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Sarcoma Andrew E Rosenberg, MD Sarcoma Andrew E Rosenberg, MD

Sarcoma Andrew E Rosenberg, MD - PowerPoint Presentation

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Sarcoma Andrew E Rosenberg, MD - PPT Presentation

Department of Pathology University of Miami Miami Florida Arosenbergmiamiedu Address basic questions about its biology Provide foundation of information greater understanding Cure Sylvester Comprehensive Cancer Center ID: 917999

sarcoma grade sarcomas tissue grade sarcoma tissue sarcomas high fusion normal genetics risk tumor osteosarcoma bone aberrant disease needle

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Slide1

Sarcoma

Andrew E Rosenberg, MD

Department of Pathology

University of Miami

Miami, Florida

Arosenberg@miami.edu

Address basic questions about its biologyProvide foundation of information – greater understanding

Cure

Sylvester Comprehensive Cancer Center

PATHOLOGY

Slide2

Derivation: New Latin, from Greek

sarkōma

,

sarkōmat

-, fleshy excrescence, from sarkoun, to produce flesh, from

sarx, sark-, flesh. Definition: Malignant tumor of connective tissue/muscleSar·co·ma (sär-kō'mə )

Slide3

Sarcoma Epidemiology

Approximately 15,000 new cases in U.S.

1 per 20,000 individuals

(625,000 breast, lung, and prostate – 1 per 480 individuals)

Account for 1% of cancers in adults and 15-20% of cancers in children

Approximately 6,200 sarcoma patients die each year – 17/day

Slide4

50%

9%

30%

20%

10%

Soft Tissue

Sarcomas

Thigh

Abdomen

Arm

Bone Sarcomas

Knee

Pelvis

Spine

Arm

10%

13%

Sarcoma Location

Slide5

Sarcoma Age Distribution

Develop in all age groups

Soft Tissue

Bone

Age (decades)

%

Slide6

Sarcoma Symptoms

Presence of a mass

Pain, swelling, progressive enlargement

Restriction of motion, bleeding

Slide7

Causes of Sarcoma

? Trauma

Underlying diseases - Paget

s disease,

bone infarction, radiation, syndromes - von Recklinghausens disease, Maffucci’s syndrome, Li Fraumani syndrome, bilateral retinoblastoma, Werner syndrome

Toxic exposuresAlterations of genetic material

Slide8

Mesenchymal Stem Cells

Expression of genes that determine phenotype (recapitulate normal tissue) and alter biologic traits

Mutations leading to

sequential and well-

orchestrated genetic changes

Slide9

Classification of Sarcoma

Phenotype - normal tissue counterpart

fibrous tissue, fat, skeletal muscle, endothelium, smooth muscle, skeletal muscle, cartilage, bone, notochord, peripheral nerve, …..

Fibrosarcoma, liposarcoma, angiosarcoma, malignant peripheral nerve sheath tumor, rhabdomyosarcoma, leiomyosarcoma, etc.

70 types and subtypes90% sarcoma have normal counterpart, 8% no counterpart, but consistent findings, 2% unique.

Slide10

Sarcomas Genetics

Slide11

Genetics of Soft Tissue Sarcoma

approx. 1/3 of all sarcomas

15 different sarcoma types

with over 25 different

translocations

• fusion genes: aberrant chimeric transcription factors (most) or aberrant kinases (some)

approx. 2/3 of all sarcomas• biology: genetic gains &

losses, chromosomal instability,

telomere dysfunction

KEY CONCEPTS

• sarcomas contain their fusion gene from earliest presentation• sarcomas with fusion genes do not show benign or premalignant phase

• fusion gene in all tumor cells

• fusion gene expressed throughout clinical course

• biology: transcriptional deregulation or aberrant signaling

Slide12

Sarcoma Genetics

Competitive advantage to cancer cells

Induces phenotype

Stimulates cell proliferation

Evade cell growth inhibitors

Avoids apoptosis Fuels angiogenesis Facilitates invasion and metastasis

Slide13

Sarcoma Genetics

Slide14

Sarcoma Diagnosis

Review clinical information

Review imaging studies

Obtain tissue

Classify and grade sarcoma

Slide15

Types of Specimens

Plan tissue retrieval

Fine needle aspiration

Needle biopsy

Open biopsy

En block resectionFrozen section laboratory

Slide16

Sarcoma Diagnosis

Needle Biopsy

Identify sarcoma type and aggressiveness

Slide17

Desmoid Tumor High grade Spindle Cell Sarcoma

Slide18

Sarcoma Grade

Grade 1 Grade 2 Grade 3

Well differentiated Fibrosarcoma Osteosarcoma

Liposarcoma

Low risk Intermediate risk High risk

Risk of developing metastases

Slide19

Grading Sarcomas

Pathologic Grade- is single most important prognostic factor

Grade 1 (low grade, 2 (intermediate grade), and 3 (high grade)

mitotic activity, % necrosis, and degree of differentiation

Clinical Grade Low grade (pathologic grade 1)

High grade (pathologic grade 2 and 3)

Normal Low grade High grade

Slide20

Grading Sarcomas

Low Grade

High Grade

Grade 1

Grade 3

Grade 2

Well Differentiated Osteosarcoma Grade 2 Osteosarcoma Grade 3 Osteosarcoma

Slide21

Arch Pathol Lab Med

2006;130:1448.

FNCLCC Grading and Outcome

Slide22

Sarcoma Resection

Slide23

Slide24

Slide25

Slide26

Slide27

What does the patient need to know about the disease?

Histologic diagnosis (what is it?)

Prognosis (how bad is it?). size, grade, Adverse features

for localized: is it all gone? (margins)

What is the best treatment?

Slide28

Picasso at work

Slide29