disease skull

AP view

Chest x-ray with diffuse involvement ribs, scapulae & humeri

Diffuse involvement pelvis and hips

Bilateral humeral involvement

Photomic showing histiocytes and eosinophils

Photomic showing giant cell, polys & histiocytes

giant cell histiocyte

poly

Case # 448

15 month male with LS skull

T-12 collapse

Femoral disease

Photomic

Case #449

3 year male with LS skull

Femoral disease

Case #450

2 year old female with LS skull

Sinus Histiocytosis

Rosai-Dorfman’s Disease

Sinus Histiocytosis

Rosai-Dorfman’s Disease

Sinus histiocytosis is a rare and new variant of the histiocytoses

which is characterized by enlarged lymph nodes in the head and

neck area in 80% of cases along with bony involvement in 40%

of cases. It is an aggressive form of the histiocytoses that is seen in

teen agers and young adults. Symptoms may include fever, weight

loss and malaise. The bony lesions may be solitary or multifocal

and suggest inflammatory disease such as chronic osteomyelitis or

EG. The pathology shows mononuclear or multinuclear giant cells

with lymphs in the cytoplasm of the giant cells. Other inflammatory

cells such as plasma cells, lymphocytes and foamy histiocytes may

be seen. 10% of those with bone lesions die of the disease from extra-

skeletal involvement of the lungs and kidneys.

Case #450.1

52 year old female with knee pain for 1 year

Rosai-Dorfman’s disease

Sag T-1 T-2

Gad

Axial T-1 T-2

Gad

Cor T-1 T-2

Gad

Post op X-ray