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Pathology F2014 > Pediatric Pathology > Flashcards

Pediatric Pathology Flashcards

1
Q

Causes of Death by Age

A

Under 1 year

  • Congenital malformations, deformations, and chromosomal anomalies
  • Disorders related to short gestation and low birth weight
  • SIDS
  • Maternal complications of pregnancy

1 to 4 years

  • Accidents (unintentional Injuries)
  • Congenital anomalies
  • Assault (homicide)
  • Malignant neoplasms
  • Heart diseases

5 to 9 years

  • Accidents (unintentional Injuries)
  • Malignant neoplasms
  • Congenital anomalies
  • Assault (Homicide)
  • Diseases of the heart

10 to 14 years

  • Accidents (unintentional injuries)
  • Malignant neoplasms
  • Assault (homicide)
  • Intentional self-harm (suicide)
  • Congenital anomalies.
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2
Q

22q11.2 Deletion Syndrome

A

Velocardiofacial Syndrome
– Congenital heart disease (outflow tracts)

– Palatal abnormalities/Facial dysmorphism

– Developmental delay

Di George Syndrome (?TBX1 gene loss)

– Thymic hypoplasia: impaired T-cell immunity

– Parathyroid hypoplasia: hypocalcemia

Psychoses in both syndromes (Schizophrenia/Bipolar disorder)

Diagnosis by FISH

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3
Q

KLINEFELTER‟S SYNDROME

A

Male hypogonadism in the presence of at least two X chromosomes and one or more Y chromosomes (XXY,XXXY etc.)

– Testicular atrophy: Sterility

– Eunuchoid with: Gynecomastia & Reduced facial, body and pubic hair.

(Prone to breast cancer,extragonadal germ cell tumours and autoimmune diseases.)

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4
Q

Down’s syndrome

A

Trisomy 21 leading to mutations in:

  • DYRK1A: ser-thr kinase
  • RCAN1: calcineurin

Sx: simian crease (double crease in palms)

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5
Q

Turner’s

A

mutations in homeobox gene SHOX: Xp22.33 -> maybe accounting for short stature

Sx:

  • coarctation
  • bicuspid aorta valve
  • horseshoe kidney
  • fibrous streak ovaries
  • ammenorrhea
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6
Q

Developmental abnormalities Environmental Etiology

A

Maternal/Placental Infections

  • Rubella
  • Toxoplasmosis
  • Syphilis
  • CMV
  • HIV

Maternal diseases

  • Diabetic embryopathy: leading to organomegaly and high birth wt
  • Phenylketonuria

Drug/Chemicals: Thalidomide, anticonvulsants, 13cis-retinoic acid, warfarin, alcohol

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7
Q

Effects of teratogens

A
  • Retinoic acid (acne) causes CNS, cardiac and craniofacial abnormalities including cleft lip and palate. May interfere with TGFβ (palatogenesis); Mice with TGFβ3 gene knockout have cleft palate.
  • Sonic Hedgehog Gene defect causes holoprosencephaly
  • Cyclopamine causes holoprosencephaly and cyclopia in sheep.
  • Homeobox Gene defect causes limb, vertebral and craniofacial abnormalities.
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8
Q

Deformation

A
  • **Uterine constraint – most common cause **

Maternal factors

  • First pregnancy
  • Small uterus
  • Leiomyomas

Fetal factors

  • Multiple fetuses
  • Oligohydramnios
  • Abnormal presentation
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9
Q

Potter sequence

A

Oligohydramnios (= lack of amniotic fluid) due to:

  • Renal agenesis/ maldevelopment
  • Amniotic fluid leak
  • Uteroplacental insufficiency

Oligohydramnios causes:

  • Pulmonary hypoplasia
  • Amnion nodosum
  • Fetal compression
    • – Worsens pulmonary hypoplasia
    • – Breech presentation
    • – Altered facies
    • – Positioning defects of feet and hands
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10
Q

Malformation Syndromes

A

The presence of >1 developmental anomalies of >2 systems due to a common etiology

With 2 exceptions, none of these disorders occurs more frequently than 1:3,000 births

  • Down syndrome - 1:660
  • XXY syndrome - 1:500 males
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11
Q

Growth Chart

A
  • Appropriate for gestational age (= AGA): Birth weight between 10th and 90th percentile for GA.
  • Small for gestational age (= SGA): Birth weight below 10th percentile for GA.
  • Large for gestational age (= LGA): Birth weight above 90th percentile for GA.
  • Premature:
  • Low-birth weight (LBW) infants:
    • Premature
    • IUGR for their gestational age i.e. SGA.
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12
Q

Major risk factors for Prematurity

A
  • Premature rupture of membranes
  • Intrauterine infections
  • Structural abnormalities of the uterus, cervix, placenta
  • Multiple gestation
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13
Q

Apgar score

A
  • Evaluated at 1 and 5 minutes.
    • – The 1-minute score determines how well the baby tolerated the birthing process.
    • – The 5-minute score assesses how well the newborn is adapting to the new environment.
    • Maximum score = 10.
  • Correlation between score and mortality during first 28 days of life is very good:
  • – Mortality = 0 when score (5 min) >7
  • – Mortality = 20% when score (5 min) = 4
  • – Mortality = 50% when score (5 min) = 0-1
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14
Q

Complications of Prematurity

A
  • Hyaline membrane disease ( RDS)
  • Necrotizing Enterocolitis
  • Intraventricular and germinal matrix hemorrhage
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15
Q

RDS of new born

A
  • aka Hyaline Membrane Disease
  • Formation of “membranes” in the peripheral airspaces.

Pathogenesis

  • Prematurity
  • Perinatal asphyxia
  • Maternal diabetes: will suppress surfactant production
  • Cesarean section before onset of labor
  • Twin gestation
  • Male sex
  • *Xray:**
  • ground glass appearance
  • bell-shaped thorax

Gross morphology

  • Normal size
  • Solid, airless, and reddish purple - resemble liver

Microscopic morphology

  • **Death within first several hours: **Necrotic cellular debris in terminal bronchioles and alveolar ducts
  • Death after 12 to 24 h:
    • Smooth homogenous pink membranes lining terminal and respiratory bronchioles and alveolar duct
    • Membranes are composed of necrotic alveolar type II pneumocytes and fibrin; neutrophilic inflammatory reaction is not associated with these membranes
  • **Death after several days: **evidence of reparative changes, including proliferation of type II pneumocytes and interstitial fibrosis, is seen.

Rx

  • Ventilatory support - oxygen
  • Surfactant replacement therapy

C&C

  • Retrolental fibroplasia (retinopathy of prematurity) fromoxygen toxicity & VEGF
  • Bronchopulmonary dysplasia
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16
Q

Brochopulmonary dysplasia

A
  • Decrease in alveolar septation resulting in large, simplified alveolar structures and a dysmorphic capillary configuration.
  • Most likely caused by an arrested development of alveolar septation at the so-called saccular stage of development.

Contributing Factors

  • Hyperoxemia
  • Hyperventilation
  • Prematurity
  • Inflammatory cytokines (IL1β, IL6 and IL8)
  • Vascular maldevelopment
  • Prevent with gentler ventilation, glucocorticoids and prophylactic surfactant

Gross & microscopic morphology

  • Hyperplasia and squamous metaplasia of bronchial epithelium
  • Peribronchialfibrosis
  • Fibrotic obliterationof bronchioles
  • Overdistended alveoli.
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17
Q

Neonatal Necrotizing Enterocolitis (NEC)

A
  • Disease of premature infants along with term infants of low birth weights (small for gestational age)
  • Mortality of 25-50%
  • Predisposing factors
    • Intestinal ischemia
    • bacterial colonization of the gut
    • administration of formula feeds

**Sx: **oral feeding and develops vomiting, blood tinged diarrhea and a distended abdomen.

  • Preterm or SGA infant with history of asphyxia requiring ventilation develops signs of obstruction after oral feedings have begun
  • Abdominal distension, bloody stools, shock, DIC progressing to death.

Dx

  • Abdominal radiographs show dilated loops of bowel
  • pneumatosis intestinalis: radiological sign

Gross morphology

  • Typically involves terminal ileum, caecum and right colon
  • bowel distended with thin and delicate wall showing spotty areas of necrosis and possible perforation.

Microscopic morphology

  • Mucosal coagulative necrosis extending into and often through the submucosa and muscular layers
  • Small air filled spaces beneath mucosa – pneumatosis intestinalis

C&C

  • Early – sepsis, shock, acute tubular necrosis, DIC, intestinal perforation
  • Delayed – short gut syndrome, malabsorption, strictures.
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18
Q

Neonatal Intraventricular hemorrhage

A
  • Bleeding into the germinal matrix with extension into ventricles and beyond
  • Germinal Matrix –source of nerve cells in embryo and fetuses (up to 33 weeks of gestation.)
  • Richly vascular area with many thin walled capillaries that are very sensitive to anoxia

Grading

I– Hemorrhage restricted to germinal matrix.

II – Intraventricular extension of hemorrhage without dilatation.

III - Intraventricular extension of hemorrhage with dilatation.

IV – Intraventricular and Intraparenchymal hemorrhage

C&C

  • Rapid death if massive hemorrhage with tears of falx cerebri or tentorium
  • In long term survivors – cavitations or pseudocysts surrounded by hemosiderin laden macrophages and gliosis.
  • Hydrocephalus seen in 10-15% of survivors
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19
Q

Pathological Jaundice

A

Any of the following features characterize pathological jaundice

  • Clinical jaundice appearing in first 24 hours
  • Total bilirubin > 15 mg/dl ( Hyperbilirubinemia)
  • Conjugated bilirubin > 2 mg/dl
20
Q

Pathological Jaundice: 2 main types

A

Unconjugated Hyperbilirubinemia

  • HDN
  • Crigler-Najjar syndrome type I & II

Conjugated Hyperbilirubinemia

  • Biliary atresia
  • Neonatal hepatitis
21
Q

HDN types

A

Determined by the extent of the hemolytic disease.

  • Death in Utero – most extreme form of disease
  • Hydrops Fetalis – most severe form in live born infants.
  • Kernicturus – bilirubin encephalopathy
22
Q

Hydrops fetalis

A

Autopsy: hepatosplenomegaly and bile stained organs

Microscopically:

– Erythroblastic hyperplasia of the bone marrow

– Extramedullary hematopoiesis in the liver, spleen.

23
Q

Kernicterus

A
  • A disorder of newborns in which very high levels of unconjugated bilirubin bind to, and injure the immature neurons of the CNS
  • Characterized by bile staining of the brain, particularly of the basal ganglia and brain stem.

Dx:

  • Amniocentesis – high levels of bilirubin
  • Human antiglobulin test (Coombs test) – Positive on fetal cord blood

Rx:

  • Exchange transfusion
  • Phototherapy

Prevention: Rhogam aka Human anti D globulin within 72 hours of delivery

24
Q

Crigler-Najjar Disease

A

Type I: AR

  • Characterized by complete absence of UDP-glucuronyltransferase activity.
  • Unremitting unconjugated hyperbilirubinemia leading to bilirubin encephalopathy.
  • Most patients die within first year of life

Type II: similar to but less severe than type I.

  • partial decrease in the activity of UDP-glucuronyltransferase activity.
  • Hepatocytes have the capacity to synthesize this enzyme on treatment with Phenobarbital.
25
Q

Biliary Atresia

A
  • Complete obstruction of the lumen of the extrahepatic biliary tree within the first three months of life.
  • Most frequent cause of chronic cholestasis in infants and children

**Clinical forms **

Embryonic or fetal type (20%)

  • – Due to aberrant intrauterine development of the extrahepatic biliary tree
  • – early onset neonatal cholestasis
  • – No jaundice free period after physiological jaundice
  • – Associated congenital anomalies (malrotation of abdominal viscera, congenital heart disease)

Perinatal type (80%)

  • – Normally developed biliary tree is destroyed following birth (virus induced injury to biliary epithelium)
  • – Late onset neonatal cholestasis (4-8 weeks)
  • – Jaundice free interval
  • – No associated congenital anomalies

Morphology

  • Inflammation and fibrosing stricture of the hepatic and common bile ducts
  • Periductal inflammation of intrahepatic bile ducts – progressive destruction of intrahepatic biliary tree
  • Features of extrahepatic biliary obstruction
    • – Marked bile ductular proliferation
    • – Portal tract edema and fibrosis
    • – Parenchymal cholestasis
26
Q

Idiopathic Neonatal Hepatitis

A

Etiology

  • Excludes known associated factors
    • Alpha-1 antitrypsin
    • Extrahepatic biliary atresia
    • Infectious agents
  • 50-60% of cases of neonatal hepatitis
  • Male : Female – 2:1

Microscopic morphology

  • Giant cell transformation: diffuse, prominent
  • Ballooning
  • Acidophilic degeneration
  • cholestasis
27
Q

SIDS

A

Morphological changes

  • Multiple petechiae (80%) in Thymus, visceral/parietal pleura and epicardium.
  • Lungs: Congestion +/- pulmonary edema
  • Hypoplasia of arcuate nucleus in the brain stem.
  • Decrease in brain stem neuronal populations in some cases.

Risk factors: Parental

  • Young maternal age (<20)
  • Smoking during pregnancy
  • Drugs in either parent
  • Late / no prenatal care
  • Low socioeconomic group
  • African American or American Indian ethnicity
  • Risk factors: Infant
  • Brain stem abnormality associated with defective arousal / cardiorespiratory control
  • Prematurity +/or low birth weight
  • Male sex
  • Product of multiple birth
  • SIDS in an earlier sibling
  • Antecedent respiratory infections
  • Germline polymorphisms in autonomic nervous system genes

Morphology

  • Multiple petechiae (80%) in Thymus,
  • visceral/parietal pleura and epicardium.
  • Lungs: Congestion +/- pulmonary edema
  • Hypoplasia of arcuate nucleus of medulla
  • Decrease in brain stem neuronal populations in some cases.
28
Q

Genetic/Metabolic defects of newborn

A

Rule out the following before DDx as SIDS:

  • Long QT syndrome (SCNSA + KCNQ1 mutations) (Na+ or K+ channel abnormalities.)
  • Fatty acid oxidation disorders (MCAD, LCHAD, SCHAD mutations) 5%
  • Histiocytoid cardiomyopathy (MTCYβ mutations)
  • Abnormal inflammatory responsiveness (Partial deletion of C4a and C4b)
29
Q

Fetal and perinatal infections

A

Hematogenous spread/Transplacental infection:

  • TORCH: Tox, Other, Rubella, CMV, HIV & Herpes
  • SLAVE: Syphilis, L. monocytogenes, Adenovirus, Varicella, Enterovirus
  • Parvovirus B19

Ascending infection via cervix: mostly bacterial and some viral resulting in

  • Chorioamnionitis
  • Funisitis
  • Pneumonia
  • Sepsis
  • Meningitis
  • Note: L. monocytogenes causes necrotizing amnionitis in late gestation

Sx:

SGA infants.

30
Q

Rubella Embryopathy: Typical manifestations

A

Ocular lesions:

  • Cataracts.
  • Corneal changes
  • Microphthalmia.

Cardiac lesions:

  • Patent ductus arteriosus.
  • Septal defects.
31
Q

Cystic Fibrosis

A
  • CFTR encoded on chr 7q31.2
  • Normal: PKA phosphorylates CFTR in apical membranes of eccrine glands.

Pathogenesis

With the absence of CFTR:

  • In the sweat gland: increased chloride and sodium concentration in sweat.
  • In the airway:
    • – Stimulated chloride secretion to the gland lumen does not occur.
    • – Increased sodium and water reabsorption leads to dehydration of the mucous layer.

Types of mutations

  • 3 base pair deletion @ F508 (70%)
  • remaining patients exhibit multiple (more than 800) different mutations.

Morphology

Lung

  • Plugging of submucosal tracheobronchial mucous glands and ducts.
  • Obstruction of bronchioles with mucus, associated with marked hyperplasia and hypertrophy of the mucus-secreting cells.
  • As a consequence of bronchiolar plugging, there is:
    • – Atelectasis.
    • – Emphysema due to destruction of parenchyma.
  • Infections result in:
    • – Chronic bronchitis.
    • – Bronchiectasis -> brochopneumonia
    • – Lung abscesses

Pancreas

  • same mucous obstruction of ducts accompanied by:
    • – Secondary dilatation and cystic changes of the distal ducts and atrophy of secretory cells.
    • – Fibrosis
    • – Destruction of parenchyma
  • These effects result in chronic pancreatitis in 85% of patients with CF.

Other organs

  • Liver - Focal biliary cirrhosis due to obstruction and bile duct hyperplasia.
  • Azoospermia and infertility in approximately 95% of males due to obstruction of the vas deferens and epididymis – atrophy & fibrosis
  • Meconium ileus in 15% of infants due to impaction of meconium in the terminal ileum with subsequent risk of perforation and peritonitis.

Sx

  • Discovered between age of 2-12 months.
  • chronic pulm infections
  • Child presents with symptoms of malabsorption secondary to pancreatic insufficiency:
  • – Foul-smelling steatorrhea.
  • – Malnutrition
    • Edema
    • Hypoalbuminemia
  • – Failure to thrive

Two clinical clues in children

  • Nasal polyps
  • **Rectal prolapse **
32
Q

PKU

A
  • AR Point mutation on 12q of Phenylalanine Hydroxylase (PAH) gene results in
    • – Hyperphenylalaninemia
    • – Formation of Phenylketones
  • Hyperphenylalaninemia causes irreversible brain damage
    • – Complete interference with amino acid transport system in brain
    • – Inhibiting the synthesis of neurotransmitters

**Sx: **Mousy odour, fair skin, blond hair and blue eyes.

33
Q

Galactosemia

A
  • AR deficiency of Galactose -1-phosphate uridyl transferase, the enzyme that catalyzes the conversion of galactose to glucose.
  • As a result galactose accumulates in the liver and other organs.

Sx:

  • Infants fed milk rapidly develop hepatosplenomegaly, jaundice and hypoglycemia.
  • Cataracts and Mental retardation
  • Cirrhosis may develop in just a few months.
  • Galactose-free diet reverses many of the morphologic changes.

Gross Morphology: extensive and uniform fat accumulation in liver and marked bile ductal proliferation, cholestasis and fibrosis.

34
Q

Dubin-Johnson syndrome

A
  • AR mutation characterized by chronic or intermittent jaundice and accompanied by a “black‟ liver.
  • Defective transport of conjugated bilirubin from hepatocytes to canalicular lumen
  • Associated defect in hepatic excretion of coproporyphrins

Microscopic: accumulation of coarse, iron free, dark brown granules in hepatocytes and Kupffer cells, aligning in shape of canaliculi.

EM: pigment is located in lysosomes and it appears to be composed of polymers of epinephrine metabolites, not bilirubin pigment

Sx:

  • Most patients are asymptomatic except for mild intermittent jaundice.
  • Serum bilirubin levels – 2-5 mg/dl
35
Q

Rotor Syndrome

A
  • Familial conjugated Hyperbilirubinemia
  • Etiology unknown.
  • defect in the excretion of conjugated bilirubin into the biliary canaliculi with the bilirubin being absorbed into the blood.

**Sx: **Characterized by jaundice, attacks of intermittent epigastric discomfort and occasionally abdominal pain, and fever.

**Microscpic: **low-grade pigment deposition, dissociation of liver cells, occasional necrotic foci, and fibrin precipitation.

36
Q

Pediatric neoplasms: Special Predisposing Factors

A

>200 genetic syndromes are associated with an increased susceptibility to cancer.

– Down syndrome (Trisomy 21) - Acute leukemia.

– Deletion 13q - Retinoblastoma.

– Wiskott Aldrich syndrome - Lymphoma.

– Agammaglobulinemia - Acute lymphoblastic leukemia (= ALL).

37
Q

“Small, Round, Blue Cell” Tumors

A
  • A name applied to many of the solid tumors of childhood because of their appearance.
  • This appearance reflects the “embryonal” origin of many of these tumors.

**Microscopic: **

  • Relatively homogeneous, densely “Small, Round, Blue Cells”
  • Despite histologic similarities, therapyand prognosis differ among these malignant neoplasms.
  • Clinicalinformation
  • Immunohistochemistry
  • Cytogenetics
  • Molecular genetics

Common types:

  • Lymphoma/Leukemia
  • Medulloblastoma(CNSneoplasm)
  • Neuroblastoma
  • Rhabdomyosarcoma (Sarcoma)
    • Embryonal
    • Alveolar
  • Wilms tumor
  • Bone tumors
    • – Ewings sarcoma/PNET & t(11q22)
    • – Small cell osteosarcoma
38
Q

Neuroblastoma

A
  • A poorly differentiated tumor arising from primitive neural crest cells that normally give rise to the adrenal medulla and sympathetic ganglia.
  • 7-10% of all childhood malignancies.
  • Second most common solid malignant tumor in children.
  • Most occur before 5 years of age.

Etiopath: Germline mutation in ALK gene – familial predisposition to neuroblastoma.

Sx:

  • < 2 years - Mass in abdomen, mediastinum, or other sites, fever, weight loss (systemic sx which other tumours may not induce)
  • Older children – Signs of metastatic disease – bone pain, respiratory or gastrointestinal symptoms.
  • Blueberry muffin baby – disseminated neuroblastoma – multiple cutaneous metastasis with deep blue discoloration of the skin

Gross

  • 40 % arise in adrenal medulla.
  • Remainder arise anywhere along the sympathetic chain
    • – paravertebral region of the abdomen (25%)
    • – posterior mediastinum (15%). .
  • Size varies from minute nodules to tumors weighing >1 kg in weight
  • With increasing size, areas of: – Necrosis.
    • – Hemorrhage.
    • – Cyst formation.
  • Gross calcification in 40-50%

Microscopic

  • Small, round, blue cell tumor with sheets of small, primitive-appearing cells with dark nuclei, scant cytoplasm, and poorly defined cell borders
    • Neuropil - a faintly eosinophilic fibrillary material that corresponds to neuritic processes of the primitive neuroblasts.
    • Homer-Wright Pseudo rosettes - the tumor cells are concentrically arranged about a central space filled with neuropil. No lumen.
    • Neurosecretory granules on electron microscopy.
    • for neuron-specific enolase and synaptophysin on immunohistochemistry.

Dx

  • Elevated blood levels of catecholamines
  • Elevated urine levels of catecholamine metabolites
  • X –ray abdomen – calcification in tumor can be picked up

C&C

Characteristically, these tumors metastasize early with spread to:

  • Bone
  • Lymph nodes
  • Liver
  • Bone marrow
  • Subcutaneous tissue

Prognostic factors

  • Younger -> better prognosis
  • Poor prognosis mutations
    • Deletion distal 1p and gain of distal 17q
    • N-myc amplification – extrachromosomal double minute chromatin bodies or homogenously staining regions (HSR) on other chromosomes
    • Telomerase overexpression
  • Good prognosis: Tyrosine kinase receptor A (Trk-A) – increased expression
39
Q

Wilms Tumor

A
  • Most common 1o renal tumour of childhood; aka nephroblastoma
  • Majority of cases are sporadic
  • 10% cases associated/risk factor with:
    • WAGR syndrome – deletion of WT1 gene
    • Denys-Drash syndrome –dominant, negative inactivating mutation of WT1 gene
    • Beckwith-Wiedemann syndrome – mutation of putative WT2 gene – overexpression of IGF2 protein
    • Mutations of β-catenin gene (10% of sporadic WT)
40
Q

Beckwith-Wiedemann Syndrome

A

Genomic imprinting disorder on putative WT-2 gene

  • Macroglossia
  • Omphalocele
  • Gigantism unilateral
  • Adrenal cortical cytomegaly
  • Visceromegaly
  • Islet cell hypertrophy
  • Renal medullary dysplasia -> immature renal cells and cartilage in kidney
41
Q

Denys-Drash Syndrome

A
  • Gonadal dysgenesis
  • Renal abnormalities
  • WT-1 gene mutation (11p13)
  • 90% chance of Wilms tumor
42
Q

Nephrogenic rests- precursor lesion to Wilms’ tumour

A

Seen in the renal parenchyma adjacent to approximately 40% of unilateral tumors and nearly 100% of bilateral tumors.

**Gross **

  • Majority are large, Solitary, well circumscribed masses
  • 10% bilateral and multicentric

Microscopic

  • Triphasic pattern: Embryonal tumor – recapitulates the normal development of the kidney.
  • Immature cells: glomeruli, tubules, etc…
  • Blastema – sheets of small round blue cells
  • Stroma – fibrocytic or myxoid in nature. May have striated muscle.
  • Epithelium - abortive tubules and glomeruli

**Anaplastic Subtype (5% of WT) **

Characterized by:

  • Cells with hyperchromatic nuclei, >3 times larger than those in adjacent cells of similar type.
  • Enlarged, bizarre, multipolar mitoses.

Correlates with acquired TP53 mutations

Sx

  • Abdominal pain (often detected when mom gives child a bath).
  • Hematuria.
  • Hypertension.
  • Acute abdominal crisis secondary to traumatic rupture -> severe hemorrhage
43
Q

Retinoblastoma sx

A
  • White pupil (= leukocoria).
  • Squint (= strabismus).
  • Poor vision.
  • Spontaneous hyphema (= hemorrhage into the anterior portion of the eye).
  • Red, painful eye.

Increased risk of:

  • Osteogenic sarcoma
  • Ewing sarcoma/PNET
  • Pinealoblastoma

**C&C: **Frequent complication is secondary glaucoma due to blockage of canal of Schlemm

44
Q

Sacrococcygeal teratoma prognosis

A
  • ≤ 4 months - ALL benign
  • At 1year 50% malignant
  • At 5years 100% malignant
45
Q

Neuroblastoma staging

A

Stage 1 – localized tumor with complete gross excision, Lymph nodes negative

Stage 2A - localized tumor with incomplete gross excision, ipsilateral, non-adherent Lymph nodes negative

Stage 2B – localized tumor with with or without complete gross excision, ipsilateral non-adherent Lymph nodes positive

Stage 3 - Unresectable unilateral tumor infiltrating across the midline with or without regional lymph node involvement, or localized unilateral tumor with contralateral regional lymph node involvement

Stage 4 – metastatic disease to the viscera, bone and/or distant lymph nodes

Stage 4S (S=Special) – Good prognosis Localized primary tumor (stages 1, 2A, or 2B) with dissemination limited to skin, liver, and/or bone marrow (<10% of nucleated marrow cells are constituted by neoplastic cells)

Pathology F2014 (17 decks)

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