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Benign
Soft Tissue Tumors
Lipoma
Hemangioma
Fibroma and Fibromatosis
Neurofibromas and Neurofibromatosis
Neurofibromatosis
Pigmented Villonodular Synovitis (PVNS)
Myositis Ossificans
Ganglion or Synovial Cyst
Malignant
Soft Tissue Tumors
Liposarcoma
Malignant Fibrous Histiocytoma (MFH)
Fibrosarcoma
Synovial Sarcoma
Leiomyosarcoma and Rhabdomyosarcoma
Angiosarcoma
Malignant Schwannoma (Neurofibrosarcoma)
Your first office visit may be overwhelming
because we attempt to confirm a diagnosis for you. You meet a lot of staff,
hear a lot of information and may have a lot of tests performed. Your first
visit maybe tiring, somewhat confusing and stressful depending on your situation.
If you brought blood results, MRI, bone or CT scans, we review all of them.
If you have had a biopsy, we consult with our pathologists to review your
pathology slides. If you have not had any of these studies done, we attempt
to schedule all of them during your first visit.
Plain x-rays, CT scans of the tumor area and of your chest, MRI, PET and bone
scans are called staging studies. These are the common studies performed to
'workup' a patient with a questionable tumor of the soft tissues or bone.
They assist us in determining whether your tumor is benign or malignant, and
whether the malignant ones (sarcomas) have traveled to other sites of your
body. When a sarcoma has traveled, this is called a metastasis. If sarcomas
metastasize, the most common site is the lung.
We will also schedule a biopsy that day if deemed necessary. A biopsy is a
needle aspirate or surgical removal of a small piece of tissue for microscopic
examination. There are four types of biopsies which are done to make a diagnosis:
1.
Fine needle aspiration (FNA): Performed
in the office by pathologists from the FNA Biopsy Service. It takes about
10-15 minutes, uses a very small needle and usually the results are ready
during your visit. A Band-Aid is placed at the biopsy site.
2. Tru-cut needle biopsy: Performed in
the office by one of the surgeons. A local anesthetic is used to numb your
skin and a larger needle is used to get several samples of tissue. You may
have some discomfort or pressure like sensation when a sample is taken. Samples
are taken to our pathologists and preliminary results are ready in an hour
or so. Many times results take 24 hours or longer. A pressure dressing is
applied to the biopsy site.
3. CT scan guided needle biopsy: Performed in the Radiology Department by one of the radiologists. It may take one hour. The CT is used to guide the radiologist to position the needle into the tumor and get several samples of tissue. Some patients have stated that the procedure is painful despite local anesthetics. A small bandage or larger pressure dressing is applied at the site. Results usually take 24 hours or longer. Radiologists prefer that you not eat or drink 6 hours before the procedure, and have someone with you to take you home. You will be medicated for the procedure, thus driving or taking public transportation is not advised.
4. Open biopsy:
A surgical procedure usually requiring general anesthesia in which a small
incision is made to take an adequate tissue sample to be examined by the pathologist.
Tumor diagnosis determines treatment plan. Thus, an open biopsy is performed
when any of the other needle biopsies can not confirm a diagnosis or in children
when this type of biopsy maybe more appropriate. An open biopsy can not be
done on the same day as the office visit since it requires booking operative
time and preadmission testing. Results may be ready following the procedure,
but usually it takes 24 hours or longer.
TUMORS: BENIGN VS. MALIGNANT
Bone and soft tissue tumors are rare. There
are a variety of types and each one has its own particular behavior, treatment
and patient outcome. The cause (etiology) of tumors is basically unknown.
Some researchers have suggested a genetic cause because of the increased incidence
in patients with certain conditions (hereditary osteocartilaginous exostosis,
Maffucci's syndrome, Paget's disease) and because certain tumors have genetic
mutations of the tumor cells. Bones in an area that has had radiation therapy
have shown an increase in rate of malignant transformation. Trauma also has
been discussed as a cause. However, most often it is more likely that the
traumatic event or injury brings attention to a tumor that has already been
present (pre-existing) rather than causing it.
Bone and soft tissue tumors are classified by their cell or tissue type and
their ability to spread beyond the original site (metastasize). Thus, bone
and soft tissue tumors fall into two major categories. They are benign (unable
to spread) or malignant (able to spread to other parts of the body, especially
to the lungs or other bones). Benign tumors are not life threatening, malignant
tumors may be. Benign tumors may heal or resolve on their own. They may be
left alone and just 'watched' over the years, or may respond well to minor
surgery if they are causing symptoms, likely to lead to fracture, or injuring
underlying bone and surrounding soft tissue, blood vessels and nerves.
Malignant tumors, bone and soft tissue, also known as sarcomas are a realtively
rare form of cancer, often require treatment with drugs (chemotherapy) and/or
radiation therapy and major surgery. Chemotherapy is used for its systemic
effect, that is, once given it circulates throughout the body to stop malignant
cells from spreading to other areas of the body. Radiation is used for its
local effect, that is, the beam of radiation penetrates the area of the tumor
and surrounding tissue to kill tumor cells causing necrosis (cell death) and
sometimes reduction in the tumor size.
Years ago, surgery meant amputation. Now surgery involves wide resection (complete
removal of the tumor and a little normal and reactive surrounding tissue)
with options to spare the limb (referred to as limb-sparing procedures). These
options involve replacing the tumor bone with a donor bone (allograft) and
securing it in place with a metal plate and screws, or replacing the tumor
bone with a metal prosthesis (hardware similar to total joint replacements
but more complex). An amputation may be necessary if the tumor invades surrounding
blood vessels and nerves.
The type of surgical procedure to remove a tumor is categorized by the amount
of surrounding tissue removed with the tumor. This border of surgical resection
is called the tumor margin. There are basically four margins, thus four general
ways to remove a tumor. A curettage or scraping of the lesion keeps the excision
or resection (removal) of the the tumor within the walls or capsule of the
tumor. A marginal excision is removal of the tumor along with a small amount
of surrounding tissue called the reactive zone. A wide excision involves removal
of the tumor, reactive zone and some normal tissue. A radical excision is
removal of the tumor and the entire bone or muscle that contained the tumor
referred to as compactment. All tumor specimens are sent to pathology to be
examined for microscopic cells left behind in the margins. Results are reported
as positive or negative margins. If margins are positive cells have been left
behind and, additional excision may be necessary, or if the tumor is malignant,
postoperative radiation therapy to the area may be advised.
In addition to the two major categories
of benign or malignant, bone and soft tissue tumors are further classified
or staged. The stage of a lesion depends on three factors:
1. the grade of the tumor which is a microscopic evaluation of the tumor cell's
biologic behavior and aggressiveness, that is, its ability to grow and extend
beyond its natural barrier (capsule).
2. whether the tumor stops in its area (compartment) or grows and penetrates
out of the compartment.
3. whether or not there are metastases.
A biopsy which involves the microscopic
evaluation of the tumor cell's biologic behavior will determine the grade.
Pathologists grade malignant tumors ranging from low grade (1) to high grade
(3). Low grade tumors are made of cells whose behavior is not aggressive,
that is they seldom spread beyond their local site (Grade 1/3). Intermediate
to high grade tumors are made of cells that behave very aggressive and most
often than not spread to other parts of the body (Grade 2 or 3).
Patients must undergo many scans (MRI, CT, bone or PET) called staging studies.
These scans are done to evaluate the extent of the tumor, locally or at distant
sites. The tumor is then staged which helps determine the best treatment plan
for that type of tumor. Many of these imaging studies need to be done prior
to the biopsy. In most circumstances the biopsy is the last part of the staging
process.
Many of our patients have expressed confusion about staging and grading of tumors because of information read in books or found on the internet. Hopefully the above information will help you understand how tumors are staged and graded.
Benign bone tumors almost never metastasize and are best classified by the matrix which the tumor cells produce such as bone, cartilage, fibrous tissue, fat or blood vessel. Some of these tumors can be described as 'aggressive' because they may recur locally after removal (resection). It is very important to determine whether a tumor is benign or malignant to avoid over or under treatment of the patient.
Osteoid Osteoma is a benign bone tumor that produces bone and is small. The patient presents with a small, painful lesion. Other symptoms include tenderness, swelling and a limp. Pain is sharp, deep, usually worse at night and relieved with aspirin. X-rays have a classic pattern which shows a small round or oval focus called a nidus. These tumors usually occur between the ages of 10-20, rarely over age thirty. They are most commonly located in the tibia and femur, but have occurred in almost every bone. Treatment used to be by excision only. Now, there is a newer, non-invasive procedure called heat ablation which is performed by our radiologists. This procedure involves placing an electrode into the tumor which is heated causing a 'microwave' treatment to the tumor. Patients receive general anesthesia for the procedure, but go home the same day with a bandaid over the probe site. Surgical excision involves a two to four day hospital stay. Incomplete excision or curettages (scraping out) of the lesion may lead to tumor recurrence.
Osteoblastoma is much less common than the osteoid osteoma. It may be confused with a malignant tumor and may behave very aggressively. These tumors occur at any age and can be found in any bone but are most common in the spine and long bones. Symptoms include tenderness, swelling, limited motion if near a joint, and pain (not as severe as the osteoid osteoma). The lesion can be seen on plain x-rays. Treatment consists of curettage (scraping out) of the lesion with bone grafting or an excision (removal) depending on the site and potential functional problems. 'Aggressive' osteoblastomas need closer monitoring to rule out recurrence.
Enchondromas are benign cartilage lesions located centrally within the middle of short, long or flat bones and occur at any age. They are the most common bone tumors of the hand. Patients usually have no symptoms (asymptomatic) unless they have fractured. Plain x-rays show the lesion. If the patient is asymptomatic and there is no deformity or fracture, the lesion can be conservatively managed/observed with x-rays. Otherwise, treatment involves curettage with bone packing taken from the patient's iliac crest hip bone (autologous) or donor bone (allograft). At times it may be difficult to distinguish enchondroma from low grade chondrosarcomas.
Multiple Enchondromatosis (Ollier's and Maffucci's syndromes) is less common than the enchondroma and does occur with increased frequency in families. These lesions are usually found at an early age and range in severity from a mild abnormality to severe deformities of the skeletal system, such as large bony masses on the hand bones or shortening and bowing of the extremities. Treatment is focused on correcting or preventing deformities which includes removing (resecting) these masses or placing metal rods in long bones to stabilize the deformed or fractured bones. These tumors can become malignant but this is not very common. Thus, the patients are carefully observed with follow-up (serial) x-rays throughout their lifetime.
Chondroblastoma is a very rare benign tumor and occurs more frequently in males especially during adolescence. Most common sites are the top (proximal) and bottom (distal) of the femur, proximal humerus and proximal tibia. There are growth plates also located in these areas. If left untreated, growth of the involved bones could be interrupted. On x-ray, the lesion is round to oval in shape. Treatment usually requires curettage and packing of the lesion with autologous or allograft bone with careful attention to avoid injury to the growth plates. These tumors have about a 20% chance of recurring after curettage.
Chondromyxoid Fibroma is also a very rare benign tumor made of fibrous myxoid elements. It may occur at any age, but usually between twenty and thirty. Most common sites are the proximal tibia and distal femur. On x-ray, it looks very unique because it expands the edges (cortex) of the bone into the soft tissues and has a scalloped inner margin. Treatment is either curettage with bone grafting or excision (removal) in suitable locations. These tumors have the highest rate of recurrence (40%) of any benign cartilage lesion. Thus, the patient must be followed closely for several years.
Solitary Osteocartilaginous Exostosis
(OCE) or Osteochondroma is
the most common benign tumor of bone probably caused by a genetic defect in
the cartilage of an embryo (unborn child). They occur at the ends of bones
at any age. The patient presents with a hard, painless mass fixed on the bone.
On x-ray they appear as bony outgrowths of the cortex (side of the bone) projecting
away from the joints. Examination of this tumor's tissue shows a cartilage
cap which causes its growth, very similar to the growth of a normal bone.
These tumors usually do not cause the patient trouble. However, if pain develops
or they fracture or interfere with surrounding vessels and nerves, the tumor
is removed (excised). Recurrence is unlikely, as is the possibility of the
benign tumor turning malignant.
The multiple hereditary form of osteocartilaginous exostosis (HMOCE) is a
genetic autosomal dominant problem in which multiple osteochondromas develop.
They may distort the skeleton and cause functional problems. Common characteristics
include short stature, bowing or knock-knee deformities of the lower legs,
forearm or hand deformity, and poor joint function. Treatment focuses on managing
these deformities in childhood and monitoring them in adulthood to see if
the benign status changes to a malignant one. In these patients there is a
very low chance of one of the osteochondromas becoming malignant in adulthood,
but this is not common. Onset of pain or sudden growth in tumor size should
cause the patient to seek medical attention for further staging studies. Bone
scans are helpful in following these lesions in adulthood. Again, treatment
consists of excision if the patient has symptoms. Malignant tumors, usually
low grade chondrosarcomas, which arise from the previously quiet benign lesions
are also excised. Additional treatment such as radiation or chemotherapy is
seldom.
Fibrous Cortical Defect and Non-Ossifying Fibroma (NOF)
are the most common benign bone tumors in children. Both types are located
in the shafts of the long bones, especially the distal femur, proximal and
distal tibia and also in the upper extremities (proximal humerus, distal radius
and ulna). Most of these tumors disappear after adolescence. They usually
do not cause symptoms unless a fracture has occurred. Biopsies to confirm
diagnosis are usually unnecessary because the tumor appearance on x-ray is
so classic. The fibrous cortical defect has a thick bony margin and slight
bulging of the edge or side (cortex) of the long bone. By adulthood, the lesions
move away from the growth plate and heal. The cause of these lesions is unclear,
but may be related to a defect in the growth plate. No metastases have ever
been reported. These tumors usually disappear (spontaneous regression). Treatment
involves observation with routine x-rays if the diagnosis can be made from
the original x-ray. If the diagnosis is in doubt, or if the lesion is likely
to weaken the bone and cause a fracture through the lesion, then biopsy and
curettage with bone grafting is advised. If a pathologic fracture occurs,
it is allowed to heal before surgical treatment because sometimes the lesion
will disappear during fracture healing. Non-ossifying fibromas are virtually
identical, but larger.
Fibrous Dysplasia is a problem of the bone tissue that probably is a developmental abnormality of the bone versus a true tumor. It is not familial or hereditary. Sometimes diagnosis is made during childhood, but it can go unrecognized until adulthood. On x-ray these tumors have a 'ground glass' appearance and can cause thinning of the cortex. The bone may be enlarged and deformed. Some patients sustain fractures through the lesion. With fibrous dysplasia, the normal bone is replaced with masses of fibrous tissue and thin weak bone. A biopsy may be necessary to rule out more aggressive lesions. Treatment involves correction of bony deformities which usually requires curettage and packing of the lesion with possible internal fixation (metal hardware) if the lesion is located near a joint or a body area requiring extra support. this tumor presents a more difficult problem in the proximal femur. These tumors rarely turn malignant.
Unicameral Bone Cysts occur
primarily in children. The cause is unknown but it is thought to be an abnormality
of the growth plate or circulation problems of the veins of the bone involved.
The cysts are usually found in the proximal humerus of a growing child but
may occur in the proximal femur and other bones. In adults, these lesions
may be found in the heel (calcaneus) or flat bones. They are usually discovered
incidentally or after a fracture through the cyst. The cyst is clearly outlined
on an x-ray. The bone is widened and the cortex is thin. The cavity of the
cyst is filled with a serum-like fluid. Treatment is by needle aspiration
of the cyst with steroid injection or bone marrow and demineralized bone matrix
(Grafton®) injection. This is a day surgery procedure under general anesthesia
with a success rate of about 80%, but it may require three or more injections
to make the cyst heal. Curettage and packing with bone graft is the more invasive
procedure requiring a surgical incision and a few days in the hospital. Malignancy
arising from a cyst almost never happens.
Aneurysmal Bone Cyst
is a benign bone lesion of unknown cause. It may occur alone or with other
types of benign bone lesions. They usually are located in the long bones and
spine in patients less than twenty years old. Typical complaints are swelling
and pain which usually follows an injury. X-rays show a clearly outlined,
bulging, destructive and eccentric lesion of the bone. The cyst tissue is
made of spongy spaces filled with unclotted blood. It is important for these
cysts to be distinguished from malignant bone tumors so biopsy is usually
required. Treatment is by curettage with bone grafting or resection depending
on the location of the cyst. The recurrence rate after currettage approaches
30% and this is one of the reasons for excision when the tumor involves an
expendable bone.
Benign Vascular Tumors of Bone are very rare. The benign hemangioma of bone is usually solitary (found only in one bone) but can affect more than one bone. Hemangiomas are found in the spine, skull, long bones and pelvis. Local pain, swelling and pathologic fracture (fracture through the bone tumor) may occur. Isolated spine lesions (tumors) usually do not cause symptoms. On x-ray, the lesions appear as lytic defects (destroyed bone). Hemangioma tissue appears as a honey combed pattern made of a conglomeration of vessels. Treatment is usually observation, but may involve resection or curettage when possible. Radiation is given for painful lesions and those located in difficult areas inaccessible to surgery.
Histiocytosis of Bone or Eosinophilic Granuloma (EG) is a benign disorder of bone involving usually no more than two or three bony sites of children and young adults. Local pain or fracture are the most common presenting symptoms. Destructive-looking lesions are seen on x-ray. Many lesions will disappear if left alone. A biopsy is usually necessary to establish the diagnosis of EG. If the bony defect is large, curettage with bone grafting is sometimes done. Steroid injections have also been successful to treat these bony lesions. There are variations of histiocytosis with more organ involvement and more generalized disease with endocrine abnormalities. Treatment options may include radiation therapy to relieve symptoms, curettage or chemotherapy to treat systemic disease.
Giant Cell Tumor (GCT)
is a benign tumor of bone but has the potential to be locally aggressive,
meaning that it may recur in the same bone or the soft tissue where the original
tumor was excised. The cause of this tumor is unknown. It occurs most often
between the ages of 20-40 years. GCT is usually located around the knee, either
the distal femur or proximal tibia. The distal radius, proximal humerus, distal
tibia and sacrum are other common sites. Unlike other benign lesions, GCT
can metastasize to a distant site such as the lungs, but this is rare.
Patients present with a deep, persistent pain in the area of tumor. The rate
of the tumor's growth seems to be accelerated during pregnancy because of
the increase in hormones. A pathologic fracture may occur, as well as adjacent
joint effusions (fluid in the joint). On x-ray, there is thinning and expansion
of the cortex, erosion and possible cortex perforation and associated soft
tissue mass. The giant cell tumor tissue is very vascular.
Treatment consists of curettage with cement packing. Local recurrences occur
in a low percentage of patients, so close follow-up is mandatory. If the tumor
is located in the proximal fibula, a resection is done. A wide resection with
allograft reconstruction is the treatment of choice for very large, poorly
defined tumors with soft tissue mass or pathologic fracture. Although rare,
giant cell tumors can spread to the lungs. Thus, a chest x-ray must always
be done at follow-up visits in addition to an x-ray of the body part to rule
out distant metastases and local recurrence. Treatment for the rare lung metastases
involves chemotherapy and resection of the lung nodules.
Malignant bone tumors are a group of lesions that vary in their tissue makeup and behavior and range from locally aggressive tumors that usually do not metastasize to high grade sarcomas with a poor prognosis. They affect all age groups and all bony sites. Diagnosis of the specific type of malignant bone tumor is critical since the treatment for each type varies. Treatment is extremely aggressive in nature usually involving one or more of the following: chemotherapy, radiation therapy, surgery (resection) and bone marrow (stem cell) transplants.
Osteosarcoma (Osteogenic
Sarcoma) is the most common primary
malignant lesion of bone identified by its ability to form bone or bone tissue.
There are a few different types of osteosarcoma which behave differently due
to their biological makeup. Central osteosarcoma is the classical form usually
occurring in adolescents located at the proximal or distal ends of long bones.
These sarcomas are rated from low grade to high grade, with low grade being
far less aggressive. Juxtacortical (both parosteal and periosteal) are usually
considered low grade sarcomas. Telangiectatic osteosarcoma is rare, high grade
and behaves as aggressively as classical osteosarcoma.
Osteosarcomas
can occur in patients with other skeletal diseases such as Paget's disease,
in bone previously treated with radiation for another type of tumor, or in
any bone within a radiation field. Osteosarcoma incidence peaks in two age
groups: 10-20 years and 50-60 (the older age group is often related to the
patient with Paget's disease or those who received radiation). It is more
common in males and occurs in areas of rapid growth. The most common site
is the distal femur, proximal tibia, proximal humerus and proximal femur,
although any bone may be involved.
The tumor is usually noted after trauma brings attention to a painful mass.
Patients present with pain, local tenderness, a bony hard mass and decreased
function of the body part. If the lesion has been untreated for a long period
of time the patient may have symptoms of weight loss, fever, chills, lack
of appetite and severe night pain not relieved by aspirin. Sometimes there
is an increase in the blood level of alkaline phosphatase. X-rays show a destructive
lesion as well as bone formation, a classical 'sun burst' streaming pattern.
The outcome of patients without metastases at diagnosis is now quite good
due to chemotherapy. Some patients have metastases at diagnosis, usually the
lungs but may be in other bones, which worsens the prognosis.
Treatment for osteosarcoma involves chemotherapy and surgery. Chemotherapy
is given before and after the surgical procedure in an attempt to reduce the
size of the tumor making resection easier, and prevent the spread of cancer
cells to other sites of the body. The chemotherapy protocol takes almost one
year in total to complete. Previously, amputation was the only surgical treatment
option to remove the tumor. Now there are limb-saving procedures which involve
resection of the entire tumor as well as a margin of normal tissue (usually
muscle and bone) around the lesion with replacement and reconstruction using
an allograft (cadaver donor bone) or metal prosthesis. Adequate bone healing
can take nine months to one year. Rehabilitation requires casting for a few
months followed by bracing and crutches (for lower extremity tumors) for almost
one year. Physical therapy is necessary to regain motion and strength, but
the progression is slow. It is important to realize that the affected limb
will not look the same nor return to normal function. Patients return to normal
daily routines but there are restrictions on physical activities. Prolonged
walking, sitting, running, and heavy lifting should be avoided with lower
extremity allografts. With upper extremity allografts, heavy lifting, pushing/pulling
objects and repetitive use of the arm should be avoided. Patients with upper
or lower extremity allografts should avoid contact sports.
Sometimes amputation may be the only surgical option due to the tumor size,
location and/or invasion of surrounding vessels. Amputation insures complete
removal of the tumor, and in most cases, the patient rapidly returns to a
functional level. Final prosthetic fittings begin within one month after surgery
if the residual limb (stump) is healed. Unlike the allograft patient, the
functional limitations of the amputee are left to the imagination and determination
of the patient.
If local recurrence or distant metastases occur, the combination of chemotherapy
drugs is changed and surgical resection and radiotherapy is considered which
involves intermittent periods of hospitalization.
Chondrosarcoma
is a malignant cartilaginous tumor so named because it makes cartilage
as it grows. It occurs most often in adults between 40-60 years of age. This
tumor affects the pelvis, hip, proximal femur and sometimes the shoulder and
knee. Patients usually complain of a dull, persistent, aching arthritic pain
with a tender mass which may slowly increase in size distorting the normal
shape of the bone and extremity. Pathologic fracture is rare, but patients
may experience pain caused by the large soft tissue mass putting pressure
on surrounding nerves and muscles. In some patients, a preexisting enchondroma
is present. A full metastatic staging workup is necessary.
Central chondrosarcomas may be low, medium or high grade. The low grade lesions
are less likely to spread. Dedifferentiated chondrosarcoma and high grade
chondrosarcoma are most likely to spread (metastasize) to other areas. Clear
cell chondrosarcoma and intermediate or middle grade chondrosarcomas are less
likely to spread to other areas. Juxtacortical and low grade chondrosarcomas
are least likely to metastasize. X-rays have a classic look which show a large,
centrally placed lesion with significant expansion of the cortex with speckled
calcifications characteristic of the the cartilaginous producing process.
Sometimes the calcifications look like a 'popcorn pattern'. Examination of
high grade chondrosarcoma tissue shows clumps of jelly-like cartilage while
lower grade tumors have a firmer consistency.
Treatment for chondrosarcoma is almost entirely surgical. Chemotherapy is
not used unless the patient presents with lung metastases at diagnosis. Radiation
is not used unless the lesion is very large or in a difficult area such as
the spine or pelvis. When possible, limb-sparing procedures are used as long
as wide margins can be achieved with reconstruction using metallic or allograft
replacement. Low grade lesions may be treated with aggressive curettage and
close observation. For high grade lesions with a large soft tissue mass, amputation
may be necessary.
Malignant Fibrous Tumors of Bone (MFT)
are much rarer than the bone and cartilage forming malignant tumors. The two
major types include the fibrosarcoma and malignant fibrous histiocytoma (MFH).
They can be grouped together and therefore considered the same in bone.
MFT of Bone usually occurs secondary to other illness such as Paget's disease,
giant cell tumor, fibrous displasia, osteomyelitis or bones that have had
radiation therapy. These tumors occur in both sexes and in all age groups
but are found most often between the ages of 40-60. They are usually located
in the long bones of the lower extremities, especially the distal femur and
proximal tibia. These lesions are very destructive, thus pathologic fractures
are common. X-rays show the destructive (lytic), poorly defined lesions, usually
with breakthrough into the cortex causing a soft tissue mass. Low grade tumors
have a more well-defined margin than the high grade tumors. The appearance
of the tissue cells (malignant fibroblastic stroma and collagen fibers) varies
with the grade or degree of tumor aggressiveness. Malignant bone and cartilage
tissue are not found in this lesion. Treatment is mainly wide surgical resections.
The treatment and outcomes are very similar to an aggressive osteosarcoma.
Radiation may have little effect but are sometimes employed in recurrent lesions.
Adamantinoma
is a very rare malignant bone tumor and gets its name from a jaw tumor because
both tissue types are very similar. The difference between lesions in the
jaw and those in the long bones is that adamantinoma of long bones can metastasize.
It occurs in all sexes and all ages, especially between the years of 20-50.
Ninety percent of the lesions occur in the tibia, but it has also been found
in the humerus, ulna, femur and other bones. Symptoms include a mild pain
or dull ache that has been present for years. Usually a firm, tender mass
can be found in the tibial area with thin and shiny skin over the tumor mass.
On x-ray, the adamantinoma is clearly outlined, varies in length, has a bubbly
appearance and causes the width of the bone to increase. The tissue type is
fibrous which replaces the marrow and cortex. The lesion may also have some
cystic (fluid-like) areas and hemorrhage (bleeding). Histologic diaganosis
can be tricky and it is sometimes difficult to distinguish from other tumors.
Treatment is by surgical wide resection of the lesion with allograft reconstruction.
Amputation may be necessary due to tumor location and/or recurrence.
Chordoma
is another rare tumor of bone believed to come from remnants of embryonic
tissue left behind in the sacrum and lower spine. It usually occurs between
the ages of 40-70 years, slightly more frequent in males. Sacral chordomas
are relentless, slow growing tumors. Patients may have long histories of mild
discomfort in the lower spine and tenderness with prolonged sitting. A large
soft tissue mass may be felt in the middle of the sacrum or during a rectal
examination. Usually neurologic symptoms occur which include constipation
because the lesion grows and pushes forward on the rectum; low back pain radiating
down the legs because the lesion is pushing against, pinching or engulfing
the nerve roots of the sacrum, and loss of sacral nerve sensation causing
lower leg weakness, loss of bowel and bladder control, and loss of sexual
function because of the the tumor's involvement with the sacral nerves. On
x-ray, the chordoma has a typical appearance because of its location in the
middle of the sacrum and its destruction of bone with poorly defined margins.
Treatment involves surgical resection of the tumor which is technically difficult
due to the location in the sacrum and the proximity (closeness) of vital organs
and nerves. Radiation therapy is sometimes given preoperatively, followed
by resection with possible intraoperative radiation, internal radiation implants
(brachytherapy) postoperatively, and/or proton beam therapy as additional
therapy. Chemotherapy has minimal effect on chordomas. Heat ablation has recently
been tried for therapy with positive results. Some patients are referred to
pain specialists until the neurologic symptoms subside.
Despite the location and difficulty in obtaining complete eradication of a
chordoma most patients have a good long-term survival prognosis. Alterations
in life style may be necessary depending upon the effect of neurologic loss
from the tumor or treatments. Tumors may recur and require more surgery or
radiation. Most patients cope well with varying degrees of loss of bowel or
bladder control or sexual function. Occasional patients have persistent pain
which can be controlled with a variety of new treatment modalities.
Ewing's Sarcoma
is a rare, highly malignant bone tumor made of small round cells of unknown
origin. It is distinguished from other round cell tumors such as malignant
lymphoma of bone, myeloma, neuroblastoma, rhabdomyosarcoma, small cell osteosarcoma
and metastatic small cell carcinoma of the lung. Ewing's sarcoma is rare in
black populations and usually occurs between the ages of 10-20. Males are
affected more frequently than females. Any bone may be involved, but the pelvis
and lower extremities are the most common sites. The patient usually presents
with pain, swelling, a mass and occasionally fevers, weight loss and fatigue
for a few months. Blood tests may reveal anemia (low red blood cell count,
low hemoglobin or hematocrit) or show an increase in the sedimentation rate
(detects inflammatory, neoplastic/cancer, infectious and necrotic/cell breakdown
decay processes). Pathologic fractures may be present. X-rays show a destructive
lesion in the bone, a soft tissue mass, and destruction of the cortex which
elicits a host response/new bone giving an 'onion skin' look around the tumor.
Tumor tissue examined under the microscope shows the closely packed, small
round cells.
Treatment for Ewing's sarcoma involves chemotherapy and surgical resection.
Radiation therapy has also been used but with caution in young children with
growth plate involvement to prevent significant growth deformity. The prognosis
(outlook for survival) is now quite good due to advances in chemotherapy.
Lung metastases may occur in 20-25% of patients at diagnosis or subsequently
and are managed with different chemotherapy, radiation, resection or at times,
stem cell transplants.
Malignant Lymphomas of Bone may occur at any age but usually in the middle to older age groups. The most common sites are the pelvis, ribs and femur. There are no distinct patient symptoms, although many patients do present with pathologic fracture. It is important to have blood tests taken to rule out leukemia. Lymphomas of bone are similar to Ewing's sarcoma and on x-ray appear as distinctive lesions not well defined with breakage of the cortex and soft tissue mass. The tumor cells are large and round varying in size and shape. Treatment consists of radiation with surgical fixation of pathologic fractures if necessary, and adjuvant chemotherapy.
Multiple Myeloma,
a malignant tumor of plasma cells, is a common malignant bone tumor that usually
develops in middle age. It is a disorder affecting the entire bone marrow,
occurring at multiple sites throughout the skeleton, but may present as a
single bone lesion referred to as a plasmacytoma. Any bone can be involved
but the spine, ribs, skull, pelvis and proximal long bones are the most common
sites. Patients complain of pain in their bones with history of fever, fatigue,
weight loss and anemia. X-rays may show a fracture, widespread osteoporosis
or small puncture holes in the skeleton which can turn into large areas with
thinning of the cortex. Skeletal surveys (plain x-rays of many bones) are
done since bone scans may not always show all the tumor site.
Patients with myeloma often do not need a biopsy, but if done, the tissue
contains sheets of plasma cells. Blood tests are very helpful to establish
the diagnosis. Ninety percent of patients have anemia and a high sedimentation
rate. Calcium levels may be elevated in patients with extensive bony disease.
The blood test immunoelectrophoresis (IEP), which profiles the immunoglobins,
demonstrates an abnormal 'g component' (myeloma protein) in ninely percent
of patients. Immunoelectrophoresis studies on the urine is positive in over
sixty percent of patients. Diagnosis is confirmed by doing a bone marrow biopsy.
Normal bone marrow contains only a small amount of plasma cells. Thus, over
eight percent of plasma cells is suspicious for myeloma and over twenty percent
confirms diagnosis.
Treatment includes radiation because myelomas respond very well to the therapy
and the pain caused by these lesions is relieved. Fractured or weakened areas
of bone are treated surgically with internal fixation (metal hardware such
as pins, rods, plates, screws and possible cement or bone grafting). New forms
of chemotherapy and bone marrow transplantation are also being used.
Metastatic
Bone Tumors of the skeleton,
that is, malignant bone tumors caused by the spread (metastatic disease) of
another type of cancer, greatly outnumber the primary (original site) malignant
bone tumors. Most of these bone metastases are from prostate, breast, thyroid,
lung or kidney cancers. The most common sites for skeletal (bony) metastases
are the bones containing blood-forming marrow such as the spine, ribs, skull,
pelvis, and the ends of long bones especially the femur and humerus. Rarely
metastases to the hands or feet occur. In children, metastatic skeletal tumors
are usually due to neuroblastoma, leukemia or Ewing's sarcoma. In teenagers
or young adults, lymphoma is the primary disease. Metastases after age 30
are usually adenocarcinoma (a type of cancer that involves the cells lining
the walls of different organs in the body). Metastatic tumors from the kidney
or thyroid are usually very vascular (made of blood vessels) whereas tumors
from prostate, breast and lung are less so. With many patients, the primary
tumor is not identified, so staging studies and biopsy are necessary to rule
out a primary malignant bone tumor. Sometimes a primary site is never identified.
In patients with a history of cancer, a bone scan will show any additional
sites of skeletal involvement.
Most patients with bony metastases present with pain in the area of the lesion
with decreased mobility and motion. Staging studies are usually done to determine
the extent of the metastatic disease and plan the most appropriate treatment.
Pain control and surgical internal fixation to provide bone stability are
the major goals of treatment. Chemotherapy, use of hormones and palliative
radiation therapy, that is radiation to treat the symptoms not cure, are other
options used to care for patients with metastatic disease.
It is important to note that many patients have a significant amount of quality
time for themselves and their families despite living with metastatic disease.
Recent advances in cancer management has made that possible. These patients
living with metastatic disease have a phenomenal amount of inner strength
and hope.
Soft tissue tumors, like bone tumors, are
either benign or malignant. They develop in connective tissue other than bone
such as the skeletal muscle, fat, tendon, fibrous tissue and nerve and blood
vessel (neurovascular) tissue. Malignant soft tissue tumors, or soft tissue
sarcomas, are more common than bone sarcomas. They usually occur in adults
and present as a large painless or painful soft tissue mass. Because benign
soft tissue "lumps" are far more common than malignant ones, the
diagnosis of a soft tissue sarcoma may be made after excision of a presumed
benign mass at a local hospital or in the local doctor's office. When the
pathologist reports a sarcoma, the patients are referred to us, a cancer center,
for staging studies to assess the local extent and/or spread of the tumor
and for further treatment (surgery, chemotherapy or radiation).
Soft tissue tumors can occur anywhere in the body but are most frequent in
the lower extremities, trunk and abdomen and upper extremities. The cause
is unknown. As with bone tumors, trauma may bring attention to a previous
existing tumor. Soft tissue tumors are also classified by their tissue type
and grade. The higher the grade, the greater the chance for aggressive growth
at the original site and for metastases to distant sites. The most common
metastatic site is the lungs. Synovial sarcoma (epithelioid sarcoma) and rhabdomyosarcoma
(the most common childhood soft tissue sarcoma) may also spread to the lymph
nodes.
Benign soft tissue tumors, like benign bone, rarely metastasize. They are
categorized according to their behavior to be aggressive or not, ranging from
inactive, to active, to aggressive. Malignant soft tissue tumors usually grow
rapidly and have the ability to metastasize to distant sites, most commonly
the lungs. Like malignant bone sarcomas, malignant soft tissue sarcomas are
categorized by their behavior to metastisize ranging from low grade (lower
chance of spread) to high grade (higher chance of spread). As with bone tumors,
staging a soft tissue tumor involves evaluating the histology (tissue grade)
of the tumor, how extensive the tumor is at the primary site, and whether
or not there are distant metastases. All of these characteristics help describe
the tumor's behavior (biologic activity), the patient's prognosis and the
best treatment option.
Patients with soft tissue sarcomas usually present with large, nontender masses
that may have been there for many months or even years. Many of these masses
are thought to be benign and are removed (excised) without staging studies
or biopsy. Any mass larger than 5 centimeters, which is located deep into
muscle under the superficial layer of fat and feels firmer than normal muscle,
should undergo all imaging studies (MRI, bone scan, CT scan) before excision.
The MRI scan is the best imaging study for soft tissue tumors because of its
sensitive technique to show differences between normal and sarcoma tissue.
It also clearly shows the relation of the tumor to nearby nerves and blood
vessels. A chest CT scan is always necessary to rule out lung metastases,
and a biopsy will determine diagnosis and treatment.
In adults the most common benign soft tissue tumor is a lipoma. In children, popliteal (Baker's) cysts, lipomas and hemangiomas are common. Benign tumors may be very large and deep but are usually soft except for the fibrous tumors which may feel firm.
Lipoma is the most common benign soft tissue tumor made of fat and is usually large and soft. They are located on the back, shoulders, abdomen and upper and lower extremities. They usually do not cause symptoms unless they begin to push on nearby nerves. Lipomas usually grow slowly with little change over many years. Many patients seek medical attention for cosmetic reasons when a lipoma grows too large. An angiolipoma which involves blood vessels usually occurs in children located deep in the muscle and is tender because it involves the vessels. On the MRI scan, the lipoma is seen as a bright, well defined mass. Treatment includes removing (excising) the mass or observing it if it is not bothersome. Recurrences are unusual.
Hemangioma
is a benign vascular (involving blood vessels) soft tissue tumor. It is the
most common soft tissue tumor in children. It can develop in the top skin
(superficial) layer or deep within the muscle. They occur more often in the
lower extremities than the upper extremities. Hemangiomas can range from being
well-defined, noninvasive and involving small vessels (capillaries) to less
defined, invasive and involving large vessels. The most common type of hemangioma
is made of both large and small vessels. The MRI scan is the best type of
imaging to show the location and extent of the tumor. Angiograms may also
be done to evaluate the large blood vessels.
Treatment is not necessary unless they cause pain. Aspirin and compression
stockings may be quite successful in relieving symptoms. Surgical excision
is sometimes necessary if symptoms persist. Although hemangiomas are vascular,
they do not spread to other areas or turn malignant. Occasionally for large
tumors that can not be removed, radiation therapy and embolization (a procedure
to plug/stop the blood supply in the tumor area preventing tumor growth) has
been attempted to treat hemangiomas with little success. Recently, treatment
has also included injections with alcohol into the tumor (sclerotherapy).
Fibroma and Fibromatosis
Benign soft tissue tumors made of fibrous tissue come in many different forms.
Congenital fibromatosis occurs in infants and usually presents as a solitary
mass. Fibrous tumors in adolescents and adults include fibromas, extra-abdominal
desmoid tumors, palmer fibromatosis and nodular fasciitis. Fibromatosis (desmoid
tumor) refers to a benign but aggressive tumor usually located in the lower
extremity. Fibromatoses are very aggressive at the original site and invade
the nearby muscle, soft tissue and bone but do not metastasize (spread to
other sites). MRI scans or CT scans are done preoperatively. Plain x-rays
will rule out any bone involvement under the soft tissue mass, and a bone
scan will show any other bony invasion.
Treatment includes removal (excision) of the soft tissue mass. In some cases
the surgical margin (area around the tumor) is not large enough because the
tumor extends microscopically beyond the main mass. Thus, there is a high
rate of recurrence. These tumors should be treated aggressively with wide
margin excisions. Radiation therapy is also used for local control.
Neurofibroma and Neurofibromatosis
are benign soft tissue tumors that are made of nerve tissue. They are also
called nerve sheath tumors or schwannomas because the tumor tissue has cells
like the nerve sheath (envelope around the nerve fibers), or Schwann cell.
Neurofibromas may occur as a single tumor or many tumors and may be found
anywhere on the body. They range from simple, small masses to gross enlargement
of an extremity (elephantiasis), to masses causing bony erosion and deformities
of the spine. Since neurofibromas come from nerve roots, those located in
the spine can press on the spinal cord causing neurologic symptoms.
Neurofibromatosis is an inherited syndrome and is the most common genetic
disorder, usually diagnosed in childhood. The patient presents with greater
than six cafe au lait spots ('coffee with milk' colored, brownish, birthmark
spots on the skin) and/or many neurofibromas (called
von Recklinghausen's disease) over their entire body. Patients with
severe disease may have large neurofibromas involving an entire extremity.
An MRI scan is necessary to evaluate the size and extent of the neurofibroma.
The mass is usually seen in connection with a major nerve. Examination of
the tumor tissue shows a tumor composed of spindle cells. The treatment involves
excision of neurofibromas if they are large, painful or interfere with function.
Single neurofibromas once excised rarely recur. The multiple neurofibromas
of neurofibromatosis may become malignant. Most often they are observed and
not excised unless they are growing or painful. If a sarcoma develops, the
patient must undergo staging studies to rule out metastatic disease. Pre-
and/or post-operative radiation therapy is usually included in the treatment
plan, and sometimes chemotherapy.
Pigmented Villonodular
Synovitis (PVNS) is a benign soft tissue
mass involving the synovial membrane (lining of a joint). It is most often
seen in the knee or hip joint of adults under age 40. Patients usually present
with a boggy, swollen, painful joint. The synovial membrane becomes thick.
Many times the disease process goes beyond the joint and causes severe degenerative
joint disease. If an aspirate (fluid withdrawn from an area with a needle)
of the joint is done, it will show a bloody, brown fluid. Other diagnoses
that must be ruled out are infection, rheumatoid arthritis, hemangioma, giant
cell tumors of tendon sheath or malignant synovial sarcoma. X-rays of PVNS
show an increase in joint fluid, a thick synovial membrane and sometimes bone
erosion. Bone scans show increased activity in the joint area, and synovial
thickening and nodularity or a mass are seen on the MRI scan. A biopsy should
be performed on large nodular tumors to rule out sarcoma. Tumor tissue reveals
many rounded synovial-like cells with a good amount of giant cells and inflammatory
cells. Treatment involves an excision of the tumor. If the PVNS is widespread,
a complete synovectomy (excision of the membrane that lines joints lubricating
them with fluid) must be done. Localized nodular synovitis is simply excised.
PVNS may recur unless the entire synovial membrane is removed. Widespread
PVNS usually causes chronic degenerative joint disease which may require total
joint replacements. Radiation is an alternative adjuvant therapy in recurrent
cases.
Myositis Ossificans is a non-tumorus soft
tissue mass that occurs after a blunt trauma. The soft tissue reacts to the
trauma by forming a bony mass. Patients present with a painful or painless
mass which is increasing in size. The tumor is usually located in the upper
arm, thigh or buttock. X-rays show a round ossified (bony) mass which may
be read as a sarcoma. However, the myositis ossificans has a distinct margin
of ossification (bone formation) with an active center containing immature
cells. Sarcomas have active and inactive tissues on the outer margin with
an inactive center. Bone scans show increased activity in the area of the
mass for about 8-12 weeks, and then the activity decreases. The CT scan is
the best imaging to show the bony mass. Treatment involves observation or
excision usually after an 8-12 week period because if it is excised before
full maturation, recurrence is likely. There lesions sometimes resolve without
treatment.
Ganglion or Synovial
Cyst
is a benign soft tissue mass 'cyst like' in nature and comes from the capsule
of the joint, synovial membrane (lining) or tendon sheath (covering). It usually
develops on the top of the wrist in adults between 25 and 45 years of age,
and are more common in women. The cause is unknown, but repeated trauma is
a contributing factor. Some patients have no symptoms, while other have tenderness,
pain and problems with wrist function. A needle aspiration should be done
if a sarcoma is suspected. Staging studies are usually not necessary because
of the ganglion cyst's classic appearance, although ultrasound is helpful
in confirming the cystic nature of the lesion. The excised cyst or nodule
has an outer fibrous layer with an inner lining made of clear colorless, gelatin-like
fluid.
Some ganglia need no treatment because they go away on their own. Usually
needle aspiration or rupture of the cyst give temporary relief. Recurrence
is common. The best treatment for a patient who has symptoms is to completely
excise the cyst down to the base of the cyst which should prevent recurrence.
Baker's cysts behind the knee in children nearly always resolve without treatment.
MALIGNANT SOFT TISSUE TUMORS (SARCOMAS)
Liposarcoma is a malignant soft tissue tumor of fat tissue containing malignant lipoblasts. It usually occurs in the thigh, abdomen area (retroperitoneum) and behind the knee (popliteal fossa). The tumor tissue has four types: myxoid (low grade), round cell (high grade), pleomorphic (giant cell-high grade), and well differentiated (low grade). Patients must undergo all staging studies. The MRI scan is more sensitive than the CT scan for this type of sarcoma. The MRI shows high grade liposarcomas to be more vascular and denser than lower grades. Treatment consists of wide excision of the tumor with radiation therapy and sometimes chemotherapy depending on the tumor size and/or presence of lung metastases. Patients with tumors larger than 8 centimeters usually have preoperative chemotherapy and radiation followed by the surgery and postoperative chemotherapy (MAID protocol). Postoperative radiation is only required if the pathology evaluation reveals positive margins (tumor cells present in the surrounding tissue).
Malignant Fibrous Histiocytoma (MFH) is a high grade malignant soft tissue tumor of fibrous tissue arranged in a pin wheel pattern along with other bizarre looking cells. It is the most common soft tissue sarcoma in older adults and is more common in men. MFH is usually located in the upper and lower extremities and the abdominal area. MFH can occur as a primary bone tumor as noted in the section on bone tumors. MFH is best imaged with an MRI scan, but chest CT scans must be done to stage the tumor. Treatment for low grade tumors involves wide excision and adjuvant therapy such as radiation. High grade tumors require resections with radical margins with both radiation and chemotherapy recommended. Patients with tumors larger than 8 centimeters will undergo the MAID protocol as discussed with liposarcoma.
Fibrosarcoma
is a malignant soft tissue tumor or sarcoma that accounts for less than 10%
of soft tissue tumors. It occurs more frequently in men, usually in the lower
extremities. Tumor tissue looks very similar to malignant fibrous histiocytoma
(MFH). In the past, most tumors of muscle and bone (musculoskeletal) were
diagnosed as fibrosarcomas. Today, pathologists are able to be more specific
with cell classification. Thus, only tumor tissue with spindle cells arranged
in a 'herringbone pattern' are called fibrosarcomas. In addition to looking
like MFH, high grade fibrosarcomas may resemble synovial sarcoma. Low grade
fibrosarcomas have to be distinguished from an aggressive fibromatosis or
desmoid-type tumors. The importance of adequate staging studies and proper
interpretation of the biopsy to determine diagnosis can not be stressed enough.
Treatment for high grade fibrosarcoma involves a wide excision, usually combined
with radiation therapy. Lower grade tumors may not need chemotherapy, but
surgical margins must be adequate or else radiation will be required. Patients
with tumors larger than 8 centimeters will undergo the MAID protocol as discussed
with liposarcoma. Children and adolescents are more likely to be diagnosed
with an aggressive benign fibromatosis instead of a low grade fibrosarcoma.
They usually do well with excision of the tumor.
Synovial Sarcoma is a malignant soft tissue
tumor that arises near, but not, as the name implies, in the joint itself.
About 10% of soft tissue sarcomas are synovial sarcomas. The patient usually
presents with a painful mass next to a joint which is growing in size. It
usually occurs in teenagers or patients under 40 years of age, most often
in the lower leg around the knee. Lower grade synovial sarcomas tend to be
found on the hands or feet, and may be confused with a benign ganglion. X-rays
show a soft tissue thick shadow with calcifications (deposits of calcium salts
in normal bone or abnormally in soft tissue) in it. A biopsy is necessary
to rule out the many other possible soft tissue masses that have calcifications
but are benign. Metastases to lymph nodes (small kidney-shaped organs of lymphoid
tissue that lie along the lymphatic vessels) are more common with synovial
sarcoma than other types of soft tissue sarcomas. The tumor may also spread
to the lungs in more advanced cases. Treatment consists of radical or wide
excision of the tumor with adjuvant therapy (chemotherapy and/or radiation
therapy). Small, low grade tumors may do well with excision only.
Leiomyosarcoma
and Rhabdomyosarcoma are malignant soft tissue
tumors of muscle. Leiomyosarcoma involves smooth muscle of blood vessels or
organ walls and rhabdomyosarcoma involves skeletal muscle. Although they are
both tumors of muscle, they have very different characteristics.
Leiomyosarcoma is a high grade soft tissue sarcoma that usually penetrates
beyond its compartment (stage IIB). It usually occurs in adults, especially
women. The most common site is the abdomen (retroperitoneum). Leiomyosarcomas
are classified by their location: retroperitoneum, subcutaneous skin, blood
vessels or extremities. Treatment is surgical excision with or without radiation,
and occasionally chemotherapy.
Rhabdomyosarcoma is the most common soft tissue sarcoma in children, occurring
more in boys than girls. About 15-20% of all soft tissue sarcomas are rhabdomyosarcomas.
Examination of the tumor tissue shows four types: embryonal, boytroid, alveolar
and pleomorphic. The location of the tumor is related to the patient's age.
Head, neck and pelvis are the most common sites in younger patients. In adolescents,
extremity and testicular tumors are the most common because rhabdomyosarcoma
has a significant rate of metastases to lymph nodes. Lymph node biopsies are
sometimes done.
Treatment for extremity rhabdomyosarcoma involves adjuvant therapy (chemotherapy
and/or radiation therapy) and usually wide margin excisions. Leiomyosarcomas
involving major nerves and blood vessels may need a resection with reconstruction
of the vessels, or an amputation. The local recurrence rate is very high if
surgical margins are not adequate.
Angiosarcoma is a high grade malignant soft tissue tumor involving blood vessel cells. Hemangioendothelioma and hemangiopericytoma are lower grade tumors. Only 1-2% of soft tissue sarcomas come from vascular origin. Angiosarcomas usually occur in men between the ages of 40 and 70. They are found in the skin, both superficial and deep soft tissue, the breast, liver and bone. The site and extent of the lesion is clearly seen on the MRI or CT scan staging studies. The bone scan shows marked uptake in the tumor area. Prognosis for low grade hemangioendothelioma and hemangiopericytoma is better than high grade angiosarcoma. Treatment for all involves a wide excision. Of course, other adjuvant therapy depends on the grade and stage of the tumor at the time of presentation.
Malignant Schwannoma
(Neurofibrosarcoma) is a malignant soft tissue
tumor involving the nerves. It is also known as neurofibrosarcoma or fibrosarcoma
of nerve sheath. The words 'malignant schwannoma' are also used to note that
the tumor cells resemble Schwann cells. These tumors may arise within a preexisting
benign schwannoma, especially in neurofibromatosis. This changing from benign
to malignant is called malignant transformation. Ten percent of all soft tissue
sarcomas are malignant schwannomas, and half of them are related to neurofibromatosis
(NF or von Recklinghausen's disease). They occur more often in men who present
with a large mass causing pain which radiates or travels down the extremity.
Examination of the tumor usually shows the nerve entering at the top of the
tumor and existing at the bottom of the tumor. The tumor cells are shaped
like commas and collect in a wavy, picket-fence type pattern. Wide excision
with limb-sparing procedures or amputation are treatment options for malignant
schwannomas, along with radiation and chemotherapy. Patients with tumors larger
than 8 centimeters will undergo the MAID protocol as discussed with liposarcoma.
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