Soft Tissue Neoplasms Flashcards

1
Q
A
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2
Q
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Osteoblastoma > 2 cm
Posterior spine
Pain unresponsive to NSAIDs
No thick rim of reactive bone
Tx: en bloc excision or curettage
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3
Q

characteristics of both osteoma and osteoblastoma

A

Round to oval
Hemorrhagic, gritty
Well circumscribed
Randomly interconnecting trabeculae of woven bone
Rimmed by osteoblasts
Loose connective tissue with congested capillaries

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4
Q
A
osteochondroma 
Most common benign bone tumor
Adolescence & early adulthood
M:F; 3:1
Metaphysis of long tubular bones (knee)
Slow growing
Benign, cartilage capped tumor with bony stalk
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5
Q
A
osteochondroma 
Sessile or pedunculated
Cap – hyaline cartilage
Endochondral ossification
New bone forms inner portion of head & stalk
Tx: simple excision
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6
Q
A
Endochondroma
Benign tumor of hyaline cartilage
Most common intraosseous cartilage tumor
20s-50s
Solitary metaphyseal lesion
Hands & feet
Circumscribed, central calcifications, sclerotic rim, intact cortex
Detected incidentally
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7
Q
A
Endochondroma
Benign hyaline cartilage tumors within medullary cavities
< 3 cm
Grey-blue
Translucent
Nodules of hyaline cartilage
Benign chondrocytes
Tx: observation or curettage
Non-hereditary disorders characterized by multiple enchondromas (Maffucci Syndrome & Ollier disease)
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8
Q

Osteosarcoma epidemiology and definition

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Most common primary malignant bone tumor
20% of primary bone cancers
75% occur in children younger than 20
Second peak in older adults with underlying conditions (Paget disease, bone infarcts, prior radiation)
M>F
Metaphysis of long bones of extremities
Approx. 50% around knee (i.e. distal femur or proximal tibia)

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9
Q

Treatment and prognosis for osteosarcoma

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Tx: chemo & surgery
Neoadjuvant chemo (assumed all patients have occult metastases)
Followed by surgery
Five year survival: 60%-70% for those without overt metastases
< 20% for those with metastases, recurrent disease, or secondary osteosarcoma

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10
Q
A

osteosarcoma
Painful, progressively enlarging mass
Large destructive lytic & blastic lesion
Infiltrative margins
Codman triangle – tumor breaks through cortex & lifts periosteum
75% have acquired genetic abnormalities
Mutations in tumor suppressor genes & oncogenes
RB, TP53, INK4a, MDM2, CDK4a
Subtypes related to
Site of origin: intramedullary, intracortical, surface
Histologic grade: low vs. high
Primary vs. secondary to pre-existing disorders
Histologic features: osteoblastic, chondroblastic, fibroblastic, telangiectatic, small cell, and giant cell
Most common subtypes: metaphysis of long bones, primary, intramedullary, osteoblastic, high grade

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11
Q
A
osteosarcoma
Bulky, gritty
Grey-white
Hemorrhage & cystic degeneration
Destroy surrounding bone
Replace bone marrow
May penetrate epiphysis or enter joint
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12
Q
A
Ostseosarcoma
Production of osteoid/bony matrix
Large hyperchromatic nuclei
Bizarre giant cells
Abundant mitoses
Vascular invasion
Extensive necrosis
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13
Q

chondrosarcoma epidemiology and definition

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Adults – 40s and older; M:F=2:1
Axial skeleton
Low grade & slow growing or high grade & aggressive
Conventional type – produce hyaline cartilage
Central (intramedullary)
Most common (approx. 90%)
Peripheral (juxtacortical)
Clear cell, dedifferentiated, mesenchymal variants
Painful, progressively enlarging masses
Direct correlation between grade & biologic behavior
Majority are grade 1
5 year survival 80%-90%
Grade 3: 5 year survival of 43%
70% of grade 3 tumors metastasize, especially to the lungs
Tx: wide surgical excision
Chemo for dedifferentiated & mesenchymal types

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14
Q
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chondrosarcoma 
Large, bulky
Glistening, grey-white, translucent
Gelatinous/myxoid matrix
Spotty calcifications
Central necrosis
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15
Q

??

A

Chondrosarcoma

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16
Q

?

A

Chondrosarcoma
Dedifferentiated variant
Low grade chondrosarcoma with high grade component that does not produce cartilage

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17
Q
A

chondrosarcoma variant

Clear cell variant
Abundant clear cytoplasm
Osteoclast type giant cells
Reactive bone formation

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18
Q
A

Chondrosarcoma

Mesenchymal variant
Islands of cartilage
Sheets of small round cells
Mimicks Ewing sarcoma

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19
Q

Lipoma

A
Benign
Soft, mobile, painless
Mature adipocytes
Proximal extremities & trunk
Tx: simple excision
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20
Q

?

A

Liposarcoma

50s-60s
Extremities & retroperitoneum
Lipoblast
Cytoplasmic vacuoles with fat

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21
Q
A

liposarcoma

Well differentiated variant
Atypical lipomatous tumor in extremities
Resemble lipomas grossly
Scattered lipoblasts
Atypical spindled cells
22
Q
A
Liposarcoma 
Dedifferentiated liposarcoma
Retroperitoneum
Heterologous elements 
MDM2 amplification
23
Q
A

Liposarcoma

Myxoid liposarcoma
Lower extremities
Prominent capillaries
Mucoid matrix
t(12;16)
FUS-DDIT3
24
Q
A
Pleomorphic liposarcoma
Highly cellular
Poorly differentiated
Tumor giant cells
Abundant mitoses
Necrosis
25
Q

Variants of liposarcoma

A

Recurrence common
Complete excision!!
Well differentiated variant – indolent behavior
Myxoid variant – intermediate behavior
Pleomorphic variant – aggressive behavior

26
Q

Nodular fasciitis

A

Self-limited
Fibroblastic & myofibroblastic proliferation
Young adults
Rapidly growing, history of trauma common
t(17;22) MYH9-USP6
Spontaneous regression

27
Q
A

Nodular Fasciitis

< 5 cm
Circumscribed or slightly infiltrative
Cellular
“Tissue culture fibroblasts”
Maturation gradient:  loose and cellular to organized and fibrous
Abundant mitoses
28
Q
A

Superficial fibrzomatosis

Infiltrative fibroblastic proliferation
Causes deformity
Clinically innocuous
M>F
Nodular proliferation of fibroblasts & dense collagen
29
Q
A
Superficial firbromatosis
Palmar – Dupuytren contracture
Puckering & dimpling
Flexion contracture
Plantar
Younger patients
No contractures
30
Q
A

Superficial fibrzomatosis

Penile – Peyronie disease
Dorsolateral penis
Abnormal curvature of shaft &/or
Urethral constriction

31
Q

Deep fibrzomatosis

A
Large & infiltrative
Recurrence common
Young patients; F>M
APC or β-catenin mutations
↑ Wnt signaling
FAP patients are predisposed
32
Q
A

Deep fibromatosis

Grey-white, firm
Poorly demarcated
Bland fibroblasts & dense collagen
Broad fascicles 
Anterior abdominal wall
Complete excision difficult
33
Q

Rhabadomyosarcoma

A

4.5% of all childhood cancers
Bimodal ages distribution
2-6 years old; 10-18 years old
3 major subtypes: embryonal*, alveolar, pleomorphic
Head & neck, GU tract & retroperitoneum, extremities
Glistening, gelatinous, grey-white or firm, rubbery, grey-white to pink-tan
Hemorrhage & necrosis

34
Q
A

Rhabdomyosarcoma: Embryonal

60%; children < 10 yo
Head & neck most common
Alternating hypercellular & loose myxoid areas
Small hyperchromatic round to spindled cells
Rhabdomyoblasts with cross striations

35
Q
A

Rhabdomyosarcoma: Ebmryonal

Botryoid variant
Polypoid growth
Mucosa-lined, hollow organs
Cambium layer
Subepithelial condensation of tumor cells
Loose stromal connective tissue
36
Q
A

Rhambomyosarcroma: Alveolar

30%; 10-25 yo
Extremities 
Irregular alveolar spaces
Central loss of cohesion
Fibrous septae & dilated vascular channels
t(1;13)  PAX3-FOXO1A
t(1;13)  PAX7-FOXO1A
37
Q
A

Rhabdomyosarcoma: Pleomorphic

Rare; 6th decade; M>F
Extremities
Haphazardly oriented pleomorphic cells
Hyperchromatic nuclei
Eosinophilic cytoplasm
Bizarre tumor giant cells
38
Q

Rhabdomyosarcoma tx and prognosis

A
Aggressive
Tx: surgery, chemo, +/- radiation
Botryoid embryonal – best prognosis
Embryonal – intermediate prognosis
Alveolar – poor prognosis
Pleomorphic – often fatal
39
Q

Leiomyoma

A

Benign smooth muscle tumor
Common in the uterus
Most common neoplasm in women
Erector pili muscles of skin, nipples, scrotum, labia
May be multiple & painful
Deep soft tissues & muscular layers of GI tract
HLRCC – uterine & cutaneous leiomyomata with RCC
Autosomal dominant mutation in fumarate hydratase gene

40
Q
A

Leiomyoma

1-2 cm
Fascicles of spindle cells
Blunt ended elongated nuclei
Minimal atypia
Tx: resection
41
Q
A

Leiomyosarcoma

10%-20% of sarcomas
Older adults; F>M
Extremities & retroperitoneum
Great vessels – IVC (more lethal)
Painless firm masses
Large & bulky in retroperitoneum
Necrosis, hemorrhage, cystic degeneration
42
Q
A

Leiomyosarcoma

Spindle cells
Hyperchromatic nuclei
Interweaving fascicles
Variable nuclear atypia
High mitotic activity  malignant
43
Q

Leiomyosarcoma tx

A

Treatment depends on size, location, & grade
Superficial/cutaneous  good prognosis
Retroperitoneum  poor prognosis
Difficult to remove
Causes death by both local extension and metastatic spread (lungs)

44
Q

Ewing Sarcoma/Primitive Neuroectodermal Tumor (PNET)

A

Bone & soft tissue tumor without obvious differentiation
6%-10% of primary bone malignancies
2nd most common bone sarcoma in children
80% < 20 yo
Present as painful, enlarging mass
Diaphysis of long tubular bones (i.e. femur)

45
Q

Ewing Sarcoma/Primitive Neuroectodermal Tumor (PNET) tx and mechanism

A
t(11;22) EWS-FLI1
Hemorrhage & necrosis
Uniform small round cells
Hyperchromatic
Scant cytoplasm
Tx: neoadjuvant chemo
Surgery +/- radiation
46
Q

Synovial Sarcoma epidemiology and gene

A

10% of all sarcomas
20s-40s
Deep seated mass present for several years
t(X;18)  SS18-SSX1, -SSX2, or –SSX4

47
Q

Types and tx for synovial sarcoma

A

Monophasic – uniform spindle cells in fascicles
Biphasic – spindle cells & gland-like structures
Staghorn vessels
Tx: surgical
+/- chemo & radiation

48
Q

Biphasic or monophonic synovial sarcoma

A
49
Q

Undifferentiated Pleomorphic Sarcoma (UPS)

A
High grade pleomorphic tumors
Diagnosis of exclusion
Largest category of adult sarcomas
Deep soft tissues of extremities (thigh)
Middle aged to older adults
50
Q
A

Undifferentiated Pleomorphic sarcoma

Grey-white & fleshy
Grow up to 20 cm
Anaplastic spindle to polygonal cells
Hyperchromatic bizarre nuclei
Abundant mitoses & necrosis
Tx: surgery +/- chemo & radiation
Poor prognosis