Term
| What are 3 things that determine the rate of growth? |
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Definition
1. percent of cells that are actively replicating
2. doubling time of cell
3. rate at which cells shed or die |
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Term
| Epithelial malignancies typically take what course of spreading? |
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Definition
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Term
| What term refers to a germ cell tumor that may contain all 3 layers of embryonic development? |
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Definition
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Term
| What is a malignant, trophoblastic, and aggressive cancer of the placenta called? |
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Definition
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Term
| What are the 3 staging criteria? |
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Definition
TMN
T- Tumor size
M- metastasis
N- lymph node involvement |
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Term
| Describe the growth pattern of capillary hemangioma. |
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Definition
| It grows rapidly, then it starts to fade at 1-3 years of age. It completely regresses by age 7 in 75% to 90% of cases |
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Term
| What is the term used to describe an extremely dilated, swollen lymphatic vessel that leads to the loss of proteins? |
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Definition
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Term
| Are fibrous tumors sparsely cellular, normocellular, or hypercellular? |
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Definition
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Term
| Congenital Infantile Fibrosarcoma is due to what novel chromosomal translocation? |
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Definition
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Term
| What are the 2 peaks of incidence for teratomas? |
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Definition
| at 2 years and during late adolescence |
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Term
| What is the frequency of sacrococcygeal teratomas? |
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Definition
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Term
| What percent of malignant tumors occur in children? |
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Definition
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Term
| Neuroblastic tumors are derived from what progenitor? |
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Definition
| neural crest cell, so they affect sympathetic ganglia, adrenal medulla |
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Term
| 1-2% of cases of Neuroblastoma are Familial. What is Familial Neuroblastoma linked to? |
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Definition
| linked to germline mutations in the ALK gene |
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Term
| What sort of metastasis route does neuroblastoma take? |
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Definition
| hemaotgenous/ periorbital space commonly affected |
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Term
| What are tumor cell arrangements around areas of fibrillarity referred to as? |
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Definition
| Homer Wright Pseudorosettes |
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Term
| In Neuroblastoma staging, what does the 4s stage mean? |
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Definition
| It is linked to infants less than 1 year old and refers to a localized primary tumor with dissemination limited to skin, liver, and/or bone marrow. |
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Term
| What is the most important prognostic indicator of neuroblastoma? |
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Definition
| N-myc oncogene amplification (high-risk regardless of stage) |
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Term
| What is the fourth most common pediatric cancer, and what is its age range? |
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Definition
| Wilms Tumor/ age range 2-5 years |
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Term
| What percent of Wilms Tumor cases have bilateral presentation? |
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Definition
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Term
| What percent of Wilms Tumor cases are syndromic? |
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Definition
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Term
| What are the 3 most common syndromes that involve Wilms Tumor? |
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Definition
| WAGR, Denys Drash, Beckwith Wiedemann |
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Term
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Definition
| Wilms Tumor, Aniridia, Genital Abnormalities, Mental Retardation |
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Term
| What percentage of Denys Drash cases is due to a mutation in the WT1 gene? |
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Definition
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Term
| Denys Drash syndrome presents with what 3 conditions? |
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Definition
| Wilms Tumor, gonadal dysgenesis, early onset nephropathy due to diffuse mesangial sclerosis |
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Term
| Beckwith Wiedemann syndrome has a Wilms Tumor component linked to a change in which gene? |
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Definition
| The WT2 gene located at 11 p15.5 site |
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Term
| Wilms Tumor is a triphasic nephroblastoma. What are the three phases? |
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Definition
Blastema- Blue Cells
Stroma- fibrous, myxoid, skeletal muscle
Epithelial cells: tubules or glomeruli |
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Term
| What genetic changes correlated with an adverse prognosis for someone with Wilms' tumor? |
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Definition
| Deletions in 11 q and 16 q, gains in 1q |
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Term
| Wilms Tumor (5% of them contain what?) |
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Definition
| Foci of anaplasia- large, hyperchromatic, pleomrophic nuclei with abnormal mitoses |
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