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rare tumours

liver tumours
1. primary malignant liver tumours
  1. hepatoblastoma (65%)
    1. high alpha-fetoprotein (AFP) detected in nearly all cases
    2. most are cured
  2. hepatocellular carcinoma (25%)
    1. hig AFP in some cases
    2. less satisfactory prognosis
2. presentation
  1. abdo distension
  2. mass
  3. rare
    1. pain
    2. jaundice
3. Mx
  1. chemo
  2. surgery
  3. liver transplant
    1. in inoperable cases
Germ cell tumours
1. benign
  1. commonest in sacrococcygeal region
2. malignant
  1. most found in gonads
  2. very sensitive to chemo
  3. good outcome expected at most sites, incl brain
3. arise form primitive germ cells which migrate from yolk sac endoderm to form gonads in embryo
4. serum markers vital to confirm diag & monitoring response to Rx
  1. AFP
  2. beta-HCG
Langerhans cell histiocytosis
1. rare
2. abnormal proliferation of histiocytes
3. not truly malignant
4. classified as a disorder of dendritic (antigen presenting) cells
5. sometimes its aggressive behaviour & response to chemo means oncologists treat it
6. clinical manifestations
  1. bone lesions
    1. at any age
    2. presentation
      1. pain
      2. swelling
      3. fracture
    3. X ray
      1. characteristic lytic lesion w/ well defined border
        skull often involved
  2. diabetes insipidus
    1. assoc w/ skull disease
      1. proptosis
      2. hypothalamic infiltration
  3. systemic LCH
    1. most aggressive form
    2. tends to present in infancy
      1. seborrhoeic rash
      2. soft tissue involvement
        gums
        ears
        lungs
        liver
        spleen
        lymph nodes
        bone marrow
    3. progressive type
    4. chemo needed
      1. can spontaneously regress

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rare tumours

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	Created by v.djabatey almost 11 years ago