Deck 35: Epidemiology of Sarcoma

Question 1

Sarcomas are rare tumors of connective tissue, primarily arising in soft tissue or bone.

Answer 1

True

Question 2

About 30% of bone sarcomas are diagnosed prior to age 20, after which the incidence steadily declines with age.

Answer 2

True

Question 3

The death curve for bone sarcomas shows an early peak during adolescence and a late peak at 75-85 years of age.

Answer 3

True

Question 4

The incidence of soft tissue sarcomas is relatively constant over the life span.

Question 5

The death curve for soft tissue sarcoma increases gradually over the life span and shows a single peak late in life.

Question 6

Reciprocal chromosomal translocations constitute the majority of specific genetic alterations associated with pediatric sarcomas.

Question 7

It is likely that the chimeric fusion proteins resulting from reciprocal translocations act as transcription activators that deregulate key genes associated with pediatric sarcoma development.

Question 8

Chimeric fusion proteins resulting from reciprocal translocations provide potential targets for the therapy of pediatric sarcomas with monoclonal antibodies.

Question 9

Adult sarcomas are often characterized by reciprocal translocations and chimeric fusion proteins.

Question 10

Lipomas are benign tumors that develop from adipose tissue.

Question 11

Uterine fibroids are benign tumors that develop from endometrial tissue.

Question 12

Lipomas and uterine fibroids often undergo malignant transformation.

Question 13

Nearly all Gastrointestinal Stromal Tumors (GIST) have been found to carry mutant forms of an important tyrosine kinase receptor gene called c-KIT.

Question 14

There is consistent evidence that radiation exposure increases the risk of developing both bone and soft tissue sarcomas.

Question 15

The primary cause of Kaposi's sarcoma is HIV infection.

Question 16

Kaposi's sarcoma arises from:

A) soft connective tissue.
B) macrophages.
C) endothelial cells of blood vessels.
D) pigmented nevi.

Question 17

The most common malignancy in sub-Saharan Africa is:

A) Burkitt's lymphoma.
B) leukemia.
C) Kaposi's sarcoma.
D) lung cancer.

Question 18

The defining genetic abnormality of gastrointestinal stromal tumors (GIST) is the presence of a mutant form of which of the following genes?

A) PAX
B) c-KIT
C) SYT
D) EWSR-1

Question 19

What is the approximate annual incidence rate of sarcoma in individuals under the age of 20 years in the US population?

A) 1 per million
B) 10 per million
C) 50 per million
D) 10 per 100,000

Question 20

Sarcomas account for about what percentage of malignant neoplasms in children and adolescents?

A) 1%
B) 13%
C) 25%
D) 50%

Question 21

Sarcomas account for about what percentage of malignant neoplasms in adults?

A) 0-1%
B) 1-5%
C) 6-10%
D) >10%

Question 22

The signature reciprocal chromosomal translocation responsible for synovial sarcoma involves which genes?

A) SYT on chromosome 18 and SSX on the X chromosome
B) FKHR on chromosome 13 and PAX on chromosomes 1 and 2
C) ETV6 on chromosome 12 and NTRK3 on chromosome 13
D) EWSR-1 on chromosome 22 and FLI-1 on chromosome 11

Question 23

The virus that causes Kaposi's sarcoma is:

A) HIV-1.
B) HIV-2.
C) HHV-2.
D) HHV-8.

Question 24

How do reciprocal chromosomal translocations cause the development of many forms of pediatric sarcoma?

A) They create new genes that code chimeric fusion proteins with oncogenic potential.
B) They increase the formation of reactive oxygen species.
C) They create new tumor suppressor genes.
D) They increase epigenetic effects.

Question 25

Chronic exposure to polyvinyl chloride increases the risk of developing which of the following types of sarcoma?

A) Fibrosarcoma
B) Liposarcoma
C) Leiomyosarcoma
D) Angiosarcoma

Question 26

Define the various subtypes of sarcoma.

Question 27

Characterize the epidemiology of pediatric sarcoma in the USA.

Question 28

Briefly characterize the epidemiology of adult sarcoma in the USA.

Question 29

Discuss the major exogenous risk factors for sarcoma.

Question 30

Discuss the pathogenesis of pediatric sarcoma.

Question 31

Describe the signature reciprocal chromosomal translocation that causes synovial sarcoma.

Question 32

Describe reciprocal chromosomal translocations that cause Ewing's sarcoma.

Question 33

Discuss the genetic mutation that is responsible for GIST.

Question 34

Describe key findings of epidemiologic studies of PVC exposure and angiosarcoma.

Question 35

Discuss the global epidemiology of Kaposi's sarcoma.