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Path - Prognosis of Neoplasms (ii) (Neuroendocrine neoplasms (Carcinoid…

- - - - Li Fraumeni syndrome (P53 germline defect)
      - Von Recklinghausen disease (13% of neurofibrosarcomas)
      - neurofibromatosis
- - - - often incidental finding
      - lumen blocked -> stasis -> inflamm -> acute appendicitis
      - rarely mets in liver
    - - haemoptysis
      - obstruction -> lobe collapse -> secondary consolidation -> infection (pneumonia)
    - - biochem dx: high urinary 5HIAAs (serotonin breakdown product)
      - due to excess serotonin which is normally detoxified in liver a bit in lung
      - serotonin regulates intestinal movement + causes bronchospasm
      - occurs when liver is bypassed (not appendix)
      - facial flushing, diarrhoea, bronchospasm, pul stenosis
- - - - in testis
      - even with mets 98% cured
      - all malignant
      - usually occurs from 20-40 y/o
      - lymphocytic infiltration
      - no chemo needed unless v late stage
    - - ovary
    - - made up of hair+skin (dermoid), or muscle or bone
      - mostly malignant in testes
      - mostly benign in ovaries
    - - in tissue that would've been placenta (trophoblast)
      - highly aggressive
      - associated with HCG (tumour marker)
      - most often occurs with a complete hydatidiform mole
      - occurs as an abnormal pregnancy (a baby may/may not develop) or also after a normal pregnancy.
    - - alpha-fetoprotein = tumour marker
    - - rare
      - occur in ovary/testis