4. Hemorrhagic (Telangiectatic) Osteosarcoma
The hemorrhagic (OGS), an extremely lytic and hemorrhagic
variant of the osteosarcoma, presents in the same age group and
location as a classic osteosarcoma but has a radiographic
appearance almost identical to that of an aggressive aneurysmal
bone cyst, making for a very difficult differential consideration
for the radiologist. At the time of biopsy the tumor is very
hemorrhagic and has the gross appearance of an aneurysmal
bone cyst. Even microscopically, many areas of the hemorrhagic
OGS will have the appearance of an aneurysmal bone cyst with
only an occasional mitotic figure. For this reason, it is very
important for the surgeon who performs the biopsy to obtain
an adequate specimen with good sampling by means of an open
biopsy as apposed to a simple needle biopsy. The microscopic
features of the hemorrhagic OGS are a large number of benign-
appearing giant cells and thus the terminology “giant cell rich”
5. osteosarcoma that is used by many pathologists. There is very
little evidence of osteoblastic acitivity in the hemorrhagic OGS
and, because it is so lytic in character, it frequently presents with
a pathologic fracture early in the course of the disease and with that
come potential problems for the treating orthopedic surgeon who
must deal with the major contamination that occurs during the
fracture. Because of these possible complications, one might consider
an early limb salvage procedure before the fracture occurs.
It was once felt that the prognosis for the hemorrhagic OGS
was worse than that of the classic OGS because of its lytic dest-
uctive nature. However, since the advent of systemic chemotherapy,
the prognosis for survival is no different than that of a classic OGS.
55. Parosteal Osteosarcoma
The parosteal (OGS) is a low grade variant arising from the surface
of a long bone that presents as an exophytic mass with dense fibro-
osseous tissue. It carries an excellent five year survival prognosis
of 85% and accounts for about 4% of all osteosarcomas. This
tumor has very little, if any, medullary involvement which clearly
separates it from the classic OGS. It is seen more commonly in
females than males and is found in a slightly older age group
than the classic OGS. By far the most common location for this
tumor is in the posterior aspect of the distal femur where it is
frequently presents with minimal symptoms of pain but with a
palpable tumor mass that might have been present many years
before medical advise was sought. Histologically, this tumor has a
very low mitotic index and in many cases can be confused with
a normal healing fracture callous with occasional areas of cartilage
being seen. Because this tumor is extremely low grade, it is not
56. responsive to adjuvant therapy such as chemotherapy or
radiation therapy. The treatment consists of a wide surgical
resection that must have safe margins, otherwise the recurrence
rate will be quite high. Recurrence can occur 10 to 15 years after
the surgery. In many cases the lesion can be resected without
sacrificing the adjacent joint, but in larger lesions the best
approach is a total joint replacement similar to that used for the
classic OGS.
110. Periosteal Osteosarcoma
The periosteal osteosarcoma is another surface type OGS that
tends to be low grade to intermediate with potential for pulmonary
metastasis in about 25% of cases. It accounts for 2% of all OGS’s
and, compared to the parosteal OGS, has a much higher percentage
of cartilagenous tissue in the tumor to the point where it can look
like a periosteal chondroma but with a much higher mitotic index.
One must find a few areas of osteoid formation to classify this as
a periosteal OGS. It is seen typically in the second decade of life
and is slightly more common in females than males. It arises from
long bones, typically the tibia or femur, and has a higher incidence
in diaphyseal bone than does OGS. Like the parosteal OGS, this
lesion is treated by aggressive wide local resection that often can
spare the adjacent joint. In most cases chemotherapy is not utilized
unless the clinical picture is more aggressive than usual.
150. Case #517
Nora’s lesion
39 year female
periosteal OGS
pseudotumor
In fact is a Nora’s
lesion or
bizarre parosteal
osteochondromatous
proliferation (BPOP)
158. Pagetic Sarcoma
There are multiple diseases of the skeletal system that can result
in a secondary form of OGS most likely brought about by a second
mutation at a later age in a patient with chronic benign disease.
These diseases include Paget’s disease, osteoblastoma, fibrous
dysplasia, benign giant cell tumor of bone, bone infarcts, and
chronic osteomyelitis. The most common of this group is Paget’s
disease, a non-specific inflammatory osteomyelitis of bone seen
in older patients that may be induced by a virus infection. Approx-
imately 1% of patients with Paget’s disease can go on to Pagetic
OGS which accounts for 3% of all OGS. The most common
location for this secondary form of OGS is in the humerus,
followed next by the pelvis and femur. The patients typically have a
long history of dull, aching pain from their inflammatory Paget’s
disease but then suddenly develop an acute new pain in the area of
the older pain with x-ray evidence of recent lysis and destruction
159. of old Pagetic reactive bone. The prognosis for survival in this
secondary form of OGS is extremely poor with only about 8%
surviving, mainly because the older age group in which the disease
occurs make it impractical to implement the aggressive protocols
used in younger age groups.
160. CLASSIC Case #113
tumor
80 year female with Pagetic sarcoma pelvis
198. Low Grade Intramedullary OGS
Low grade intramedullary OGS is another rare low grade fibro-
osseous variant of OGS that is unique because it is totally confined
within the cortical anatomy of a long bone, most typically around
the knee joint. It is found in an older age group than the classic
OGS and is typically seen between the ages of 15 and 55 years;
it affects males and females equally. The radiologic picture is that
of a diffuse sclerotic change within the metaphysis of the long
bone with no periosteal response or lytic destruction of the cortical
anatomy. The smoky appearance of metaphyseal bone suggests
the diagnosis of chronic osteomyelitis or perhaps fibrous dysplasia.
Microscopically, the tumor has a histological appearance similar
to parosteal OGS and because of this carries the same excellent
prognosis for survival as we see in parosteal sarcoma. Likewise,
treatment is similar without the use of chemotherapy or radiation.
These lesions must be treated with complete wide resection that
frequently involves a TKA, similar as in the classic OGS.
221. Radiation-induced Osteosarcoma
One of the most malignant forms of OGS is the secondary type
induced by radiation therapy, usually over 3000 rads, for some
type of either benign or malignant disease process in the past.
One of the most common types of radiation-induced OGS is
seen in patients with breast cancer who receive local radiation
following radical mastectomy and than develop OGS in the
shoulder girdle area. Other malignant diseases that can result in
OGS after radiation therapy include Ewing’s sarcoma and
lymphomas. Benign diseases that can result in OGS from
radiation therapy include GCT, ABC, and fibrous dysplasia. The
average delay for the occurrence of secondary OGS is 15 years,
with a range from 3 to 55 years. The prognosis for this variant is
extremely poor, similar to Pagetic OGS. It has a very high rate
of metastasis to the lung for which chemotherapy is not very
effective.
255. Multicentric Osteosarcoma
The multicentric variant of OGS is an extremely rare variant
occurring in approximately 1% of all OGS. It has two distinct
categories: (1) Synchronous multicentric OGS occurring in child-
hood and adolescence. This is the more severe variant, considered
to be extremely high grade with a very poor prognosis associated
with it. This form presents with multiple sclerotic lesions seen in a
fairly symmetrical fashion in long bones, mostly in the lower
extremities and because of the heavy tumor burden associated
with multiple lesions throughout the skeleton, the alkaline phos-
phatase is frequently elevated. (2) Metachronous multicentric OGS
occurring mainly in adults is less aggressive than the synchronous
form seen in children, presenting usually with a solitary lesion.
Then, later on, more lesions develop that are considered multi-
focal in nature. The possibility of metastasis can not be ruled out.
These forms of OGS are quite resistant to chemotherapy and
surgical treatment is frustrating because of the multi focal disease.
317. Soft Tissue Osteosarcoma
OGS can be seen in soft tissue outside the skeletal system. It
accounts for 4% of all OGS and is typically in large muscle groups
around the pelvis and thigh area. It occurs most often in patients
over 40 years of age and hits males and females equally. Soft
tissue OGS, with its mature appearing bone in the central area of
the lesion and aggressive, poorly mineralized tissue at the
periphery, must be differentiated from myositis ossificans, which
has a typical zonal pattern with peripheral maturation of bone
formation. As with any soft tissue sarcoma, the treatment consists
of wide local resection. Because of the poor prognosis, worse
than that of bone osteosarcoma, systemic chemotherapy is utilized
extensively as one would use for a typical medullary OGS.
341. Intracortical Osteosarcoma
The intracortical OGS is perhaps the rarest variant of OGS with
only 14 cases described in the world literature since 1960. It
occurs between the ages of 10 and 47 years, equally between
males and females, and is seen most typically in the femur or
tibia as a metadiaphyseal lesion with a radiographic appearance
very similar to that of osteoid oasteoma. The prognosis is usually
quite good with a total of three deaths in the world literature. It
is usually treated by wide resection without chemotherapy. A
few cases are higher grade and carry a poor prognosis similar
to the classic OGS.
342. CLASSIC Case #119
42 year female with intracortical OGS femur