Oncology Flashcards
What other conditions are linked to neuroblastoma?
Other neural crest disorders: • Neurofibromatosis 1 • Hirschsprung's Disease • Beckwidth Wiedemann • Central hypoventilation syndrome (PHOX2B)
What are the disease characteristics of a child <12 months old, with subcutaneous nodules, positive urine HMA/VMA, and liver metasteses?
Age and characteristics indicative of Neuroblastoma MS.
Favourable prognosis, majority is treated with observation, taking care to monitor for symptoms re: liver mets or potential spinal cord compression.
Differentiating Ewing Sarcoma from Osteosarcoma
Ewing Sarcoma
• Central axis lesions i.e. ribs, spine, hips
• Onion skinning pattern on x-ray (lytic lesion w/ periosteal reaction)
• No predisposing conditions
• Small round blue cell
Osteosarcoma
• Metaphysis of long bones i.e. femur, humerus
• Sunburst pattern on x-ray (sclerotic)
• Associated with Li-Fraumeni, Retinoblastoma, Paget disease, Rothmund-Thomson
• Spindle cell
Where do metastatic spread of Ewing Sarcoma and Osteosarcoma tend to go?
Bone and lung
Clinical presentation of langerhans cell histiocytosis
Varied depending on tissues involved
• Bone: lytic bone lesions, pathologic fractures, pain, swelling, growth retardation
• Skin: scaly, difficult to treat lesions resembling seborrheic dermatitis on scalp, diaper, axilla or occipital regions
• Ears: chronically draining ears
• Endocrine: diabetes insipidus, primary hypothyroidism
• Eyes: bilateral exophthalmos
How would you confirm and treat suspected langerhans cell histiocytosis?
- biopsy of suspected area must be done for diagnostic purposes
- bone scan looking for bony involvement, as well as bloodwork to rule out additional system involvement
- localized treatment with low dose radiation therapy, corticosteroid injection, currettage or bone graft
Girl with hypernatremia with high output, low specific gravity urine, improves with DDAVP (improved Na, concentrated urine specific gravity. MRI shows thickened pituitary stalk. What is the diagnosis?
a) Langerhans Cell Histiocytosis
b) Craniopharyngioma
c) Prolactinoma
d) Hypothalamic hamartoma
Langerhans Cell Histiocytosis
Note: a thickened pituitary stalk would most likely occur in the setting of a craniopharyngioma post-surgically, otherwise more likely to be supra-sellar
What findings on flow cytometry are linked to different types of ALL?
- CD19 = B-cell marker
- CD10 = Early B-cell marker
- CD3 = T-cell marker
- CD33 = myeloid marker
- MPO = myeloid marker
What syndromes are linked to a greater risk of ALL? (Name 6)
- Down syndrome
- Bloom syndrome
- Ataxia-telangiectasia
- Fanconi anemia
- Diamond-Blackfan anemia
- Shwachman-Diamond Syndrome
- Kostmann Syndrome
- NF-1
- SCID
- Li-Faumeni syndrome
What chromosomal characteristics are associated with greater risk?
- Hypodiploidy
- Philadelphia chromosome
- KMT2A (MLL)
What non-chromosomal factors at diagnosis are associated with higher risk?
- initial WBC count >50
- age <1 or >10 yoa
- MRD+ after induction therapy
- testicular disease
What are the main findings in tumour lysis syndrome?
- Hyperkalemia
- Hyperphosphatemia
- Hypercalcemia –> hypocalcemia
- High urate
How does allopurinol assist with TLS? Rasburicase?
- Allopurinol blocks synthesis of uric acid by inhibition/blocking the action of xanthine oxidase
- Rasburicacse expedites urinary excretion through allantoin
What treatments should be offered in the case of tumour lysis syndrome?
- Hyperhydration
- Allopurinol
- Rasburicase
- Diruesis (furosemide)
- Aluminum hydroxide for hyperphosphatemia
- Cardioprotection (calcium gluconate) in case of hyperkalemia
What oncological conditions are associated with EBV?
- Nasopharyngeal carcinoma
- Burkitt lymphoma
- Hodgkin disease
- Lymphoproliferative disorders
- Leiomyosarcoma in immunodeficient states (AIDS)
