Malignancies in Childhood

Question 1

A 3 yr. old patient presents with malaise, fever, bone/joint pain, bruising, lymphadenopathy, hepatosplenomegaly. Duration 2-3 months. You assume it is the most common childhood cancer. What is it?

Answer 1

ALL

Question 2

ALL common lab findings

Answer 2

WBC: 50% of all cases have normal WBC. Blasts on smear.
Hgb: Low, normal (MCV WNL to increased)
Platelets: Moderately or severely decreased.

Question 3

What is Leukemic Hiatus

Answer 3

-Peripheral smear has mature granulocytes and very immature cells (blast cells) but nothing else.

Question 4

DDX for ALL

Answer 4

• EBV/CMV
• HIV
• Pertussis
• Other leukemias (AML, CML)
• JIA
• SLE
• Aplastic anemia
• Others…

Question 5

What is the most common location for a brain tumor? What are common presentations of the patient? Most common histologies?

Answer 5

• Posterior fossa
• Wide-based unsteady gait, Ascending cranial nerve palsy, N/V, HA
• Medulloblastoma, Astrocytoma

Question 6

What type of brain tumor is associated with drops mets, sugar coated mets, and is the most common single histology? In brainstem and cerebellum.

Answer 6

Medulloblastoma

Question 7

Most common brain tumor in children

Answer 7

Astrocyte

Question 8

Treatment of Brain tumor

Answer 8

• Surgery is definitive
• Chemo
• Radiation

Question 9

What is this? Arises in sympathetic chain/adrenal medulla, peak incidence is 1-2 years, metastatic sites include bone, marrow, lymph nodes, skin, lungs.

Answer 9

Neuroblastoma

Question 10

Common presentation of neuroblastoma

Answer 10

• Painless mass
• Nerve compression
• Fever, irritability
• Limp/Bone pain
• HTN
• Opsomyoclonua
• FTT

Question 11

What is this: Painelss abdominal mass arising from kidney. Peak incidence 2-3 years. May be part of congenital syndromes (Beckwith-weideman, WAGR, Denys-Drash). May be associated with chromosomal loss or advanced paternal age.

Answer 11

Wilms tumor

Question 12

Early stage Wilms tumor treatment

Answer 12

-Resection and mild chemo. Higher stage require more aggressive treatment.

Question 13

What is this: Associated with EBV. Charascteristic path=Reed Sternberg Cell. Rare in very young. Till late, confined to Lymph nodes.

Answer 13

Hodgkins Lymphoma

Question 14

What is this: No reed sternberg cell. Not necessarily assoc. with EBV. Often associated with immune disorders. Occurs in all age groups. Not confined to lymph nodes. Frequently seen in patients with immune deficiency disorders, post-transplant, metabolic derangements during induction chemo.

Answer 14

Non-Hodgkins Lymphoma

Question 15

What is this: Tumor arising in connective tissues, muscle, or bone.

Answer 15

Sarcoma

Question 16

Common metastatic sites for sarcomas

Answer 16

Lung, Other bones

Question 17

Common sarcomas in kids and treatment.

Answer 17

• Rhabdomyosarcoma, osteosarcoma, ewing sarcoma.

- Treatment is multimodal: Surgery, chemo, occasional radiation.

Question 18

Osteosarcoma arises in what part of bone

Answer 18

Diaphyseal areas of long bones

Question 19

Ewing sarcoma occurs in what part of bone

Answer 19

-Mid-shaft of bones and may arise in axial skeleton and pelvis, as well as soft tissue structure

Question 20

What is this: Acute electrolyte disturbance due to renal failure secondary to release of uric acid and phosphate as cancer cells die abruptly and in large numbers. Most common cause is Lymphoma, High WBC ALL.

Answer 20

Tumor Lysis Syndrome

Question 21

Symptoms and treatment of tumor lysis

Answer 21

Symptoms: Range form none to renal failure and cardiac arrhythmias depending on severity and electrolyte derangement.
Treatment: Prevention: Hydration with 2x maintenance IV fluid. Avoid all KCL in IV fluids. Lower uric acid levels with Allopurinol or Raspburicase. Careful monitoring.

Question 22

Nausea/vomiting after chemotherapy. How would you treat?

Answer 22

Acute: within 24 hours of chemo dose respond to 5HT receptor antagonists- ex: Zofran/kytril, Emend

Chronic: greater than 72 hours after chemo responds poorly to 5HT receptor antagonists. Dexamethasone

Question 23

Common problems with transfusions include:

1) volume overload.
2) HLA immunization
3) Graft vs host reaction
4) viral infection
- how would you fix?

Answer 23

1) loop diuretic
2) Leuko-depletion
3) radiation of blood product
4) volunteer blood donor, avoid family donors, shorten window of testing

Question 24

Neutropenic fever pathophys:

Answer 24

Chemo kills all fast growing cells.

-Marrow is killed resulting in decreased WBC production. Disruption of physical barriers (Gut, skin)

Question 25

Neutropenia:

1) High risk
2) Moderate risk

Answer 25

1) ANC range 0-200
2) ANC range 200-500
Normal= 1500-4500

Question 26

Management of Neutropenia

Answer 26

• Basic data set
• CVL
• Perianal
• CBC
• Blood cultures
• UA and Culture
• CXR

Question 27

Treatment of neutropenia

Answer 27

• Prophylactic AB: Cefepime, vanco?, tobramicin?

- Antifungal: FLuconazole