Pediatric Neoplasias

Question 1

Signs and symptoms of Acute Lymphoblastic Leukemia (ALL)

Answer 1

Typical patient is 5 year old boy with a limp, fever, URI symptoms, hepatosplenomegaly, and petechiae.
■ Bone/joint pain/swelling, limping
■ Anemia, thrombocytopenia, leukopenia (pallor, bruising, petechiae, infections)
■ Low grade fever
■ Hepatosplenomegaly due to leukemic infiltration
■ Lymphadenopathy (generalized, firm, non-tender)
■ Gingival hyperplasia

Question 2

Diagnosis of Acute Lymphoblastic Leukemia (ALL)

Answer 2

Bone marrow aspirate, lymphoblasts > 20%. TdT+, CD34+.

Question 3

Treatment of Acute Lymphoblastic Leukemia (ALL)

Answer 3

■ 1. Induction chemo (often CHOP) + CNS prophylaxis (systemic +
intrathecal chemo + cranial radiation)
● Prophylaxis for tumor lysis syndrome: hydrate, allopurinol

■ 2. Then consolidation: chemo in low risk, stem cell transplant in high risk

■ 3. Maintenance: chemo precautions
● Risk of “testicular relapse” - chemo didn’t penetrate leukemia
cells in testes

*CHOP=
Cyclophosphamide
Doxorubicin (Hydroxydaunorubicin)
Vincristine( Oncovin)
Prednisone

Question 4

Signs and symptoms of Neuroblastoma

Answer 4

Most common tumor of adrenal medulla in children.
○ Usually < 4 y/o.
Palpable, irregular abdominal mass that can cross the midline (diff from Wilms, which is smooth and unilateral).
○ Can present with opsoclonus-myoclonus (dancing eyes-dancing feet) and
raccoon eyes (bilateral orbital hemorrhage).
Can have Horner syndrome.

Question 5

Diagnosis of Neuroblastoma

Answer 5

Increased HVA and VMA (catecholamine metabolites) in urine.
Overexpression of N-myc oncogene.

Question 6

Treatment of Neuroblastoma

Answer 6

-Surgery whenever feasible.

-Chemotherapy :
○Cyclophospamide, cisplatin, carboplatin, Vincristine, Doxorubicin, Etoposide, Irinotecan, Topotecan.

-Radiation therapy

-Immunotherapy

Question 7

Wilm’s Tumor (Nephroblastoma) important information.

Answer 7

-Wilms/Nephroblastoma: most common renal malignancy of early childhood.

-Age 2-4. Large, palpable, unilateral flank mass +/- hematuria

-Can present with :
○Hypertension ( due to high renin)
○Polycythemia (due to high EPO)
○Varicocele and inguinal hernia (due to mass effect)
○IVC obstruction.

-Associated with WAGR, Denys-Drash, Beckwith-Widemann syndromes :

*WAGR= Wilms tumor, Aniridia, Genitourinary abnormalities, and intellectual disability.

**Denys-Drash=early-onset nephrotic syndrome, edema and low albumin levels.

***Beckwith-Widemann :
○Macrosomia at birth
○Macroglossia.
○Visceromegaly (enlargement of internal organs).
○Increased risk of childhood tumors (such as Wilms tumor and hepatoblastoma)
○Abdominal wall defects (such as omphalocele or umbilical hernia)
○Other: Ear creases or pits, hypoglycemia (low blood sugar) in infancy, and developmental abnormalities.

Question 8

Pediatric brain tumous important information and findings.

Answer 8

-Pediatric brain tumours usually affect the cerebellum.
-Usually presents with signs of increased Intracranial Pressure (ICP) which manifests as morning headaches, emesis, lethargy +- focal seizures.
-Types:
○ Pilocytic Astrocytoma: low-grade, most common childhood brain tumor. Usually in cerebellum.

○ Medulloblastoma: Most common malignant childhood brain tumor. Usually
cerebellum. Can compress 4th ventricle, causing hydrocephalus. Drop metastases to spinal cord.

○ Ependymoma: Usually 4th ventricle. Poor prognosis.

○ Craniopharyngioma: most common childhood supratentorial tumor. Causes
bitemporal hemianopia (like pituitary)

-Treatment :
○ Typically start with surgery to relieve pressure, and do chemo/radio after.
!!! Don’t use radiation if the child is < 2 years old, beacuse it will cause permanent brain
damage !!!

Question 9

Give the differential diagnosis of pediatric abdominal tumours.

Answer 9

Wilms tumor
Neuroblastoma
Leukemia
Lymphoma
Hepatoblastoma/other hepatic tumors
Ovarian tumors
Soft tissue sarcoma