Oncological presentations

Question 1

What is lymphoma and how is it Mx?

Answer 1

Begins with a malignant change in a lymphocyte (B or T cell), lymph node cell or lymphatic tissue of the marrow

Non-Hodkins (CHILD) = painless LN swelling, fatigue, weight loss, fever, night sweats, asymptomatic (low grade)

Hodkins = LN enlargement, B symptoms, pruritus, alcohol-induced LN pain, cough, SOB, presence of Reed-Sternberg cell

Mx =

• Low-grade = watchful waiting, DXT, chemo, rituximab (follicular lymphoma)
• High grade = chemo, stem cell transplant
• Hodgkins = DXT (when localised), chemo (when systemic)

Question 2

What is a neuroblastoma

Answer 2

Tumours arising from neural crest cells of the sympathetic NS + adrenal medulla

Most frequently starts from one of the adrenal glands (40-60%) but can also develop in the neck, chest, abdomen, or spine.

Ix = serum and urine catecholamines (raised in 90%), imaging, genetic testing, CT/MRI, biopsy

Sx = bone pain, abdo mass, neck, or chest, or a painless bluish lump under the skin, fever, weight loss, sweating, pallor, hepatomegaly, limp

Mx = chemo, surgery, RT, monoclonal Ab therapy

Question 3

What is a Wilm’s tumour

Answer 3

Malignant tumour that develops in the kidney from nephroblasts

Medium age 3.5y

Also called a nephroblastoma

S+S = a painless palpable abdo mass, loss of appetite, abdo pain, fever, N+V, haematuria, HTN

Ix = USS, biopsy, staging (lungs)

Mx = nephrectomy, chemo, RT

Question 4

Outline the aetiology of pathophysiology of ALL

Answer 4

Acute Lymphocytic Leukaemia - greatest risk for ALL is in the first 5 years of life

Fast growing cancer of the blood and bone marrow

Trisomy 21 = increased risk of ALL

Characterized by

• Uncontrollable growth of non-functional lymphoblasts
• Blockade of normal marrow cells (marrow failure)

Question 5

What are the S+S of ALL?

Answer 5

• Anaemia (lethargy, looking pale)
• Thrombocytopaenia (easy bruising/bleeding)
• Leukopaenia (fevers/infections)
• Bone pain (increased pressure from hyperplastic BM)
• Weight loss
• Malaise
• Lymphadenopathy
• Hepatosplenomegaly

Question 6

How should suspected ALL be Ix?

Answer 6

FBC = pancytopaenia, anaemia, thrombocytopaenia with a significant lymphocytosis

Blood film = blast cells

CXR = exclude mediastinal mass

BM aspirate/trephine = morphology, immunophenotyping, cytogenetics

LP = check for CNS involvement

Question 7

How should diagnosed ALL be Mx?

Answer 7

Mediastinal mass = risk of airway becoming compromised - steroids

Concurrent infection/sepsis is present = broad spectrum Abx

UKALL 2011 protocol = chemo given IV, orally, and intra-thecally (into the CSF)

Throughout Tx = blood products (red cells, platelets) and prophylactic anti-fungal therapy

Question 8

Outline a DDx for ALL

Answer 8

Bruising = immune thrombocytopenia, trauma, non-accidental injury

Recurrent infections = immune deficiency

Lymphadenopathy = reactive lymphadenopathy

CNS symptoms = infection, RICP

Pancytopaenia = neuroblastoma, aplastic anaemia

Question 9

Give 2 examples of bone tumours that effect children

Answer 9

Osteosarcoma

Ewings sarcoma

(typically older children)