Peds Oncololgy Flashcards
Leading cause of death in pediatric patients aged 1-19
Cancer
Still, it’s pretty rare
Why is pediatric cancer difficult to diagnose in early stages?
Non specific associate Sx
Mimic other more common concerns
Lack of clinician experience
Clinicians reluctant to consider it as a Dx
Optimal treatment for childhood cancer requires…
High level of suspicion
Early referral to PEDS onco
Warning signs for childhood cancer (using the “CHILD CANCER” acronym)
Continued unexplained weight loss
Headaches (often w vomiting or early night/early morning)
Increased swelling or pain in bones, joints, back, legs
Lump/mass in abdomen, neck, chest, pelvis (LAD that DOESNT go away)
Development of excessive bruising, bleeding, rash
Constant infections
A whitish color behind the pupil
Nausea which persists or vomiting without nausea
Constant tiredness or noticeable paleness
Eye or vision changes that occur suddenly and persist
Recurrent fevers of unknown origin
Seriously, this is the worst acronym ever
The most common malignancy of childhood
Acute Lymphoblastic Leukemia (ALL)
ALL results from uncontrolled proliferation of …
Immature lymphocytes
85% B-precursor lineage
Peak incidence for ALL
2-5 years of age (85% Dx 2-10 years)
Genetic factor contributing to ALL
Down syndrome (14%)
Associations with ALL
Viral infections
Exposure to radiation
Clinical presentation of ALL
Intermittent fever, fatigue, pallor Bleeding (50%) - petechiae, purpura BONE PAIN (25%) - esp pelvis, vertebral bodies, and legs (consider in a little kid limping for unknown reason or refusing to walk) Hepatosplenomegaly (64/61%) Lymphadenopathy (50%)
Laboratory findings in ALL
Anemia and/or thrombocytopenia with normal or depressed WBD (but WBC can be >50K in 20% of patients - more likely to have increased WBC if acute)
Differential shows neutrocytopenia (ANC<1000)
Peripheral smear shows LYMPHOBLASTS
Dx standard for ALL
Bone marrow biopsy: leukemic blasts (lymphoblasts) replacing normal marrow
Treatment modalities for ALL
Chemotherapy
Hematopoietic stem cell transplant (HSCT) - best if from a matched sibling
Tx may take 2-3 years to complete
Prognosis for ALL
> 85% overall survival rate
Oncologic emergency that must be anticipated when initiating chemotherapy (esp if starting aggressive chemo regime)
Tumor Lysis Syndrome
Massive tumor cell lysis: • Hyperkalemia • Hyperuricemia • Hyperphosphatemia • Acute renal failure
Acute Myeloid Leukemia (AML) arises from…
Precursor cells (Myeloblasts) committed to the myeloid line of cellular development —> reduced capacity to differentiate into more mature cells
AML accounts for only ______% of leukemia in children
15-25%
BUT it’s responsible for 1/3 of deaths from leukemia in children/teenagers
Predisposing factors for AML
Often no identifiable risk factors
Congenital: Down Syndrome, Neurofibromatosis
Environmental exposures: radiation, benzene (smoking), and previous chemotherapy
Clinical findings in AML
Fatigue, pallor, bleeding, or infection (fever)
CNS involvement present in 5-15% at diagnosis (HA, lethargy, mental status changes, cranial nerve palsies)
Laboratory findings in AML
Anemia (44%)
Thrombocytopenia (33%)
Neutropenia (ANC<1000 in 69%)
WBC >100,000 in 20% of patients at diagnosis (HYPERLEUKOCYTOSIS)
Auer rods on peripheral smear
Circulating myeloblasts ≥20%
Auer Rods on a peripheral smear is pathognomonic for…
Acute Myeloid Leukemia (AML)
Auer rods = granular structures in the cytoplasm
A WBC > 75,000 is a medical emergency called ___________, associated with life-threatening complications from _________.
Hyperleukocytosis —> AML
Dx of Acute Myeloid Leukemia (AML)
Requires BOTH:
BM biopsy: 20% or more blasts
The leukemic cells must be of myeloid origin
Tx for AML
Chemo
HSCT
Prognosis: 65-75% overall survival rate
Childhood cancer associated with Philadelphia chromosome?
Chronic myeloid leukemia
Myeloproliferative disorder characterized by uncontrolled proliferation of mature and maturing granulocytes
Chronic Myeloid Leukemia (CML)
CML is rare, accounting for only ____% of childhood leukemia
5%
A history of __________ is something to look for when suspecting CML
Ionizing radiation exposure
Because it can lead to the mutation —> Philadelphia chromosome
What IS the Philadelphia chromosome?
Reciprocal translocation between chromosomes 9 and 22
Associated with chronic myeloid leukemia (CML) - ask about exposure to ionizing radiation in patient history
CML presents in what three phases?
Chronic phase —> accelerated phase —> blast phase
Clinical presentation of CML
20-50% asymptomatic
Bone pain
B symptoms (fever, night sweats, fatigue)
Pallor, ecchymosis
Massive splenomegaly, variable hepatomegaly
Lab findings for CML patients
Anemia, thrombocytosis, and marked leukocytosis (>100,000/µl)
Peripheral smear: myeloid cells in all stages of maturation, increased basophils and blast cells
Blast cells >20% in a blast crisis
CML confirmed if Philadelphia chromosome present (not always there but diagnostic if it is)
Treatment for CML
Tyrosine kinase inhibitor allows for dysregulated cellular proliferation
But Hematopoietic Stem Cell Transplant is the only consistently curative intervention
50% of childhood lymphomas are ___________
Hodgkin Lymphoma
Gender predominance in HL
4:1 male predominance in the first decade
Epstein-Barr virus is associated with what childhood cancer?
Hodgkin Lymphoma
Diagnostic peripheral smear finding for HL
Reed-Sternberg cells
Terminal-center B cells that have undergone malignant transformation
Prognosis for HL in children vs adults
Children with Hodgkin lymphoma have a better response to treatment than adults
90% 5-10 year survival rate
Clinical presentation for HL
PAINLESS cervical (70-80%) or supraclavicular (25%) LAD
MEDIASTINAL MASS (75%) - caution for superior vena cava syndrome
Weight loss, fever, night sweats, fatigue (B SYMPTOMS)
Splenomegaly +/- hepatomegaly
What is superior vena cava syndrome?
Dyspnea, cough, orthopnea, facial/upper extremity edema due to mediastinal mass
Associated with Hodgkin Lymphoma
Dx of HL
Tissue biopsy showing presence of Reed-Sternberg cells
How do we stage HL patients?
CXR, CT scan (chest, abdomen, and pelvis) and bone marrow biopsy
Treatment for HL
Chemo
Radiation if indicated
Autologous HSCT may improve survival rates
5th most common pediatric cancer for patients <15 years of age
Non-Hodgkin Lymphoma (NHL)
Gender predominance for Non-Hodgkin Lymphoma
3:1 male predominance
Non-Hodgkin Lymphoma can arise …
In any site of lymphoid tissue
Can spread anywhere but usually spreads to distant nodes
Conditions associated with Non-Hodgkin Lymphoma
Congenital and acquired immunodeficiency syndrome
EBV
Unlike adult NHL, virtually all childhood Non-Hodgkin Lymphomas are…
Rapidly proliferating, high-grade, diffuse malignancies
Clinical findings in Non-Hodgkin Lymphoma
Fast, usually 1-3 weeks
Common: enlarging, non-tender LAD
May see abdominal pain, fevers, cough, dyspnea, weight loss, night sweats
Hepatomegaly/Splenomegaly in advanced stage
Dx of NHL is via…
Tissue biopsy
Treatment for Non-Hodgkin Lymphoma
Chemotherapy - WATCH FOR TUMOR LYSIS SYNDROME
Autologous or allogenic HSCT is an option for those who relapse
Prognosis for Non-Hodgkin Lymphoma
90% have long term survival
The most common solid tumor of childhood
Brain tumor
Children have a better prognosis than adults though, so there’s that
Modalities for evaluating for brain tumors
Performed even in neonatal/infant exams
Measure head circumference
Observe gait
30% of pediatric brain tumors present with this triad…
AM headache
Vomiting
Papilledema
SSx of brain tumors more common in younger children
Vomiting, unsteadiness, lethargy, irritability, macrocephaly
Failure to thrive, delayed development
SSx of brain tumors more common in older children
Headache, visual symptoms, seizure, focal neurological deficits
School failure and personality changes
Preferred diagnostic study for pediatric brain tumors
MRI is first choice - HOWEVER, it’s hard to get kids to lay still for the required 30 minutes
CT is an alternative (only takes 10 min)
Final diagnosis is by tissue biopsy
Two categories of brain tumors accounting to the cell of origin
Glial tumors
• Most common = astrocytomas
• Ependymomas
Nonglial tumors
• Medulloblastoma
Initial approach for the treatment of pediatric brain tumors
Surgical removal of as much of the tumor as possible
Add radiation and chemo when indicated
Prognosis for pediatric brain tumors
60-90% 5-10 year survival with low grade astrocytoma
Most common abdominal tumor in children
Neuroblastoma
BUT, it makes up only 7-10% of pediatric malignancies
Most common solid neoplasm outside of the CNS
Neuroblastoma
Most common primary site for Neuroblastomas
Adrenal gland (40% of them start here)
How soon do neuroblastomas usually get diagnosed?
90% are diagnosed < 5 years of age
50% are diagnosed < 2 years of age
Clinical manifestations of neuroblastomas
Abdominal mass (65%) - firm, fixed, and irregular in shape, often extending beyond midline
Bone pain from metastatic disease (60%)
Fever, weight loss, irritability, abdominal pain, anorexia
Laboratory findings for neuroblastomas
Anemia is present in 60% of children
Urinary catecholamines elevated in 90% at diagnosis (b/c adrenal gland is common primary site)
Chemicals made by the nervous system (dopamine, NE, epi)
Treatment for neuroblastoma
The mainstay of therapy is surgical resection coupled with chemo
Surgery alone if low grade
Radiation sometimes necessary as well
Prognosis for neuroblastoma
Long term survival <40% in children with high-risk disease
Second most common abdominal tumor in children
Nephroblastoma or Wilms Tumor (2nd after neuroblastoma)
5-6% of cancers in children <15 years
Most common between 2-5 years of age
Most have solitary tumor (5-7% have bilateral kidney involvement)
Clinical findings in nephroblastoma
Asymptomatic abdominal mass/swelling (83%)
• Smooth, firm, well demarcated
• Rarely crosses midline
• can extend inferiorly into the pelvis
Fever, hematuria, HTN
Diagnosing nephroblastoma
Imaging - ultrasound or CT of the abdomen
CT of the chest (b/c commonly spreads to lungs)
Dx by histologic confirmation from biopsy or from surgical excision
Treatment for nephroblastoma
Surgical exploration, chemo, and radiation
90% overall 5 year survival
Most common primary bone malignancy in pediatric patients
Osteosarcoma
Most common demographics for osteosarcoma patients
Male predominance with peak incidence between 13-16 years (or 10-19) - the time of rapid bone growth
Most common location for osteosarcoma to develop
In long bones (metaphysic)
40% in distal femur
Cardinal signs of bone tumors
Bone pain at the site
Mass formation
Fracture through the area of cortical destruction
Presentation: bone pain over involved area w or w/o associated soft tissue mass/swelling and antalgic gait
Primary site for metastasis of osteosarcoma
Lungs
Imaging modalities for osteosarcoma
X-ray: will see destruction of normal trabecular pattern and irregular margins
MRI: to evaluate soft tissue involvement
Bone scan and CT scan of the chest for evaluation of possible metastasis
Dx via imaging, with histological confirmation from tissue
Treatment and prognosis for osteosarcoma
Tx with surgery and chemo
Prognosis is 70-75% long term survival with a localized tumor
Second most common primary bone tumor in pediatrics
Ewing Sarcoma
Who gets Ewing sarcoma?
Primarily affects white males during their second decade of life
Most often arises in the long bones (diaphysis)
• Extremities and pelvis
• Rarely in soft tissue
Clinical findings for Ewing Sarcoma
Worsening localized pain +/- swelling
Bone pain worse at night
Fatigue, fever, +/- weight loss
Dx of Ewing Sarcoma
Imaging: X-ray and CT/MRI of primary lesion
Staging: CT scan of chest, bone scan, bone marrow aspirated and biopsy
Dx confirmed by tissue biopsy
Treatment and prognosis for Ewing sarcoma
Tx with chemo, surgery, radiation, or a combo if needed
Prognosis is 70-75% long term survival if small localized tumor
10-15% of all cancers diagnosed in the first year of life
Retinoblastoma
90% of tumors diagnosed before age 5
Most common intraocular tumor in pediatrics
Retinoblastoma
BL in 20-30% of cases
Causes 5% of cases of childhood blindness
DDx to consider if BL white pupillary reflex
Retinopathy of prematurity (more likely to be bilateral than retinoblastoma)
Genetic factors related to retinoblastoma
Can be inherited - parent may be a silent carrier
Metastasis is rare with retinoblastoma, but if they occur…
Death within a year is typical
Clinical presentation of retinoblastoma
Leukocoria (white pupillary reflex) in patients under 2
• most common sign (60% of patients) but not definitive
Other SSx: strabismus, nystagmus, and red inflamed eye
Diagnosing retinoblastoma
Detailed ophthalmologist exam under anesthesia
Chalky off white retinal mass with soft, friable consistency is diagnostic
Ocular ultrasound
MRI of the brain and orbits
Treatment of retinoblastoma
Variety of options available for children, including several “vision-sparing” therapies
External beam irradiation
Chemo if confined to the globe
Prognosis: 90% 5 year survival if confined to the retina
Most common soft tissue sarcoma in childhood
Rhabdomyosarcoma
Rare: 7% of all childhood cancers
70% of children are diagnosed before age 10 years
Slight male predominance
Any body part can be affected in rhabdomyosarcoma but _____________ most common
Head and neck
Can resemble fat, fibrous tissue and muscle
Clinical presentation of rhabdomyosarcoma
Painless, progressively enlarging mass
Orbital rhabdomyosarcoma:
• Proptosis/exophthalmos
Bladder Rhabdomyosarcoma:
• Hematuria
• Urinary obstruction (palpate distended bladder)
• Pelvic mass
Diagnosis of rhabdomyosarcoma
Imaging: xray and CT or MRI of mass
Chest CT to rule out pulmonary mets
Skeletal survey to rule out bone mets
Treatment of rhabdomyosarcoma
Any combo of surgery, chemo, radiation
Prognosis: 70-75% 3 year survival if localized
________ of liver masses found in childhood are malignant
2/3rds
90% of hepatic malignancies are either hepatoblastoma or hepatocellular carcinoma
___________ is often elevated and is an excellent marker for response to treatment of hepatic tumors
Serum alpha-fetoprotein (AFP)
Clinical presentation of hepatic tumors
Usually present because of an enlarging abdomen - 10% found on routine exam
Dx and Tx of Hepatic tumors
Imaging: abdominal ultrasound, CT scan, or MRI
Tx: Surgery or chemo, but complete resection is essential for survival
Liver transplantation when tumors are unresectable
Prognosis: up to 1/3 with complete resection are long term survivors
