Pediatric Solid Tumors

Front 1

What clinincal syndromes are associated with the development of Wilms' Tumors?

Back 1

Clinical syndromes associated with Wilm's tumors:

1. WAGR syndrome
2. Denys-Drash syndrome
3. Beckwith-Wiedemann syndrome

Front 2

What are the characteristics associated WAGR syndrome?

Back 2

Characteristics of WAGR syndrome:

1. Wilm's tumor
2. aniridia
3. genitourinary malfromation
4. mental retardation

Front 3

Which characteristics are associated with Denys-Drash syndrome?

Back 3

Characteristics of Denys-Drash syndrome:

1. WIlm's tumor
2. Intersex disorder
3. Progressice neuropathy

Front 4

Which characteristics are associated Beckwith-Wiedemann syndrome?

Back 4

Characteristics associated with Beckwith-Wiedemann syndrome:

1. omphalocoele
2, visceromegaly
3. macroglossia
4. hypoglycemia

Front 5

What serologic and urinary tests should be ordered in pediatric patients with solid abdominal masses? How do these tests help with the differential diagnosis?

Back 5

Serologic and urinary work up for pediatric solid abdominal masses:

1. serum metanephrine, normetanephrine (neuroblastoma)
2. serum alpha feto protein (hepatoblastoma)
3. Urinary homovanillic acid, urinary vanillylmandelic acid (neuroblastoma)

Front 6

What are the most common pediatric solid tumors?

Back 6

Most common pediatric solid tumors:

1. Neuroblastoma
2. Wilm's Tumor
3. rhabdomyosarcoma
4. hepatoblastoma
5. Sacrococcygeal tumor

Front 7

Where do neuroblastomas most commonly metastasize? What symptoms are associated with each site of metastasis?

Back 7

Common sites of meuroblastoma metastasis:

1. cortical bone: swelling, tenderness, pain, pathologic fractures
2. bone marrow: anemia, weakness, easy bruising
3. liver: hepatomegaly, respiratory compromise
4. periorbital bone: proptosis, ecchymosis (racoon's eye)
5. skin: blue subcutaneous nodules (blueberry muffin syndrome)

Front 8

Which serologic tests are poor prognostic indicators for neuroblastoma?

Back 8

Poor prognostic indicators for neuroblastoma:

1. elevated lactate dehydrogenase
2. elevated neuron-specific enolase
3. elevated serum ferritin

Front 9

Amplification of which proto-oncogene is a poor prognostic indicator for neuroblastoma? What is the function of the gene product?

Back 9

Amplification of the N-myc proto-oncogene is a poor prognostic indicator for neuroblastoma. The N-myc gene produces a transcription factor that binds to DNA via basic helix-loop-helix moiety.