Pediatric Oncology Flashcards
Acute Leukemias
- Myeloid or Lymphoid
- Most Pediatric Acute Leukemias have
NO clear cause
What differentiates the Two main types of Acute Leukemia?
- Epidemiology is different
- Diagnostic findings are Similar, a few important differences
Pathophysiology of ALL
Lymphoid Progenitor Cells
in the bone marrow become
malignant & begin to proliferate
uncontrolled
Pathophysiology of AML
Myeloid Progenitor Cells become malignant & proliferate uncontrolled
_____ = MOST COMMON childhood malignancy!
ALL
ALL epidemiology
- Incidence of ALL peaks at the age of 5 years (3-7)
■ ALL = MOST COMMON childhood malignancy! - Incidence ↓ after 5 yo, then ↑ after 50 yo
- 80% in kids
- 20% in adults
Epidemiology of AML
- 80% of all new cases of
ADULT leukemias - Adults over 50 years
of age (mean = 60) - It can occur in children & young adults, but less often
Which leukemia is associated with Trisomy 21?
AML
Clinical Presentation (AML & ALL)
- Chief complaint may be fever, fatigue,
weight loss, irritability, arthralgias,
bleeding
(often the gums → “pink in the sink”) - If CNS involvement, may have headache,
stiff neck, visual disturbances,
or vomiting - Gingival hypertrophy from leukemic
infiltration is common
Physical exam of ALL and AML may reveal:
○ Pallor
○ Petechiae
○ Ecchymosis
○ Papilledema
○ Retinal hemorrhages
○ Focal neurologic deficits
○ Hepatomegaly (more common in ALL)
○ Splenomegaly (more common in ALL)
○ Lymphadenopathy (more common in ALL)
You are more likely to see hepatomegaly, splenomegaly and lymphadenopathy in ____
ALL
Hyperleukocytosis
■ High circulating blasts in the blood
leads to clumping blasts in capillaries
● Impaired circulation
● Headache, confusion, dyspnea
● Tx = emergent leukapheresis
& chemotherapy
● MOST EXTREME presentation
MOST EXTREME presentation in AML and ALL?
Hyperleukocytosis
Diagnosis of Acute leukemias
- Bone Marrow Biopsy
○ Hypercellular
■ > 20% Blasts - CBC
o Normocytic anemia
o Thrombocytopenia
o Often neutropenia - WBC count can be low, normal, or high
- Chest x-ray
- ALL
- Possible Anterior Mediastinal mass
○ specifically T-cell ALL - Peripheral Blood Smear in
AML may show Auer Rods
Hallmark of both AML and ALL?
- Pancytopenia and circulating blasts
- Lymphoid or myeloid
CNS infiltration is common in ____
ALL
Management & Referral of Acute leukemias
- Acute Leukemia tx’d by Hematology Oncology
- Treatment for both ALL & AML includes “Induction Chemotherapy”
- CNS infiltration is common in ALL
- Intrathecal chemotherapy
What is “Induction Chemotherapy”?
- aggressive combination chemo
- goal = “inducing” remission
Childhood ALL – ___ % tx
success within 4 weeks
98
Adult ALL & AML outcomes
- Remission ~60-80%
- Complete cure is less likely in adults
Hodgkin’s Lymphoma epidemiology
● Bimodal age distribution
1. ↑ incidence in young-teen to young-adulthood
■ Average around 20 years of age (20’s)
2. 2nd peak occurs later in life at about 50 years of age (50’s)
● ~85 % male
Characterized by the presence of
Reed-Sternberg cells
Hodgkin’s Lymphoma
Some studies have suggested a
correlation between EBV infection
& lymphomas with _____
Hodgkin’s Lymphoma
Hodgkin’s Lymphoma Pathophysiology
○ Starts in a localized region of lymph
nodes
○ Spreads in an orderly, predictable
fashion to adjacent sites of lymph tissue
■ Opposite of Non-Hodgkin’s
○ Often starts in the cervical or
supraclavicular regions
Hodgkin’s Lymphoma Presentation
■ Painless lump in neck area
■ Fevers/chills
■ Night sweats
■ Weight loss
■ Anorexia
■ General malaise
■ Excessive fatigue in children
○ May report a febrile pattern called Pel-Ebstein pattern
Interestingly, adults with Hodgkin’s will
experience pain/soreness at lymph
node with _____
ETOH ingestion
Hodgkin’s Lymphoma diagnosis
Lymph node biopsy
■ Excisional biopsy is preferred over fine-needle aspiration
○ CT chest/abdomen/pelvis required for Ann Arbor Staging
Management & Referral of Hodgkin’s Lymphoma
○ General surgeon or ENT surgeon for biopsy
○ If diagnosis is established, contact Oncology right away to set up consultation
Prognosis of Hodgkin’s Lymphoma
■ 5 year survival rate for Stage 1 is > 95%
● For Stage 4, about 65%
Non-Hodgkin’s Lymphoma
A group of dozens of forms of Lymphoma all classified as
Non-Hodgkin’s Lymphoma (Non-Hodgkin’s Disease)
T/F Non-Hodgkin’s is much more common than Hodgkin’s
T
Characteristic Signs & Symptoms of Non-Hodgkin’s Lymphoma
○ Like Hodgkin’s, NHL is characterized by
painless lymphadenopathy
■ However, NHL lymphadenopathy seems to be more generalized rather than localized to one region
■ 2/3rd have retroperitoneal, mesenteric, or pelvic nodal involvement
Constitutional symptoms (e.g. fever, night sweats, weight
loss) can occur, but a much less common in ____
NHL
Non-Hodgkin’s Lymphoma diagnosis
Tissue biopsy is required for
definitive diagnosis
○ Peripheral blood smear is usually normal,
Treatment of NHL
○ If diagnosis is confirmed, treatment usually involves a chemotherapy & possibly radiation
■ Treatment is based on stage & apparent aggression of the malignancy
■ If indolent, may not treat initially
○ 70-95% of pediatric patients with NHL can be cured with current therapies
____ tumors are the most common solid tumors seen in
children
Brain & spinal cord
Brain & Spinal Cord Tumors clinical presentation
○ Clinical presentation
depends upon tumor
location
■ Headaches
■ Bulging fontanelle
■ Irritability
■ Nausea & vomiting
■ Imbalance (cerebellar involvement)
■ Neck or back pain
■ Eye/facial movement problems
Diagnostic Techniques for Brain & Spinal Cord Tumors
If H&P suggests concern for CNS tumor,
imaging is immediately indicated
○ CT scan possible
○ Prefer MRI with & without contrast
■ Brain or spine, depending on
presentation
■ Biopsy/Excision confirms Dx
The most common type of primary
CNS malignancy seen in children!
Medulloblastoma
Medulloblastoma frequently spreads to
other locations within the CNS by way
of ____
the CSF seeding
Pilocytic Astrocytoma
○ Tumors arising from astrocytes
○ “Low-grade” or “High-grade”
○ Slow growing, well demarcated
considered mostly noncancerous
Most common pediatric brain tumor
Pilocytic Astrocytoma
Management of Brain & Spinal Cord Tumors
○ Treatment depends on the type of tumor, location of the tumor, & age of the child
○ Surgery, radiation therapy, & chemotherapy are often utilized
○ In general, 3 out of 4 children will survive at least 5 years after being diagnosed
____ is the 2nd most common solid tumor in children
Neuroblastoma
Neuroblastoma
Neuroendocrine cancer arising
from the neural crest element of
the sympathetic nervous system
Most commonly originates in an adrenal gland
● May also develop in nervous tissue of the chest, abdomen, pelvis, or neck
● Incidence peaks before 2 yo
Neuroblastoma Most commonly originates in _____
an adrenal gland
Neuroblastoma clinical presentation
○ Abdominal mass or swelling
○ Neck mass
○ Horner’s Syndrome with Chest Mass
○ Back pain
○ Bowel or bladder dysfunction
○ Bulging eye with bruising
○ Bone pain
○ Anemia bleeding
Diagnostic Techniques for Neuroblastoma
■ CT scan of the body
■ MRI scan is preferred if concern
of spinal column involvement
■ Nuclear medicine scans
■ CMP & CBC
■ Bone marrow studies
Management of Neuroblastoma
○ Surgery for complete total excision is preferred as long as vital organs have not been invaded
○ Chemotherapy & radiation
Retinoblastoma
Rare tumor of the immature cells of the retina
* Generally occurs in children less than
5 years old
○ Peak incidence at 18-20 mo
T/F Retinoblastoma can run in families
T
○ Important that siblings of affected
children are evaluated by an
ophthalmologist as well
○ 1⁄3 of cases are bilateral
White light reflex is called
Leukocoria
Common Clinical Presentation of Retinoblastoma
○ Worsening vision over weeks to months
○ Misaligned eyes develop (weeks to
months)
■ A growing retinal tumor starts to
displace the eye
○ Change in the pupil size
Leukocoria = white light reflex
Retinoblastoma diagnostic techniques
○ MRI of the brain with contrast
○ Eye exam under anesthesia performed by an ophthalmologist, with possible biopsy (unless classic appearance)
Management of retinoblastoma
○ Chemotherapy is often utilized
○ Radiation is avoided, but is
sometimes necessary
○ If recovering full vision with
chemotherapy is not possible,
removal of the eye is
recommended
○ Untreated retinoblastoma is
deadly, however, with
current treatments, prognosis
is generally favorable if
caught early
Osteosarcoma
Most common malignant bone tumor in youth
Most Common Location of Osteosarcoma = _____
Metaphysis of long bones
○ Distal femur, proximal humerus, tibia or fibula,
Common Clinical Presentation of Osteosarcoma
○ Pain in the affected bone (most common)
○ Visible swelling or malformation
○ Limited range of motion
○ Pathologic fracture may be first sign
Diagnostic Techniques for Osteosarcoma
○ X-ray is a good initial test
○ MRI or CT for more detail
○ CT of the chest, abdomen, pelvis should be performed to rule out metastases
○ Biopsy is always required
○ Labs: ↑ALP, LDH, ESR
Osteosarcoma management
○ Most children can be cured
○ Treatment depends on size,
position, & stage of the disease
○ Chemotherapy is often used
to shrink the tumor before surgery
○ Radiation is rarely used;
only if surgery is not an option
○ Tumor removal is necessary; may require amputation
Ewings sarcoma
Less common bone cancer that osteosarcoma
● May arise anywhere in the body, but more common in the
pelvis, axial skeleton, femur
● Although more often
occurs in bone, it can
develop in the soft
tissues near bone as well
● Most common during
teenage years
Common Clinical Presentation of Ewings sarcoma
○ Bone pain is the most common symptom
○ Swelling or enlargement of affected bone
○ Pathologic fracture with minor event
○ Fever
○ Fatigue
○ Weight loss
○ Anemia
Diagnostic Techniques of Ewings sarcoma
○ XR, CT, MRI, Biopsy
○ Same as osteosarcoma
Management of Ewings sarcoma
○ Usually a combination of chemotherapy,
radiation therapy, & surgery
○ Depends on size & spread of tumor
○ Most patients can be cured
Rhabdomyosarcoma
Cancer of muscle tissue
● Accounting for approximately
3-4% of childhood cancers
● It can appear in any muscle in the
body, but more often arises in
head/neck region, extremities,
abdominal or chest wall, orbit, or
pelvic cavity (psoas or pelvic floor)
Rhabdomyosarcoma is Most commonly diagnosed in patients under the age of ___
6
Rhabdomyosarcoma clinical presentation
○ Symptoms usually arise as the mass
grows to a point where it pushes
on other tissue (mass effect)
Diagnostic Techniques for Rhabdomyosarcoma
○ CT scan or MRI will both provide very useful images of the mass, with characteristics suggesting a muscular origin
○ Biopsy is necessary for diagnosis
Rhabdomyosarcoma management
○ Chemotherapy is usually used initially to
shrink the size of the mass
○ Surgery and/or radiation therapy are
then utilized to treat local sites of tumor
visible on imaging
○ >60% of patients will survive
○ If detected early, cure rate = 80%+
Nephroblastoma is also known as a
“Wilm’s Tumor”
Nephroblastoma
“Wilm’s Tumor”
● Cancer of the kidneys that occurs in
children under the age of 9 years
○ Peak incidence at ages 2-5
○ About 10% of Wilm’s Tumor
patients will have malignant
tumor in BOTH kidneys
Wilm’s can spread to lymph nodes in:
- Abdomen
- Chest
- Occasionally to the liver
Nephroblastoma clinical presentation
○ Abdominal swelling
○ Palpable abdominal mass
○ Abdominal pain
○ Poor appetite
○ Constipation or bowel obstruction
○ Hematuria
○ Hypertension
○ Fatigue & generalized malaise
Diagnostic Techniques for a Nephroblastoma
○ After a detailed H&P reveals a mass…
■ CT of chest, abdomen, & pelvis
■ Oral & IV contrast
■ Ultrasound may be used to assess
vascular flow around the tumor
■ Urinalysis looking for hematuria
■ CMP & CBC
■ If both kidneys are involved, biopsy
may be done to confirm Dx
Management of a Nephroblastoma
○ If the tumor only involves one
kidney & is not extending into major blood vessels, surgery will entail removal of the entire involved kidney
○ Children do well with 1 remaining
kidney
○ Chemotherapy is important in tx
○ If both kidneys are involved,
chemotherapy will be used to
shrink the kidneys before
considering resection surgery
○ Overall cure rate = 85%
