CRANIOPHARYNGIOMA

CRANIOPHARYNGIOMA

  • Benign well-circumscribed lesion from the remnants of Rathke\’s pouch.
  • Calcified with complex-cystic component containing cholesterol "crankcase oil."
  • The typical adamantinous craniopharyngioma is from rest cells from along the pituitary stalk; Has less common SCCA in adults (solid no cysts)
  • Located in the suprasellar and sellar regions. Difficult to resect due to adjacent structures—oc, on, circle of willis
  • 70% are retro-chiasmatic and can grow superiorly into the third ventricle/along the hypothalamus.
  • 30% pre-chiasmatic between the optic nerves, more easily respectable; Multi-cystic lesion may extend to post fossa.
  • Present with ICP, visual disturbances (field defits) and endocrine deficits (GH, diabetes insipidus, TSH/ACTH) personality and cognitive changes.

Treatment

Main issue

  • outcome with radical surgery and radiation is similar so the difference is QOL
  • limited surgery and RT had improved QOL mainly due to hypothalamic damage
  • also radical surgery may be attempted after limited surgery and RT but not after radical surgery has been attempted.

Surgery

  • Gross total resection (curative) can be performed in about 50% to 80% so no need for adjuvant RT. 10 to 30% recur after GTR.
  • 3% operative mortality,
  • 10%—20%major neurologic morbidity (vision loss, neurologic defect)
  • 85% diabetes insipidus
  • morbid hypothalamic obesity \~50%
  • 70% of sub-totally resected lesions recur within 2-3 years.; Ca2+ flecks=residual
  • Second surgery less efficacious

RT

  • Just as effective as surgery as primary therapy
  • if incomplete surgery, unresectable, or recurrent disease then RT to 54/30 at 1.8Gy daily.

Royal Marsden

  • showed 15-year survival at 96%

Boston

  • 86% with limited surgery + RT.

Delayed RT

  • does not work as good as immediate post-op RT following sub-total resection.
  • For recurrence after EBRT, P32 can be given to the cyst wall or RSG. There is no role for chemotherapy.
  • Expect late endocrinopathies after RT –decrease in GH, TSH, ACTH and vascular effects (DI rare after RT).

Results

  • 10-year survival is about 75-95%.
  • Cyst enlargement in the 1st few weeks after RT not recurrence

RT

  • Vol/Tec: GTV=residual solid tumor + diminished cyst vol; CTV = GTV + 1 cm (or 2mm for RSG); PTV= 3mm + CTV
  • May need to aspirate cyst during RT
  • RSG: Only after NGTR or limited local recurrence.
  • Intracranial radionuclide: < 4 yr, largely cystic recurrent after sg+RT; P32, Yt90200Gy to cyst wall
  • Dose 54 to 55/180cGy

EJCRH Pre-RT-1 Experience

Thyroid Adrenal GH Gonado DI Seizure
Surgery alone 100% 93% 93% 27% 73% 40%
Limited Surgery + RT 93% 67% 93% 53% 33% 0%

Quality of Life

qol
  • hypothalamic injury changes personality
  • hyperphagic appetitie and lack of initiative
  • RT has loss of 10 IQ points.
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