Pediatrics - Oncology & Neurology (Kaplan Chapters 20 & 21)

Front 1

What is the most common malignancies in children?

Back 1

Leukemias

Front 2

What is the most common malignancy in children?

Back 2

Acute Lymphoblastic Leukemia
(77% of all childhood leukemias)

Front 3

Initial, non-specific symptomes of ALL?

Back 3

Fever, anorexia, fatigue

Bone & Joint pain

---> especially in lower extremities

Front 4

What are the signs of bone marrow failure, seen later in ALL?

Back 4

Pallor
Bruising

Epistaxis (nose bleed)
Petechiae/Purpura

Mucous Membrane Bleeding

Lymphadenopathy
Hepatosplenomegaly

Joint Swelling

Hint 4

Purpura = "Condition of red or purple discolored spots on the skin that do not blanch on applying pressure. Spots are caused by bleeding underneath the skin usually secondary to vasculitis or scurvy" - Wikipedia

Front 5

Advanced clinical signs of ALL (exclusively severe signs of Bone Marrow failure) ?

Back 5

1. Petechiae

2. Lymphadenopathy

3. Hepatosplenomegaly

4. Joint Swelling

Front 6

What will you find on a peripheral blood smear in ALL?

Is this the definitive diagnosis?

Back 6

Peripheral blood smear will show -->

1. Anemia

2. Thrombocytopenia

3. Leukemic cells NOT often seen

4. Most WBC <10,000/mm3

---> No.

Front 7

The definitive diagnostic finding for ALL on a peripheral blood test is?

Back 7

Lymphoblasts

Front 8

The treatment for ALL is devided into three important phases.

Describe

Back 8

1. Remission induction with chemotherapy

2. CNS Tx (Intrathecal + intensive systemic chemotherapy)

3. Maintenance phase --> 2-3 yrs

Front 9

How is the chemotherapy administered in ALL?

Is radiotherapy used in ALL?

Back 9

Intrathecally (into the spinal chord)

No.

Front 10

Relapses of ALL consist mainly of?

Back 10

1. CNS (increased ICP or isolated cranial nerve palsies)

2. Testicular relapse in 1-2% of boys

Front 11

Patients with ALL will be at increased risk of infections. Which infection in particular?

Back 11

Pneumocystis carinii pneumonia

Front 12

Tumor lysis syndrome, in case of starting therapy in a patient with a large tumor cell load. Many cells will be dying simultaneously, causing which disturbances?

Back 12

1. Hyperuricemia

2. Electrolyte imbalance --> K, P, Ca

3. Renal failure

Front 13

Tx of Tumor Lysis Syndrome?

Back 13

1. Good hydration

2. Alkalinize urine

3. Prevent uric acid formation (Allopurinol)

Front 14

Prognosis of ALL?

Factors predisposing to worse prognosis?

Back 14

>80% 5-yrs survival

1. Age <1 or >10 yrs at Dx

2. >100,000/mm3 WBC

3. Slow response to initial Tx

4. Chromosomal anomalies

Front 15

The two age peaks of Hodgkin Disease are?

Back 15

15-19 yrs !!

> 50 yrs

Front 16

The characteristic pathologic finding in Hodgkin Disease is which type of cell?

Does immunideficiency predispose to Hodgkin Disease?

Back 16

Reed-Sternberg cell
--> Large cell with multiple or multilobulated nuclei

Yes.

Front 17

EBV is associated with both Hodgkin Disease and non-Hodgkin.

Which of these has the higher association?

Back 17

Non-Hodgkin

Front 18

Clinical presentation of Hodgkin Disease?

What is the most common presenting sign?

Back 18

!Clinical - depends on location!

1. Painless, firm cervical or supraclavicular nodes (most common presenting sign)

2. Anterior mediastinal mass

3. Night sweats, fever, weight loss, lethargy, anorexia, pruritus

Front 19

What is the most common presenting sign in Hodgkin Disease?

Back 19

Painless, firm cervical or supraclavicular nodes

Front 20

What is the best way to Dx suspected Hodgkin Disease?

Back 20

Excisional node biopsy of affected lymph node

"When in doubt, take it out"

Front 21

What are the Staging-Non-Invasive tests done for Hodgkin Disease?

Back 21

1. CXR (look for metastasis)

2. CBC

3. ESR, ferritin, copper

4. CT scan of chest/abdomen

5. Bone marrow if stage III/IV

Front 22

Staging of Hodgkin Disease:

Stage I - IV

Name the stages

Back 22

I --> Single node or site

II --> Two or more; same side of diaphragm

III --> Both sides of diaphragm

IV --> Diffuse

Front 23

Tx of Hodgkin Disease consists of?

Back 23

Chemotherapy and Radiation

Front 24

Define Non-Hodgkin Lymphoma (NHL)

Back 24

"Malignant proliferation of lymphocytes of T, B, or intermediate cell origin" - Kaplan

Front 25

EBV has a major role in which type of Non-Hodgkin Lymphoma?

Geographically, where is this mostly found?

Back 25

Burkitt lymphoma

Equatorial Africa

Front 26

Does immunodeficiency predispose to Hodgkin Disease or Non-Hodgkin Lymphoma (NHL)?

Back 26

Both

Front 27

What are the 3 subtypes of NHL?

Back 27

1. Lymphoblastic -usually T cells

2. Small, noncleaved cell lymphoma - B cell

3. Large cell - T, B or intermediate

Front 28

Children with an Anterior mediastinal mass causing respiratory symptomes, or an abdominal mass causing pain should always be considered for?

Back 28

Hodgkin Disease or NHL.

Do CXR

Front 29

What is the Dx of NHL?

Does NHL present as a fast- or slow-growing tumor?

Back 29

Biopsy of the mass in question

Fast! Aggressive disease!

Front 30

St. Jude staging system for NHL defining tumor extent.

Stages I - IV

Back 30

I --> Localized disease

II --> Regional, w/o mediastinal

III --> Mediastinal/extensive disease

IV --> Disseminated; CNS and/or bone marrow

Front 31

Main Tx of NHL?

Secondary, supportive Tx?

Back 31

Surgical excision of the tumor(s)

Chemo/Radio

Front 32

Prognosis of NHL for stages I & II?

Back 32

90% cure rate (disease free <5 yrs)

Front 33

What are the most common solid tumors in children?

Back 33

Brain tumors

---> 2nd most frequent malignancy in children (after leukemia)

Front 34

What is the mortality in case of brain tumors in children?

Back 34

45 %

Front 35

Brain tumors are most common under what age?

Back 35

<7 yrs

Front 36

What specific location of Brain tumors is most common in children?

Back 36

Infratentorial (2/3)

Front 37

Best test for Dx of suspected Brain tumor?

Back 37

Head CT scan

Front 38

Brain tumors in children <1 yrs of age
--> most common location?

--> 2 most common tumor types?

Back 38

Supratentorial

Choroid plexus tumors & Teratomas

Front 39

Brain tumors in children 2-10 yrs of age

--> most common location?

--> 2 most common tumor types?

Back 39

Infratentorial

Juvenile pilocytic astrocytoma & Medulloblastoma (supratentorial)

Front 40

Brain tumors in children >10 yrs of age

---> most common location?

---> Most common tumor type?

Back 40

Supratentorial

Diffuse astrocytoma (most common)

Front 41

What is the most common type of Infratentorial Brain Tumor in children?

Back 41

Juvenile Pilocytic Astrocytoma

Front 42

What is the most common site for a Juvenile Pilocytic Astrocytoma?

Back 42

Cerebellum

Front 43

A child wakes up in the morning, vomits, then feels fine for the rest of the day. Next morning, repeat.

Which test should be used to check for pathology in this child?

Back 43

Head CT for brain tumor blocking the flow of CSF

Front 44

What is the most common embryonal tumor (90%)?

Back 44

Medulloblastoma (2nd most common brain tumor)

Front 45

Most common location and age group/sex of Medulloblastomas?

Back 45

Midline Cerebellar Vermis

Males age 5-7

Front 46

Medulloblastoma, with their common location, may present with which two typical symptomes in patients?

Back 46

1. Wide spaced gait

2. Ataxia

Front 47

Which common brain tumor may block the 4th ventricle and cause hydrocephalus?

Back 47

Medulloblastoma

Front 48

Prognosis and Tx for Medulloblastoma?

Back 48

60-70% survival

Chemo/radio

Front 49

3rd most common brain tumors in children?

Back 49

Brainstem tumors

Front 50

A patient presenting with:
Motor weakness, CN deficits, Cerebellar deficits (gait), signs of increased ICP
---> may be suffering from which type of tumor?

Back 50

Brainstem tumor

Front 51

Brainstem tumors prognosis?

Tx?

Back 51

Poor.

Radiation --> mean survival 12 months

Palliative chemotherapy

Front 52

What is the most common Supratentorial tumor?

Back 52

Craniopharyngioma

Front 53

The morbidity in this tumor is

1. Short stature/Growth failure

2. Visual disturbances/Visual loss

3. Panhypopituitarism

--> which type of Brain tumor?

Back 53

Craniopharyngioma

Front 54

Tx of Craniopharyngioma

What is not used?

Back 54

Surgery and radiation

No chemo

Front 55

Patients with neurofibromatosis will be at increased risk of acquiring?

Back 55

Optic Nerve Glioma

Front 56

Symptomes of patients with Optic Nerve Glioma include?

Back 56

1. Unilateral vision loss/Optic atrophy

2. Proptosis

3. Eye deviation

4. Nystagmus

5. Strabismus

Front 57

First Tx of Optic Nerve Glioma?

Rule for when to do surgery in case of Optic Nerve Glioma?

Back 57

First --> Observation

Surgery when the patient starts to get visual problems

Front 58

Nephroblastoma, also called ______________ is usual in which age group?

Back 58

Wilms Tumor

Usual in age 2-5 yrs

Front 59

What is the 2nd most common malignant abdominal tumor?

This tumor is most commonly uni- or bilateral?

Back 59

Wilms Tumor/Nephroblastoma

Unilateral (bilateral in 7%)

Front 60

What are the two most common clinical associations with Wilms Tumor/Nephroblastoma?

Back 60

1. Hemihypertrophy

2. Aniridia

Front 61

What is the best inital test when suspecting a Wilms Tumor?

Back 61

USG

Front 62

What is the confirmatory test used when suspecting a Wilms Tumor?

Which screening is also useful with this diagnosis?

Back 62

Abdominal CT

CXR to look for lung involvement

Front 63

Tx of Wilms Tumor?
1st, then 2nd?

Back 63

1st --> Surgery

2nd --> Chemo/Radio

Front 64

The Wilms Tumor is most commonly an asyptomatic abdominal tumor.
If it turns symptomatic, which symptomes may the patient experience?

Back 64

1. HTN 2ndary to renal ischemia

2. Hematuria

3. Abdominal pain, vomiting

Front 65

Staging of Wilms Tumor

Stage I - V

Back 65

I --> Confined to the kidney

II --> Kidney, but capsule is penetrated

III --> Postsurgical residual nonhematogenous extension, confined to abdomen

IV --> Hematogenous metastasis --> lungs + liver

V --> bilateral renal

Front 66

Tx of bilateral Wilms Tumor?

Back 66

Unilateral nephrectomy and contralateral partial (plus chemo/radio)

Front 67

Prognosis of Wilms Tumor?

Back 67

4 yrs: 54-97%

Front 68

Where can the Neuroblastoma occur?

Where does it most commonly occur?

Back 68

Trick question --> It can occur anywhere!

Most commonly occurs in the abdomen (from Adrenal Gland)

Front 69

Which type of cells do Neuroblastomas arise from?

Back 69

Neural Crest Cells

Front 70

What is the typical age for getting a Neuroblastoma?

Median age is given

Back 70

90% <5 yrs

Median age 2 yrs

Front 71

What is the most frequent cancer of neonates?

Back 71

Neuroblastoma

Front 72

Which cancer may resolve on its own during the first year of life?

Back 72

Neuroblastoma

Front 73

What is the DDx of "Dancing eyes and dancing feet" ?

Back 73

1. Neuroblastoma

2. Horner syndrome

Front 74

DDx of a firm, palpable abdominal mass in a child?

Back 74

1. Nephroblastoma/Wilms Tumor

2. Neuroblastoma
--> May get calcification and hemorrhage

Front 75

Most of the neuroblastomas present in the abdomen from either Adrenal Glands or Retroperitoneal sympathetic Ganglia

30% of Neuroblastomas present elsewhere. Which 3 other places are common?

Back 75

1. Cervical

2. Thoracic

3. Pelvic ganglia

Front 76

Common sites of metastasis for Neuroblastoma?

Back 76

1. Long bones and skull

2. Bone marrow

3. Liver

4. Lymph nodes

5. Skin

Front 77

Dx of suspected Neuroblastoma?

Back 77

CT scan of the entire body + MRI

Front 78

Opsomyoclonus is a typical neurologic sign associated with which kind of tumor?

Back 78

Neuroblastoma

Front 79

In 95% of cases, a Neuroblastoma will present with increased values of which two tumor markers in a patient's urine?

Back 79

HVA (homovanillic acid)

&

VMA (Vanillylmandelic acid)

Front 80

Neuroblasts seen in a bone marrow aspiration will point to Dx of which tumor?

Back 80

Neuroblastoma

Front 81

Staging of Neuroblastoma

Stage I - IV

Back 81

I --> Confined to organ of origin

II --> beyond organ, but not across midline

III --> beyond midline

IV --> disseminated

Front 82

Tx of Neuroblastoma


Last resort?

Back 82

1. Surgery

2. Chemo/Radio

Last --> Bone marrow or stem cell transplant

Front 83

Pheochromocytoma secretes which type of compound?

Most common location, specifically?

Back 83

Catecholamines

Adrenal medulla

Front 84

Although the Pheochromocytoma is most commonly located in the Adrenal Medulla, it can also be located?

Back 84

Anywhere along abdominal sympathetic chain, i.e. bladder, urethral walls, thorax, cervix

Front 85

Pheochromocytoma
--> Typical Age?
--> Right/Left?

--> Bilateral?

--> Inheritance?

Back 85

6-14 yrs

Right more common

20% Bilateral

Autosomal Dominant

Front 86

Pheochromocytoma
Associations with other diseases/conditions?

Back 86

Neurofibromatosis

MEN-2A & MEN-2B

Tuberous Sclerosis
Sturge-Weber

Front 87

A child of 7 years presents with sustained HTN, palpitations, headache and abdominal pain.

On CT, an abdominal mass is found.
Likely diagnosis?

Back 87

Pheochromocytoma

Front 88

Retinal exam in children with Pheochromocytoma may reveal which 3 findings?

Back 88

1. Papilledema

2. Hemorrhages

3. Exudate

Front 89

What is the one maker to look for the urine of children when suspecting Pheochromocytoma?

Back 89

VMA (Vanillylmandelic Acid)

Front 90

Differences of Pheochromocytoma and Neuroblastoma?
--> HTN?

--> Metabolites?

Back 90

HTN in Pheo, not Neuro

Metabolites
Neuro --> dopmaine and HVA

Pheo ---> norepinephrine and VMA

Front 91

Dx method used when suspecting Pheochromocytoma?

Back 91

CT scan

Front 92

Tx of Pheochromocytoma is removal of the tumor. What has to be administered before starting the procedure?

Back 92

Preoperative alpha and beta blockade + fluid administration to counter-act any sudden catecholamin release during the procedure

Front 93

The mildest form of Neural Tube Defect is?

Back 93

Spina Bifida Occulta

Front 94

The three terms describing bleeding into the skin (differentiated by size) are?

Back 94

Petechia (<3mm)

Purpura (3 - 10 mm)

Echhymosis (>1cm)

Front 95

A patient with a Tethered Cord
Best Dx test?
Tx?

Back 95

MRI

Surgery

Front 96

Define Tethered Cord

Back 96

"Ropelike filum terminale persists and anchors the conus below L2" -- Kaplan

Front 97

Tethered Cord is also known by another name (?)

Back 97

Occult Spinal Dysraphism

Front 98

In Occult Spinal Dysraphism, what symptom can be visible, suggesting the Dx?

Back 98

Midline Skin Lesion

Front 99

Tethered Cord patients will need
Definite Dx?
Tx?

Back 99

MRI

Surgery

Front 100

Define Menigocele

Back 100

"Meninges herniate through defect in posterior vertebral arches" -- Kaplan

Front 101

Is a "Dermal Sinus Tract" dangerous pathologically?

Back 101

Depends,

--> Asymptomatic non-hollow pits are harmless

--> Actual tracts connecting with spinal cord can become infected or produce neurologic deficits

Front 102

Meningocele
In case of a fluctuant midline mass with thin skin protruding through a dermal sinus tract, what should be done clinically (Dx & Tx)?

Back 102

1. MRI to assess extent of involvement

2. Head CT (to check for hydrocephalus)

3. Surgery (to close the defect)

Front 103

Maternal periconceptional use of _____________ reduces risk of Myelomeningocele by half

Back 103

Folate

Front 104

Myelomeningocele may occur anywhere along the neural tube, but mostly occurs in which area?

Back 104

The lumbosacral area

Front 105

Myelomeningocele
Low Sacral Lesion will present with which clinical features?

Back 105

"Bowel and bladder incontinence and perineal anesthesia without motor impairment" -- Kaplan

Front 106

Which known defect of the hindbrain usually accompanies a Myelomeningocele?

Back 106

Arnold-Chiari Malformation
type I

Front 107

Myelomeningocele
Midlumbar lesion will present with which clinical features?

Back 107

"Flaccid paralysis below the level of the lesion is most common; no deep tendon reflexes (DTRs), no response to touch and pain.
Urinary dribbling, relaxed anal sphincter"

Front 108

What will a patient with Myelomeningocele require for
Dx?

Back 108

MRI & CT scan (looking for hydrocephalus and extent of lesion, or to rule out other pathology)

Front 109

Tx of Myelomeningocele?

Back 109

Ventriculoperitoneal shunt and correction of defect

Front 110

What sort of bladder problems will patients with Myelomenigocele have, and what will this predispose for?

Back 110

Neurogenic bladder, a bladder that doesn't empty normally, causing stasis

----> UTI

Front 111

A patient with Myelomeningocele is a a higher risk of developing which particular allergy?

Back 111

Latex (due to repeated exposure during surgery)

Front 112

Hydrocephalus occurs due to?

Back 112

Impaired circulation and absorption of CSF

or

Increased CSF prod. from choroid plexus papilloma

Front 113

Hydrocephalus is apparent clinically how?

Back 113

A 2-montyh of infant is noted to have a head circumference greater than the 95th percentile

Front 114

Two types of Hydrocephalus?

Back 114

Obstructive (noncommunicative) &

Nonobstructive (communicative)

Front 115

The clinical presentation of Hydrocephalus depends on?

Back 115

The rate of rise of ICP

Front 116

Clinical presentation of Hydrocephalus in infants?

Back 116

Bulging anterior fontanel

Distended Scalp veins

"Setting Sun"- sign

Spasticity, clonus

Front 117

Define "Setting Sun"-sign

Back 117

Up-gaze paresis, pupils look to be forced downward. Lower lid may cover the pupil

Front 118

Clinical presentation of Hydrocephalus in older children

Back 118

Irritability, Lethargy

Vomiting

Headache

Papilledema

Sixth nerve palsy

Front 119

Tx for all types of hydrocephalus?

Back 119

Shunting, ventriculoperitoneal hunt

Front 120

Dandy-Walker malformation is a form of Hydrocephalus; define it

Back 120

Cystic expansion of the 4th ventricle due to absence of roof

Front 121

Impairment of the arachnoid villi may cause hydrocephalus. Which conditions may cause impairment of the arachnoid villi?

Back 121

Meningitis or an interventricular hemorrhage

Front 122

When would you suspect Epilepsy as a cause of seizures in a child?

Back 122

When at least 2 unprovoked seizures occur >24 hours apart

Front 123

What is the age occurence and peak of Febrile Seizures?

Back 123

--> 9 months to 5 years

Peak --> 14-18 months

Front 124

Define Postictal State

Back 124

Altered state of consciousness after an epileptic seizure last between 5 and 30 minutes

Front 125

Describe a typical Febrile Seizure

Back 125

Generalized tonic-clonic seizure, <10-15 minutes; brief or non-occuring postictal period
No more seizures after this.

Front 126

Describe an atypical Febrile Seizure

Back 126

<15 minutes, more than one in a day + focal findings

Front 127

Describe the Simple Partial Seizure

Back 127

1. Focal, most of face, neck and extremities. 10-20 seconds

2. May have aura

3. No postictal period

Front 128

Tx of Simple Partial Seizure?

Back 128

Phenytoin and other Anticonvulsants

Front 129

Describe complex partial seizures

Back 129

1. Impaired consciousness at some point

2. Automatism (unconscious actions like lip smacking)

Front 130

MRI in Complex Partial Seizures will show?

Back 130

Abnormalities in Temporal Lobe
(Sclerosis, hamartoma, cyst, infarction)

Front 131

Medication used to Tx Complex Partial Seizure?

Back 131

Carbamazepine

Front 132

Generalized Seizures
Describe the Absence (petit mal) seizure

Back 132

Sudden cessation of motor activity or speech with blank stare and flickering eyes

---> No aura

Front 133

Absence (petit mal) Seizure

Common age and sex?

Back 133

Uncommon <5 yrs

More common in girls

Front 134

Tx of Absence Seizure?

Back 134

Ethosuxamide (DoC)

Valproate (2nd line)

Front 135

Generalized Seizures

Describe Tonic-Clonic Seizures

Back 135

1. Aura - focal onset; may indicate site of pathology

2. Loss of Consciousness (LOC)
--> eyes roll back, tonic contractions, apnea

Front 136

Generalized Seizures
Describe the Postictal phase in case of Tonic-Clonic Seizures

Back 136

Semicomatose up to 2 hours with vomiting and bilateral frontal headache

Front 137

Generalized Seizures
Tx for Tonic-Clonic Seizures

Back 137

Valproate, Phenobarbital, Carbamazepine

Front 138

Describe Myoclonic Seizures

Back 138

Repetitive brief symmetric muscle contraction and loss of body tone; falling forward
--> "Jackknife seizure"

Front 139

Describe Infatile Spasms

Back 139

"Symmetric contractions of neck, trunk and extremities, at times with extension episodes" --Kaplan
---> May have multiple episodes (100s)
--> Looks like moro response

Front 140

Typical age group for Infantile Spasms?

Back 140

4-8 months

Front 141

Typical EEG finding of Infantile Spasms?

Back 141

Hypsarrhythmia

Front 142

Infantile Spasms
Biochemical abnormality?

Tx?

Back 142

Increased corticotropin releasing factor

---> Neuronal hyperexcitability

Tx --> ACTH

Front 143

Describe the presentation of Neonatal Seizures

Back 143

Chewing
Excess salivation

Apnea

Blinking

Pedaling movements

Nystagmus

Front 144

Common etiologies of Neonatal Seizures?

Back 144

1. Hypoxic ischemic encephalopathy

2. CNS infection or hemorrhage

3. Structural abnormalities

4. Inborn errors of metabolism

5. Drug withdrawal

Front 145

Tx of Neonatal Seizures?

Back 145

Lorazepam & Phenobarbital

Front 146

Neurofibromatosis
Mode of inheritance?

Back 146

Autosomal Dominant

Front 147

Neurofibromatosis type 1 (NF-1) is known by another name. Which?
Chromosome?

Back 147

von Recklinghausen

17q11.2

--> more common than NF-2

Front 148

Neurofibromatosis type 2 (NF-2) is on which chromosome?

Back 148

Chromosome 22

Front 149

NF-1 is a clinical diagnosis, two of the following criteria are needed
-->

Back 149

1. >/6 cafe-au-lait spots (5 mm prepubertal, 15 mm postpubertal)

2. Axillary/inguinal freckling

3. >2 iris Lisch nodules

4. >2 neurofibromas, or 1 plexiform neurofibroma

5. Osseous lesion -- scoliosis, sphenoid dysplasia

6. Optic glioma

Front 150

What are the CNS complications of Neurofibromatosis?

Back 150

1. Low-grade gliomas, hamartomas

2. Malignant neoplasms

3. Cerebral vessel abnormalities

4. Seizures

5. Cognitive defects

Front 151

What are the renal compliations of Neurofibromatosis?

Back 151

1. Renovascular HTN

2. Pheochromocytoma

Front 152

Dx of Myasthenia Gravis?

Back 152

Give Edrophonium or Neostigmine and see the child's fatiguability fade away. They become very happy, and then very sad