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
What histological features of Osteosarcoma are most noteworthy?
X-ray: sunburst pattern
Histo: Pleomorphic cells
Lace-like architecture with osteoid
What is the most common soft-tissue sarcoma in children?
Rhabdomyosarcoma
What are the four msot notable types of rhabdomyosarcoma?
Embryonal RMS
Alveolar RMS
Spindle cell/sclerosing RMS
Pleimorphic RMS
Rank the four types of RMS from least to most favorable
Pleiomorphic RMS < Alveolar RMS < Spindle cell RMS < Embryonal RMS
What is notable about Embryonal RMS?
Most common type (60%)
subclass: Botyroid RMS
median age 6.5 years
What is notable about alveolar RMS?
median age 12 years
t (2;13) PAX3-FOXO1a (agg)
t(1;13) PAX7-FOXO1a
more common in deep muscles of extremities
What is notable about Spindle cell/sclerosing RMS?
age dependent prognosis (more favorable for children)
MYOD1 &NCOA2 mutations
What is notable about Pleiomorphic RMS?
What is the etiology of cancer initiated by polycyclic aromatic hydrocarbons?
G to T DNA transversions
What are the two most carcinogenic compounds found in cigarette smoke?
polycyclic aromatic hydrocarbons and nitrosamines
What are two other risk factors for cancer besides smoking and air quality?
Radon and Asbestos
What is the leading cause of lung cancer?
smoking (85%)
What is the second greatest cause of lung cancer?
Radon
When should screening for lung cancer be done?
At age 55
> 30 pack year history
currently smoke or quit in last 15 years
What is the usual sequence of diagnosis for lung cancer?
What type of cancer best fits this epidemiologic profile?
Small cell lung cancer
What type of cancer best fits this etiology? What is most significant?
Small cell lung cancer; arises from neuroendocrine cells, and rapidly grows and metastasizes
What paraneoplastic syndromes are associated with small cell lung cancer?
What is the most common staging for small cell lung cancer?
What type of cancer is best represented by this histological profile?
Small cell lung cancer
An immunohistochemistry report involving the following would suggest what type of cancer?
Small cell lung cancer
What treatment would be adminstered for small cell lung cancer?
What is the staging for NSCLC?
What paraneoplastic syndrome is associated with squamous cell carcinoma?
What type of cancer best matches this histological profile?
Squamous cell carcinoma of the lung
What types of lung cancer are more common in men? Which types are centrally located?
small cell lung cancer
squamous cell carcinoma
What type of lung cancer is peripherally located?
adenocarcinoma of the lung
What are the associated paraneoplastic syndromes of adenocarcinoma of the lung?
In what three enzymes do patients with adenocarcinoma of the lung often have mutations?
What is the most common lung cancer of non-smokers?
adenocarcinoma of the lung
Increased expression of TTF-1 and/or Cytokeratin 7 would be suggestive of what type of cancer?
adenocarcinonma of the lung
Adenocarcinoma of the lung is most common in what three demographics?
Asians
women
non-smokers
This histological profile best matches what type of cancer?
Adenocarcinoma of the lung
This profile best matches what type of cancer?
Large cell carcinoma
This profile is most consistant with what type of neoplasm?
Carcinoid