Overview

• Acute Lymphoblastic Leukaemia
  • Bone Marrow Failure → anaemia (lethargy and pallor) + neutropenia (frequent or severe infections) + thrombocytopenia (easy bruising, petechiae).
    • Other Sx → lymphadenopathy, bone pain, splenomegaly, hepatomegaly, fever.
    • Raised lymphocytes (blast cells).
  • Definitive diagnosis via bone marrow aspiration and biopsy.
    • Offer a very urgent FBC (within 48 hours) if any of following → pallor, persistent fatigue, unexplained fever, unexplained persistent infection, generalised lymphadenopathy, persistent or unexplained bone pain, unexplained bruising, unexplained bleeding.
  • Poor Prognostic Factors ⇒
    • age <2 years or >10 years
    • WBC >20 x 10^9/l at diagnosis
    • T or B cell surface markers
    • non-Caucasian
    • male sex
• Sarcoma
  • Cancers originating in the muscles, bones or other types of connective tissue.
  • Types of Bone Sarcoma:
    • Osteosarcoma → most common form of bone cancer. Persistent pain worse at night time, described as dull and unremmiting. Most common in femur and tibia.
    • Chondrosarcoma → cancer originating from the cartilage. Very rare in children.
    • Ewing Sarcoma → a form of bone and soft tissue cancer most often affecting children and young adults. (Onion skin appearance on x-ray).
  • Key Features that raise suspicion → soft tissue lump (particularly if growing, painful or large), bone swelling, persistent bone pain.
    • Unexplained bone swelling and persistent pain in children → need to make a urgent (48-hour) referral for an X-ray to assess for bone sarcomas.
  • Investigations → x-ray is the initial investigation for bony lumps or persistent pain. Biopsy is required to look at the histology of the cancer.
• Retinoblastoma
  • Common ocular malignancy in children, average age of diagnosis 18 months. Autosomal dominant condition. Mutations in RB1 gene on chromosome 13.
  • Features → abscence of red-reflex (replaced by white pupil - leukocoria) - MOST COMMON PRESENTING SYMPTOM + strabismus (squint).
  • Mx → enucleation (removal of eye - if significant risk cancer could spread), external beam radiation therapy, chemotherapy (for earlier stages/less aggresive), photocoagulation.
    • Indications for Enucleation → anterior chamber involvement, glaucoma, intra-orbital inflammation.
  • Excellent prognosis, >90% survive into adulthood.
    • Survivors require lifelong monitoring due to the risk of developing non-ocular secondary malignancies, such as malignant melanoma, sarcoma, brain tumours, leukaemia, and osteosarcoma.
• Neuroblastoma
  • Tumour arising from neural crest tissue of the adrenal medulla and sympathetic nervous system.
  • Sx → abdominal mass, pallor, weight loss, bone pain, limp, hepatomegaly, proptosis.
  • Ix → raised urinary catecholamine metabolites (VMA and HVA).
• Nephroblastoma
  • Mx → nephrectomy + chemotherapy.

Making Diagnosis

Clinical Features

Investigations

Management Plan

Complications →

Prognosis →