vol 13 ppt
DESCRIPTION
TRANSCRIPT
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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
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Adamantinoma
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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
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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.
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CLASSIC
Case #214
25 year male with
adamantinoma tibia
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Lateral view
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Wide resection specimen
opened on back table
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Photomic
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Surgical specimen after
3 mins in autoclave
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Autoclaved tibia
replaced over IM
nail and rotated 180
degrees
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Cancellous autogenous
iliac chips placed
between tibia and
roughed up fibular
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1 year later showing
union of prox tib-fib
step ladder synostosis
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3 years post op with solid
union to autoclaved bone
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7 years post op with
symptoms of ankle pain
Turns HIV positive
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One year later with
osteopenia about
painful and swollen
ankle
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Sagittal T-1 MRI
shows recurrent tumor
above ankle
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Cut amputation specimen
showing excellent
osseointegration between
fibula & autoclaved tibia
tumor
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Photomic at tibia-fibula interphase
autoclaved tibia
reactive live bone
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Case #215
22 year female
adamatinoma
distal tibia
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Photomic
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Another photomic
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Wide resection
distal tibia biopsy
site
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Autoclaved specimen
cemented over IM nail
including total ankle
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Composite reconstruction
completed including
total ankle
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Post op lateral x-ray
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AP x-ray showing
fibular recurrence
resulting in BK amp
2 years later
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Case #216
11 year female with
adamantinoma arising
from osteofibrous dysplasia
tibia
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AP view
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Bone scan
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Low power photomic with osteofibrous dysplasia
to right and adamantinoma to left
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Photomic with cords and nests of epithelioid cells
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Higher power of cords and nests of epithelioid cells
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4 years following
segmental resection
and allograft recon
over IM nail
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Case #217
25 year female
adamantinoma tibia
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Close up AP view
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Low power photomic
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Case #218
58 year female
adamantinoma
proximal tibia
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Lateral view
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Bone scan
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Coronal Gad
Contrast MRI
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Sagittal STIR MRI
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Axial T-2 MRI
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Another axial T-2 MRI showing cystic fluid-fluid levels
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Photomic
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Another photomic
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Case #1094
15 year female with
adamantinoma arising
from osteofibrous dysplasia
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AP view several years later
showing slow progression and
fibular involvement as well
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Lateral view showing
path fracture
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Photomic showing combined fibro-osseous and adamantinoma histology
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Photomic showing mostly adamantinoma histology
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Another photomic
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6 months following
curettement and cancellous
allograft placement
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9 months later
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AP view same time
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3.5 years later showing
good healing and no
progression of disease
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Lateral view at same time
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Case #1095
58 year female with
adamantinoma tibia
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Sagittal T-1 MRI
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Sagittal T-2 MRI
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Case #1096
18 year male with
adamantinoma tibia
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Bone scan
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Sagittal T-2 MRI
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Axial T-2 MRI
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Case #1096.1
78 year male with chronic osteomyelitis tibia since age 2 years
with recent fungating lesion anterior tibia
Pseudo adamantinoma
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Case #1097
14 year male with
adamantinoma distal
tibia and fibula
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Coronal T-1 MRI
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Photomic
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Case #1098
16 year male with
adamantinoma mandible
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AP x-ray
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Oblique view
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Surgical exposure
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Resected specimen
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Photomic
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Another photomic
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Chordoma
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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
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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
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fifteen years after the original surgery. Pulmonary metastases are
extremely rare and systemic chemotherapy is not indicated for this
tumor.
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CLASSIC Case #219
45 year male with chordoma sacrum
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Lateral view
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Sagittal T-1 MRI
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Coronal T-1 MRI
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Axial T-1 MRI
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Axial T-2 MRI
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Resected specimen cut in path lab
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Photomic showing physaliferous cells
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Case #220 Sagittal T-1 MRI
74 year male with chordoma sacrum
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Sagittal T-2 MRI
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Case #220.1 CT scan
48 year male with 4 mo history of sacral pain
Sacral chordoma
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Bone scan
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Axial MRI
T-1
T-2
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Sag T-2
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Cor T-2 Gad
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Post op x-ray
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Case #221
82 year male with chordoma lower sacrum
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Lateral view
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Sagittal T-1 MRI
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Sagittal T-2 MRI
tumor
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Axial T-1 MRI
tumor
![Page 100: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/100.jpg)
Case #222 CT scan
65 year female with chordoma sacrum and buttock
![Page 101: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/101.jpg)
Axial T-2 MRI
tumor
![Page 102: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/102.jpg)
Axial T-2 MRI at higher level
![Page 103: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/103.jpg)
Sagittal T-2 MRI
tumor
femur
![Page 104: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/104.jpg)
Photomic
![Page 105: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/105.jpg)
T-2 MRI
Sacral chordoma
65 yr male
Case #222.1
![Page 106: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/106.jpg)
Rebar and cement reconstruction
![Page 107: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/107.jpg)
Case #223
49 year male with recurrent chordoma sacrum
![Page 108: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/108.jpg)
CT scan
tumor
![Page 109: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/109.jpg)
Sagittal T-2 MRI
tumor
![Page 110: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/110.jpg)
Axial T-2 MRI
tumor
![Page 111: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/111.jpg)
Wide resection entire
sacrum and recon with
CD rods and Steinman
pins prior to cementation
CD rod
Steinman pins
L-5
![Page 112: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/112.jpg)
After cementation
CD rods
cement sacrum
![Page 113: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/113.jpg)
Post op x-ray
cement
![Page 114: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/114.jpg)
Lateral view
cement coccyx
L-4
L-5
cement
![Page 115: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/115.jpg)
Case #224 Sagittal T-1 MRI
55 year male with chordoma lumbar spine
L-3
S-1
![Page 116: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/116.jpg)
Resection L-4, L-5 and part of S-1
![Page 117: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/117.jpg)
Resected vertebrae
![Page 118: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/118.jpg)
Photomic
![Page 119: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/119.jpg)
Anterior recon with large bone allograft and plate
sacrum
![Page 120: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/120.jpg)
Post op lateral x-ray
CD rods
allograft
![Page 121: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/121.jpg)
Case #1099
53 year female with chordoma sacrum
![Page 122: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/122.jpg)
Bone scan
![Page 123: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/123.jpg)
Oblique bone scan
![Page 124: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/124.jpg)
CT scan one year later
tumor
![Page 125: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/125.jpg)
Sagittal T-2 MRI
![Page 126: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/126.jpg)
Sagittal T-2 MRI
tumor
![Page 127: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/127.jpg)
tumor
Axial T-2 MRI
![Page 128: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/128.jpg)
Photomic
![Page 129: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/129.jpg)
Case #1100 CT scan
61 year male with chordoma distal sacrum
![Page 130: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/130.jpg)
Bone scan
bladder
![Page 131: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/131.jpg)
Sagittal T-1 MRI
tumor
![Page 132: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/132.jpg)
Sagittal T-2 MRI
tumor
![Page 133: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/133.jpg)
Axial T-1 MRI
![Page 134: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/134.jpg)
Photomic
![Page 135: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/135.jpg)
Case #1101 Axial T-1 MRI
60 year female with chordoma sacrum
![Page 136: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/136.jpg)
Axial T-2 MRI
![Page 137: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/137.jpg)
Sagittal T-2 MRI
![Page 138: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/138.jpg)
Case #1102
Axial and coronal CT scan
37 year male with chordoma in body of L-4
![Page 139: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/139.jpg)
CT scan myelogram with block at L-4
![Page 140: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/140.jpg)
Myelogram showing block
at the L-4 level
![Page 141: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/141.jpg)
Oblique myelogram views
![Page 142: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/142.jpg)
Photomic
![Page 143: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/143.jpg)
Post op x-ray following
removal of L-4 body
and recon with fibular
strut from L-3 to L-5
![Page 144: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/144.jpg)
Lateral view of fibular
strut reconstruction
![Page 145: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/145.jpg)
8 mos later with
collapse of L-3 on L-5
![Page 146: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/146.jpg)
10 more months
and further collapse
of L-3 on L-5
![Page 147: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/147.jpg)
Sagittal T-1 MRI showing collapse and kinked dural sac
![Page 148: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/148.jpg)
X-ray following restoration
of collapsed vertebral space
with iliac strut graft in
front and CD rods behind
![Page 149: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/149.jpg)
AP view
![Page 150: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/150.jpg)
Case #1103
34 year male with
chordoma L-5
![Page 151: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/151.jpg)
CT scan at L-5 shows chondroid like tumor arising from
the postero-lateral elements of L-5
tumor
![Page 152: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/152.jpg)
Another CT cut
tumor
![Page 153: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/153.jpg)
Another
![Page 154: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/154.jpg)
Axial T-1 MRI
tumor
![Page 155: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/155.jpg)
Axial T-2 MRI
![Page 156: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/156.jpg)
Another axial T-2 MRI
![Page 157: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/157.jpg)
Sagittal T-2 MRI
![Page 158: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/158.jpg)
Another sagittal T-2 cut
![Page 159: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/159.jpg)
Photomic
![Page 160: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/160.jpg)
Case #1104
65 year male with a
chordoma of L-2
Myelographic study
showing complete
block at L-2 level
![Page 161: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/161.jpg)
Surgical exposure of paraspinous mass at L-2 level
![Page 162: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/162.jpg)
Post op x-ray showing
recon with cement
and pins in tumor defect
![Page 163: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/163.jpg)
Lateral view
![Page 164: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/164.jpg)
Case #1104.1
49 year male with back
pain for 6 mos and recent
paraparesis
Chordoma L-1
![Page 165: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/165.jpg)
Axial CT L-1 level
![Page 166: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/166.jpg)
Axial T-1 T-2
Gad
![Page 167: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/167.jpg)
Sag T-1 T-2
![Page 168: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/168.jpg)
PO x-ray
![Page 169: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/169.jpg)
PO Cor CT Sag
![Page 170: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/170.jpg)
Case #1105
54 year male with
chordoma C-spine
![Page 171: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/171.jpg)
Case #1106
42 year female with chordoma base of skull
![Page 172: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/172.jpg)
Autopsy specimen
showing lobulated
chondroid looking
mass in post fossa
![Page 173: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/173.jpg)
Macro section
![Page 174: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/174.jpg)
Photomic
![Page 175: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/175.jpg)
Photomic
![Page 176: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/176.jpg)
Histiocytoses Of
Bone
![Page 177: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/177.jpg)
Eosinophilic
Granuloma
![Page 178: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/178.jpg)
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
![Page 179: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/179.jpg)
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-
![Page 180: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/180.jpg)
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.
![Page 181: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/181.jpg)
CLASSIC
Case #417
17 year male
EG distal femur
![Page 182: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/182.jpg)
Lateral view
![Page 183: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/183.jpg)
Coronal T-1 MRI
![Page 184: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/184.jpg)
Sagittal T-2 MRI
![Page 185: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/185.jpg)
Coronal STIR MRI
![Page 186: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/186.jpg)
Biopsy photomic
eosinophil histiocyte
![Page 187: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/187.jpg)
Case #417.1
6 yr male with pain in left thigh for 6 months
![Page 188: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/188.jpg)
CT scan
![Page 189: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/189.jpg)
Axial T-1 T-2
Gad
![Page 190: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/190.jpg)
Cor Gad Sag Gad
![Page 191: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/191.jpg)
Case #417.2
6 year male with pain in thigh for 1 mo.
10/08 11/08 12/08
![Page 192: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/192.jpg)
11/08 1/09
Cor T-1 T-2 T-1 T-2
![Page 193: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/193.jpg)
Case #418
2 year male
EG mid femur
![Page 194: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/194.jpg)
Case #419
8 year male
EG mid femur
![Page 195: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/195.jpg)
Case #420
3 year female
path fracture
EG femur
![Page 196: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/196.jpg)
Lateral view
![Page 197: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/197.jpg)
Case #421
3 year male
EG mid femur
![Page 198: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/198.jpg)
Lateral view
![Page 199: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/199.jpg)
Case #422
7 year female with
EG femur
![Page 200: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/200.jpg)
Case # 423
14 year male with EG clavicle
![Page 201: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/201.jpg)
Case #424
16 year male with EG mid clavicle
![Page 202: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/202.jpg)
Macro section of resection specimen
![Page 203: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/203.jpg)
Photomic
![Page 204: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/204.jpg)
Case #425
6 year male with EG proximal humerus
![Page 205: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/205.jpg)
Case # 426
12 year male with
EG mid humerus
![Page 206: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/206.jpg)
Arteriogram
![Page 207: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/207.jpg)
Bone Scan
![Page 208: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/208.jpg)
Case #427
2.5 year male
EG distal humerus
![Page 209: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/209.jpg)
Lateral view
![Page 210: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/210.jpg)
Case #428
2.5 female with EG proximal ulna
![Page 211: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/211.jpg)
AP view
![Page 212: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/212.jpg)
CT scan showing reactive involucrum formation
![Page 213: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/213.jpg)
More proximal CT cut
![Page 214: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/214.jpg)
Axial T-2 MRI
edema
![Page 215: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/215.jpg)
Case #429
23 year female with EG right ilium
![Page 216: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/216.jpg)
Bone scan
![Page 217: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/217.jpg)
CT scan
![Page 218: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/218.jpg)
Axial STIR MRI
![Page 219: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/219.jpg)
Case #429.1
51 year female with 6 mos pelvic pain second to EG
![Page 220: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/220.jpg)
Bone scan
![Page 221: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/221.jpg)
Axial T-2 MRI
![Page 222: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/222.jpg)
Case #429.2
39 year male with dull aching pain left hip for 9 mos.
EG pelvis
![Page 223: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/223.jpg)
Axial CT scan
![Page 224: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/224.jpg)
Cor and Sag CT
![Page 225: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/225.jpg)
Axial
T-1
T-1
T-2
T-2
![Page 226: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/226.jpg)
Cor T-1
Sag T-2
![Page 227: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/227.jpg)
Case #430
12 year male
EG ilium
![Page 228: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/228.jpg)
Case #431
20 year male with EG scapula
![Page 229: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/229.jpg)
CT scan
![Page 230: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/230.jpg)
Coronal T-2 MRI
![Page 231: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/231.jpg)
Axial T-2 MRI
![Page 232: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/232.jpg)
Case #432
4 year male
EG mid fibula
![Page 233: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/233.jpg)
Case #433
12 year male with EG posterior rib
![Page 234: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/234.jpg)
24 year male with EG skull
x-ray resection specimen
Case #434
![Page 235: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/235.jpg)
CT scan of EG skull
![Page 236: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/236.jpg)
Photomic
![Page 237: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/237.jpg)
Case #435
4 year female
EG right mandible
![Page 238: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/238.jpg)
Oral photo of submucosal mass
![Page 239: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/239.jpg)
Mandibular view
![Page 240: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/240.jpg)
Full mouth dental x-ray view
![Page 241: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/241.jpg)
Axial CT scan
![Page 242: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/242.jpg)
Photomic
![Page 243: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/243.jpg)
Case #436
3 year male
vertebra plana from
EG thoracic vertebra
![Page 244: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/244.jpg)
Sagittal CT scan
![Page 245: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/245.jpg)
Sagittal T-1 MRI
![Page 246: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/246.jpg)
Sagittal T-2 MRI
![Page 247: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/247.jpg)
Sagittal gad contrast MRI
![Page 248: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/248.jpg)
Case #438
7 year male with EG T-11
![Page 249: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/249.jpg)
Sagittal gad contrast MRI
![Page 250: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/250.jpg)
Sagittal T-2 MRI showing protrusion into vertebral canal
![Page 251: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/251.jpg)
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
![Page 252: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/252.jpg)
Case # 440
7 year female with
healing collapsed
lumbar vertebra
![Page 253: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/253.jpg)
Case # 441
15 year male with
EG lumbar vertebra
![Page 254: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/254.jpg)
Case #442
5 year female
EG C-3
![Page 255: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/255.jpg)
Case #443
10 year female
EG C-7
![Page 256: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/256.jpg)
Case #444
8 year male
EG skull with
EG dermatitis
skull defect
![Page 257: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/257.jpg)
Severe EG dermatitis back
![Page 258: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/258.jpg)
Case #444.1
5 year old male with pain
in heel and knee for 6 mos
Multifocal osteomyelitis
EG pseudotumor
![Page 259: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/259.jpg)
Sag T-1 T-2 Gad
![Page 260: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/260.jpg)
Cor T-1 Sag T-2
Axial T-2
![Page 261: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/261.jpg)
Case #444.2
2 year old male with painful
foot for 2 months
EG
![Page 262: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/262.jpg)
Sag T-1 T-2
Gad
![Page 263: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/263.jpg)
Axial T-1 T-2
Gad
![Page 264: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/264.jpg)
Hand-Schiller-
Christian Disease
![Page 265: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/265.jpg)
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
![Page 266: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/266.jpg)
10-30%. Treatment consists of local surgical treatment plus systemic
treatment consisting of therapeutic protocols similar to those used in
leukemic patients.
![Page 267: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/267.jpg)
CLASSIC Case # 445
5 year male with HSC disease skull
![Page 268: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/268.jpg)
Photomic from edge of skull lesion
reactive bone
granuloma
![Page 269: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/269.jpg)
Photomic showing foam cells
![Page 270: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/270.jpg)
Higher power of foam cells
![Page 271: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/271.jpg)
Photomic showing cholesterol deposits
![Page 272: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/272.jpg)
Case # 446
6 year female with HSC disease skull, spine and pelvis
![Page 273: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/273.jpg)
Pelvis
![Page 274: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/274.jpg)
Spinal lesions
![Page 275: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/275.jpg)
Biopsy photomic
![Page 276: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/276.jpg)
Photomic showing cholesterol deposits
![Page 277: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/277.jpg)
Letterer-Siwe
Disease
![Page 278: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/278.jpg)
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.
![Page 279: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/279.jpg)
CLASSIC Case #447
3 year female with LS disease skull
![Page 280: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/280.jpg)
AP view
![Page 281: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/281.jpg)
Chest x-ray with diffuse involvement ribs, scapulae & humeri
![Page 282: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/282.jpg)
Diffuse involvement pelvis and hips
![Page 283: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/283.jpg)
Bilateral humeral involvement
![Page 284: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/284.jpg)
Photomic showing histiocytes and eosinophils
![Page 285: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/285.jpg)
Photomic showing giant cell, polys & histiocytes
giant cell histiocyte
poly
![Page 286: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/286.jpg)
Case # 448
15 month male with LS skull
![Page 287: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/287.jpg)
T-12 collapse
![Page 288: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/288.jpg)
Femoral disease
![Page 289: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/289.jpg)
Photomic
![Page 290: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/290.jpg)
Case #449
3 year male with LS skull
![Page 291: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/291.jpg)
Femoral disease
![Page 292: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/292.jpg)
Case #450
2 year old female with LS skull
![Page 293: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/293.jpg)
Sinus Histiocytosis
Rosai-Dorfman’s Disease
![Page 294: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/294.jpg)
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.
![Page 295: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/295.jpg)
Case #450.1
52 year old female with knee pain for 1 year
Rosai-Dorfman’s disease
![Page 296: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/296.jpg)
Sag T-1 T-2
Gad
![Page 297: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/297.jpg)
Axial T-1 T-2
Gad
![Page 298: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/298.jpg)
Cor T-1 T-2
Gad
![Page 299: Vol 13 ppt](https://reader034.vdocuments.net/reader034/viewer/2022052217/54b805f04a7959d5118b45b6/html5/thumbnails/299.jpg)
Post op X-ray