Which solid tumor is slightly higher incidence in females?
wilms
What does WAGR syndrome stand for and what is it associated with?
Wilms Tumor. Syndrome assoc with genetic deletions of 11p13. W - wilms A- aniridia G- GU abnormalities like cryptorchidism R - mental retardation
What is the usual first symptoms of Wilms tumor?
firm mass in abdomen
Why do you not palpate Wilms tumors?
they can easily rupture.
What is the most common met site for Wilms?
Lung
Wilms treatment?
surgery and chemo, sometimes radiation
What are the overall cure rates for Wilms?
85-92%. much lower after recurrence
Where does rhabdomyosarcoma arise?
striated muscle tissue, can be virtually anywhere
Treatment for rhabdo?
surgery (at least biopsy, sometimes resection), radiation sometimes and chemo
Overall surivorship for rhabdo?
70%
What does ‘TNM’ mean in regard to rhabo?
Staging: tumor, node, metastasis.
3 most common sites for ostesarcoma?
distal femur, proximal tibia, proximal humerus
What is a usual treatment course for osteosarcoma?
Biopsy only for diagnosis, then indution chemo to shrink tumor before surgery. After surgery, consolidation chemo. Usually resistant to XRT.
What are two new therapies that are being studied for OS?
monoclonal antibodies and inhaled GMCSF with pulmonary met patients because it is an immunomodulator.
What kind of surgery is standard of care for OS?
limb salvage - using a prosthesis or bone graft to replace the joint and allow some movement.
Where does Ewings originate?
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
Treatment for Ewings?
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.
Which tumor has a possible association with HPV?
retinoblastoma
What is the penetrance of retinoblastoma?
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.
When might an opthal tru to save the eye in retinoblastoma?
If low stage the eye may be saved. If bilateral disease, they may try to save the eye with the least amount of tumor.
When to use chemo for retinoblastoma?
bilateral disease, or if unilateral, only if there are tumor cells beyond a certain point in the eye/optic nerve on path review.
What are 4 genetic predispositions to hepatoblastoma?
Hemihypertrophy, Beckwith Wiedemann, fetal alcohol syndrome, and familial adenomatous polyposis (inherited polyp disorder)
What is the classic marker for hepatoblastoma?
elevated AFP.
Classic treatment for hepatoblastoma?
4 rounds chemo (5FU, VCR and cisplat) followed by resection, aimed at GTR. does not respond to XRT
Hepatoblastoma prognosis with and without GTR?
GTR - greater than 75%
unresectable - 20%
Where do germ cell tumors originate?
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
What percentage of germ cell tumors are benign?
50%
A teen could have a positive pregnancy test but not be pregnant due to what tumor?
germ cell
non-malignant, rashes in 50% of cases, treated as a malignanacy, could be:
LCH
Which solid tumor may be misdiagnosed as cradle cap or diaper rash?
LCH
Why would you get a skeletal survery for a new LCH?
bony lesions are very common. the may change spontaneously or jsut be watched - otherwise they may need chemo/steroids
17 yo has swelling and a limp with increasing pain. possible onc diagnosis?
bone cyst, OS, Ewings, or another rare sarcoma. Trauma may injure the tumor, but does not cause it.
What lifelong healthcare precaution is needed after limb salvage therapy?
endocarditis prophy, notify dentist and take abx prior to dental work
Does hepatoblastoma respond to xrt?
No
