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114 Cards in this Set

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  • Back
What % of malignant tumors occur in infancy and childhood?
2%
What percent of childhood deaths are accounted for by neoplastic disease?
9%
What type of neoplasms are more common in childhood?
Benign
What is the tissue origin of the most common neoplasms in childhood?
Mesenchymal
What are neoplasms in childhood frequently seen in relationship with?
Abnormal development
What do childhood neoplasms have the potential for?
-Spontaneous regression
-Cytodifferentiation
What is Heterotopia?
Microscopically NORMAL cells or tissue in ABNORMAL locations/
What are 2 examples of heterotopia?
-Pancreatic tissue in the stomach or GI wall
-Adrenal cells in the kidney lung or ovary
What is another name for heterotopia?
Choristoma
What is a Hamartoma?
Excessive LOCALIZED overgrowth of mature cells/tissue that are normal in that organ, but abnormally arranged.
What are 5 examples of Hamartomas?
-Hemangiomas
-Lymphangiomas
-Adenomas of the liver
-Rhabdomyomas of the heart
-Developmental cysts of the kidneys lungs or pancreas
What are the 4 most common Benign tumors seen in infancy and childhood?
1. Hemangioma
2. Lymphatic tumors
3. Fibrous tumors
4. Teratomas
What is the most common tumor of infancy? Where is it frequently located?
Hemangioma on the skin of the face and scalp.
What do hemangiomas look like?
Flat to elevated irregular red-blue masses.
What tissue proliferation causes hemangiomas?
Small or large vessels
What is a hereditary disorder that hemangiomas may be associated with?
von Hippel-Lindau disease
Are hemangiomas representative of heterotopia or hamartomas?
Hamartomas
What are lymphatic tumors the result of?
Hamartoma/neoplastic processes
What do lymphatic tumors consist of?
-Cystic/cavernous spaces filled with lymph
Where do lymphatic tumors typically arise?
-Skin
-Deeper tissues of neck, axilla, mediastinum, retroperitoneum
What can happen as lymphatic tumors fill with fluid?
Encroachment on vital structures
How is lymphangiectasis different from lymphatic tumors?
It is the abnormal dilation of PRE-EXISTING Lymphatics
What does Lymphangiectasis often result in?
Deformation of the extremities
What is the mechanism for development of Fibromatosis?
Hypocellar spindle cells proliferate/infiltrate locally.
What does the tendancy for recurrence of Fibromatosis depend on in the patient?
-Age
-Location
-Mode of therapy
How is congenital/infantile fibrosarcoma different from Adult malignant fibrosarcoma?
-Hypercellular spindle cell proliferation
-Has excellent prognosis
-Has a characteristic translocation associated with it
What is the characteristic translocation ass w/ Congenital infantile Fibrosarcoma?
(12;15) (p13;q25)
What is pediatric Myofibromatosis?
Proliferation of myofibroblasts involving the skin, soft tissue, and organs.
What are 2 types of myofibromatosis? Which is clinically benign?
-Solitary (clinically benign)
-Multifocal
What feature are morbidity/mortality associated with in multifocal myofibromatosis?
Impingement on vital organ function.
What is Fibrous hamartoma of infancy?
Benign fibrous growth in the dermis or subcutaneous tissue during the first 2 yrs of life.
What sex is Fibrous hamartoma more commonly seen in?
Males
What is the most common location of Fibrous hamartomas of infancy?
In the anterior/posterior axillary folds.
What is the morphology seen in Fibrous hamartoma of infancy?
Organoid patterns with
-Fibrous trabeculae
-Islands of loosely arranged spindle-shaped cells
-Mature fat
What is the main way to differentiate between Fibromatosis and Fibrous hamartoma of infancy?
Fibromatosis = bland looking
Fibrous hamartoma = more mixed in nature
What are Teratomas usually derived from?
All 3 germ layers
What is the most common location of teratomas in childhood? In what sex?
-Sacrococcyx
-In females
What is a malignant tumor in the hematopoietic system called?
Leukemia/lymphoma
What are 3 types of malignant tumors in central nervous tissue?
-Posterior fossa juvenile astrocytoma
-Ependymoma
-Medulloblastoma
What are 2 types of peripheral nervous tissue malignant tumors?
-Neuroblastoma (adrenal medulla)
-Retinoblastoma (retina)
What is a malignant tumor of soft tissue called?
Rhabdomyosarcoma
What is a malignant tumor of bone called?
-Ewing sarcoma or Osteosarcoma
What is a malignant tumor of the kidney called?
Wilms tumor - nephroblastoma
What is a malignant tumor in the liver called?
Hepatoblastoma
What are malignant pediatric neoplasms designated as?
-blastomas
What do the morphologic features of pediatric malignant neoplasms often demonstrate?
Attempts at recapitulating the site of origin organ development
What are malignant pediatric tumors generally referred to as?
Pediatric Small Round Blue Cell tumors
What is required for diagnosing and evaluating prognosis in pediatric tumors, since their appearance is often primitive?
-Adjunctive staining
-EM
-cytogenetic analysis
What type of malignant neoplasm is generally limited to pediatrics age 0-4?
Teratoma
Wilm's tumor
At what age are leukemia, retinoblastoma, and neuroblastoma mostly seen?
0-9 yrs
What is the age group in which osteosarcoma, thyroid cancer and hodgkins disease are mostly seen?
10-14
What are neuroblastic tumors and what are they derived from?
Tumors of the sympathetic chain ganglia and adrenal medulla
-Derived from neural crest
What is the most common childhood malignancy? 2nd most common?
1. Brain tumor
2. Neuroblastoma
What is most important for determining prognosis in neuroblastic tumors?
Age and Stage
Are neuroblastic tumors more sporadic or familial?
Sporadic
What are the 3 types of neuroblastic tumors?
1. Neuroblastoma
2. Ganglioneuroblastoma
3. Ganglioneuroma
What is the basis of differentiating the 3 types of neuroblastic tumors?
Degree of maturity
Which type of neuroblastic tumor is most primitive? Which is most mature?
Neuroblastoma = primitive

Ganglioneuroma = mature
What is the morphology consistent in Neuroblastoma?
-Sheets of small primitive neuroblasts w/ dark nuclei
-Pale eosinophilic fibrillary background
How does morphology in ganglioneuroblastoma compare to neuroblastoma?
Cells become larger with more abundant cytoplasm and Ganglion cell differentiation is seen
What additional cell types are seen in ganglioneuroblastomas?
-Schwann cells
-Fibroblasts
What does the morphology consist of in Ganglioneuromas?
-Mature ganglion cells
-Schwannian stroma
What are telltale clinical symptoms of neuroblastomas?
-Large abdominal mass
-Fever
-Weight loss
What is more commonly seen in older pediatric patients with neuroblastoma?
Metastasis to bone, lung, GI, and bladder.
What is a common clinical sign of metastasized neuroblastoma?
Periorbital proptosis and ecchymosis
What is a clinical symptom of cutaneous neuroblastoma metastases in neonates?
Blueberry muffin baby
What is the very important diagnostic finding in neuroblastomas?
Elevated urine levels of catecholamine metabolites
-VMA and HVA
What age of pediatric patients have excellent prognosis in neuroblastoma?
<1 yr old - REGARDLESS OF STAGE
In the staging of neuroblastomas, what stages are favorable? Unfavorable?
Favorable = 1, 2a, 2b, and 4s

Unfavorable = 3 and 4
What stage tumors do most children present with?
3 and 4
What is Stage 4s?
A special stage for infants under 1 yr old
What is stage 4s defined as?
Localized primary tumor w/ dissemination limited to skin, liver, and/or bone marrow
Why is stage 4s a good prognostic indicator?
Because 80% have a 5 yr survival with only minimal therapy.
What are the morphologic prognostic indicators in neuroblastic tumors?
-Degree of differentiation
-Mitotic rate
-Mitotic/karyorrhexis index
-Calcification (a good sign)
What types of DNA ploidy are good prognostic indicators?
-Hyperploidy
-Near-triploidy
What types of DNA ploidy are poor prognostic indicators?
-Diploidy
-Near-diploidy
-Near-tetraploidy
What is the most important genetic abnormality used in risk stratification in Neuroblastic tumors? Is it good or bad?
The N-myc oncogene on Ch' 2; presence is BAD.
What 2 chromosome abnormalities are bad prognostic indicators in neuroblastic tumors?
-Gain of 17q
-Loss of 1p
What are 4 general Favorable Factors in neuroblastic tumors?
-Presence of Trk-A/CD44
-Absent MRP/telomerase expression
-Normal serum ferritin
-Low lactate dehydrogenase
Gross hematuria.. think...
Wilms tumor
Where does Wilms tumor rate in terms of frequency in solid malignancies in children?
4th
What is the average age of patients diagnosed with WT?
3 yrs old
Are the majority of WT's uni or bilateral?
Unilateral
What age tendency is seen in bilateral WT cases?
Younger
What are 4 conditions associated with increased risk of Wilms tumor?
1. WAGR syndrome
2. Denys-Drash syndrome
3. Beckwith-Wiedemann syndrome
4. Familial nephroblastoma
What does WAGR stand for?
-Wilms
-Aniridia
-Genital anomaly
-Retardation
What is the chromosomal abnormality associated with WAGR syndrome?
A constitutional deletion in 11p13
What genes are deleted in WAGR syndrome?
-WT1 gene
-PAX6 gene
What 4 things does Denys-Drash syndrome consist of?
-Gonadal dysgenesis
-Male pseudohermaphroditism
-Nephropathy
-Wilms tumor
What is the cause of Denys-Drash syndrome?
A germ line mutation in 11p13
What is the acronym for Beckwith-Wiedemann syndrome?
MOARCHO
What does MOARCHO stand for?
-Macroglossia
-Organomegaly
-Adrenal cytomegaly
-Renal medullary cysts
-Hemihypertrophy
-Omphalocele
What is the abnormal chromosome and gene delation that causes Beckwith Wiedemann syndrome?
Ch' 11p15.5, deletion of WT2
What is the chromosome and gene abnormaltiy in Familial nephroblastoma?
Loss of heterozygosity on long arm of Ch' 16, WT3 gene
What does WT1 gene encode?
A transcription factor
What happens when there is a complete, homozygous gross deletion of WT1 in mice?
Renal agenesis
What happens when there are heterozygous gross deletions of WT1?
WAGR syndrome
What happens when there is a dominant point mutation of WT1?
Denys drash syndrome
What will abdominal exam commonly show in WT presentation?
Asymptomatic unilateral mass
What are 3 associated signs and symptoms in WT presentation?
-Malaise
-Pain
-Micro/macroscopic hematuria
Where does WT most commonly metastasize?
to the lungs
What is the cell type that composes Wilms tumors?
Embryonic metanephric blastemal tissues from the developing kidney.
What does the presence of nephrogenic rests persisting beyond 36 wks gestation indicate?
Increased risk of developing a contralateral Wilms
What does the characteristic Triphasic morphology of WT include?
1. Undifferentiated blast cells
2. Stromal spindle cells
3. Epithelial elements
What do the morphologic characteristics of WT indicate?
Recapitulation of normal kidney tissue - glomeruloid structures
What is a common finding in WT morphology?
Heterotopic cell types - smooth/skeletal muscle, fat, cartilage, bone..
What is the most favorable histology in WT?
Absence of anaplasia
What is considered Unfavorable histology in WT?
Diffuse anaplasia
What is Anaplasia defined as?
3 fold nuclear enlargement compared with adjacent nuclei of the same cell tye
-Hyperchromasia of enlarged nuclei
-Atypical enlarged multipolar mitoses
What gene mutation is associated with anaplasia?
p53
What is considered diffuse anaplasia?
>10 per high power field
What is the prognosis for patients with WT?
Good - 90% have long term survival with combined surgery chemo and radiation
What is associated with the increased survival in WT patients?
Increased risk of 2nd malignancy
Flowers!
yay