9.2 - Soft Tissue Tumors

11/23/13 /Classification of Soft Tissue Tumors/Soft Tissue Tumors
section 2 Soft Tissue Tumors
I. INTRODUCTION
Soft tissue tumors are common. They may appear as small lumps or large masses.
A. Classification: Soft tissue tumors can be broadly classified as benign or malignant
(sarcoma) or characterized by reactive tumor-like conditions (Box 9-2). Lesions are
classified according to the direction of differentiation of the lesion: the tumor tends to
produce collagen (fibrous lesion), fat, or cartilage.
1. Benign soft tissue tumors: These tumors may occur in all age groups. The biologic behavior
of these lesions varies from asymptomatic and self-limiting to growing and symptomatic. On
occasion, benign lesions grow rapidly and invade adjacent tissues.
2. Malignant soft tissue tumors (sarcomas): Sarcomas are rare tumors of mesenchymal origin.
In the United States, there are approximately 9000 new cases of soft tissue sarcoma each
year.
▪ Diagnosis: Patients often experience an enlarging painless or painful soft tissue
mass, which is the most common reason for seeking medical attention.
□ Most sarcomas are large (>5 cm), deep, and firm.
□ In some instances, they are small and may be present for a long time before
they are recognized as tumors (synovial sarcoma, rhabdomyosarcoma,
epithelioid sarcoma, and clear cell sarcoma).
□ Initial radiographic evaluation begins with radiographs in two planes.
□ MRI is the best imaging modality for defining the anatomy and helping
characterize the lesion. When a mass is judged to be indeterminate, an open
incisional or needle biopsy is performed. A definitive histologic diagnosis must be
established before treatment is planned.
□ CT scan of the chest is required in order to evaluate for metastasis. CT
scan of the abdomen and pelvis is obtained for liposarcoma because of
synchronous retroperitoneal liposarcoma.
▪ Treatment: Radiation therapy is an important adjunct to surgery in the treatment of soft

tissue sarcomas.
□ Ionizing radiation can be delivered preoperatively, perioperatively with
brachytherapy after loading tubes, or postoperatively.
□ Treatment regimens are often designed to use combinations of the three types of
preoperative, postoperative, and external beam irradiation. Poor prognostic
factors include the presence of metastases, high grade, size greater than 5 cm,
and location below the deep fascia.
B. Diagnosis: The evaluation of patients with soft tissue tumors must be systematic to avoid
errors.
1. Unplanned removal of a soft tissue sarcoma is the most common error.
▪ Residual tumor may exist at the site of the operative wound, and repeat
excision for all patients with an unplanned removal should be performed.
2. Delay in diagnosis may also occur if the clinician does not recognize that the lesion is
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malignant.
3. Patients who have a new soft tissue mass or one that is growing or causing pain should
undergo MRI.
4. The MRI scan should be carefully reviewed with a radiologist to characterize the nature of the
mass. If it can be determined that the lesion is a benign process such as a lipoma, ganglionic
cyst, or muscle tear, then it is classified as a determinate lesion, and treatment can be
planned without a biopsy. In contrast, if the exact nature of a lesion cannot be
determined, the lesion is classified as indeterminate, and either a needle or open
biopsy is necessary to determine the exact diagnosis. Then treatment can be planned.
5. Excisional biopsy should not be performed when the clinician does not know the origin of a
soft tissue tumor.
C. Metastasis: Most soft tissue sarcomas metastasize to the lung.

1. Lymph node metastasis occurs with 5% of soft tissue sarcomas. Rhabdomyosarcoma,
clear cell sarcoma, epithelioid sarcoma, and synovial sarcoma are the tumors that
most commonly metastasize via the lymphatic vessels.
II. TUMORS OF FIBROUS TISSUE

Fibrous tumors are common, and their characteristics range widely, from small, self-limiting, benign
conditions to aggressive, invasive, benign tumors. The malignant fibrous tumors are fibrosarcoma and
malignant fibrous histiocytoma.
A. Calcifying aponeurotic fibroma
1. Manifests as a slow-growing, painless mass in the hands and feet in children and young
adults 3 to 30 years of age.
2. Radiographs may reveal a faint mass with stippling.
3. Histologic examination reveals a fibrous tumor with centrally located areas of calcification and
cartilage formation.
4. After local excision, the tumor often recurs (in up to 50% of cases); however, the condition
appears to resolve with maturity.
B. Fibromatosis
1. Palmar (Dupuytren) and plantar (Ledderhose) fibromatosis: These disorders consist of firm
nodules of fibroblasts and collagen that develop in the palmar and plantar fascia. The nodules
and fascia become hypertrophic, producing contractures.
2. Extraabdominal desmoid tumor
▪ Most locally invasive of all benign soft tissue tumors.
▪ Commonly occurs in adolescents and young adults.
□ Patients with Gardner syndrome (familial adenomatous polyposis) have
colonic polyps and a 10,000-fold increased risk of developing desmoid
tumors.
▪ On palpation, the tumor has a distinctive “rock-hard” character.

▪ Multiple lesions may be present in the same extremity (10% to 25%).
▪ Histologically, the tumor consists of well-differentiated fibroblasts and abundant
collagen. The lesion infiltrates adjacent tissues. Immunohistochemistry study
reveals positivity for estrogen receptor β, and inhibitors have been used for
treatment.
▪ Surgical treatment is aimed at resecting the tumor with a wide margin.
▪ Local recurrence is common.
▪ Radiotherapy has been used as an adjunctive treatment to prevent recurrence and
progression.
▪ Behavior of the tumor is capricious: Recurrent nodules may remain dormant for years
or grow rapidly for some time and then stop growing.
C. Nodular fasciitis
1. A common reactive lesion that manifests as a painful, rapidly enlarging mass in a
young person (15 to 35 years of age).
2. Half of these lesions occur in the upper extremity.
3. Short, irregular bundles and fascicles; a dense reticulum network; and only small amounts of
mature collagen characterize the lesion histologically. Mitotic figures are common, but
atypical mitoses are not a feature.
4. Treatment consists of excision with a marginal line of resection.
D. Malignant fibrous soft tissue tumors: Malignant fibrous histiocytoma and fibrosarcoma are
the two malignant fibrous lesions.
1. Diagnosis
▪ Similar clinical and radiographic manifestations; treatment methods are similar
▪ Patients are generally between the ages of 30 and 80 years.
▪ Most common manifestation is an enlarging, generally painless mass.
▪ MRI often shows a deep-seated, inhomogeneous mass that has a low signal on
T1-weighted images and a high signal on T2-weighted images. The two lesions
may be similar histologically, but there are distinctive features:
□ Malignant fibrous histiocytoma: The spindle and histiocytic cells are arranged in
a storiform (cartwheel) pattern. Short fascicles of cells and fibrous tissue appear
to radiate about a common center around slitlike vessels. Chronic inflammatory
cells may also be present.
□ Fibrosarcoma: There is a fasciculated growth pattern, with fusiform or spindle-
shaped cells, scanty cytoplasm, and indistinct borders, and the cells are
separated by interwoven collagen fibers. In some cases, the tissue is organized
into a herringbone pattern, which consists of intersecting fascicles in which the
nuclei in one fascicle are viewed transversely but in an adjacent fascicle are
viewed longitudinally.
▪ Treatment is by wide-margin local excision. Radiation therapy is employed in many

cases when the size of the tumor exceeds 5 cm.
▪ A common scenario is to deliver radiation preoperatively (5000 cGy), followed by
resection of the lesion. A final radiation boost (1400 to 2000 cGy) is then administered
postoperatively or with brachytherapy afterloading tubes if the margins are very close or
positive.
▪ Postoperative external beam irradiation (6300 to 6600 cGy) yields equal local
control rates, with a lower postoperative wound complication rate but a higher
incidence of postoperative fibrosis.
E. Dermatofibrosarcoma protuberans
1. Rare, nodular, cutaneous tumor that occurs in early to middle adulthood
2. Low grade, with a tendency to recur locally, but it only rarely metastasizes (often after
repeated local recurrence)
3. In 40% of the cases, it occurs on the upper or lower extremities. The tumor grows slowly but
progressively.
4. The central portion of the nodules shows uniform fibroblasts arranged in a storiform pattern
around an inconspicuous vasculature.
5. Wide-margin surgical resection is the best form of treatment.
III. TUMORS OF FATTY TISSUE

There is a wide spectrum of benign and malignant tumors of fat origin. Each has a particular biologic
behavior that guides evaluation and treatment.
A. Lipomas: common benign tumors of mature fat
1. Occur in a subcutaneous, intramuscular, or intermuscular location
2. History of a mass is long, but sometimes the mass was only recently discovered.
3. Not painful
4. Radiographs may show a radiolucent lesion in the soft tissues if the lipoma is deep within the
muscle or between the muscle and bone.
5. CT scan or MRI shows a well-demarcated lesion with the same signal characteristics
as those of mature fat on all sequences. On fat suppression sequences, the lipoma
has a uniformly low signal. If the patient experiences no symptoms and the
radiographic features are diagnostic of lipoma, no treatment is necessary.
6. If the mass is growing or causing symptoms, excision with a marginal line of
resection or an intralesional margin is all that is necessary.
7. Local recurrence is uncommon.
8. Several variants:
▪ Spindle cell lipoma
□ Commonly occurs in men (45 to 65 years of age)
□ Manifests as a solitary, painless, growing, firm nodule
□ Histologically characterized by a mixture of mature fat cells and spindle cells.
There is a mucoid matrix with a varying number of birefringent collagen fibers.
□ Treatment is excision with a marginal margin.
▪ Pleomorphic lipoma
□ Occurs in middle-aged patients
□ Manifests as a slow-growing mass
□ Histologically characterized by lipocytes; spindle cells; and scattered, bizarre
giant cells
□ May be confused with different types of liposarcoma
□ Treatment is by excision with a marginal margin.
▪ Angiolipoma
□ The only lipoma that is very painful when palpated.
□ Manifests with small nodules in the upper extremity that are intensely painful
□ MRI may show a small, fatty nodule or completely normal appearance.
□ Consists of mature fat cells (as in a typical lipoma) and nests of small arborizing
vessels
B. Liposarcomas
1. Type of sarcoma; direction of differentiation is toward fatty tissue
2. Heterogeneous group of tumors, having in common the presence of lipoblasts (signet ring–
shaped cells) in the tissue
3. Liposarcomas virtually never occur in the subcutaneous tissues.
4. They are classified into the following types:
▪ Well-differentiated liposarcoma (low grade)
□ Lipoma-like
□ Sclerosing
□ Inflammatory
▪ Myxoid liposarcoma (intermediate grade)

▪ Dedifferentiated (high grade)
▪ Round-cell liposarcoma (high grade)
▪ Pleomorphic liposarcoma (high grade)
5. Liposarcomas metastasize according to the grade of the lesion:

▪ Well-differentiated liposarcomas have a very low rate of metastasis
(<10%)
▪ The metastasis rate of intermediate-grade liposarcomas is 10% to 30%.
▪ The metastasis rate of high-grade liposarcomas is more than 50%.
IV. TUMORS OF NEURAL TISSUE

The two benign neural tumors are neurilemoma and neurofibroma. Their malignant counterpart is
neurofibrosarcoma.
A. Neurilemoma (benign schwannoma)
1. Benign nerve sheath tumor
2. Occurs in young to middle-aged adults (20 to 50 years of age)
3. Patients have no symptoms except for the presence of the mass.
4. Tumor grows slowly and may wax and wane in size (cystic changes).
5. MRI studies may demonstrate an eccentric mass arising from a peripheral nerve, or they may
show only an indeterminate soft tissue mass (low signal on T1-weighted images and high
signal on T2-weighted images).
6. Histologically, the lesion is composed of Antoni A and B areas.
▪ Antoni A area:
□ Compact spindle cells usually having twisted nuclei; indistinct cytoplasm; and
occasionally clear, intranuclear vacuoles
□ There may be nuclear palisading, whorling of cells, and Verocay bodies.
□ When the lesion is predominantly cellular (Antoni A), the tumor may be confused
with a sarcoma.
□ Treatment: removing the eccentric mass while leaving the nerve intact
▪ Antoni’a B area:
□ Less orderly and cellular
□ Arranged haphazardly in the loosely textured matrix (with microcystic changes,
inflammatory cells, and delicate collagen fibers)
□ Vessels are large and irregularly spaced.
B. Neurofibroma
1. Solitary or multiple (neurofibromatosis)
2. Superficial, slow-growing, and painless
3. When they involve a major nerve, they may expand it in a fusiform manner.
4. Histologic study shows interlacing bundles of elongated cells with wavy, dark-
staining nuclei.
5. Cells are associated with wirelike strands of collagen.
6. Small to moderate amounts of mucoid material separate the cells and collagen
7. Treatment: excision with a marginal margin.
C. Neurofibromatosis (von Recklinghausen disease)

1. Autosomal dominant trait (both peripheral and central forms)
2. Café au lait spots (smooth) and Lisch nodules (melanocytic hamartomas in the iris)
3. Variable skeletal abnormalities (metaphyseal fibrous defect [nonossifying fibroma], scoliosis,
and long-bone bowing)
4. Malignant changes occur in 5% to 30% of affected patients.
5. Pain and an enlarging soft tissue mass may herald conversion to a sarcoma.
D. Neurofibrosarcoma
1. Rare tumor that arises in a de novo manner or in the setting of neurofibromatosis
2. High-grade sarcoma; treated in a manner similar to that for other high-grade sarcomas
V. TUMORS OF MUSCLE TISSUE

A. Leiomyosarcoma
1. Manifests as a small nodule or a large extremity mass
2. May or may not be associated with blood vessels
3. Low or high grade
B. Rhabdomyosarcoma
1. The most common sarcoma in young patients; may grow rapidly
2. Composed of spindle cells in parallel bundles, multinucleated giant cells, and racquet-shaped
cells
3. Cross-striations within the tumor cells (rhabdomyoblasts)
4. Rhabdomyosarcomas are sensitive to multiagent chemotherapy and wide-margin surgical
resection after induction of chemotherapy. External beam irradiation plays a prominent role in
treatment.
VI. VASCULAR TUMORS

A. Hemangioma
1. Commonly seen in children and adults
2. Cutaneous, subcutaneous, or intramuscular location
3. Large tumors have signs of vascular engorgement (aching, heaviness, swelling).
4. MRI scans demonstrate a heterogeneous lesion with numerous small blood vessels and fatty
infiltration.
5. It is important to examine the patient in both the supine and standing positions. (The lower
extremity often fills with blood after several minutes).
6. Radiographs may reveal small phleboliths.
7. Nonoperative treatment: NSAIDs, vascular stockings, and activity modification if local
measures adequately control discomfort
8. Can be treated by application of a sclerosing agent such as alcohol
B. Angiosarcoma
1. Cells resemble the endothelium of blood vessels.
2. Highly malignant
3. Infiltrative; with local excision, the rate of failure is high.
4. Amputation may be necessary to achieve local control.

5. Pulmonary metastases are common.
VII. SYNOVIAL DISORDERS

A. Ganglia
1. Out-pouching of the synovial lining of an adjacent joint
2. Common locations include the wrist, foot, and knee.
3. Filled with gelatinous, mucoid material
4. Paucicellular connective tissue without a true epithelial lining
5. MRI: homogeneously low signal on T1-weighted images and a very bright signal on T2-
weighted images; contrast agent such as gadolinium is useful in differentiating a cyst from a
solid neoplasm because cysts do not enhance (except for a small rim at the periphery) but
active neoplasms usually do.
B. Pigmented villonodular synovitis (PVNS)

1. Reactive condition (not a true neoplasm) characterized by an exuberant proliferation of
synovial villi and nodules
2. May occur locally (within a joint) or diffusely
3. The knee is affected most often, followed in frequency by the hip and shoulder.
4. Manifests with pain and swelling in the affected joint
5. Recurrent, atraumatic hemarthrosis is the hallmark (arthrocentesis demonstrates a bloody
effusion).
6. Cystic erosions may occur on both sides of the joint.
7. Highly vascular villi are lined with plump, hyperplastic synovial cells; hemosiderin-
stained, multinucleated giant cells; and chronic inflammatory cells.
8. Treatment is aimed at complete synovectomy by arthroscopy for resection of all the
intraarticular disease, followed by open posterior synovectomy to remove the
posterior extraarticular extension.
▪ The local form of PVNS may be treated with partial synovectomy.
9. Local recurrence is common (30% to 50% of cases) despite complete synovectomy.

10. External beam irradiation (3500 to 4000 cGy) can reduce the rate of local recurrence to 10%
to 20%.
C. Giant cell tumor of tendon sheath

1. Benign nodular tumor occurs along the tendon sheaths (hands/feet).
2. Moderately cellular (sheets of rounded or polygonal cells) zones; hypocellular, collagenized
zones; multinucleated giant cells are common, as are xanthoma cells.
3. Treatment: resection with a marginal margin
4. Local recurrence is common (usually treated with repeat excision).
D. Synovial chondromatosis
1. Synovial proliferative disorder that occurs within joints or bursae, ranging in appearance from
metaplasia of the synovial tissue to firm nodules of cartilage
2. Typically affects young adults, who present with pain, stiffness, and swelling
3. The knee is the most common location.
4. Radiographs may demonstrate fine, stippled calcification.
5. Treatment: removal of the loose bodies and synovectomy
E. Synovial sarcoma
1. Highly malignant, high-grade tumor that occurs near joints, most commonly around the knee
2. Although the name implies that it arises from synovial cells, it rarely arises from an
intraarticular location.
▪ Typically manifests between the ages of 15 and 40 years
3. May be present for years or may manifest as a rapidly enlarging mass

4. Lymph nodes may be involved.
5. Most common synovial sarcoma is in the foot.
6. Radiographs or CT scans may show mineralization within the lesion in up to 25% of
cases (spotty mineralization may even resemble the peripheral mineralization seen
in heterotopic ossification).
▪ Irregular contour differentiates these lesions from hemangioma.
7. The tumor is often biphasic, with both epithelial and spindle cell components.
▪ The epithelial component may show epithelial cells that form glands or nests, or they
may line cystlike spaces.
9. The tumor may also be composed of a single type of cell (monophasic); the monophasic
fibrous type is much more common than the monophasic epithelial type.
10. Translocation between chromosome 18 and the X chromosome—t(X;18)—is always
present in tumor cells, and staining of the tumor cells yields positive results for
keratin and epithelial membrane antigen.
11. The balanced translocation results in gene fusion products. The two most common
are SYT-SSX1 and SYT-SSX2.
12. Wide-margin surgical resection with adjuvant radiotherapy is the most common method of
treatment.
13. Metastases develop in 30% to 60% of cases.
14. Larger tumors (>5 to 10 cm) are more prone to distant spread.
VIII. OTHER RARE SARCOMAS

A. Epithelioid sarcoma
1. Rare nodular tumor that commonly occurs in the upper extremities of young adults
2. May also occur about the buttock/thigh, knee, and foot
3. The most common sarcoma of the hand
4. May ulcerate and mimic a granuloma or rheumatoid nodule
5. Lymph node metastases are common.
6. Cells range in shape from ovoid to polygonal, with deeply eosinophilic cytoplasm (cellular
pleomorphism is minimal).
7. Often misdiagnosed as benign processes.
8. Wide-margin surgical resection is necessary to prevent local recurrence.
B. Clear cell sarcoma

1. Manifests as a slow-growing mass in association with tendons or aponeuroses
2. Usually occurs about the foot and ankle but may also involve the knee, thigh, and hand
3. Characterized by compact nests or fascicles of rounded or fusiform cells with clear

cytoplasm; multinucleated giant cells are common
4. Wide-margin surgical resection with adjuvant irradiation is the treatment of choice.
C. Alveolar cell sarcoma

1. Manifests as a slow-growing, painless mass in young adults (15 to 35 years of age)
2. Occurs in the anterior thigh

3. Dense, fibrous trabeculae dividing the tumor into an organoid or nestlike arrangement; cells
are large and rounded and contain one or more vesicular nuclei with small nucleoli
4. Treatment: wide-margin surgical resection with adjuvant irradiation in selected cases
IX. POST-TRAUMATIC CONDITIONS

A. Hematoma
1. Hematoma may occur after trauma to the extremity.
2. Organizes and resolves with time
3. Sarcomas may spontaneously hemorrhage into the body of the tumor or after minor
trauma and masquerade as a benign process. A lack of fascial plane tracking and
subcutaneous ecchymosis suggests that the bleeding is contained by a
pseudocapsule; this is an important physical examination finding.
4. Clinicians should monitor patients with hematomas at 6-week intervals until the mass
resolves.
5. MRI scanning is often not able to distinguish a simple hematoma from a sarcoma with
spontaneous hemorrhage.
B. Myositis ossificans (heterotopic ossification)

1. Develops after single or repetitive episodes of trauma (occasionally, patients cannot recall the
traumatic episode)
2. Most common locations are over the diaphyseal segment of long bones (in the middle aspect
of the muscle bellies).
3. As maturation progresses, radiographs show peripheral mineralization with a central lucent
area.
4. Lesion is not attached to the underlying bone, but in some cases, it may become fixed to the
periosteal surface.
5. Zonal pattern, with mature, trabecular bone at the periphery and immature tissue in the center
6. Nonoperative treatment is all the management that is necessary.
Classification of Soft Tissue Tumors
TUMORS AND TUMOR-LIKE LESIONS OF FIBROUS TISSUE

Benign
Fibroma
Nodular fasciitis
Proliferative
fasciitis
Fibromatoses
Superficial fibromatoses
Palmar and plantar fibromatoses
Knuckle pads
Deep fibromatoses (extra-abdominal
fibromatoses)
Malignant
Adult fibrosarcoma
Postirradiation
fibrosarcoma
FIBROHISTIOCYTIC TUMORS
Benign
Fibrous histiocytoma
Atypical
fibroxanthoma
Intermediate
Dermatofibrosarcoma
protuberans
Malignant (malignant histiocytoma)

Storiform-pleomorphic
Myxoid (myxofibrosarcoma)
Giant cell (malignant giant cell tumor of soft parts)
Inflammatory (malignant xanthogranuloma,
xanthosarcoma)
TUMORS AND TUMOR-LIKE CONDITIONS OF ADIPOSE TISSUE

Benign
Lipoma (cutaneous, deep, and
multiple)
Angiolipoma
Spindle cell and pleomorphic lipoma
Lipoblastoma and lipoblastomatosis
Intramuscular and intermuscular
lipoma
Hibernoma
Malignant
Liposarcoma
Well-differentiated (lipoma-like, sclerosing,
inflammatory)
Myxoid
Round cell (poorly differentiated myxoid)
Pleomorphic
Dedifferentiated
TUMORS OF MUSCLE TISSUE

Smooth Muscle
Benign
Leiomyoma (cutaneous and
deep)
Angiomyoma (vascular
leiomyoma)
Malignant
Leiomyosarcoma
Striated Muscle
Benign
Adult
rhabdomyoma
Malignant
Rhabdomyosarcoma: predominantly embryonal (including botryoid), alveolar,
pleomorphic, and mixed
TUMORS OF LYMPH VESSELS

Benign (lymphangioma)
Cavernous
Cystic (cystic
hygroma)
Malignant
Lymphangiosarcoma
Postmastectomy
lymphangiosarcoma
TUMORS AND TUMOR-LIKE LESIONS OF SYNOVIAL TISSUE

Benign
Giant cell tumor of tendon
sheath
Localized (nodular
tenosynovitis)
Diffuse (florid synovitis)
Malignant
Synovial sarcoma (malignant synovioma), predominantly biphasic or monophasic
(either fibrous or epithelial)
Malignant giant cell tumor of tendon sheath
TUMORS AND TUMOR-LIKE LESIONS OF PERIPHERAL NERVES

Benign
Traumatic neuroma
Morton neuroma
Neurilemoma (benign schwannoma)
Neurofibroma, solitary
Neurofibromatosis (von Recklinghausen
disease)
Localized
Plexiform
Diffuse
Malignant
Malignant schwannoma
Peripheral tumors of primitive neuroectodermal
tissues
TUMORS AND TUMOR-LIKE LESIONS OF CARTILAGE AND BONE-FORMING TISSUES

Benign
Panniculitis ossificans
Myositis ossificans
Fibrodysplasia (myositis) ossificans
progressiva
Extraskeletal chondroma
Extraskeletal osteoma
Malignant
Extraskeletal
chondrosarcoma
Well-differentiated
Myxoid (choroid sarcoma)
Mesenchymal
Extraskeletal osteosarcoma
TUMORS AND TUMOR-LIKE LESIONS OF PLURIPOTENTIAL MESENCHYME

Benign mesenchymoma
Malignant
mesenchymoma
TUMORS AND TUMOR-LIKE CONDITIONS OF BLOOD VESSELS

Benign
Hemangioma
Deep hemangioma (intramuscular, synovial,
perineural)
Glomus tumor
Intermediate
Hemangioendothelioma
Malignant
Hemangiosarcoma
Malignant
hemangiopericytoma
TUMORS AND TUMOR-LIKE CONDITIONS OF DISPUTED OR UNCERTAIN HISTOGENESIS

Benign
Tumoral calcinosis
Myxoma (cutaneous and
intramuscular)
Malignant
Alveolar soft-part sarcoma
Epithelioid sarcoma
Clear cell sarcoma of tendons and
aponeuroses
Extraskeletal Ewing sarcoma
UNCLASSIFIED SOFT TISSUE TUMORS AND TUMOR-LIKE LESIONS
Box 9-2
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11/23/13 /Classification of Soft Tissue Tumors/Soft Tissue Tumors

section 2 Soft Tissue Tumors

▪ Treatment: Radiation therapy is an important adjunct to surgery in the treatment of soft

C. Metastasis: Most soft tissue sarcomas metastasize to the lung.

II. TUMORS OF FIBROUS TISSUE

▪ On palpation, the tumor has a distinctive “rock-hard” character.

or grow rapidly for some time and then stop growing.

▪ Treatment is by wide-margin local excision. Radiation therapy is employed in many

5. Wide-margin surgical resection is the best form of treatment.

III. TUMORS OF FATTY TISSUE

□ Treatment is excision with a marginal margin.

▪ Myxoid liposarcoma (intermediate grade)

5. Liposarcomas metastasize according to the grade of the lesion:

IV. TUMORS OF NEURAL TISSUE

C. Neurofibromatosis (von Recklinghausen disease)

V. TUMORS OF MUSCLE TISSUE

VI. VASCULAR TUMORS

4. Amputation may be necessary to achieve local control.

VII. SYNOVIAL DISORDERS

B. Pigmented villonodular synovitis (PVNS)

9. Local recurrence is common (30% to 50% of cases) despite complete synovectomy.

C. Giant cell tumor of tendon sheath

3. May be present for years or may manifest as a rapidly enlarging mass

VIII. OTHER RARE SARCOMAS

B. Clear cell sarcoma

3. Characterized by compact nests or fascicles of rounded or fusiform cells with clear

C. Alveolar cell sarcoma

2. Occurs in the anterior thigh

IX. POST-TRAUMATIC CONDITIONS

B. Myositis ossificans (heterotopic ossification)

Classification of Soft Tissue Tumors

TUMORS AND TUMOR-LIKE LESIONS OF FIBROUS TISSUE

Malignant (malignant histiocytoma)

TUMORS AND TUMOR-LIKE CONDITIONS OF ADIPOSE TISSUE

TUMORS OF MUSCLE TISSUE

TUMORS OF LYMPH VESSELS

TUMORS AND TUMOR-LIKE LESIONS OF SYNOVIAL TISSUE

TUMORS AND TUMOR-LIKE LESIONS OF PERIPHERAL NERVES

TUMORS AND TUMOR-LIKE LESIONS OF CARTILAGE AND BONE-FORMING TISSUES

TUMORS AND TUMOR-LIKE LESIONS OF PLURIPOTENTIAL MESENCHYME

TUMORS AND TUMOR-LIKE CONDITIONS OF BLOOD VESSELS

TUMORS AND TUMOR-LIKE CONDITIONS OF DISPUTED OR UNCERTAIN HISTOGENESIS

UNCLASSIFIED SOFT TISSUE TUMORS AND TUMOR-LIKE LESIONS

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