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114 Cards in this Set
- Front
- Back
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What % of malignant tumors occur in infancy and childhood?
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2%
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What percent of childhood deaths are accounted for by neoplastic disease?
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9%
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What type of neoplasms are more common in childhood?
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Benign
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What is the tissue origin of the most common neoplasms in childhood?
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Mesenchymal
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What are neoplasms in childhood frequently seen in relationship with?
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Abnormal development
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What do childhood neoplasms have the potential for?
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-Spontaneous regression
-Cytodifferentiation |
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What is Heterotopia?
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Microscopically NORMAL cells or tissue in ABNORMAL locations/
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What are 2 examples of heterotopia?
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-Pancreatic tissue in the stomach or GI wall
-Adrenal cells in the kidney lung or ovary |
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What is another name for heterotopia?
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Choristoma
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What is a Hamartoma?
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Excessive LOCALIZED overgrowth of mature cells/tissue that are normal in that organ, but abnormally arranged.
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What are 5 examples of Hamartomas?
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-Hemangiomas
-Lymphangiomas -Adenomas of the liver -Rhabdomyomas of the heart -Developmental cysts of the kidneys lungs or pancreas |
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What are the 4 most common Benign tumors seen in infancy and childhood?
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1. Hemangioma
2. Lymphatic tumors 3. Fibrous tumors 4. Teratomas |
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What is the most common tumor of infancy? Where is it frequently located?
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Hemangioma on the skin of the face and scalp.
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What do hemangiomas look like?
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Flat to elevated irregular red-blue masses.
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What tissue proliferation causes hemangiomas?
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Small or large vessels
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What is a hereditary disorder that hemangiomas may be associated with?
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von Hippel-Lindau disease
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Are hemangiomas representative of heterotopia or hamartomas?
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Hamartomas
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What are lymphatic tumors the result of?
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Hamartoma/neoplastic processes
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What do lymphatic tumors consist of?
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-Cystic/cavernous spaces filled with lymph
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Where do lymphatic tumors typically arise?
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-Skin
-Deeper tissues of neck, axilla, mediastinum, retroperitoneum |
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What can happen as lymphatic tumors fill with fluid?
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Encroachment on vital structures
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How is lymphangiectasis different from lymphatic tumors?
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It is the abnormal dilation of PRE-EXISTING Lymphatics
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What does Lymphangiectasis often result in?
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Deformation of the extremities
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What is the mechanism for development of Fibromatosis?
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Hypocellar spindle cells proliferate/infiltrate locally.
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What does the tendancy for recurrence of Fibromatosis depend on in the patient?
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-Age
-Location -Mode of therapy |
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How is congenital/infantile fibrosarcoma different from Adult malignant fibrosarcoma?
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-Hypercellular spindle cell proliferation
-Has excellent prognosis -Has a characteristic translocation associated with it |
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What is the characteristic translocation ass w/ Congenital infantile Fibrosarcoma?
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(12;15) (p13;q25)
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What is pediatric Myofibromatosis?
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Proliferation of myofibroblasts involving the skin, soft tissue, and organs.
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What are 2 types of myofibromatosis? Which is clinically benign?
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-Solitary (clinically benign)
-Multifocal |
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What feature are morbidity/mortality associated with in multifocal myofibromatosis?
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Impingement on vital organ function.
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What is Fibrous hamartoma of infancy?
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Benign fibrous growth in the dermis or subcutaneous tissue during the first 2 yrs of life.
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What sex is Fibrous hamartoma more commonly seen in?
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Males
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What is the most common location of Fibrous hamartomas of infancy?
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In the anterior/posterior axillary folds.
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What is the morphology seen in Fibrous hamartoma of infancy?
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Organoid patterns with
-Fibrous trabeculae -Islands of loosely arranged spindle-shaped cells -Mature fat |
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What is the main way to differentiate between Fibromatosis and Fibrous hamartoma of infancy?
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Fibromatosis = bland looking
Fibrous hamartoma = more mixed in nature |
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What are Teratomas usually derived from?
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All 3 germ layers
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What is the most common location of teratomas in childhood? In what sex?
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-Sacrococcyx
-In females |
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What is a malignant tumor in the hematopoietic system called?
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Leukemia/lymphoma
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What are 3 types of malignant tumors in central nervous tissue?
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-Posterior fossa juvenile astrocytoma
-Ependymoma -Medulloblastoma |
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What are 2 types of peripheral nervous tissue malignant tumors?
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-Neuroblastoma (adrenal medulla)
-Retinoblastoma (retina) |
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What is a malignant tumor of soft tissue called?
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Rhabdomyosarcoma
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What is a malignant tumor of bone called?
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-Ewing sarcoma or Osteosarcoma
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What is a malignant tumor of the kidney called?
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Wilms tumor - nephroblastoma
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What is a malignant tumor in the liver called?
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Hepatoblastoma
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What are malignant pediatric neoplasms designated as?
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-blastomas
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What do the morphologic features of pediatric malignant neoplasms often demonstrate?
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Attempts at recapitulating the site of origin organ development
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What are malignant pediatric tumors generally referred to as?
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Pediatric Small Round Blue Cell tumors
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What is required for diagnosing and evaluating prognosis in pediatric tumors, since their appearance is often primitive?
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-Adjunctive staining
-EM -cytogenetic analysis |
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What type of malignant neoplasm is generally limited to pediatrics age 0-4?
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Teratoma
Wilm's tumor |
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At what age are leukemia, retinoblastoma, and neuroblastoma mostly seen?
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0-9 yrs
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What is the age group in which osteosarcoma, thyroid cancer and hodgkins disease are mostly seen?
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10-14
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What are neuroblastic tumors and what are they derived from?
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Tumors of the sympathetic chain ganglia and adrenal medulla
-Derived from neural crest |
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What is the most common childhood malignancy? 2nd most common?
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1. Brain tumor
2. Neuroblastoma |
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What is most important for determining prognosis in neuroblastic tumors?
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Age and Stage
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Are neuroblastic tumors more sporadic or familial?
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Sporadic
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What are the 3 types of neuroblastic tumors?
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1. Neuroblastoma
2. Ganglioneuroblastoma 3. Ganglioneuroma |
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What is the basis of differentiating the 3 types of neuroblastic tumors?
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Degree of maturity
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Which type of neuroblastic tumor is most primitive? Which is most mature?
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Neuroblastoma = primitive
Ganglioneuroma = mature |
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What is the morphology consistent in Neuroblastoma?
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-Sheets of small primitive neuroblasts w/ dark nuclei
-Pale eosinophilic fibrillary background |
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How does morphology in ganglioneuroblastoma compare to neuroblastoma?
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Cells become larger with more abundant cytoplasm and Ganglion cell differentiation is seen
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What additional cell types are seen in ganglioneuroblastomas?
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-Schwann cells
-Fibroblasts |
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What does the morphology consist of in Ganglioneuromas?
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-Mature ganglion cells
-Schwannian stroma |
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What are telltale clinical symptoms of neuroblastomas?
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-Large abdominal mass
-Fever -Weight loss |
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What is more commonly seen in older pediatric patients with neuroblastoma?
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Metastasis to bone, lung, GI, and bladder.
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What is a common clinical sign of metastasized neuroblastoma?
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Periorbital proptosis and ecchymosis
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What is a clinical symptom of cutaneous neuroblastoma metastases in neonates?
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Blueberry muffin baby
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What is the very important diagnostic finding in neuroblastomas?
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Elevated urine levels of catecholamine metabolites
-VMA and HVA |
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What age of pediatric patients have excellent prognosis in neuroblastoma?
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<1 yr old - REGARDLESS OF STAGE
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In the staging of neuroblastomas, what stages are favorable? Unfavorable?
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Favorable = 1, 2a, 2b, and 4s
Unfavorable = 3 and 4 |
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What stage tumors do most children present with?
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3 and 4
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What is Stage 4s?
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A special stage for infants under 1 yr old
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What is stage 4s defined as?
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Localized primary tumor w/ dissemination limited to skin, liver, and/or bone marrow
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Why is stage 4s a good prognostic indicator?
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Because 80% have a 5 yr survival with only minimal therapy.
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What are the morphologic prognostic indicators in neuroblastic tumors?
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-Degree of differentiation
-Mitotic rate -Mitotic/karyorrhexis index -Calcification (a good sign) |
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What types of DNA ploidy are good prognostic indicators?
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-Hyperploidy
-Near-triploidy |
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What types of DNA ploidy are poor prognostic indicators?
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-Diploidy
-Near-diploidy -Near-tetraploidy |
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What is the most important genetic abnormality used in risk stratification in Neuroblastic tumors? Is it good or bad?
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The N-myc oncogene on Ch' 2; presence is BAD.
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What 2 chromosome abnormalities are bad prognostic indicators in neuroblastic tumors?
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-Gain of 17q
-Loss of 1p |
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What are 4 general Favorable Factors in neuroblastic tumors?
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-Presence of Trk-A/CD44
-Absent MRP/telomerase expression -Normal serum ferritin -Low lactate dehydrogenase |
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Gross hematuria.. think...
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Wilms tumor
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Where does Wilms tumor rate in terms of frequency in solid malignancies in children?
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4th
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What is the average age of patients diagnosed with WT?
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3 yrs old
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Are the majority of WT's uni or bilateral?
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Unilateral
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What age tendency is seen in bilateral WT cases?
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Younger
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What are 4 conditions associated with increased risk of Wilms tumor?
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1. WAGR syndrome
2. Denys-Drash syndrome 3. Beckwith-Wiedemann syndrome 4. Familial nephroblastoma |
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What does WAGR stand for?
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-Wilms
-Aniridia -Genital anomaly -Retardation |
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What is the chromosomal abnormality associated with WAGR syndrome?
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A constitutional deletion in 11p13
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What genes are deleted in WAGR syndrome?
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-WT1 gene
-PAX6 gene |
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What 4 things does Denys-Drash syndrome consist of?
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-Gonadal dysgenesis
-Male pseudohermaphroditism -Nephropathy -Wilms tumor |
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What is the cause of Denys-Drash syndrome?
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A germ line mutation in 11p13
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What is the acronym for Beckwith-Wiedemann syndrome?
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MOARCHO
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What does MOARCHO stand for?
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-Macroglossia
-Organomegaly -Adrenal cytomegaly -Renal medullary cysts -Hemihypertrophy -Omphalocele |
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What is the abnormal chromosome and gene delation that causes Beckwith Wiedemann syndrome?
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Ch' 11p15.5, deletion of WT2
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What is the chromosome and gene abnormaltiy in Familial nephroblastoma?
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Loss of heterozygosity on long arm of Ch' 16, WT3 gene
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What does WT1 gene encode?
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A transcription factor
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What happens when there is a complete, homozygous gross deletion of WT1 in mice?
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Renal agenesis
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What happens when there are heterozygous gross deletions of WT1?
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WAGR syndrome
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What happens when there is a dominant point mutation of WT1?
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Denys drash syndrome
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What will abdominal exam commonly show in WT presentation?
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Asymptomatic unilateral mass
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What are 3 associated signs and symptoms in WT presentation?
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-Malaise
-Pain -Micro/macroscopic hematuria |
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Where does WT most commonly metastasize?
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to the lungs
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What is the cell type that composes Wilms tumors?
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Embryonic metanephric blastemal tissues from the developing kidney.
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What does the presence of nephrogenic rests persisting beyond 36 wks gestation indicate?
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Increased risk of developing a contralateral Wilms
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What does the characteristic Triphasic morphology of WT include?
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1. Undifferentiated blast cells
2. Stromal spindle cells 3. Epithelial elements |
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What do the morphologic characteristics of WT indicate?
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Recapitulation of normal kidney tissue - glomeruloid structures
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What is a common finding in WT morphology?
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Heterotopic cell types - smooth/skeletal muscle, fat, cartilage, bone..
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What is the most favorable histology in WT?
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Absence of anaplasia
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What is considered Unfavorable histology in WT?
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Diffuse anaplasia
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What is Anaplasia defined as?
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3 fold nuclear enlargement compared with adjacent nuclei of the same cell tye
-Hyperchromasia of enlarged nuclei -Atypical enlarged multipolar mitoses |
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What gene mutation is associated with anaplasia?
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p53
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What is considered diffuse anaplasia?
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>10 per high power field
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What is the prognosis for patients with WT?
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Good - 90% have long term survival with combined surgery chemo and radiation
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What is associated with the increased survival in WT patients?
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Increased risk of 2nd malignancy
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Flowers!
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yay
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