3. 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
4. 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.
76. 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
77. 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
78. fifteen years after the original surgery. Pulmonary metastases are
extremely rare and systemic chemotherapy is not indicated for this
tumor.
79. CLASSIC Case #219
45 year male with chordoma sacrum
178. 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
179. 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-
180. 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.
251. Case #439
new collapse
19 year male with EG collapse new collapse
at 3 different levels but at old
different times old
The lower levels show height
restoration as a spontaneous
healing process seen in children
old
old
252. Case # 440
7 year female with
healing collapsed
lumbar vertebra
265. 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
266. 10-30%. Treatment consists of local surgical treatment plus systemic
treatment consisting of therapeutic protocols similar to those used in
leukemic patients.
267. CLASSIC Case # 445
5 year male with HSC disease skull
278. 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.
279. CLASSIC Case #447
3 year female with LS disease skull
294. 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.
295. Case #450.1 Rosai-Dorfman’s disease
52 year old female with knee pain for 1 year
