8 Solid Tumors

Question 1

Which solid tumor is slightly higher incidence in females?

Answer 1

wilms

Question 2

What does WAGR syndrome stand for and what is it associated with?

Answer 2

Wilms Tumor. Syndrome assoc with genetic deletions of 11p13. 
W - wilms
A- aniridia
G- GU abnormalities like cryptorchidism
R - mental retardation

Question 3

What is the usual first symptoms of Wilms tumor?

Answer 3

firm mass in abdomen

Question 4

Why do you not palpate Wilms tumors?

Answer 4

they can easily rupture.

Question 5

What is the most common met site for Wilms?

Answer 5

Lung

Question 6

Wilms treatment?

Answer 6

surgery and chemo, sometimes radiation

Question 7

What are the overall cure rates for Wilms?

Answer 7

85-92%. much lower after recurrence

Question 8

Where does rhabdomyosarcoma arise?

Answer 8

striated muscle tissue, can be virtually anywhere

Question 9

Treatment for rhabdo?

Answer 9

surgery (at least biopsy, sometimes resection), radiation sometimes and chemo

Question 10

Overall surivorship for rhabdo?

Answer 10

70%

Question 11

What does ‘TNM’ mean in regard to rhabo?

Answer 11

Staging: tumor, node, metastasis.

Question 12

3 most common sites for ostesarcoma?

Answer 12

distal femur, proximal tibia, proximal humerus

Question 13

What is a usual treatment course for osteosarcoma?

Answer 13

Biopsy only for diagnosis, then indution chemo to shrink tumor before surgery. After surgery, consolidation chemo. Usually resistant to XRT.

Question 14

What are two new therapies that are being studied for OS?

Answer 14

monoclonal antibodies and inhaled GMCSF with pulmonary met patients because it is an immunomodulator.

Question 15

What kind of surgery is standard of care for OS?

Answer 15

limb salvage - using a prosthesis or bone graft to replace the joint and allow some movement.

Question 16

Where does Ewings originate?

Answer 16

A primary bone tumor wit ha soft tissue component; or an extraosseous outside the bone that may or may not have bony involvement. Diagnosed by exclusion

Question 17

Treatment for Ewings?

Answer 17

Surgery and XRT for local control, chemo for systemic. No chance without both parts. If surgery has very clear margins, sometimes they can avoid XRT.

Question 18

Which tumor has a possible association with HPV?

Answer 18

retinoblastoma

Question 19

What is the penetrance of retinoblastoma?

Answer 19

Autosomal dominant in the hereditary form. So 45% chance of it occurring in the offspring of survivors of bilateral disease.
There is also a nonhereditary form.

Question 20

When might an opthal tru to save the eye in retinoblastoma?

Answer 20

If low stage the eye may be saved. If bilateral disease, they may try to save the eye with the least amount of tumor.

Question 21

When to use chemo for retinoblastoma?

Answer 21

bilateral disease, or if unilateral, only if there are tumor cells beyond a certain point in the eye/optic nerve on path review.

Question 22

What are 4 genetic predispositions to hepatoblastoma?

Answer 22

Hemihypertrophy, Beckwith Wiedemann, fetal alcohol syndrome, and familial adenomatous polyposis (inherited polyp disorder)

Question 23

What is the classic marker for hepatoblastoma?

Answer 23

elevated AFP.

Question 24

Classic treatment for hepatoblastoma?

Answer 24

4 rounds chemo (5FU, VCR and cisplat) followed by resection, aimed at GTR. does not respond to XRT

Question 25

Hepatoblastoma prognosis with and without GTR?

Answer 25

GTR - greater than 75%

unresectable - 20%

Question 26

Where do germ cell tumors originate?

Answer 26

Germ cells are first released in the human embryo yolk sac at 4 to 6 weeks gestation and then travel to the ovaries or testes.
Germ cell tumors (GCTs) arise from the primitive germ cells or embryonal cells and can occur in the gonads or sites along the midline migration path (the path from the yolk sac to the testes or ovaries in gestation). Germ cells develop in the embryo yolk sac. As a result, most GCTs typically arise in the gonads or in tissues along the midline migration path and include intracranial, mediastinal, retroperitoneal, or sacrococcygeal sites.
Associated with abnormal sex chromosomes, CNS and GU abnormalities, and malformations of the lower spine

Question 27

What percentage of germ cell tumors are benign?

Answer 27

50%

Question 28

A teen could have a positive pregnancy test but not be pregnant due to what tumor?

Answer 28

germ cell

Question 29

non-malignant, rashes in 50% of cases, treated as a malignanacy, could be:

Answer 29

LCH

Question 30

Which solid tumor may be misdiagnosed as cradle cap or diaper rash?

Answer 30

LCH

Question 31

Why would you get a skeletal survery for a new LCH?

Answer 31

bony lesions are very common. the may change spontaneously or jsut be watched - otherwise they may need chemo/steroids

Question 32

17 yo has swelling and a limp with increasing pain. possible onc diagnosis?

Answer 32

bone cyst, OS, Ewings, or another rare sarcoma. Trauma may injure the tumor, but does not cause it.

Question 33

What lifelong healthcare precaution is needed after limb salvage therapy?

Answer 33

endocarditis prophy, notify dentist and take abx prior to dental work

Question 34

Does hepatoblastoma respond to xrt?

Answer 34

No