AKT · Child health/Oncology

Childhood cancer (incl. retinoblastoma)

Leukaemia commonest; retinoblastoma is the classic eye tumour

Overview

Leukaemia (mostly ALL) is the commonest childhood cancer, then CNS tumours and lymphoma. Retinoblastoma is the classic intraocular malignancy (leukocoria). Each has a discriminating presentation the exam tests.

Recognise

ALL: pancytopenia — pallor, bruising/petechiae, infections, bone pain, hepatosplenomegaly, lymphadenopathy
Retinoblastoma: LEUKOCORIA (white pupillary reflex / absent red reflex), squint
Neuroblastoma: abdominal mass crossing midline, raised catecholamines; Wilms: abdominal mass NOT crossing midline

Red flags

Any white pupillary reflex (leukocoria) → urgent ophthalmology; persistent unexplained bruising/pallor/bone pain; a child who is systemically unwell

Differentials & how to tell them apart

Fanconi anaemiapancytopenia WITH congenital anomalies (short stature/absent radius) — flips away from ALL

ITPisolated low platelets, otherwise well, normal film

Neuroblastoma vs Wilms tumourneuroblastoma crosses the midline + catecholamines; Wilms does not cross the midline

Congenital cataractalso leukocoria — but retinoblastoma needs urgent exclusion

Investigations

ALL: FBC/blood film (blasts), bone marrow. Retinoblastoma: examination under anaesthesia, MRI (avoid biopsy). Solid tumours: imaging, tumour markers, biopsy.

Management

Urgent paediatric oncology referral; chemotherapy (and tumour-specific therapy)

1Urgent paediatric oncology referral. ALL: chemotherapy. Retinoblastoma: refer same-day to ophthalmology; focal therapy/enucleation.Gate: Leukocoria (white pupillary reflex) → urgent retinoblastoma referral, do NOT biopsy the eye
2Tumour-specific multimodal therapy; genetic counselling (heritable retinoblastoma — RB1).

Chemotherapy — mainstay for ALL and many solid tumours

Enucleation / focal therapy — retinoblastoma, eye-preserving where possible

Key points

High-yield discriminators: leukocoria = retinoblastoma; midline-crossing abdominal mass + catecholamines = neuroblastoma; non-crossing mass = Wilms; pancytopenia + congenital anomaly = Fanconi not ALL.

Monitor & prognosis

Treatment response; late effects; (retinoblastoma) second tumours in heritable form.

Childhood ALL now has high cure rates; retinoblastoma survival high with early treatment.

Source: NICE; CCLG; ophthalmology