3 most common childhood cancers
- Leukemia
- Brain tumors
- Lymphoma
Down Syndrome cancers
ALL
Sometimes AML
Turner Syndrome cancers
Gonadoblastoma
Trisomy 13 Cancer
Leukemia
Teratoma
Trisomy 18 cancers
Wilm’s tumor
Neurogenic tumors
Klinefelter’s syndrome cancers
Leukemia
Germ cell tumors
Breast cancer
Fanconi anemia cancers
Leukemia
Xeroderma Pigmentosum cancers
Basal and squamous cell carcinoma
Melanoma
Ataxia Telangectasia cancers
Hodgkin’s and non-Hodgkin’s
Leukemia
Sarcomas
Bloom syndrome cancers
Leukemia
Lymphoma
GI malignancies
Solid tumors
Beckwith-Wiedemann syndrome cancers
Wilm’s tumor
Hepatoma stroma
Rhabdomyosarcoma
Adrenocortical carcinoma
NFT type I cancers
Brain tumors
Lymphoma
Leukemia
Malignant schwannoma
NFT type II Cancer
Acoustic neuroma
MC childhood cancer
ALL (L1 classification)
Also most commonly Pre-B cell phenotype with CALLA (+)
Usually occurs at 2-6 years of age
Favorable ALL prognostic factors
1-9 years of age
Female
White people
Diagnosis of leukemia
Requires bone marrow exam
Pts can have a normal CBC
ALL phases of treatment (3)
- Induction
- intrathecal MTX is always used occasionally alongside corticosteroids, vincristine, and L-asparaginase
- Consolidation
- continue intrathecal MTX, sometimes use intracranial radiation if absolutely necessary
- Maintenance
- chemotherapy for up to 3 yrs
Tx. Of neutropenia associated infxn following chemotherapy
IV Broad spectrum antibiotics
-Should also consider opportunistic infxns like PCP, Aspergillus, and fungi
Tumor lysis syndrome complications
Hyperuricemia ➡️ renal insufficiency
Hyperkalemia ➡️ cardiac Dysrhythmia
Hyperphosphatemia ➡️ Hypocalcemia with tetany
Drugs causing medication induced pancreatitis
L-asparaginase
Corticosteroids
AML classifications
M1= acute myeloblastic leukemia (no maturation)
M2= acute myeloblastic leukemia (some maturation)
M3= acute promyelocytic leukemia (Auer rods common)
M4= acute myelomonocytic leukemia
M5= acute Monocytic leukemia
M6= erythroleukemia
M7= acute megakaryocytic leukemia (assoc. W/ Down’s)
AML differences in clinical signs from ALL
CNS involvement
Gingival hypertrophy
DIC
BM management after chemo is recommended
(Both have fever, hepatosplenomegaly, bruising, bleeding)
Juvenile CML
Least common type of leukemia and is due to abnormalities on Cr. 7 and 8 (NOT the Philadelphia chromosome)
Signs/Symptoms: Fever ***Eczemalike facial rash ***Suppurative lymphadenopathy Petechiae/Purpura Moderate leukocytosis (
Hodgkin’s Disease
Lymphoma assoc. w/ EBV infection! !
-Commonly presents w/ painless lymphadenopathy in the supraclavicular or cervical regions
Signs/Symptoms: (opposite of non-Hodgkins)
- Slow, indolent onset
- Cervical and supraclavicular nodes (+)
- Presents w/ systemic signs of cancer (nausea, night sweats)
- Rare abdominal findings
- Painless adenopathy
Staging of Hodgkin’s Lymphoma
I: Single node or extralymphatic site
II: Involvement of 2 or more nodes on same side of diaphragm or extension to extralymphatic site and one or more nodes on same side of diaphragm
III: involvement of nodes on both sides of diaphragm
IV: disseminated involvement of organs
**A=lack of systemic symptoms
B=Systemic symptoms
Complications of Hodgkin’s Tx.
Growth retardation (radiation)
Secondary malignancies (AML, non-Hodgkin’s)
***Male sterility
Hypothyroidism
Lymphoblastic lymphoma
non-hodgkin’s assoc. w/ immunodeficiency states that is ***T-cell in origin
***Presents w/ an anterior mediastinal mass (SVC, Airway obstruction, Horner’s)
Small, noncleaved cell lymphoma
B-cell origin lymphoma that presents as intussusception, abdominal pain, or mass
**CONSIDER THIS IN ANY KID OLDER THAN 3 YEARS WITH INTUSSUSCEPTION
MC lymphoma in childhood
Burkitt’s Lymphoma
B-cell in origin; presents as a large jaw mass
Large Cell lymphoma
B-cell in origin; presents as enlarged tonsils, adenoids, or Peyer’s patches
Most common solid tumors in children (and their categories and sites)
- Glial cells (includes astrocytoma, most common supratentorial)
- Primitive neuroectodermal tumors (includes medulloblastoma, common infratentorial)
- Ependyomas
- Craniopharyngiomas
* Infratentorial location more common
Optic glioma
Cranial tumor assoc. w/ diminished vision, visual field defects, and strabismus
Craniopharyngioma
Tumor assoc. w/ growth retardation, delayed puberty, bitemporal hemianopsia, diabetes insipidus
Preferred imaging for brain tumors
MRI; better imaging of temporal lobes, cranial base, and brainstem
Second most common solid tumors in children
Neuroblastoma; can arise anywhere along sympathetic ganglia and in the adrenal medulla
*Peak incidence is before 5 yrs; 75% reoccurrence rate
Acute cerebellar atrophy
Symptoms of ataxia, opsoclonus (eye jerks), and myoclonus that occurs w/ neuroblastomas
Neuroblastoma staging (which do you not have to treat?)
I: localized to structure of origin
II: extends beyond structure of origin but not beyond the midline
III: extends past the midline
IV: metastasizes to bone, lymph nodes, bone marrow, or soft tissue
IVS: local tumor at Stage I or II but with distant metastasis to any organ but bone (spontaneously regresses!)
*Poor prognostic factors include stages III or IV, presence of n-myc, diploidy
Most common childhood renal tumor
Wilm’s tumor
Presents as an abdominal mass that doesn’t cross midline
Signs/Symptoms:
- Hematuria
- Abdominal pain
- HTN
- Fever, anorexia
- GU malformations
- Hemihypertrophy
- Sporadic aniridia
Beckwith-Wiedemann Syndrome
BMW
Big tongue
heMihypertrophy
Wilm’s tumor
-Also hepatoblastoma, visceromegaly
WAGR syndrome
Wilm’s tumor
Aniridia
Genitourinary malformation
Retardation of the mental kind
Wilm’s tumor staging
I: Limited to kidney and excised intact w/o rupture
II: extends locally but still resected w/o residual disease
III: residual tumor remains in abdomen or spills during resection
IV: distant metastasis to lung, liver, bone, brain
V: bilateral renal involvement
MC site of metastasis for Wilm’s tumor
Lung
Most common soft tissue sarcoma of childhood
Rhabdomyosarcoma; pts. w/ NFT 1 at increased risk
***MC sites= Head and neck (may present as proptosis, chemosis, epistaxis, airway obstruction, or hoarseness)
-Also occur at GU tract (hematoma, obstruction) or extremities (painless mass)
Most common malignant bone tumor
Osteogenic sarcoma; peak incidence is during MALE GROWTH SPURT
-Assoc. w/ Rb, Paget’s, radiation therapy
Tx: Surgery; remove pulmonary metastases as well
Osteogenic sarcoma findings (from table)
Site: Metaphysis of long bones
-Distal femur, proximal tibia, proximal humerus
Signs/Symptoms: Pain, swelling, soft tissue mass
*No systemic symptoms
Radiographic findings: Periosteal reaction w/ SUNBURST appearance
-Lytic lesions
Metastases: Lungs
Ewing’s Sarcoma findings (from table)
Site: Flat bones and diaphysis of long bones
-Pelvis, humerus, femur
Signs/Symptoms: Pain, swelling, soft tissue mass
***Fever, malaise, weight loss, leukocytosis, increased ESR
Radiographic findings: Periosteal rxn w/ “ONION SKIN’
Metastases: Slightly more common, also goes to lung mostly but also bone marrow
Second most common malignant bone tumor
Ewing’s Sarcoma; assoc. w/ 11;21 translocation
-Bone scan, chest CT, and bone marrow usually examined for metastasis
***Chemotherapy important due to risk of metastasis
MC liver tumor in childhood
Hepatoblastoma; assoc. w/ Beckwith-Wiedemann syndrome
- Presents as RUQ abdominal mass w/ anorexia, weight loss
- NO JAUNDICE
- Slightly better prognosis than hepatocellular carcinoma
Hepatocellular carcinoma
Presents in kids w/ chronic HBV infxn as well as biliary atresia, a1-antitrypsin deficiency, hereditary tyrosinemia
- RUQ abdominal mass, anorexia, weight loss
- NO JAUNDICE
Most common teratoma during first year of life
Sacrococcygeal teratoma; mostly in females
-Soft tissue mass arising from the coccyx that is mostly benign
MC testicular tumor
Germinoma; yolk-sac type (will see increased AFP)
-Assoc. w/ cryptorchid testes
Amount of malignant ovarian tumors
1/3; increase in frequency during puberty
Eosinophilic granuloma
Sub-type of Langerhans Cell Histiocytosis assoc. w/ localized bony changes
***MC involves the SKULL
-May see pathologic fractures, chronic draining ears due to mastoid involvement, ***SEBORRHEIC DERMATITIS (skin involvement), nonspecific systemic signs
Letterer-Siwe disease
Langerhan’s cell histiocytosis assoc. w/ DISSEMINATED DISEASE