Quiz 3 Cancers Flashcards
What is the etiology of Retinoblastoma?
What percent of Retinoblastoma patients inherited their “first hit” from a parent?
40%
The pathology seen in this photo is suggestive of what kind of cancer?
Retinoblastoma
What kind of tumor is most commonly found in patients with an average age of 3 years, and is the most common malignant renal tumor in children?
Wilms tumor
What three syndromes have a high propensity for developing a Wilms tumor?
WAGR syndrome- WT1 deletion
Denys-Drash syndrome- WT1 zinc finger point mutation
Beckwith-Wiedemann syndrome- WT2 overexpression
What is notable about WAGR syndrome?
WT1 deletion
Nephrogenic rest formation
30% risk of Wilms tumor
What is notable about Denys-Drash syndrome
What is notable about Beckwith-Wiedemann syndrome?
What cancer is suggested by these clinical symptoms?
Wilms tumor
What is notable about Wilms tumor histology?
Small round blue cells (blastemal cells)
stromal and epithelial patterns
Anaplasia correlates with loss of p53
CD99+
How is Wilms tumor treated?
Depending on extent, nephrectomy and chemotherapy
What kind of cancer is the most common extracranial solid tumor in children?
Neuroblastoma
What is the etiology of Neuroblastoma?
What cancer best matches this presentation of symptoms?
Neuroblastoma
What histological features of a Neuroblastoma are most significant?
Presence of neuropils and
Homer-Wright pseudorosettes
What pediatric musculoskeletal tumor is most common in boys under 15 yo?
Ewing sarcoma
What is the difference between a Ganglioneuroblastoma and a Ganglioneuroma?
Both have ganglion cells and schwannian stroma but only GNB has neuropil
What type of cancer is suggested by this etiology? What is especially important to note?
Ewing Sarcoma
t(11;22) translocation causing EWS and FLI1 fusion
more common in diaphysis of long bones
These symptoms may suggest what type(s) of cancer?
Osteosarcoma
Ewing sarcoma
Which type of cancer may be indicated by these histological findings? What is most significant?
Ewing sarcoma
Little or no pleiomorphism
Homer-Wright pseudorosettes
What type of cancer best fits these epidemiologic characteristics?
Osteosarcoma
What characteristics of osteosarcoma are most significant?
Production of osteoid
most common in metaphysis
Often found with tumor supressor mutation
For what type of pedatric musculoskeletal cancer can this treatment be used?
Osteosarcoma