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MSS > Soft Tissue Tumors > Flashcards

Soft Tissue Tumors Flashcards

1
Q

What are soft tissue tumors?

A

Nonepithelial extra skeletal tumors
Exclude: reticuloendothelial system, glia and supporting tissue of parenchymal organs

Mesenchymal tumors are classified according to the tissue they recapitulate (muscle, fat, fibrous tissue, vessels and nerves)

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2
Q

What is the cause of most soft tissue tumors?

A

Unknown – majority occur sporadically.

Association following radiation therapy.
Rare instances following chemical burns, thermal burns or trauma.

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3
Q

Are any soft tissue tumors genetically associated?

A

Yes.

Neurofibromatosis type 1 – neurofibroma, malignant schwannoma
Gardner syndrome – fibromatosis
Li-Fraumeni syndrome – soft tissue sarcomas
Osler-Weber-Rendu syndrome – telangiectasia

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4
Q

Where do the majority of sarcomas occur?

A

About 40% occur in the lower extremities, especially the thigh.

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5
Q

What types of of sarcomas tend to appear in childhood? Young adulthood? Later adulthood?

A

Childhood: Rhabdomyosarcoma
Young adulthood: Synovial Sarcoma
Later adultlife: Liposarcoma and pleomorphic or undifferentiated sarcomas

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6
Q

What are a couple of important diagnostic features for soft tissue tumors?

A

Cell morphology

Architectural arrangement

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7
Q

What is the grading for soft tissue sarcoma based on?

A

Degree of differentiation/pleomorphism
Average number of mitoses per high-power field
Extent of necrosis (presumably a reflection of rate of growth)

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8
Q

What other prognostic factors are important to consider?

A

Size (> or < 5cm)
Depth (above or under fascia)
Stage (I-IV)

In general, tumors arising in superficial locations have better prognosis than deep lesions.

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9
Q

What is the treatment for soft tissue sarcomas?

A

Usually treated with wide surgical excision (frequently limb-sparing)

Irradiation and systemic therapy reserved for large high-grade tumors.

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10
Q

Adipose tissue

A

Lipoma
Hibernoma
Liposarcoma

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11
Q

Nerve

A

Neurofibroma
Schwannoma
Perineuriroma
Malignant peripheral nerve sheath tumor (MPNST)

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12
Q

Muscle

A

Smooth Muscle: leiomyoma, leiomyosarcoma

Skeletal Muscle: Rhabdomyoma, Rhabdomyosarcoma

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13
Q

Fibrous tissue

A

Fibroma
Fibromatosis
Fibrosarcoma

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14
Q

Blood vessels

A

Hemangioma
Hemangioendothelioma
Angiosarcoma

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15
Q

Miscellaneous

A

PVNS, myositis ossificans, angiofibroma

Synovial sarcoma, alveolar soft part sarcoma, epithelioid sarcoma

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16
Q

What is the most common soft tissue tumor of adulthood?

A

Lipoma

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17
Q

What are some characteristics of conventional lipomas?

A

The most common subtype

Soft, yellow, well-encapsulated masses

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18
Q

What is the histology of conventional lipomas??

A

Consist of mature white fat cells with no pleomorphism

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19
Q

What is the genetic alteration in conventional lipomas?

A

12q14-q15

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20
Q

What is one of the most common sarcomas of adulthood?

A

Liposarcoma

21
Q

Where are liposarcomas generally located?

A

Deep soft tissues of proximal extremities and retroperitoneum, may develop into large tumors

22
Q

How can liposarcomas histologically present and what is there behavior?

A

Well-differentiated is relatively indolent
Myxoid/round cell type is intermediate
Pleomorphic variant usually aggressive and may metastasize

23
Q

How does a lipoblast differ from mature adipocyte?

A

Smaller size, has vacuolated bubbles, nucleus seems to be closer to center of cell and pushed/indented by other cells around it – indicates liposarcoma

24
Q

What characterizes WD-LPS and myxoid/round LPS genetically?

A

WD-LPS: supernumerary ring chromosomes, amplification of 12q14-q15, containing MDM2

Myxoid/round LPS: t(12;16)(q13;p11)

25
Q

What are pseudosarcomatous proliferations?

A

Reactive non-neoplastic lesions that develop in response to some form of local trauma or are idiopathic
Develop suddenly and grow rapidly
Hypercellularity, mitotic activity, and primitive appearance mimic sarcoma

26
Q

What are two types of pseudosarcomatous proliferations?

A

Nodular fasciitis: deep dermis, subcutis, or muscle; several centimeters with poorly defined margins

Myositis ossificans: proximal extremities, young adults, trauma in >50% of cases; presence of metaplastic bone– eventually the lesion ossifies and the intertrabecular spaces become filled with bone marrow. Must be distinguished from extraskeletal osteosarcoma.

27
Q

What are fibromatoses?

A

Benign soft tumors with similar characteristics

28
Q

Examples of superficial fibromatosis

A

Palmar (Dupuytren contracture)
Plantar
Penile (Peyronie disease)

May stabilize and resolve spontaneously. Some recur.

29
Q

Some characteristics of deep-seated fibromatosis (desmoid tumors)

A

Behavior lies between benign fibrous tumors and low-grade fibrosarcomas
Frequently recur after incomplete excision
Most frequent in the teens to 30s
Some associated with Gardner syndrome
Mutations in APC or B-catenin genes

30
Q

What are fibrosarcomas?

Where do they usually occur?

A

Malignant tumors composed of fibroblasts.

Occur mostly in adults. Usually in deep tissues of the thigh, knee, and retroperitoneum.

Agressive tumors, recur in >50% of cases, metastasize in >25%.

31
Q

What is the most common neoplasm in women? What kind of tumor is it?

A

Uterine leiomyomas – smooth muscle tumor

32
Q

What are some characteristics of smooth muscle tumors?

A

Can arise in skin and deep soft tissues
Usually <1-2 cm
Solitary lesions easily cured
Multiple tumors may be difficult to remove

33
Q

What are some characteristics of leiomyosarcoma?

A

10-20% of soft tissue sarcomas
Occur in adults, F > M
Located in skin and deep soft tissues of the extremities and retroperitoneum
Superficial leiomyosarcomas are usually small and have good prognosis
Those in retroperitoneum are large, cannot be entirely excised – local extension and metastatic spread.

34
Q

What are some characteristics of rhabdomyosarcoma?

A

Most common soft tissue sarcoma of childhood and adolescence, usually before age 20.

Most commonly in head and neck or genitourinary tract, usually at sites where there is little normal skeletal muscle.

35
Q

What are the three subtypes of rhabdomyosarcoma?

A

Embryonal
Alveolar
Pleomorphic

36
Q

Most common type of RMS

A

Embryonal – 49% of RMS

37
Q

What population does it normally affect and where?

A

More frequent in <10 years of age

In head and neck, particularly in orbit and parameninges; genitourinary tract; deep soft tissues of the extremities, pelvis and retroperitoneum

38
Q

What are some variants of Embryonal RMS?

A

Sarcoma botryoides (“grape-like”)
Spindle cell
Anaplastic

39
Q

How common is Alveolar RMS?

A

31% of RMS

40
Q

In what population is Alveolar RMS commonly seen?

A

More frequent between 10 and 25 years of age

41
Q

Where does Alveolar RMS commonly occur?

A

In deep soft tissues of the extremities

Less frequently in head and neck, perineum, pelvis, retroperitoneum.

42
Q

What genetically confirms a diagnosis of Alveolar RMS?

A

t(2;13)/PAX3-FKHR
OR
t(1;13)/PAX7-FKHR

present in most (80-85%) of cases of alveolar FMS

43
Q

What are some tumors of unknown histogenesis?

A

Synovial sarcoma
Epithelioid sarcoma
Alveolar soft part sarcoma

44
Q

How common is synovial sarcoma?

A

5-10% of all soft tissue sarcomas

Young adults, more commonly in males

45
Q

Where do synovial sarcomas commonly occur?

A

Over 80% in deep soft tissue of extremities, especially around the knee

46
Q

What is genetically characteristic of synovial sarcoma? Histologically??

A

t(X;18)(p11;q11)

Histologically biphasic – two types of cells or monophasic – one type of cell

47
Q

How are synovial sarcomas treated?

A

Aggressively with limb-sparing surgery and chemotherapy.

5 year survival rates range from 25% to 62%; only 10-30% of patients live longer than 10 years.

48
Q

What are some common metastatic sites of synovial sarcoma?

A

Lung, bone and regional lymph nodes

MSS (24 decks)

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