Module 8:PEDS: Neoplasms Flashcards Preview

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Flashcards in Module 8:PEDS: Neoplasms Deck (24):
1

What are the common malignant neoplasms in 0-4 yr olds?

Leukemia
Retinoblastoma
Neuroblastoma
Wilm's Tumor
Hepatoblastoma
Soft Tissue Sarcoma
Teratoma
CNS tumor

2

What are the common malignant neoplasms in 5-9 yr olds?

Leukemia
Retinoblastoma
Neuroblastoma
Hepatocellular carcinoma
Soft tissue sarcoma
Ewing tumor
CNS tumor
Lymphoma

3

What are the common malignant neoplasms in 10-14 yr olds?

Heptocellular Carcinoma
Soft tissue sarcoma
Osteogenic sarcome
Thyroid carcinoma
Hodgkin disease

4

What are some general features of pediatric neoplasms?

Magnitude of pediatric malignancy is much lower than in adult
-most pediatric tumors are embryonal, not carcinomas
--pediatric tumors dont occur in the same location as adult tumors
--pediatric tumors are more responsive to therapy
--better prognosis and survival rate than adults

5

What are the predisposing factors for pediatric neoplasms?

Down syndrome and agammaglobulinemia
Deletion 13q (retinoblastoma)
Wiskott Aldrich Syndrome (lymphoma)

6

What are the tumors of embryonal origin?

Small, round, blue cell tumors
-lymphoma/leukemia
-medulloblastoma
--neuroblastoma
--rhabdomyosarcoma (embryonal and alveolar)
-wilms tumor
-bone tumors (ewings sarcoma and small cell osteosarcoma)

7

What is the origin of neuroblastoma?

Poorly differentiated tumor of NCC that normally give rise to the adrenal medulla and sympathetic due to germline mutation in ALK gene
--second most common malignant tumor in kids

8

What is the presentation for neuroblastoma?

less than 2 years old: mass in abdomen or mediastinum, fever or weight loss
Older children: signs of metastatic disease (bone pain, resp and GI symptoms)
Blueberry Muffin baby: disseminated neuroblastoma

9

What investigations are done for neuroblastoma?

Gross: calcification in 40-50%
Microscopy: neurophil (eosinophilic fibrillary material) and Homer-Wright Pseudorosettes (tumor cells arrange around neurophil without a lumen)
EM: neurosecretory granules
IHC: neuron-specific enolase and synaptophysin

10

What confirms diagnosis of neuroblastoma?

Increased blood catecholamines
Increased urine levels of catecholamines (VMA and HVA)
MIBG scintigraphy: concentrates over 90% of tumors -- used for assessment of spread and response to therapy

11

What are the good and poor prognostic factors for neuroblastoma?

Good: stage 4S and increased expression of tyrosine kinase receptor A
Poor: deletion distal 1p and gain of distal 17q; n-myc amplification and telomerase overexpression

12

Moving on to nephroblastoma (Wilm's Tumor), what is this?

Malignant embryonal neoplasm from nephrogenic blastemal cells
--most common primary renal tumor of childhood

13

What are the various etiologies for Wilm's Tumor?

Most cases are sporadic
10% are associated with:
--WAGR syndrome: WT-1 gene mutation
--Denys-Drash syndrome: WT-1 gene mutation
-Beckwith-Wiedemann syndrome
-Mutations of Beta-Catenin Gene

14

What is the precursor lesion for Wilm's Tumor?

Nephrogenic rests

15

What is presentation for Wilm's Tumor?

Detection of abdominal mass by parent while bathing the child

16

What is seen on histology for Wilm's Tumor?

Triphasic pattern due to divergent differentiation
--blastema: sheets of small round blue cells
-stroma: myxoid
-epithelium: abortive tubules and glomeruli

17

What are complications of Wilm's Tumor?

Restricted pattern of metastasis = regional LNs, lungs and liver

18

What is the anaplastic subtype of Wilm's Tumor?

5% of cases: characterized by hyperchromatic nuclei with greater than 3 times larger than those in adjacent cells
--correlates with acquired TP53 mutations

19

Next is retinoblastoma, what is the etiology?

Malignant neoplasm arising from retina
--40% congenital: RB1 gene: multiple, bilateral tumors
(increased susceptibility to other malignant tumors: osteogenic sarcoma, Ewing's sarcoma, pinealoblastoma)
--60% sporadic: unifocal and unilateral tumors

20

What is the presentation for retinoblastoma?

Leukocoria
Strabismus
Poor Vision
Spontaneous Hyphema
Red and Painful eye

21

What is found on histology for retinoblastoma?

Flexner-Wintersteiner Rosettes: clusters of cuboidal cells arranged around a central lumen

22

What are complications of retinoblastoma?

Extension into optic nerve and/or subarachnoid space with intracranial spread
Invasion of blood vessels
Secondary Glaucoma

23

Finally where do teratomas arise from?

Most arise in the sacro-coccygeal region
--other sites = testes and ovaries

24

What is the prognosis for teratomas?

Less than 4 months = benign
1 year = 50% malignant
5 years = 100% malignant
--proportion of immature elements is important in gauging degree of malignant potential

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